Safety profile and probe placement accuracy of intraspinal pressure monitoring for traumatic spinal cord injury: Injured spinal cord pressure evaluation study - Phang I.; Zoumprouli A.; Saadoun S.; Papadopoulos M.C. (Journal of Neurosurgery: Spine, 2016)
OBJECTIVE: A novel technique for monitoring intraspinal pressure and spinal cord perfusion pressure in patients with traumatic spinal cord injury was recently described. This is analogous to monitoring intracranial pressure and cerebral perfusion pressure in patients with traumatic brain injury. Because intraspinal pressure monitoring is a new technique, its safety profile and impact on early patient care and long-term outcome after traumatic spinal cord injury are unknown. The object of this study is to review all patients who had intraspinal pressure monitoring to date at the authors' institution in order to define the accuracy of intraspinal pressure probe placement and the safety of the technique. METHODS: At the end of surgery to fix spinal fractures, a pressure probe was inserted intradurally to monitor intraspinal pressure at the injury site. Postoperatively, CT scanning was performed within 48 hours and MRI at 2 weeks and 6 months. Neurointensive care management and complications were reviewed. The American Spinal Injury Association Impairment Scale (AIS) grade was determined on admission and at 2 to 4 weeks and 12 to 18 months postoperation. RESULTS: To date, 42 patients with severe traumatic spinal cord injuries (AIS Grades A-C) had undergone intraspinal pressure monitoring. Monitoring started within 72 hours of injury and continued for up to a week. Based on postoperative CT and MRI, the probe position was acceptable in all patients, i.e., the probe was located at the site of maximum spinal cord swelling. Complications were probe displacement in 1 of 42 patients (2.4%), CSF leakage that required wound resuturing in 3 of 42 patients (7.1%), and asymptomatic pseudomeningocele that was diagnosed in 8 of 42 patients (19.0%). Pseudomeningocele was diagnosed on MRI and resolved within 6 months in all patients. Based on the MRI and neurological examination results, there were no serious probe-related complications such as meningitis, wound infection, hematoma, wound breakdown, or neurological deterioration. Within 2 weeks postoperatively, 75% of patients were extubated and 25% underwent tracheostomy. Norepinephrine was used to support blood pressure without complications. Overall, the mean intraspinal pressure was around 20 mm Hg, and the mean spinal cord perfusion pressure was around 70 mm Hg. In laminectomized patients, the intraspinal pressure was significantly higher in the supine than lateral position by up to 18 mm Hg after thoracic laminectomy and 8 mm Hg after cervical laminectomy. At 12 to 18 months, 11.4% of patients had improved by 1 AIS grade and 14.3% by at least 2 AIS grades. CONCLUSIONS: These data suggest that after traumatic spinal cord injury intradural placement of the pressure probe is accurate and intraspinal pressure monitoring is safe for up to a week. In patients with spinal cord injury who had laminectomy, the supine position should be avoided in order to prevent rises in intraspinal pressure.
Keywords: dalteparin; noradrenalin; adult; American Spinal Injury Association impairment scale; article; clinical article; computer assisted tomography; evaluation study; female; hematoma; human; intensive care; intraspinal pressure monitoring; laminectomy; liquorrhea; male; meningitis; mental deterioration; monitor; neurologic examination; nuclear magnetic resonance imaging; patient positioning; patient safety; perfusion pressure; postoperative complication; postoperative period; pressure measurement; pseudomeningocele; spinal cord injury; spine fracture; supine position; surgical approach; surgical technique; surgical wound; thrombosis; thrombosis prevention; tracheostomy; wound infection; MX800 bedside monitoring system

Congenital neuromuscular diseases with neonatal respiratory failure excluding myotonic dystrophy type 1 and infantile spinal muscular atrophy. Diagnosis strategy according to a 19-child series - Raignoux J.; Walther-Louvier U.; Espil C.; Berthomieu L.; Uro-Coste E.; Rivier F.; Cances C. (Archives de Pediatrie, 2016)
Apart from spinal muscular atrophy (SMA) and myotonic dystrophy type 1 (DM1), congenital neuromuscular diseases with early neonatal symptoms mean diagnostic and prognostic challenges mainly when infants require ventilatory support. Objectives Consider a standardized strategy for infants suspected of congenital neuromuscular disease from analysis of the literature and retrospective experience with floppy and ventilatory support-dependent infants, after exclusion of well-known diseases (DM1, SMA). Patients and methods Floppy infants requiring ventilatory support in their 1st month of life, but showing no evidence of DM1, SMA, Prader-Willi syndrome, or encephalopathy. The retrospective multicenter study was based on the response of regional referent neuropediatricians in the Reference Centre for Neuromuscular Diseases of Greater Southwest France to an inquiry about prenatal and perinatal history, investigations, diagnosis, and outcome of the child and family. It was conducted between 2007 and 2012. Results Among the 19 newborns studied, all had severe hypotonia. Prenatal and perinatal features were similar. Their outcome was generally severe: the median survival as measured by the Kaplan-Meier method was 6.9 months. Thirteen children died at a median age of 61 days; ten of them were treated with a palliative procedure. Five children had achieved respiratory independence but suffered from a small delay in motor development. Among the three children who continuously required ventilatory support, only one survived (follow-up period: 23 months); he was the only one undergoing tracheostomy in the cohort. Diagnostic processes were different, leading to pathological and genetic diagnosis for only six infants. There was only histological orientation for seven and no specific diagnostic orientation for the last six. These difficulties have led us to propose an exploration process based on the literature. Conclusion This study highlights difficulties in obtaining a diagnosis and a precise prognosis for floppy ventilated infants. An exploration-standardized process for infants suspected of congenital neuromuscular diseases was made in order to standardize procedures. It could be used as a tool for all professionals involved.
Keywords: article; brain disease; clinical article; developmental disorder; disease severity; human; infant; muscle hypotonia; myotonic dystrophy; neuromuscular disease; Prader Willi syndrome; respiratory failure; retrospective study; spinal muscular atrophy

Therapeutic hypothermia in neonatal cervical spine injury - Montaldo P.; Oliveira V.; Lally P.J.; Chaban B.; Atreja G.; Kirmi O.; Thayyil S. (Archives of Disease in Childhood: Fetal and Neonatal Edition, 2016)
Keywords: lactic acid; n acetylaspartic acid; Apgar score; arm weakness; assisted ventilation; breathing; case report; cervical spine injury; contusion; cooling; disease severity; electroencephalography; human; hypoxic ischemic encephalopathy; induced hypothermia; male; newborn; newborn disease; note; nuclear magnetic resonance imaging; priority journal; quadriplegia; shoulder dystocia; tendon reflex; tracheostomy

Length of stay of Spinal Cord Injury (SCI) patients and their outcome while in Neuro ICU - Hilasque C. (Intensive Care Medicine Experimental, 2016)
Introduction Why do patients with Spinal Cord Injury (SCI) stay in Neuro ICU for a long time? Objectives A survey about the reasons why SCI patients stayed longer in NICU between 2013 and 2015 and their outcome Methods A local database (Wardwatcher) was used to search and collection of data for patients admitted with SCI between 2013 and 2015. Results Out of 3,539 admissions in NICU, 326 cases were related to spinal injury and 137 were considered as spinal cord injury and 93 % of admissions were related to trauma and 6 patients were readmitted while in the hospital between 2013 and 2015. There was an increase trend of admissions in NICU between 2013 and 2015 and the data showed that the average length number of days of patient stay was 3.6 days while spinal patients were 8.6 days. The Guidelines for the Provision of Intensive Care Services (2015) recommends a discharge from critical care to ward must be within 4 hours from the decision of the consultant, the data showed that 71 % of between 9 to 14 hours delayed discharges from NICU to ward are mainly caused by shortage of ward beds followed by delay on ward. 60-66 % of SCI patients required advance cardiac and respiratory organ support and 36 % were on neurological support. 86 % of the patients had tracheostomy. Only 19 patients were qualified for SCI study called Injured Spinal Cord Pressure Evaluation (ISCoPE) where the intraspinal pressure was measured in relation to traumatic spinal cord injury and this requires the patient to be in the spinal monitoring pressure for at least 7 days. On patients outcome, 75 % had improved, 13 % died and 12 % of the patients remain unchanged. While the hospital patient outcome on discharge, 83 % lived and 17 % died. Conclusions Though there were not many SCI patients admitted in the unit compared to other patients. SCI patients stayed longer by 5 days on average. Three main reasons were identified why they stayed longer; one was because of ISCoPE research, the other one was their dependency on advance organ support and lastly, the delayed discharges because of shortage of ward beds. The delayed discharges could indicate that the hospital needs to increase the bed capacity. The patients' outcome appears encouraging with their improved outcome. A further audit is necessary to see any changes.
Keywords: consensus development; consultation; controlled study; data base; evaluation research; female; heart; hospital bed capacity; hospital patient; human; length of stay; major clinical study; male; monitoring; neurological intensive care unit; organ; spinal cord injury; tracheostomy

Surgical outcomes for acute type A aortic dissection with aggressive primary entry resection - Inoue Y.; Minatoya K.; Oda T.; Itonaga T.; Seike Y.; Tanaka H.; Sasaki H.; Kobayashi J. (European Journal of Cardio-thoracic Surgery, 2016)
OBJECTIVES: An entry located at aortic arch in acute type A aortic dissection (AAAD) is uncommon. It remains controversial whether or not aggressive primary entry resection should be routinely performed in such patients. We have adopted an aggressive strategy of entry site resection, including total arch replacement (TAR) in patients with arch tears. The purpose of this study was to investigate the efficacy of our surgical management approach, using aggressive primary entry resection. METHODS: Between January 2000 and December 2014, we retrospectively reviewed the records of 334 patients with AAAD who underwent emergent surgery. The mean age was 67 ± 13 years (range, 20-95 years). Ninety-five patients (28%) presented with shock vital status, and 84 patients (25%) manifested malperfusion of branched arteries. Primary entry resection was achieved in 95% of patients under an aggressive surgical strategy [hemiarch replacement for 173 (52%) patients and TAR for 161 (48%) patients] concomitant with 22 coronary artery bypass grafts and 38 root replacements. Ninety-six percent of hospital survivors (298/311) were followed for a median of 39 months (range, 0-179 months). RESULTS: Operation, cardiopulmonary bypass, cardiac arrest, antegrade cerebral perfusion and lower body circulatory arrest times were 447 ± 170, 236 ± 93, 112 ± 74, 115 ± 81 and 54 ± 18 min, respectively. The 30-day mortality rate was 5.4%. The in-hospital mortality rate was 8.4% (6.9% at our hospital). Incidences of postoperative permanent neurological dysfunction, tracheotomy and newly permanent haemodialysis were 6.9, 8 and 2%, respectively, with no spinal cord injuries observed. Complete false lumen thrombosis was achieved in 57% of patients as visualized by postoperative computed tomography angiography. After 3, 5 and 10 years, overall survival rates were 81, 74 and 65%, respectively, and the percentages of patients free from downstream dissection-related reoperation were 89, 86 and 80%, respectively. Multivariable analysis demonstrated that the risk factors for downstream aortic reoperation were patent false lumen, residual primary entry tear and connective tissue disorder. CONCLUSIONS: The surgical outcomes following aggressive treatment of AAAD are satisfactory. False lumen thrombosis can be achieved in a relatively high proportion of patients using this technique, resulting in a low rate of subsequent downstream aortic reoperations.
Keywords: acute disease; acute type A aortic dissection; adult; aged; aggressive primary entry resection; aortic dissection; aortic surgery; aortic root surgery; article; brain perfusion; cardiopulmonary bypass; cardiovascular mortality; computer assisted tomography; connective tissue disease; coronary artery bypass graft; emergency surgery; female; follow up; heart arrest; hemodialysis; hospital mortality; human; life expectancy; major clinical study; male; neurologic disease; operation duration; outcome assessment; overall survival; postoperative complication; priority journal; reoperation; retrospective study; shock; spinal cord injury; surgical mortality; tracheostomy; tracheotomy

A severe spinal muscular atrophy phenotype associated with a novel BICD2 mutation - Kichula E.A.; Medne L.; Zackai E.H.; Estilow T.; Harding B.; Banwell B.; Yum S.W. (Journal of the Peripheral Nervous System, 2016)
BICD2 has previously been reported as a rare cause of a distal-predominant spinal muscular atrophy (SMA). Here we report a case involving a De novo and novel mutation 2080C>T resulting in a more severe phenotype than has previously been reported. The patient was diagnosed antenatally with arthrogryposis congenital with normal fetal cardiac echocardiogram and brain MRI. She was born with severe arthrogryposis: flexion contractures of elbows, ulnar deviation at the wrist, bilateral flexion contractures of index fingers, severe hip flexion and knee extension contractures. She needed respiratory support with CPAP at birth. NCS/EMG done shortly after birth demonstrated unobtainable sensory responses. Motor responses in the lower extremities were absent and significantly decreased in amplitude with normal conduction velocity in the arm. EMG showed evidence of chronic denervation/reinnervation. Muscle biopsy demonstrated extensive fibrous and fatty tissue replacement of muscle with groups of tiny muscle fibers embedded in connective tissue, consistent with severe neurogenic atrophy. She continued to have significant respiratory requirements as well as difficulty handling secretions leading to tracheostomy. She has had gradual improvement since birth. By age of 21 months, she was able to reach her arms antigravity to grab for toys and bring hands to her mouth. She still had only minimal movement in her lower extremities. She had a few words, but with significant cognitive delay. She continues with tracheostomy, but primarily on CPAP while awake. This case has expanded the phenotype and genotype associated with BICD2 mutation.
Keywords: endogenous compound; adipose tissue; arthrogryposis; bodily secretions; brain; case report; denervation; diagnosis; echocardiography; elbow; female; fetus; flexion contracture; gene frequency; genetic predisposition; genotype; gravity; hereditary motor sensory neuropathy; hip; human; index finger; infant; intellectual impairment; knee; mouth; muscle biopsy; mutation; nuclear magnetic resonance imaging; phenotype; positive end expiratory pressure; reinnervation; skeletal muscle cell; spinal muscular atrophy; tracheostomy; ulna; velocity; wakefulness; wrist

Transverse Myelitis in Turner syndrome - Kopacek C.; Vieira S.; Capelo L.; Quadros F.; Kieling R.; Da Silva C.A. (Hormone Research in Paediatrics, 2016)
Background: Transverse Myelitis (TM) is an auto-immune syndrome with neural injury to the spinal cord. The TM may be first clinical manifestation of Multiple Sclerosis (MS). It is known that Turner's Syndrome (TS) is associated to the presence of autoimmune diseases. Case Report: A 15-year old female, began with manifestations of loss of strength on the lower limbs evolving rapidly with sensorial loss, tetraparesis and hemodynamic instability, requiring intubation. Two days later she was transferred to the intensive care unit (ICU). The patient had hypothyroidism diagnosed at the age of 2 and TS at age of 11, and had been in use of Vitamin D and calcium carbonate, conjugated estrogen, progestogen, levothyroxine, oxandrolone and growth hormone. On examination, she had BMI 29.5, Glasgow 15, flaccid tetraplegia and areflexia. MRI showed extensive hypointense signs on T1 and T2 on the central region of the cervical spine. She was diagnosed with TM and received 7 cycles of plasmapheresis, pulse therapy with methylprednisolone for 5 days followed by Rituximab with partial improvement of the strength and weaning of vasoactive drugs and mechanical ventilation. She was discharged from the ICU 2 months after admission, tracheostomized and bedridden. One month later she was discharged with prednisolone and azathioprine. Conclusion: TS is associated to the presence of autoimmune diseases (AID), though its association with TM or MS has been rarely reported. Despite the strong association between TS and AID is well known, the underlying immunopathogenic mechanism remains unexplained. Recent studies have displayed that TS patients do not show an excess of immunogenic risk markers. This is evocative for a higher responsibility of X-chromosome abnormalities in the development of AID. Early diagnosis and regular screening for potential associated autoimmune conditions are essential in the medical follow-up of TS patients.
Keywords: azathioprine; calcium; calcium carbonate; conjugated estrogen; endogenous compound; gestagen; growth hormone; levothyroxine; methylprednisolone; oxandrolone; prednisolone; rituximab; vitamin D; adolescent; areflexia; artificial ventilation; asthenia; autoimmune disease; body mass; case report; cervical spine; child; chromosome aberration; diagnosis; drug therapy; early diagnosis; female; follow up; hemodynamics; human; hypothyroidism; immobility; intensive care unit; intubation; lower limb; multiple sclerosis; myelitis; nuclear magnetic resonance imaging; plasmapheresis; preschool child; quadriplegia; responsibility; school child; screening; Turner syndrome; weaning

Postoperative management - Schraag S. (Best Practice and Research: Clinical Anaesthesiology, 2016)
Most patients undergoing major aortic surgery have multiple comorbidities and are at high risk of postoperative complications that affect multiple organ systems. Different aortic pathologies and surgical repair techniques have specific impact on the postoperative course. Ischemia–reperfusion injury is the common denominator in aortic surgery and influences the integrity of end-organ function. Common postoperative problems include hemodynamic instability due to the immediate inflammatory response, renal impairment, spinal cord ischemia, respiratory failure with prolonged mechanical ventilation, and gastrointestinal symptoms such as ileus or mesenteric ischemia. Focused care bundles to establish homeostasis and a team working toward an early functional recovery determine the success of effective rehabilitation and outcomes after aortic surgery.
Keywords: glucose; acute kidney failure; adult respiratory distress syndrome; analgesia; aortic surgery; artificial ventilation; aspiration pneumonia; bleeding; blood clotting disorder; body temperature monitoring; cerebrovascular accident; checklist; chest infection; chylothorax; cognitive defect; delirium; fluid therapy; glucose blood level; heart arrhythmia; heart failure; heart muscle ischemia; heart tamponade; hemodynamics; heparin induced thrombocytopenia; human; hypertension; hypotension; intensive care; intensive care unit; liver failure; malabsorption; mesenteric ischemia; metabolic regulation; neurologic examination; pancreatitis; pleura effusion; pneumonia; postoperative care; postoperative complication; priority journal; prognosis; respiratory failure; review; spinal cord ischemia; thoracotomy; tracheostomy; transfusion related acute lung injury; treatment outcome

Cardiac arrest due to pneumoperitoneum after PEG insertion in ALSpatient - Ceylan B.G.; Karabacak P.; Saygın H.; Demirel H.F.; Eroğlu F. (Journal of Clinical and Analytical Medicine, 2016)
Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, is characterized by motor neuron degeneration in the primary cortex, brainstem, and spinal cord. Percutaneous endoscopic gastrostomy (PEG) is a preferable method of nutritional support in patients with normal gastrointestinal function who can-not be fed orally for various reasons. PEG tube placement is recommended in amyotrophic lateral sclerosis (ALS) patients with dysphagia to provide reliable access for medications and nutrition. We report a case of a 63-year-old man with amyotrophic lateral sclerosis presenting with dysphagia and pneumoperitoneum following percutaneous endoscopic gastrostomy (PEG) placement. We also report on the intensive care period of this patient. PEG is a widely used nutrition therapy in these cases but complications such as pneumoperitoneum result in long term ICU stays and higher mortality rates.
Keywords: piperacillin plus tazobactam; riluzole; abdominal pain; adult; amyotrophic lateral sclerosis; article; case report; computer assisted tomography; dysphagia; dyspnea; heart arrest; hoarseness; human; male; middle aged; muscle atrophy; nasogastric tube; necrotizing enterocolitis; nutritional status; percutaneous endoscopic gastrostomy; pneumonia; pneumoperitoneum; reflex disorder; respiratory arrest; resuscitation; T tube; thorax radiography; tracheotomy; weakness; body weight loss

W44X mutation in the WWOX gene causes intractable seizures and developmental delay: A case report - Elsaadany L.; El-Said M.; Ali R.; Kamel H.; Ben-Omran T. (BMC Medical Genetics, 2016)
Background: WW domain containing oxidoreductase (WWOX) gene was cloned in 2000; alteration has been seen in many cancer cells. It acts as a tumor suppresser by blocking cell growth and causing apoptosis. WWOX protein showed different expression of mice brain and spinal cord, for which deletion causes seizure and early death. Case presentation: Clinical and molecular characteristics of a consanguineous family show a homozygous mutation of WWOX gene at specific bases, causing a debilitating syndrome characterized by growth retardation, intractable epilepsy, intellectual disability, and early death. Using Whole Exome Sequencing (WES), a novel homozygous mutation in the WWOX gene is identified in a consanguineous Arab family from Qatar with two daughters who presented with intractable seizure and developmental delay. Conclusion: The study presents the importance of human WWOX gene for brain development and the association between gene mutation and epileptic encephalopathy. It also highlights the power of WES particularly in clinically challenging cases.
Keywords: clobazam; clonazepam; genomic DNA; levetiracetam; messenger RNA; phenobarbital; phenytoin; topiramate; acute respiratory failure; article; artificial ventilation; aspiration; birth weight; brain atrophy; brain disease; bronchiolitis; case report; child; consanguineous marriage; demyelination; developmental disorder; disease course; disease severity; lack of drug effect; electroencephalogram; epileptic discharge; exome; feeding difficulty; female; fever; frontal lobe; gastrointestinal symptom; gene; gene mutation; genetic variation; head circumference; hospital admission; human; hypersalivation; intractable epilepsy; muscle contracture; muscle hypertonia; muscle strength; muscle weakness; myoclonus; nasogastric tube; neuroimaging; nuclear magnetic resonance imaging; pathogenesis; persistent vegetative state; positive end expiratory pressure; proximal humerus fracture; psychomotor retardation; respiratory failure; school child; scoliosis; seizure; sequence analysis; stomach tube; tachypnea; temporal lobe; tonic seizure; tracheostomy; treatment response; visual evoked potential; WWOX gene

Neuropathologic and neurobiologic characterization of early onset amyotrophic lateral sclerosis associated with a novel TDP43 S375G variant - Newell K.; Ghetti B.; Murrell J.; Romano M.; Salis E.; Stuani C.; Buratti E. (Journal of Neurochemistry, 2016)
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with onset most frequently occurring in middle age. Familial ALS may have an earlier onset. A 22 year-old woman presented with painless weakness of the right foot and leg. An EMG showed denervation involving the muscles of the right lower extremity. Weakness progressed to the left lower extremity and subsequently to the upper extremities. The clinical diagnosis was ALS. By the age of 24, she used a wheelchair and required a tracheostomy. She died at age 26. A family history of ALS was reported in distant relatives. An autopsy was carried out, and the neuropathologic evaluation showed upper and lower motor neuron loss, corticospinal tract degeneration, and neurogenic changes in skeletal muscle. TDP43 and FUS-immunoreactive neuronal and glial inclusions were present in motor cortex, putamen, globus pallidus, thalamus, substantia nigra, inferior olivary nucleus, and anterior horns of the spinal cord. DNA was extracted from brain tissue, and a TDP43 S375G change was found. A C9ORF72 expansion was not identified. To determine whether the TDP43 variant is pathogenic, we have studied a recombinant protein carrying the S375G change via transfection in HeLa cells. Our results show that this variant does not appear to alter the predominantly nuclear localization of TDP43. However, the variant protein shows increased ability to induce CFTR exon 9 skipping in a minigene-based add-back splicing assay with respect to wild-type TDP43. This result suggests that the S375G substitution may act through a gain-of-function mechanism. We tested a nearby TDP43 mutation, N378D, previously identified in other patients, and found that it also caused a similar gain-of-function effect on the splicing ability of TDP43. Taken together, these results highlight the possible presence in the C-terminus of a novel region capable of affecting the splicing regulation function of TDP43.
Keywords: recombinant protein; TAR DNA binding protein; adult; amyotrophic lateral sclerosis; autopsy; brain tissue; case report; denervation; diagnosis; DNA structure; family study; female; foot; gene mutation; genetic transfection; globus pallidus; human; inferior olivary nucleus; motoneuron; motor cortex; putamen; pyramidal tract; RNA splicing; skeletal muscle; spinal cord ventral horn; substantia nigra; thalamus; tracheostomy; upper limb; weakness; wheelchair; wild type

A case report of acute injury in ankylosing spondylitis - Yeh Y.-H.; Su Y.-J. (Journal of Acute Disease, 2016)
Ankylosing spinal disorders tend to fracture after minor trauma because of its changed biomechanical properties. Herein we presented an ankylosing spinal disorders case with cervical spine fracture in a trauma after neck protection and surgical airway application. On CT scan of cervical spine, ankylosing spondylitis with an extension C4–C5 fracture was found. The patients' family decided not to receive operation to immobilize his cervical spine, then he was admitted to our surgical intensive care unit. He was discharged against medical advice two months later with a permanent tracheostomy with ventilator dependence due to high-level spinal cord injury. Timely identification of unstable fractures is instrumental in avoiding adverse neurologic sequela.
Keywords: adult; ankylosing spondylitis; article; blunt trauma; case report; cervical collar; cervical spine fracture; computer assisted tomography; dyspnea; fracture immobilization; human; hypoxia; lung ventilation; male; middle aged; motor vehicle; priority journal; quadriplegia; spinal cord injury; tracheostomy; tracheotomy; traffic accident; unconsciousness; ventilator

Tracheostomy following anterior cervical spine fusion in trauma patients - Binder H.; Lang N.; Tiefenboeck T.M.; Bukaty A.; Hajdu S.; Sarahrudi K. (International Orthopaedics, 2016)
Purpose: Traumatic injuries to the cervical spine are frequently accompanied by cervical spinal cord injuries—often necessitating tracheostomy. The purpose of this study was to evaluate patient characteristics and outcomes after undergoing anterior cervical spine fusion (ACSF) with tracheostomy. Methods: All patients with cervical spine injury (CSI) who underwent ACSF and tracheostomy between December 1992 and June 2014 were included in this retrospective data analysis. The study group consisted of 32 men (84 %) and six women (16 %), with an average age of 47 ± 20 years. Blunt trauma to the cervical spine was the cause of CSI in all 38 patients. Results: The mean Injury Severity Score (ISS) was 30.50 ± 6.25. Eighteen patients sustained severe concomitant injuries related to the spinal injury. In 15 patients (39.5 %), traumatic brain injury (TBI) with fractures of the cranium and/or intracranial lesions were observed. The mean Glasgow Coma Scale (GCS) score was 11 ± 4.5 (range 3–15). Two tracheostomies (5.3 %) were performed simultaneously with ACSF. The remaining 36 were performed with an average “delay” of 15 ± ten days. We observed no difference in time to tracheostomy among patients initially presenting with an American Spinal Injury Association (ASIA) score of either A, B, C or D. Only two patients (5.3 %) were identified as having an infection at the site of ACSF after placement of a tracheostomy. There were no deaths directly related to airway difficulties in our cohort. Conclusions: Our data show that tracheostomy is safely performed after an average of 15 days post-ACSF, thereby being associated with a very low rate of complications. However, future prospective randomised studies are needed to identify the optimal timing of tracheostomy placement after ACSF. Level of evidence: IV; retrospective case series.
Keywords: adult; adult respiratory distress syndrome; American Spinal Injury Association impairment scale; anterior spine fusion; article; blunt trauma; brain damage; cervical spinal cord injury; cervical spine; cervical spine injury; clinical article; female; follow up; Glasgow coma scale; hospital admission; hospitalization; human; infection rate; intensive care unit; limb fracture; male; middle aged; priority journal; retrospective study; skull fracture; surgical technique; thorax injury; tracheostomy; traumatic brain injury

Needs assessment of adults post-intensive care rehabilitation department in Amiens university hospital - Bacari-Risal G.; Doutrellot P.L.; Vaysse B.; Inan M.; Khelfoun K.; Nasserallah M.; Maizel J.; Legrand-Monteil C.; Tasseel-Ponche S. (Annals of Intensive Care, 2016)
Introduction A part of patients transferred from intensive care unit (ICU) to continuing care services is due to the persistence of important functional failures. The role of early rehabilitation in the management of these patients has shown interest. The creation of unit, with both expertise of intensivists and physics Doctor, would improve the healthcare network. But physical and rehabilitation medicine requires specialized human resources and equipment. This retrospective epidemiological pilot study was to determine the needs of Amiens University Hospital in post-intensive care rehabilitation department (PICRD). Patients and methods We determined the number of patients eligible to PICRD, during 2013. The Programme Médicalisé des Systèmes d'Information allowed to count all patients admitted to medical-surgical ICUs in Amiens University Hospital. Patients who had brain injury, spinal cord injury, or severe peripheral neurological damage regardless of origin were included in the study among the ICU patients. Even if they did not suffer from those pathologies, patients with duration of hospitalization (DH) over 7 days in the ICU were also included. The need for PICRD has been evaluated by computing the number of eligible patients in PICRD. They were eligible to PICRD: patients with persistent neurological impairment in their day 10 of hospitalization, without hemodynamic disability in absence of catecholamine, with a tracheotomy when a ventilator weaning was impossible. It was applied to the group “eligible” a new release date, which corresponded to day 2 after the realization of tracheotomy and/or stopping of catecholamine, and/or stopping continuous invasive treatments. This allowed calculating a new DH and therefore a new average length of stay. Over 70 and under 18 years old and those with undergoing neoplasia were excluded from the study. Results A total of 2690 hospitalizations in ICU were counted at the Amiens University Hospital in 2013. Seven hundred and seventeen patients were included in the study; after application of the exclusion criteria, a total of 388 were analyzed. This group of patients consisted of 72 % men, and the median age was 54 years old (IQR = 20.25). The median SAPS II was 42 (IQR = 28) with a median length of stay of 12 days (IQR = 14) and a mortality rate of 19.6 %. As regards the reasons for hospitalization: over half, 56 %, fell to neurological impairment, with stroke (25 %), severe traumatic brain injury (STBI = 22 %) and spinal cord injury (SCI = 6 %). The other half consisted mostly shocks (19 %), acute respiratory failure (8 %), acute respiratory distress syndrome (ARDS = 4.5 %), severe sepsis (5 %), cardiopulmonary arrest (2.5 %). Nearly 22 % of patients (n = 84) were eligible for PICRD. They were young: median of age 55 years old (IQR = 20), mostly men (81 %). The median SAPS II was 38 (IQR = 23.25). The main diseases were also neurological for 46 % with: stroke, STBI and SCI. Other causes were: shock for 14 %, acute respiratory distress and ARDS for a total of 28 %, severe sepsis for 11 %. Seventy-seven of them came out alive from the ICU, 20 % in intensive care in “Berck”, 10 % in physical and rehabilitation medicine unit, 50 % in medicine or surgery services. After application of the new date of stay, a significant difference in hospital stay of 40 days (p < 0.0001) was demonstrated. Conclusion There is a need of adults PICRD at Amiens University Hospital. It would allow an early medical and rehabilitative treatment, improving the fluidity of the post-intensive care discharge, and lead to a reduction in socioeconomic costs of care of these patients who need no more ICU care.
Keywords: catecholamine; intensive care; university hospital; rehabilitation center; resuscitation; society; adult; needs assessment; human; patient; hospitalization; spinal cord injury; sepsis; length of stay; rehabilitation; cerebrovascular accident; tracheotomy; Simplified Acute Physiology Score; male; rehabilitation medicine; adult respiratory distress syndrome; cardiopulmonary arrest; pathology; intensivist; brain injury; traumatic brain injury; mortality; neoplasm; invasive procedure; artificial ventilation; pilot study; acute respiratory failure; health care; physician; disability; surgery; respiratory distress; diseases; physics; intensive care unit

Osteopathia striata with cranial sclerosis (OSCS): review of the literature and case report demonstrating challenges of spinal fusion after trauma - Katsevman G.A.; Turner R.C.; Lucke-Wold B.P.; Sedney C.L.; Bhatia S. (Acta Neurochirurgica, 2016)
Osteopathia striata with cranial sclerosis (OSCS) is a rare but well-described pathology characterized by abnormalities in bone deposition in the axial and cranial skeleton as well as other abnormalities and associated deficits. These skeletal abnormalities can lead to significant intra-operative challenges for the surgeon and influence outcomes for the patient. In this report, we present a case of a patient with OSCS who was involved in a traumatic motor vehicle crash and underwent posterior cervico-thoracic fusion for a T4 chance fracture. Bony abnormalities in the cervico-thoracic spine presented a significant operative challenge due to alterations in bony anatomy and bone architecture. This case serves as an example of the challenges that the spine surgeon faces when dealing with OSCS, and highlights the differences between OSCS and commoner skeletal hyperplasias such as osteopetrosis.
Keywords: suxamethonium; acute disease; adult; Albers Schoenberg disease; arachnoid cyst; article; bone malformation; bone microarchitecture; brain cyst; brain ventricle dilatation; case report; cervico thoracic fusion; chondrodysplasia; cleft palate; computer assisted tomography; corpus callosum; corpus callosum agenesis; crush trauma; diffuse axonal injury; fasciotomy; female; femur subtrochanteric fracture; frontal bossing; Glasgow coma scale; hospitalization; human; hyperlipidemia; hypothyroidism; lung contusion; medial collateral ligament; medical history; mental health; middle aged; motor vehicle; nuclear magnetic resonance imaging; optic nerve; optic nerve disease; optic nerve impingement; osteopathia striata with cranial sclerosis; osteosclerosis; percutaneous endoscopic gastrostomy; posterior fossa; postoperative period; priority journal; rib fracture; spine fusion; spine radiography; stomach tube; subarachnoid hemorrhage; thoracic spine; tracheostomy; traumatic brain injury

Postoperative extradural hematoma of the cervical spine: A rare but avoidable complication - Tomar G.S.; Kumar S.; Dube S.K.; Goyal K. (Journal of Clinical Anesthesia, 2016)
Keywords: anesthetic agent; adult; bleeding; breathing exercise; case report; cervical spine injury; comorbidity; emergency surgery; epidural hematoma; erythrocyte concentrate; erythrocyte transfusion; human; iatrogenic disease; immobility; incidence; intensive care unit; intubation; letter; male; massage; middle aged; nuclear magnetic resonance imaging; postoperative complication; priority journal; reoperation; spinal cord compression; spine surgery; tracheobronchial toilet; tracheostomy; Valsalva maneuver

Nothing will keep me down: A case of descending weakness in a patient with a pure motor guillain-barre syndrome variant - Leung P. (Journal of General Internal Medicine, 2016)
LEARNING OBJECTIVE #1: To recognize the presentation of a motor variant of Guillain-Barre Syndrome with atypical features LEARNING OBJECTIVE #2: To manage a patient with an acute motor axonal neuropathy CASE: 66 year old woman with no significant past medical history presents with descending weakness and pain. Four days prior to admission, she developed bilateral proximal upper extremity dull, aching pain followed by difficulty raising her arms above her head. She went to an emergency department and was discharged with pain medicines. Two days prior to presentation, she had difficulty ambulating due to lower extremity weakness and pain. On arrival, she was unable to stand. She denied any confusion, vision changes, jaw pain, chest pain, shortness of breath, abdominal pain, bowel/bladder incontinence, or other sensation changes. One week prior to hospitalization, she hiked effortlessly on a well-paved trail in the Mid-Atlantic and did not recall any bug bites. She also described a brief diarrheal illness around the same time. She denied recent travel, recent influenza vaccination, ingestion of canned foods, infected wounds, and history of intravenous drug use. Her physical exam is notable for pain with any movement of her arms and legs. Her muscles are tender on palpation. No rashes are appreciated. Her speech is intact. She has intact cranial nerves. She has 3/5 strength at bilateral deltoids and hips, 4/5 at bilateral wrists and knees, and 5/5 with dorsiflexion and plantarflexion. She is intact to light touch and hypo-reflexive throughout. She had down-going toes. Her CBC, BMP, and creatine kinase were all within normal limits. MRI brain did not reveal any acute intracranial pathology. MRI cervical spine only showed C3-C4 disc protrusion without spinal cord impingement. Lumbar puncture showed 1 wbc, 1 rbc, negative gram stain, glucose 55, and protein 69, consistent with cytoalbuminologic dissociation. Her negative inspiratory force (NIF) was at -30. Given rapid progression in symptoms and picture most consistent with Guillian-Barre Syndrome (GBS), plasma exchange (PLEX) was initiated. Electromyography (EMG) was consistent with pure motor axonal GBS. She was also ganglioside antibody positive. Infectious work up was largely negative. Despite receiving plasma exchanges, her NIF progressively worsened to -18 and she was intubated for airway protection. Subsequently, she was given intravenous immunoglobulin (IVIG) and later required a tracheostomy. Repeat EMG 1 month after admission showed minimal improvement in motor function. Ultimately, she was discharged to acute rehabilitation. DISCUSSION: GBS is an immune-mediated polyradiculoneuropathy with a mean age of onset of over 40 and with a male predominance. Worldwide incidence of GBS is quoted at 0.6 to 4/100,000 people. Typically, GBS presents with hyporeflexia and neuropathic pain in a symmetric ascending pattern. Our patient's descending presentation (less commonly seen in GBS) led us to consider botulism. However, this idea was quickly dismissed given lack of typical exposures to botulinum toxin. Her notable proximal muscles weakness also triggered concerns over possible acute myopathy/myositis. However with such a quick onset and a normal creatine kinase, the likelihood was low. This patient's clinical pattern is most consistent with acute motor axonal neuropathy (AMAN), a pure motor axonal subtype of GBS rarely seen in North America (3-5 % of all GBS cases in North America). It is frequently associated with Campylobacter jejuni enteritis and the molecular mimicry of human gangliosides by C. jejuni lipo-oligosaccharides. Compared to the most common GBS subtype, acute inflammatory demyelinating polyradiculopathy (AIDP), AMAN typically has preserved reflexes (unlike this patient), less cranial nerve involvement, less autonomic dysfunction, and a more rapid progression of disease. Interestingly, despite having a pure motor GBS, this patient had significant pain on arrival. Unlike typical neuropathic pain that results from the degeneration of sensory nerve fibers as seen in AIDP, the pain she experienced in the acute phase of her disease was likely of nociceptive origin secondary to inflammation or inflammatory mediators. Another notable difference between more common forms of GBS is that recovery from axonal degradation (as in AMAN) takes longer than recovery from demyelination (as in AIDP). Regaining the ability to walk, if possible, may range from months to years. Supportive care plays a large role in AMAN as roughly 30% of GBS cases progress to respiratory failure. Frequent telemetry and respiratory monitoring is critical. PLEX (to remove autoantibodies, immune complexes, and other inflammatory mediators) and IVIG (to inhibit costimulatory molecules involved in antigen presentation and attenuate autoantibody-initiated inflammation) had both been shown in large randomized control trials to be effective therapies. However, patients in these studies were predominantly diagnosed with AIDP rather than AMAN. It is debated whether combined PLEX and IVIG (which our patient received) offer improved outcomes over monotherapy with PLEX or IVIG. Corticosteroids have no role in the treatment of GBS. Despite usage of these immunemodulating treatments, approximately 20 % of patients are left with severe disability.
Keywords: autoantibody; creatine kinase; immunoglobulin; ganglioside; ganglioside antibody; glucose; protein; botulinum toxin; oligosaccharide; corticosteroid; patient; human; Guillain Barre syndrome; society; internal medicine; weakness; pain; arm; acute motor axonal neuropathy; North America; thorax pain; neuropathic pain; inflammation; plasmapheresis; leg; cranial nerve; muscle; abdominal pain; hip; dyspnea; speech; rash; jaw pain; palpation; drug use; wound; rehabilitation; polyradiculoneuropathy; onset age; botulism; exposure; Campylobacter enteritis; wrist; male; canned food; airway; electromyography; ingestion; influenza vaccination; dissociation; Gram staining; travel; diseases; emergency ward; medical history; recall; leukocyte; disability; protection; tracheostomy; motor performance; therapy; hyporeflexia; lumbar puncture; antigen antibody complex; spinal cord; hospitalization; cervical spine; sensation; pathology; molecular mimicry; demyelination; radiculopathy; reflex; brain; nerve fiber; toe; sensory nerve; degeneration; respiratory failure; telemetry; monitoring; autonomic dysfunction; knee; antigen presentation; monotherapy; incontinence; nuclear magnetic resonance imaging; female; electromyogram

The incidence of airway complications following posterior occipitocervical spine fusion - Sheshadri V.; Moga R.; Manninen P.; Venkatraghavan L. (Anesthesia and Analgesia, 2016)
INTRODUCTION: The management of the airway may be challenging in patients undergoing occipitocervical spine fusions (OCF). Limited information is available regarding the incidence, severity, the mechanism, and the risk factors for postoperative airway complications after OCF. Changes in the occipitocervical angle (dOC2A) of fusion after surgery may result in acute airway obstruction, dyspnea and/or dysphagia.1,2 The aim of this study was to determine the incidence, nature, and risk factors for postoperative airway complications in patients undergoing OCF and to determine the relationship between the dOC2A and airway complications. METHODS: After IRB approval, we retrospectively reviewed the charts of all patients who underwent OCF from 2005-2013. We excluded patients who had combined anterior/posterior or revision surgeries and those already intubated or with tracheostomy. Data collected included patient demographics, airway management, anesthesia and surgical data, and postoperative complications. Plain lateral radiographs or computed tomography were used to measure the dOC2A (Figure 1). Immediate postoperative airway complications included in the analysis were the need for reintubation and the delay of extubation in the operating room. Delayed complications were tracheostomy, pneumonia and mortality. Statistical analyses were done using unpaired t test, Mann-Whitney U test, Chi-square test and Fisher's exact test, as appropriate. P-value of <0.05 was considered significant. RESULTS: Records of 59 patients were reviewed. Demographic data are shown in Table 1. Common indications for surgery included degenerative, rheumatoid arthritis, metastases and fracture. Following extubation in the operating room (OR), there were no complications in 43 (73%) patients (Group 1). Airway complications were seen in 16 (27%) patients (Group 2); 4 patients required re intubation (2 in the OR, 2 in post anesthetic care unit), and 12 had delayed extubation and were taken to the intensive care unit intubated. The number of vertebral levels fused, presence of difficult intubation and duration of surgery were significantly associated with airway complications. There was no significant difference in the dOC2A between the groups (-1.070±5.527 versus -4.375±10.788, p=0.127) (Table 2). CONCLUSIONS: Airway management in patients undergoing OCF poses a challenge for anesthesiologists. The incidence of airway complications was 27%. The decision to extubate needs to be individualized, and factors such as difficult intubation, number of vertebral levels fused and the duration of the surgery has to be considered. We could not find a significant correlation between dOC2A and postoperative airway complications. The risk factors for postoperative airway complications are multifactorial and there is a need for prospective study to identify the risk factors.
Keywords: endogenous compound; airway obstruction; anesthesia; anesthesist; chi square test; complication; computer assisted tomography; demography; dysphagia; dyspnea; extubation; fracture; human; information processing; intensive care unit; intubation; major clinical study; metastasis; mortality; operating room; pneumonia; postoperative complication; prospective study; rank sum test; respiration control; rheumatoid arthritis; risk factor; spine fusion; statistical significance; Student t test; surgery; tracheostomy; vertebra; X ray film

The ugly truth of sagliker syndrome - Shepherd K.J.; Barker B.R. (Journal of General Internal Medicine, 2016)
LEARNING OBJECTIVE #1: Recognize the clinical features of Sagliker Syndrome (SS). LEARNING OBJECTIVE #2: Recognize the importance of early diagnosis and treatment of ESRD to slow the progression of SS. CASE: A 23-year-old female with history of end stage renal disease (ESRD) of unknown etiology on emergent dialysis since the age of 18, secondary hyperparathyroidism, seizures, chronic respiratory failure with a tracheostomy was admitted to the ICU for pneumonia, requiring ventilator support and continuous renal replacement therapy. Her exam was particularly notable for short stature with height of 3 ft and a very thin and distorted trunk and extremities due to frequent fractures. She was wheelchair bound and unable to ambulate. Her head comprised approximately 1/3 of her body mass with severe maxillary and mandibular bony enlargement. Her oral exam revealed extensive tooth loss and bleeding gums. Her appearance was distinctly different from pictures her family shared from just 3 years prior. In addition, her lab values showed substantial abnormalities including PTH >5000 pg/mL, calcium 7 mg/dL; phosphorous 2 mg/dL, vitamin D-25-OH 9.8 ng/dL, and alkaline phosphatase 827 units/L. This pattern of physical exam findings and extreme abnormalities was felt consistent with the unusual disorder, Sagliker Syndrome. Her secondary hyperparathyroidism was refractory to optimal medical management, and her hospital course was further complicated by endocarditis, right atrial thrombus, and inability to be weaned off of the ventilator. After nearly 90 days in the hospital, she developed acute neurologic changes, and CT head showed massive frontal lobe hemorrhage. The family ultimately decided to withdraw care. DISCUSSION: Yahya Sagliker, M.D. described Sagliker Syndrome (SS) in 2004. By 2012, only 60 cases had been recognized, however, Sagliker estimates the observed incidence to be near 0.5 %. Sagliker described this special population of patients with ESRD with an “uglifying” facial appearance, short stature, severe maxillary and mandibular changes, finger abnormalities, dental abnormalities, soft tissue tumors of the mouth, and neurologic and psychiatric symptoms. These patients are most often children who had a delay in treatment of renal insufficiency and are most frequently seen in underdeveloped countries. It has also seen in adults related to a severe form of renal osteodystrophy with parathyroid hyperplasia leading to lab abnormalities, as seen in our patient. These patients suffer from severe peripheral and central nervous system complications from the chaotic bone growth, including spinal cord compression, deafness, blindness, cortical deficits, as well as respiratory failure due to thoracic bone involvement. Vascular and neurologic impairment are the leading causes of death. While there is no treatment and it is unclear why certain patients develop this syndrome, a small study concluded that SS should be added to indications for total parathyroidectomy as this treatment has improved psychiatric symptoms and lab abnormalities. However, it could not reverse the skeletal malformations. Ultimately, there is no treatment beyond slowing the progression of the disease with optimal medical management.
Keywords: fenticlor; alkaline phosphatase; vitamin D; calcium; parathyroid hormone; internal medicine; society; human; patient; ventilator; hospital; disease management; end stage renal disease; maxilla; mental disease; short stature; secondary hyperparathyroidism; bleeding; pneumonia; limited mobility; tracheostomy; fracture; endocarditis; population; body mass; height; chronic respiratory failure; etiology; female; diseases; early diagnosis; cerebral blindness; death; skeleton malformation; seizure; continuous renal replacement therapy; heart atrium thrombosis; frontal lobe; clinical feature; facies; soft tissue tumor; spinal cord compression; child; bone growth; central nervous system; parathyroid hyperplasia; renal osteodystrophy; periodontal disease; adult; hearing impairment; developing country; respiratory failure; bone; kidney failure; parathyroidectomy; dialysis

Percutaneous tracheostomy in post-cervical spine fixation patients: Challenging the dogma - Gupta S.; Singh P.; Soni K.D.; Aggarwal R.; Yadav D. (American Journal of Emergency Medicine, 2016)
Keywords: artificial ventilation; bronchoscopy; bronchospasm; cervical spine fixation; endotracheal tube; female; heart arrest; human; letter; percutaneous dilatational tracheostomy; perioperative period; postoperative period; priority journal; quadriplegia; spine stabilization; surgical patient; tracheostomy; traffic accident; ultrasound; X ray

En Bloc Resection of Primary Malignant Bone Tumor in the Cervical Spine Based on 3-Dimensional Printing Technology - Xiao J.-R.; Huang W.-D.; Yang X.-H.; Yan W.-J.; Song D.-W.; Wei H.-F.; Liu T.-L.; Wu Z.-P.; Yang C. (Orthopaedic Surgery, 2016)
Objective: To investigate the feasibility and safety of en bloc resection of cervical primary malignant bone tumors by a combined anterior and posterior approach based on a three-dimensional (3-D) printing model. Methods: Five patients with primary malignant bone tumors of the cervical spine underwent en bloc resection via a one-stage combined anteroposterior approach in our hospital from March 2013 to June 2014. They comprised three men and two women of mean age 47.2 years (range, 26–67 years). Three of the tumors were chondrosarcomas and two chordomas. Preoperative 3-D printing models were created by 3-D printing technology. Sagittal en bloc resections were planned based on these models and successfully performed. A 360° reconstruction was performed by spinal instrumentation in all cases. Surgical margins, perioperative complications, local control rate and survival rate were assessed. Results: All patients underwent en bloc excision via a combined posterior and anterior approach in one stage. Mean operative time and estimated blood loss were 465 minutes and 1290 mL, respectively. Mean follow-up was 21 months. Wide surgical margins were achieved in two patients and marginal resection in three; these three patients underwent postoperative adjuvant radiation therapy. One vertebral artery was ligated and sacrificed in each of three patients. Nerve root involved by tumor was sacrificed in three patients with preoperative upper extremity weakness. One patient (Case 3) had significant transient radiculopathy with paresis postoperatively. Another (Case 4) with C 4 and C 5 chordoma had respiratory difficulties and pneumonia after surgery postoperatively. He recovered completely after 2 weeks’ management with a tracheotomy tube and antibiotics in the intensive care unit. No cerebrovascular complications and wound infection were observed. No local recurrence or instrumentation failure were detected during follow-up. Conclusion: Though technically challenging, it is feasible and safe to perform en bloc resection of cervical primary bone tumors. This is the most effective means of managing cervical spine tumors. Preoperative 3-D printing modelling enables better anatomical understanding of the relationship between the tumor and cervical spine and can assist in planning the surgical procedure.
Keywords: adjuvant radiotherapy; adult; aged; article; cancer radiotherapy; cancer recurrence; cancer staging; cancer surgery; cancer survival; chondrosarcoma; chordoma; clinical article; computed tomographic angiography; computed tomography scanner; diaphragm paralysis; dysphagia; en bloc resection; feasibility study; female; follow up; Horner syndrome; human; image reconstruction; intensive care unit; male; middle aged; nuclear magnetic resonance imaging; operation duration; outcome assessment; paresis; pneumonia; postoperative hemorrhage; preoperative period; radicular pain; radiculopathy; recurrent laryngeal nerve palsy; retrospective study; spine surgery; spine tumor; survival rate; three dimensional printing; tracheotomy; tumor diagnosis; wound infection; CT scanner

ALS patients with ability to communicate after long-term mechanical ventilation have confined degeneration to the motor neuron system - Mochizuki Y.; Hayashi K.; Nakayama Y.; Shimizu T.; Kamide M.; Ogino M.; Komori T.; Hasegawa M.; Isozaki E.; Nakano I. (Journal of the Neurological Sciences, 2016)
Objective To clarify the position in the amyotrophic lateral sclerosis (ALS) spectrum, of a subgroup of patients who maintained the ability to communicate after long-term mechanical ventilation (LTMV) by tracheostomy. Methods We undertook a clinicopathological investigation of sporadic ALS in three patients who maintained the ability to communicate after approximately 30-year survival on LTMV by tracheostomy. Results The age of onset and duration of disease was 48 years and 31 years in patient 1, 55 years and 29 years in patient 2, and 31 years and 33 years in patient 3, respectively. Each patient displayed slow disease progression. In all patients, both upper and lower motor neurons were markedly degenerated, while other neuronal systems and the brainstem tegmentum were spared. A few normal-looking motor neurons remained in the anterior horn of the spinal cord. There were no TAR DNA-binding protein 43-immunoreactive inclusions in the lower motor neurons in any patient and only occasional inclusions in the cerebral cortex of one patient. Conclusion The clinicopathological findings of these three patients suggest that there is a distinct subgroup of ALS patients characterized by the above-mentioned features.
Keywords: TAR DNA binding protein; adult; amyotrophic lateral sclerosis; arm weakness; article; artificial ventilation; brain cortex; brain stem; brain tissue; case report; cell inclusion; clinical feature; disease course; disease duration; human; human tissue; inferior olivary nucleus; interpersonal communication; male; medulla oblongata; middle aged; motoneuron; muscle atrophy; neuropathology; onset age; priority journal; reticular formation; spasticity; spinal cord; spinal cord atrophy; spinal cord ventral horn; survival; tegmentum; tracheostomy; tumor spheroid; Western blotting

A case series expanding the phenotypic variations in spinal muscular atrophy type I - Lopez M.; Wagle M.; Abid F. (Neurology, 2016)
Objective: To expand the spectrum of phenotypes in patients with Spinal Muscular Atrophy Type I Background: Spinal Muscular Atrophy (SMA) is the most common infant motor neuron disease and a leading cause of infant death. Those with Type I SMA present with symptoms within the first 6 months and never roll, sit, walk, and run. Death is expected before the age of 2 years, with one paper demonstrating some survival past 4 years with respiratory intervention. Methods: The medical records of our patients were reviewed for pertinent findings associated with SMA. Results: We have identified a series of eight patients between the ages of 17 months and 14 years who clinically fit the phenotype of SMA Type I. Each patient presented with symptoms of hypotonia, delayed motor skills, weakness, areflexia, and fasciculations within the first six months of age. Genetic testing for all patients revealed homozygous deletions of exons seven and eight of SMN1. Patients of 17 months, 2 years, 5 years, and 14 years of age were not dependent on tracheostomy and mechanical ventilation. See Table 1 for pulmonary status by age. One patient was tested and found to have only three copies of SMN2. Table 1. Age Pulmonary 17 mo Cough assist 2 yrs BiPAP 3 yrs Trach/Mechanical ventilation 5 yrs Cough assist, BiPAP at night, IPV 6 yrs Trach/mechanical ventilation 7 yrs Trach/mechanical ventilation 12 yrs Trach/mechanical ventilation 14 yrs Noninvasive ventilation (PPIV) Conclusions: We report a series of eight patients with clinical presentation and genetic testing consistent with SMA Type I. Presently, six out of eight patients in the series have survived more than two years. This series expands the phenotype of SMA Type I, which has important implications for prognosis. It suggests that future investigations into gene modifying factors, such as SMN2 copy number, may be beneficial.
Keywords: phenotypic variation; spinal muscular atrophy; American; neurology; case study; human; patient; air conditioning; phenotype; genetic screening; non continuous ventilator; motor neuron disease; areflexia; motor performance; weakness; muscle hypotonia; prognosis; medical record; exon; tracheostomy; night; infant; survival; artificial ventilation; death; noninvasive ventilation; child death; modifier gene

A case series expanding the phenotypic variations in spinal muscular atrophy type I - Lopez M.; Wagle M.; Abid F. (Neurology, 2016)
Objective: To expand the spectrum of phenotypes in patients with Spinal Muscular Atrophy Type I Background: Spinal Muscular Atrophy (SMA) is the most common infant motor neuron disease and a leading cause of infant death. Those with Type I SMA present with symptoms within the first 6 months and never roll, sit, walk, and run. Death is expected before the age of 2 years, with one paper demonstrating some survival past 4 years with respiratory intervention. Methods: The medical records of our patients were reviewed for pertinent findings associated with SMA. Results: We have identified a series of eight patients between the ages of 17 months and 14 years who clinically fit the phenotype of SMA Type I. Each patient presented with symptoms of hypotonia, delayed motor skills, weakness, areflexia, and fasciculations within the first six months of age. Genetic testing for all patients revealed homozygous deletions of exons seven and eight of SMN1. Patients of 17 months, 2 years, 5 years, and 14 years of age were not dependent on tracheostomy and mechanical ventilation. See Table 1 for pulmonary status by age. One patient was tested and found to have only three copies of SMN2. Table 1. Age Pulmonary 17 mo Cough assist 2 yrs BiPAP 3 yrs Trach/Mechanical ventilation 5 yrs Cough assist, BiPAP at night, IPV 6 yrs Trach/mechanical ventilation 7 yrs Trach/mechanical ventilation 12 yrs Trach/mechanical ventilation 14 yrs Noninvasive ventilation (PPIV) Conclusions: We report a series of eight patients with clinical presentation and genetic testing consistent with SMA Type I. Presently, six out of eight patients in the series have survived more than two years. This series expands the phenotype of SMA Type I, which has important implications for prognosis. It suggests that future investigations into gene modifying factors, such as SMN2 copy number, may be beneficial.
Keywords: phenotypic variation; spinal muscular atrophy; American; neurology; case study; human; patient; air conditioning; phenotype; genetic screening; non continuous ventilator; motor neuron disease; areflexia; motor performance; weakness; muscle hypotonia; prognosis; medical record; exon; tracheostomy; night; infant; survival; artificial ventilation; death; noninvasive ventilation; child death; modifier gene

Physiotherapy and noninvasive respiratory management as adjuvant to prevent early tracheostomy in spinal muscular atrophy type i - Magalhães P.A.F.; D'Amorim A.C.G.; Mendes A.P.A.; Ramos M.E.A.; De Almeida L.B.S.; Do Carmo Menezes Bezerra Duarte M. (Pediatric Pulmonology, 2016)
Objective: Spinal atrophy type 1 (SMA1) is an autosomal recessive genetic disease which promotes cell death ofmotor neurons located in the ventral horn of the spinal cord and brainstemnuclei engines. Earlymorbidity andmortality in this group is associated with bulbar dysfunction and respiratory failure requiring hospitalization and artificial ventilation. A large proportion of this population is considered difficult to wean from mechanical ventilation. Tracheostomy is considered treatment of choice for children with SMA 1. However, recent studies have been suggested noninvasive respiratory management as adjuvant to prevent early tracheostomy. The aim of the present study was to promulgate awareness of maintaining a patient with SMA 1 without the use of invasive ventilation and with a personalized physiotherapy protocol, thereby providing better quality of life and integration with family members. Case description: a case study of a minor diagnosed withSMA1, who, at the age of elevenmonths, underwent invasive mechanical ventilation (IMV) for 76 days, with successful weaning after application of a respiratory physiotherapy protocol, including use of mechanically assisted coughing and non-invasive ventilation (NIV). Discussion: despite the difficulties and complications observed, the assistance proposed achieved the objective of removal of IMV and transfer to home care using non-invasive ventilation. IMV via tracheostomy is the treatment of choice in Brazil but families need to be informed of the irreversibility of the disease and the strategic possibilities of current therapies (IMV, NIV and palliative care) for management of a child with severe SMA 1.
Keywords: adjuvant; awareness; Brazil; breathing exercise; case report; child; coughing; diagnosis; family study; home care; human; noninvasive ventilation; palliative therapy; prevention; quality of life; tracheostomy; weaning; Werdnig Hoffmann disease

Cerebral and spinal anomalies in one monochorionic twin with schimmelpenning-feuerstein-mims syndrome - Gruber-Sedlmayr U.; Hubmann H.; Haidl H.; Brunner-Krainz M.; Schwerin-Nagel A.; Schwerin-Nagel A.; Kortschak A.; Haber E.; Sorantin E. (Neuropediatrics, 2016)
Background: Schimmelpenning-Feuerstein-Mims syndrome (SFM) is a neurocutaneous disorder with nevus sebaceous in association with cerebral, ocular or skeletal defects. Postzygotic HRAS (chromosome 11p15.5) and KRAS (chromosome 12p12.1) mutations can be found in the lesions. Case Report: We report a 2-year-old boy, born preterm by cesarian section after complicated twin pregnancy because of fetofetal transfusion syndrome. He presented with multiple nevus sebaceous predominantly on the right side of the head and thorax and with a malformation of the right bulbus oculi and eyelid. HRAS mutation was found in the naevi. Because of cyanotic attacks, a tracheostoma and a percutaneous endoscopic gastrostoma were necessary at the age of 6 months. When he was 11 months old, he developed focal seizures partly with generalization. The cerebral MRT showed malformation with polygyria, nearly absent myelinization, signs of atrophy, a cystic defect of the right occipital lobe, and enlarged cervical nerve roots. A MRT of the whole spine was performed. It showed increased thickness of the nerve roots cervical, thoracal and lumbar and three lipomas extramedullary. The boy is developmentally retarded, but is able to grasp with both hands and he can move both legs without signs of spinal cord compression. The epilepsy is controlled by ketogenic diet in combination with topiramate and levetiracetam. Result: We describe the first patient with SFM syndrome confirmed by genetic analysis with enlargement of all nerve roots. The prognosis with regard to spinal compression of this patient is unclear.
Keywords: endogenous compound; levetiracetam; topiramate; atrophy; case report; cesarean section; child; eyeball; eyelid; female; focal epilepsy; gastrostomy; gene mutation; genetic analysis; human; ketogenic diet; leg; lipoma; lumbar region; male; myelination; nerve root; nevus; occipital lobe; oncogene c H ras; preschool child; prognosis; spinal cord compression; spinal cord malformation; spine; thickness; thorax; trachea stoma; twin pregnancy; twin twin transfusion syndrome

Diaphragmatic pacing stimulation: Anesthetic management at institut guttmann - Alegret Monroig N.; Serra P.; Pere Pessas J. (Journal of Neurosurgical Anesthesiology, 2016)
Background: Diaphragm pacing (DP) has been shown to successfully replace mechanical ventilators for patients affected by spinal cord injuries, amyotrophic lateral sclerosis, and other neurological injuries with chronic respiratory insufficiency, improving quality of life and decreasing morbidity, mortality, and health costs. From an anesthetic perspective, both the surgery and the patient population present several unique challenges. The aim of this study is the analysis of the anesthetic management and intraoperative complications of patients undergoing insertion of a DP in our institution. Methods: With IRB approval, retrospective review was conducted from December 2007 to July 2015. Data register of patient previous state, anesthetic technique, and intraoperative complications due both to anesthetic technique or surgery were collected. Results: We included 16 patients (5 pediatric) with DP indication due to spinal cord injuries, 63%; amyotrophic lateral sclerosis, 25%; or other neurological diseases, 12%. General anesthesia was required for the abdominal laparoscopy; we used intravenous 87% versus inhalatory induction 13% and total intravenous anesthesia (TIVA) 50% versus balanced 50% for maintenance anesthesia. Succinylcholine was administered to 31% of the patients for orotracheal intubation. Anesthetic deepening was needed during the surgery for pneumoperitoneum tolerance in 50% of the cases in the balanced anesthesia group and in 25% of the cases in the TIVA group. Registered complications were: 31% mechanical ventilation difficulty during laparoscopy, pneumotorax 12.5%, and autonomic dysreflexia 6%. Discussion: Although few studies about perioperative management for DP surgery and none in pediatric patients are available, all agree in the need of diaphragmatic contraction visualization during the procedure, so neuromuscular blocking agents should be avoided during surgery. Our report is the first using depolarizing neuromuscular blocker at the anesthetic induction with no consequences on the diaphragmatic mapping. We are describing for first time inhalatory induction through tracheostomy and TIVA for maintenance anesthesia in DP surgery with satisfactory results. Our incidence of complications is comparable to other studies. Conclusions: The implantation of DP is a safe technique with the potential to improve the quality of life of patients dependent on mechanical ventilation. Appropriate intraoperative care is fundamental to achieving better results. Nevertheless larger, randomized, prospective studies are required.
Keywords: neuromuscular blocking agent; suxamethonium; amyotrophic lateral sclerosis; artificial ventilation; autonomic dysreflexia; balanced anesthesia; child; clinical article; clinical trial; controlled clinical trial; controlled study; diaphragm; endotracheal intubation; general anesthesia; human; implantation; intravenous anesthesia; laparoscopy; muscle contractility; peroperative care; peroperative complication; pneumoperitoneum; prospective study; quality of life; randomized controlled trial; retrospective study; spinal cord injury; surgery; tracheostomy

Pneumocephalus leading to the diagnosis of cerebrospinal fluid leak and esophageal perforation after cervical spine surgery - Goodwin C.R.; Boone C.E.; Pendleton J.; Elder B.D.; Wei Z.; Hsu W.; Sciubba D.M.; Witham T.F. (Journal of Clinical Neuroscience, 2016)
Pneumocephalus is a collection of air within in the intracranial cavity, most commonly seen following traumatic injury or cranial surgeries. Esophageal injury and cerebrospinal fluid (CSF) leak are rare complications that may occur following anterior cervical discectomy and fusion (ACDF). We present a novel case of pneumocephalus arising from unrestricted leakage of CSF via coincident esophageal injury and durotomy in a patient who underwent an ACDF after trauma. A 21-year-old man presented to an outside hospital with C5/C6 subluxation, complete spinal cord injury, and quadriplegia from a motor vehicle accident. He underwent an ACDF, during which a CSF leak was observed. He was then transferred to our institution for rehabilitation and tracheostomy placement 1 week after the ACDF surgery. Following the tracheostomy, the patient developed intractable fevers and nonspecific symptoms. A CT scan demonstrated frontal pneumocephalus without mass effect. Air was found in the retropharyngeal space. There were no accumulations of CSF in the neck. Extravasation of contrast around instrumentation at C5/C6 on a cine esophagogram demonstrated an esophageal perforation at that level. Pneumocephalus may form when large volumes of CSF escape from the intracranial space and air is drawn into the space by the negative pressure. In this unusual case, the esophageal perforation promoted the formation of the pneumocephalus. Treatment included closure of both defects, disrupting the suspected communication between the intracranial space and the esophagus.
Keywords: antibiotic agent; macrogol; abscess; adult; anterior cervical discectomy and fusion; article; case report; cerebrospinal fluid culture; cervical abscess; computer assisted tomography; debridement; esophagography; esophagoscopy; esophagus perforation; extravasation; fever; Gram negative bacterium; Gram positive bacterium; hematoma; hospital discharge; human; intraoperative period; laryngoscopy; liquorrhea; male; pneumocephalus; postoperative period; priority journal; quadriplegia; rehabilitation care; spinal cord injury; spine surgery; surgical drainage; symptom; tracheostomy; traffic accident; vertebra dislocation; young adult

Planned ventral hernia following damage control laparotomy in trauma: an added year of recovery but equal long-term outcome - Zosa B.M.; Como J.J.; Kelly K.B.; He J.C.; Claridge J.A. (Hernia, 2016)
Purpose: Significantly injured trauma patients commonly require damage control laparotomy (DCL). These patients undergo either primary fascial closure during the index hospitalization or are discharged with a planned ventral hernia. Hospital and long-term outcomes of these patients have not been extensively studied. Methods: Patients who underwent DCL for trauma from 2003 to 2012 at a regional Level I trauma center were identified and a comparison was made between those who had primary fascial closure and planned ventral hernia. Results: DCL was performed in 154 patients, 47 % of whom sustained penetrating injuries. The mean age and injury severity score (ISS) were 40 and 25, respectively. Hospital mortality was 19 %. Primary fascial closure was performed in 115 (75 %) of those undergoing DCL during the index hospitalization. Of these, 11 (9 %) had reopening of the fascia. Of the surviving patients, 22 (19 %) never had primary fascial closure and were discharged with a planned ventral hernia. Patients with primary fascial closure and those with planned ventral hernia were similar in age, gender, ISS, and mechanism. Those with planned ventral hernias underwent more subsequent laparotomies (3.0 vs 1.3, p < 0.001), and had more enteric fistulas (18.2 vs 4.3 %, p = 0.041) and intra-abdominal infections (46 vs 15 %, p = 0.007), and had a greater number of hospital days (38 vs 25, p = 0.007) during the index hospitalization. Sixteen (73 %) patients with a planned ventral hernia had definitive reconstruction (mean days = 266). Once definitive abdominal wall closure was achieved, the two groups achieved similar rates of return to work and usual activity (71 vs 70 %, p = NS). Conclusions: Following DCL for trauma, patients with a planned ventral hernia have definitive reconstruction nearly 9 months after the initial injury. Once definitive abdominal wall closure has been achieved; patients with primary fascial closure and those with planned ventral hernia have similar rates of return to usual activity.
Keywords: abdominal infection; abdominal wall closure; abdominal wall hernia; adult; article; damage control laparotomy; female; follow up; hospitalization; human; injury; injury scale; intestine fistula; laparotomy; major clinical study; male; mortality; mortality rate; outcome assessment; penetrating trauma; priority journal; recurrent disease; retrospective study; return to work; spinal cord injury; survival; survivor; tracheostomy

Relationship between quality-of-life after 1-year follow-up and severity of traumatic brain injury assessed by computerized tomography - Prieto-Palomino M.A.; Curiel-Balsera E.; Arias-Verdu M.D.; Der Kroft M.D.-V.; Munõz-Lopez A.; Fernandez-Ortega J.F.; Quesada-Garcia G.; Sanchez-Cantalejo E.; Rivera-Fernandez R. (Brain Injury, 2016)
This paper studies the relationship between computed tomography (CT) scan on admission, according to Marshall's tomographic classification, and quality-of-life (QoL) after 1 year in patients admitted to the Intensive Care Unit (ICU) with traumatic brain injury (TBI). Methods: This study used validated scales including the Glasgow Outcome Scale and the PAECC (Project for the Epidemiologic Analysis of Critical Care Patients) QoL questionnaire. Results: We enrolled 531 patients. After 1 year, 171 patients (32.2%) had died (missing data = 6.6%). Good recovery was seen in 22.7% of the patients, while 20% presented moderate disability. The PAECC score after 1 year was 9.43 ± 8.72 points (high deterioration). Patients with diffuse injury I had a mean of 5.08 points vs 7.82 in those with diffuse injury II, 11.76 in those with diffuse injury III and 19.29 in those with diffuse injury IV (p > 0.001). Multivariate analysis found that QoL after 1 year was associated with CT Marshall classification, depth of coma, age, length of stay, spinal injury and tracheostomy. Conclusions: Patients with TBI had a high mortality rate 1 year after admission, deterioration in QoL and significant impairment of functional status, although more than 40% were normal or self-sufficient. QoL after 1 year was strongly related to cranial CT findings on admission.
Keywords: adult; age; article; coma; computer assisted tomography; convalescence; disability; disease classification; female; follow up; Glasgow outcome scale; hospital admission; human; injury severity; intensive care; length of stay; major clinical study; male; Marshall classification; middle aged; mortality rate; Project for the Epidemiologic Analysis of Critical Care Patients; prospective study; quality of life; quality of life assessment; spine injury; tracheostomy; traumatic brain injury

Effect of Intrathecal Baclofen on Delayed-Onset Paroxysmal Dystonia due to Compression Injury Resulting from Congenital and Progressive Spinal Bone Deformities in Chondrodysplasia Punctata - Okazaki T.; Saito Y.; Ueda R.; Sugihara S.; Tamasaki A.; Nishimura Y.; Ohno K.; Togawa M.; Ohno T.; Horie A.; Honda M.; Takenaka A.; et al. (Pediatric Neurology, 2016)
Background Dystonia due to spinal lesions in adult patients is characterized by the provocation and/or amelioration of the spasm by somatosensory stimulation with a sensory trick. Patient Description An infant with brachytelephalangic chondrodysplasia punctata developed flaccid tetraplegia due to cervical cord compression resulting from congenital atlantoaxial dislocation. Episodic, tonic extension of the extremities, neck, and trunk had appeared daily since age two years and was often provoked by tactile stimulation. Although decompression surgery was performed at age three years, progressive spinal deformity resulted in the aggravation of episodic dystonia thereafter, lasting for hours. Foot dorsiflexion and wearing a truncal brace for scoliosis inhibited these spasms. Intrathecal baclofen bolus injection transiently ameliorated the paroxysmal dystonia and detrusor-sphincter dyssynergia in the lower urinary tract. Conclusion Paroxysmal dystonia is unusual in children with spinal cord lesions; however, it should be recognized for appropriate individualized clinical management.
Keywords: baclofen; eperisone; phenobarbital; article; aspiration pneumonia; atlantoaxial dislocation; bolus injection; brace; case report; cervical cord compression; child; chondrodysplasia punctata; decompression surgery; detrusor dyssynergia; disease course; drug effect; dyspnea; somatosensory evoked potential; flaccid paralysis; gastrostomy; human; lower urinary tract symptom; male; muscle hypotonia; neurogenic bladder; neuroimaging; nuclear magnetic resonance imaging; paroxysmal dystonia; positive end expiratory pressure; preschool child; priority journal; quadriplegia; recurrent aspiration; scoliosis; sleep disordered breathing; spinal cord compression; stomach fundoplication; tactile stimulation; tracheotomy

Hybrid lateral mass screw sublaminar wire construct: A salvage technique for posterior cervical fixation in pediatric spine surgery - Quinn J.C.; Patel N.V.; Tyagi R. (Journal of Clinical Neuroscience, 2016)
We present a novel salvage technique for pediatric subaxial cervical spine fusion in which lateral mass screw fixation was not possible due to anatomic constraints. The case presentation details a 4-year-old patient with C5-C6 flexion/distraction injury with bilateral jumped facets. Posterior cervical fixation was attempted; however, lateral mass fracture occurred during placement of screws. Using a wire-screw construct, an attempt was made to provide stable fixation. The patient was followed post-operatively for assessment of outcomes. After the patient had progressive kyphosis following initial closed reduction and external orthosis, internal reduction with fusion/fixation was performed. Lateral mass fracture occurred during placement of lateral mass screws. After placement of a sub-laminar wire-lateral mass screw construct, intra-operative evaluation determined stability. Post-operatively, the procedure resulted in stable fixation with evidence of bony fusion on follow-up. Pediatric subaxial cervical spine instrumentation provides rigid fixation however is technically difficult due to anatomic and instrumentation related constraints. In the presented patient, the wire-screw construct resulted in stable fixation and bony fusion on follow-up. A modified sublaminar wire-lateral mass screw construct is an example of a salvage technique that provides immediate stability in the event of instrumentation related lateral mass fracture.
Keywords: article; atlantoaxial subluxation; bone screw; brace; brain hypoxia; case report; cervical collar; cervical spine fracture; cervical spine injury; cervical spine radiography; child; computer assisted tomography; conservative treatment; disease course; disease severity; fluoroscopy; follow up; fracture reduction; hospital discharge; hospitalization; human; kyphosis; male; peroperative care; posterior spine fusion; postoperative period; preschool child; priority journal; rehabilitation care; spinal cord injury; spine instability; spine stabilization; stomach tube; surgical technique; surgical wire; tracheostomy; traffic accident; visual stimulation; Mountaineer

Thoracic endovascular repair first for extensive aortic disease: The staged hybrid approach - Vivacqua A.; Idrees J.J.; Johnston D.R.; Soltesz E.G.; Svensson L.G.; Roselli E.E. (European Journal of Cardio-thoracic Surgery, 2016)
OBJECTIVES: Repair of extensive aortic disease carries a significant risk of death and morbidity, the most feared complication being spinal cord ischaemia. Objectives of this study are to characterize patients, describe repair methods and assess feasibility and safety of hybrid staged repair for treatment of extensive aortic disease. METHODS: From to 2001 to 2013, 22 patients underwent extensive aortic repair that included a thoracic endovascular aortic repair (TEVAR) first followed by an open completion repair extending through the visceral and infrarenal aorta for degenerative aneurysm and dissection. At the time of initial repair, all patients were deemed to be at a high risk for conventional open repair and had extensive disease. Indications for open completion included emergency failure of TEVAR (n = 3), early two-stage approach (n = 6) and delayed disease progression after TEVAR (n = 13). The median interval between stages was 6.5 months. The mean age was 56 ± 14 years, 5 patients had connective tissue disorder and the mean maximum aortic diameter was 58 ± 16 mm preoperatively. RESULTS: There was no death or major complication after initial TEVAR, but the operative mortality rate was 9% (n = 2) after the open procedure. One of these patients died from intraoperative myocardial infarction during emergency repair, and the other had disseminated intravascular coagulation during delayed repair for disease progression after TEVAR. Other complications included paralysis in 1 (4.5%), tracheostomy in 2 (9%) and dialysis in 1 (4.5%), and there was 1 reoperation for bleeding (4.5%). The median follow-up was 37 (range 3.3-93) months and there were no late deaths. There were four late reoperations for proximal disease progression leading to Type 1 endoleak (n = 2), Type A dissection (n = 1) and root aneurysm (n = 1).
Keywords: adult; aortic disease; aortic reconstruction; article; cardiac patient; cardiovascular risk; clinical article; descending aortic surgery; dialysis; disease course; disseminated intravascular clotting; endovascular aneurysm repair; female; follow up; heart death; heart infarction; human; hybrid staged repair; male; middle aged; morbidity; outcome assessment; paralysis; postoperative hemorrhage; priority journal; reoperation; spinal cord ischemia; surgical mortality; surgical patient; surgical technique; tracheostomy

Acute spinal cord injury - Kirke Rogers W.; Todd M. (Best Practice and Research: Clinical Anaesthesiology, 2016)
Acute spinal cord injury (SCI) is a devastating event with high mortality and, among survivors, a high degree of morbidity due to both motor and sensory deficits. The damage that occurs with a SCI is recognized to be pathophysiologically biphasic: the initial insult to neural tissue can be followed by a secondary process of progressive ischemia that may worsen the severity of dysfunction. The extent of this secondary insult is potentially modifiable, and a variety of interventions have been studied in an attempt to improve motor and sensory outcomes. What follows is a brief review of some such procedural and pharmacologic interventions, including early decompressive surgery, use of methylprednisolone, and blood pressure and respiratory management, which have been proposed to improve outcomes after SCI.
Keywords: ganglioside GM1; methylprednisolone; naloxone; protirelin; tirilazad; acute disease; acute spinal cord injury; article; artificial ventilation; blood pressure; decompression surgery; deep vein thrombosis; human; hypotension; induced hypothermia; intensive care unit; Medline; nonhuman; priority journal; spinal cord injury; thrombosis prevention; tracheostomy

Multi-center analysis of operative safety of surgery in patients with amyotrophic lateral sclerosis: The diaphragm pacing experience - Onders R.; Carlin A.; Dunkin B.; Jossart G.; Marohn M.; Menegaux F.; Morton J.; Smith C.D. (Surgical Endoscopy and Other Interventional Techniques, 2016)
Introduction: With the growing prevalence of patients with amyotrophic lateral sclerosis (ALS) there is a need to describe and analyze the correct perioperative management. ALS is a progressive neurodegenerative disease that has devastating effects to respiratory muscles resulting in respiratory failure death in 80 % of patients. Diaphragm pacing (DP) replaces ventilators in spinal cord injury patients and delays death and tracheostomy in ALS patients. This report outlines the peri-operative outcomes to 90 days for patients in a multicenter pivotal FDA trial of DP in ALS. Methods: The study was conducted under an investigational device exemption (IDE G040142, clinicaltrials.gov NCT00420719) from the U.S. FDA and IRB approval. Patients underwent three pre-operative neurophysiologic assessments to quantify diaphragm function. Inclusion criteria included chronic hypoventilation with intact diaphragm motor units to stimulate. A standardized ALS functional rating scale (ALSFSR-R) was completed serially pre and post-operatively. Patients underwent general anesthesia with no paralytics with laparoscopic implantation of the diaphragm pacing electrodes in each hemi-diaphragm. Post-operative ALSFSR-R results and complications were noted. Data were analyzed with Stata 13.0. Results: At eight worldwide sites, 107 patients went to surgery with 106 patients successfully being implanted with DP. One patient had a reaction to anesthesia requiring cancellation of surgery. Twenty-eight patients received a simultaneous percutaneous gastrostomy tube (PEG). There were 73 males and 36 females with a median age of 57.9 years (range 32-76). The mean lead-in time was 2.7 ± 0.6 months from consent to implant. The mean post-op period was 3.8 ± 1.0 months from implant to data collection. There were no deaths within 30 days post implant. Three in the non-PEG group, died within 90 days. The median ALSFRS-R score at implant was 28 points (on the 48 point scale). The lead-in period rate of decline was compared on a paired, patient-by-patient, basis to the rate of decline for the post-operative treatment period. There was no significant change in the rate of decline of this functional scale (lead-in slope -0.80 vs post-op slope -0.73 with p value = 0.50). There were 4 serious adverse effects: 2 capnothorax, 1 respiratory failure following complications from surgery (dislodgement of gastrostomy); and 1 post-operative chest pain. Conclusion: There was no degradation in function for ALS patients undergoing surgical procedures when no paralytics are used and DP is placed. The diagnosis of ALS should not prevent a patient from being considered for a surgical procedure when necessary and to improve their quality of life.
Keywords: human; patient; amyotrophic lateral sclerosis; surgery; diaphragm; society; American; surgeon; safety; implant; death; surgical technique; respiratory failure; prevalence; spinal cord injury; ventilator; female; information processing; adverse drug reaction; thorax pain; diagnosis; tracheostomy; devices; stomach tube; anesthesia; electrode; breathing muscle; implantation; general anesthesia; rating scale; motor unit; degenerative disease; hypoventilation; statistical significance; gastrostomy; United States; quality of life; male; food and drug administration

Spinal muscular atrophy with respiratory distress type 1 (SMARD1): Report of a Spanish case with extended clinicopathological follow-up - Millan B.S.; Fernandez J.M.; Navarro C.; Reparaz A.; Teijeira S. (Clinical Neuropathology, 2016)
Background: Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a clinically and genetically distinct and uncommon variant of SMA that results from irreversible degeneration of α-motor neurons in the anterior horns of the spinal cord and in ganglion cells on the spinal root ganglia. Aims: To describe the clinical, electrophysiological, neuropathological, and genetic findings, at different stages from birth to death, of a Spanish child diagnosed with SMARD1. Patient and methods: We report the case of a 3-monthold girl with severe respiratory insufficiency and, later, intense hypotonia. Paraclinical tests included biochemistry, chest X-ray, and electrophysiological studies, among others. Muscle and nerve biopsies were performed at 5 and 10 months and studied under light and electron microscopy. Post-mortem examination and genetic investigations were performed. Results: Pre- and post-mortem histopathological findings demonstrated the disease progression over time. Muscle biopsy at 5 months of age was normal, however a marked neurogenic atrophy was present in post-mortem samples. Peripheral motor and sensory nerves were severely involved likely due to a primary axonal disorder. Automatic sequencing of IGHMBP2 revealed a compound heterozygous mutation. Conclusions: The diagnosis of SMARD1 should be considered in children with early respiratory insufficiency or in cases of atypical SMA. Direct sequencing of the IGHMBP2 gene should be performed.
Keywords: binding protein; corticosteroid; immunoglobulin mu binding protein 2; oxygen; unclassified drug; areflexia; article; artificial ventilation; autopsy; bronchiolitis; case report; corticosteroid therapy; female; gastrostomy; gene mutation; heart arrhythmia; human; hypoventilation; infant; limb weakness; lung auscultation; lung infection; muscle hypotonia; neurogenic bladder; oxygen therapy; clubfoot; pleura effusion; priority journal; recurrent infection; respiratory distress; respiratory failure; spinal muscular atrophy; spinal muscular atrophy with respiratory distress type 1; tendon contracture; thorax radiography; tracheostomy; urinary tract infection

Complete Transection of the Cervical Trachea due to Blunt Trauma: Report of a Case - Shimoyama T. (Kyobu geka. The Japanese journal of thoracic surgery, 2016)
We herein report a case of complete transection of the cervical trachea due to blunt trauma. A 50-year-old man was transported to our hospital because of blunt neck trauma. His body surface showed only slight bruising on the neck. However, he exhibited see-saw breathing, so we suspected upper airway injury. The patient was carefully intubated using a bronchofiberscope, revealing complete transection of the cervical trachea. Chest computed tomography demonstrated destruction of the cervical tracheal structure. The patient also had a cervical spinal cord injury;thus, we performed tracheoplasty after 3 days of hospitalization. A tracheostomy was necessary because of the cervical cord injury and paralysis of the bilateral recurrent nerve. The patient's postoperative course was uneventful.
Keywords: accident; case report; human; injuries; male; middle aged; neck injury; trachea; tracheostomy

Proton beam radiation therapy results in significantly reduced toxicity compared with intensity-modulated radiation therapy for head and neck tumors that require ipsilateral radiation - Romesser P.B.; Cahlon O.; Scher E.; Zhou Y.; Berry S.L.; Rybkin A.; Sine K.M.; Tang S.; Sherman E.J.; Wong R.; Lee N.Y. (Radiotherapy and Oncology, 2016)
Background As proton beam radiation therapy (PBRT) may allow greater normal tissue sparing when compared with intensity-modulated radiation therapy (IMRT), we compared the dosimetry and treatment-related toxicities between patients treated to the ipsilateral head and neck with either PBRT or IMRT. Methods Between 01/2011 and 03/2014, 41 consecutive patients underwent ipsilateral irradiation for major salivary gland cancer or cutaneous squamous cell carcinoma. The availability of PBRT, during this period, resulted in an immediate shift in practice from IMRT to PBRT, without any change in target delineation. Acute toxicities were assessed using the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. Results Twenty-three (56.1%) patients were treated with IMRT and 18 (43.9%) with PBRT. The groups were balanced in terms of baseline, treatment, and target volume characteristics. IMRT plans had a greater median maximum brainstem (29.7 Gy vs. 0.62 Gy (RBE), P < 0.001), maximum spinal cord (36.3 Gy vs. 1.88 Gy (RBE), P < 0.001), mean oral cavity (20.6 Gy vs. 0.94 Gy (RBE), P < 0.001), mean contralateral parotid (1.4 Gy vs. 0.0 Gy (RBE), P < 0.001), and mean contralateral submandibular (4.1 Gy vs. 0.0 Gy (RBE), P < 0.001) dose when compared to PBRT plans. PBRT had significantly lower rates of grade 2 or greater acute dysgeusia (5.6% vs. 65.2%, P < 0.001), mucositis (16.7% vs. 52.2%, P = 0.019), and nausea (11.1% vs. 56.5%, P = 0.003). Conclusions The unique properties of PBRT allow greater normal tissue sparing without sacrificing target coverage when irradiating the ipsilateral head and neck. This dosimetric advantage seemingly translates into lower rates of acute treatment-related toxicity.
Keywords: brain stem; cancer size; clinical article; conference paper; dermatitis; distant metastasis; distant metastasis free survival; dysgeusia; dysphagia; fatigue; female; follow up; head and neck cancer; human; intensity modulated radiation therapy; lymph vessel metastasis; male; mouth cavity; mucosa inflammation; nausea; overall survival; priority journal; progression free survival; proton radiation; radiation injury; salivary gland cancer; skin carcinoma; spinal cord; stomach tube; tracheostomy

Airway management in laryngotracheal injuries from blunt neck trauma in children - Chatterjee D.; Agarwal R.; Bajaj L.; Teng S.N.; Prager J.D. (Paediatric Anaesthesia, 2016)
Summary Pediatric laryngotracheal injuries from blunt neck trauma are extremely rare, but can be potentially catastrophic. Early diagnosis and skillful airway management is critical in avoiding significant morbidity and mortality associated with these cases. We present a case of a patient who suffered a complete tracheal transection and cervical spine fracture following a clothesline injury to the anterior neck. A review of the mechanisms of injury, clinical presentation, initial airway management, and anesthetic considerations in laryngotracheal injuries from blunt neck trauma in children are presented.
Keywords: fentanyl; midazolam; oxygen; rocuronium; sevoflurane; anesthesiological procedure; blunt neck trauma; blunt trauma; breathing; case report; cervical soft tissue; cervical spinal cord injury; cervical spinal fusion; chest tube; child; clinical feature; clothesline injury; computer assisted tomography; consciousness level; drug intermittent therapy; emergency care; end to end anastomosis; endotracheal tube; face mask; fiberoptic bronchoscopy; hemodynamics; human; intensive care; intubation; laryngoscopy; laryngotracheal injury; larynx injury; male; neck injury; nuclear magnetic resonance imaging; oxygen saturation; patient transport; pneumothorax; positive end expiratory pressure; priority journal; rapid sequence induction; respiration control; review; school child; skin bruising; soft tissue; subluxation; trachea injury; trachea reconstruction; tracheostomy; treatment outcome

An infant with MLH3 variants, FOXG1-duplication and multiple, benign cranial and spinal tumors: A clinical exome sequencing study - Kansal R.; Li X.; Shen J.; Samuel D.; Laningham F.; Lee H.; Panigrahi G.B.; Shuen A.; Kantarci S.; Dorrani N.; Reiss J.; Shintaku P.; et al. (Genes Chromosomes and Cancer, 2016)
A 4-month-old male infant presented with severe developmental delay, cerebellar, brainstem, and cutaneous hemangiomas, bilateral tumors (vestibular, hypoglossal, cervical, and lumbar spinal), and few café-au-lait macules. Cerebellar and lumbar tumor biopsies revealed venous telangiectasia and intraneural perineuroma, respectively. Sequencing NF1, NF2, and RASA1 (blood), and NF2 and SMARCB1 (lumbar biopsy) was negative for pathogenic mutations. Clinical exome sequencing (CES), requested for tumor syndrome diagnosis, revealed two heterozygous missense variants, c.359T>C;p.Phe120Ser and c.3344G>A;p.Arg1115Gln, in MLH3 (NM_001040108.1), a DNA mismatch repair (MMR) gene, Polyphen-predicted as probably damaging, and benign, respectively. Sanger sequencing confirmed both variants in the proband, and their absence in the mother; biological father unavailable. Both biopsied tissues were negative for microsatellite instability, and expressed MLH1, MSH2, PMS2, MSH6, and MLH3 immunohistochemically. Chromosomal microarray showed a 133 kb segment copy number duplication of 14q12 region encompassing FOXG1, possibly explaining the developmental delay, but not the tumors. The presence of MLH3 variants with multiple benign neural and vascular tumors was intriguing for their possible role in the pathogenesis of these neoplasms, which were suspicious for, but not diagnostic of, constitutional MMR deficiency. However, functional assays of non-neoplastic patient-derived cells showed intact base-base MMR function. Also, no previous FOXG1-aberrant patient was reported with tumors. We now report a 3-year-old FOXG1-duplicated patient with a yet undescribed tumor syndrome with clinical features of neurofibromatosis types I and II, where several validation studies could not ascertain the significance of CES findings; further studies may elucidate precise mechanisms and diagnosis for clinical management, including tumor surveillance.
Keywords: etiracetam; forkhead box g1 protein; mismatch repair protein PMS2; protein; MutL protein homolog 1; protein MLH3; DNA mismatch repair protein MSH2; protein MSH6; transcription factor; unclassified drug; article; benign neoplasm; bilateral cancer; bilateral hearing loss; bone biopsy; brain biopsy; brain development; brain stem; brain tumor; cafe au lait spot; cancer diagnosis; case report; cerebellum disease; cerebellum injury; chromosomal instability; clinical exome sequencing; clinical feature; computer assisted tomography; copy number variation; cranial nerve; developmental disorder; differential diagnosis; DNA repair; DNA sequence; eye movement; face dysmorphia; facial nerve paralysis; family history; follow up; gastrostomy; gene duplication; gene sequence; human; human tissue; hypoglossal nerve disease; immunohistochemistry; infant; infant disease; infant feeding; internal auditory canal; leg length inequality; lumbar spinal cord; male; medical history; microsatellite instability; mismatch repair; missense mutation; multiple cancer; neurilemoma; neurofibromatosis type 1; neurofibromatosis type 2; neuroimaging; nuclear magnetic resonance imaging; pathogenesis; perineural invasion; peripheral lymphocyte; physical examination; priority journal; ptosis (eyelid); radiodiagnosis; recurrent aspiration; scoliosis; seizure; skin defect; skin hemangioma; spinal cord tumor; telangiectasia; tracheostomy; tumor biopsy; vascular lesion; vascular tumor; vestibular disorder; vestibulocochlear nerve disease; vocal cord paralysis

A study of risk factors for tracheostomy in patients with a cervical spinal cord injury - Tanaka J.; Yugue I.; Shiba K.; Maeyama A.; Naito M. (Spine, 2016)
Study Design. A retrospective, consecutive case series. Objective. To determine the risk factors for a tracheostomy in patients with a cervical spinal cord injury. Summary and Background Date. Respiratory status cannot be stabilized in patients with a cervical spinal cord injury (CSCI) for various reasons, so a number of these patients require longterm respiratory care and a tracheostomy. Various studies have described risk factors for a tracheostomy, but none have indicated a relationship between imaging assessment and the need for a tracheostomy. The current study used imaging assessment and other approaches to assess and examine the risk factors for a tracheostomy in patients with a CSCI. Methods. Subjects were 199 patients who were treated at the Spinal Injuries Center within 72 hours of a CSCI over 8-year period. Risk factors for a tracheostomy were retrospectively studied. Patients were assessed in terms of 10 items: age, sex, the presence of a vertebral fracture or dislocation, ASIA Impairment Scale, the neurological level of injury (NLI), PaO2, PaCO2, the level of injury on magnetic resonance imaging (MRI), the presence of hematoma-like changes (a hypointense core surrounded by a hyperintense rim in T2-weighted images) on MRI, and the Injury Severity Score. Items were analyzed multivariate logistic regression, and P<0.05 was considered to indicate a significant difference. Results. Twenty-three of the 199 patients required a tracheostomy, accounting for 11.6% of patients with a CSCI. Univariate analyses of the risk factors for tracheostomy revealed significant differences for six items: age, Injury Severity Score, presence of fracture or dislocation, ASIA Impairment Scale A, NLI C4 or above, and MRI scans revealing hematoma-like changes. Multivariate logistic regression analyses revealed significant differences in terms of two items: NLI C4 or above and MRI scans revealing hematoma-like changes. Thirty patients had both an NLI C4 or above and MRI scans revealing hematoma-like changes. Of these, 17 (56.7%) required a tracheostomy. Conclusion. Patients with an NLI C4 or above and MRI scans revealing hematoma-like changes were likely to require a tracheostomy. An early tracheostomy should be considered for patients with both of these characteristics.
Keywords: adolescent; adult; age; aged; American Spinal Injury Association impairment scale; article; blood carbon dioxide tension; blood oxygen tension; blunt trauma; cervical spinal cord injury; female; hematoma; human; injury scale; major clinical study; male; nuclear magnetic resonance imaging; patient assessment; priority journal; retrospective study; risk factor; spine fracture; tracheostomy; vertebra dislocation; very elderly

Risk factors for dysphagia in acute cervical spinal cord injury - Hayashi T.; Sakai H.; Maeda T.; Shiba K. (Journal of Orthopaedic Research, 2016)
INTRODUCTION: Dysphagia following traumatic cervical spinal cord injury (CSCI) is an under-recognized complication that can lead to aspiration pneumonia, which is a significant cause of morbidity and mortality. Several authors have investigated dysphagia associated with CSCI, however, risk factors for dysphagia are still not well understood. The objective of this study was to elucidate the incidence and risk factors of dysphagia in patients with acute CSCI. Methods: A total of 464 consecutive patients with traumatic cervical spinal injury with and without spinal cord damage were treated at our institute and were registered in a database from January 2007 to December 2014. All patients underwent CT, MRI, and neurological examination on admission. We retrospectively selected 298 patients based on following criteria: (1) admission within 3 days following injury, (2) patients with paresis or paralysis, (3) patients without brain injury. Neurological impairment scale was evaluated according to ASIA impairment scale (AIS), and level of injury was identified using CT and MRI. We analyzed the factors postulated to increase the risk for dysphagia, including the patient's age, neurological impairment scale grade, level of injury tracheostomy, and operative treatment, using a multiple logistic regression model to compute odds ratios (ORs) and 95% confidence intervals (95% CI). The institutional review board at our institute approved this study. Results: 298 eligible patients (256 males and 42 females) with an average age 61.4±17.3 (range, 14-91 yr.) were identified during 8-year study period. 21 of 298 patients appeared to be suffering from dysphagia after CSCI (7.0%). All of them experienced evident aspiration and had to stop eating their meals due to aspiration. The neurological status revealed that 13 of those patients were AIS A, 6 patients were AIS B, and 2 patients were AIS C. 12 of 21 patients (57.1%) received tracheostomy. Multivariable logistic regression analysis revealed that age > 72 years (OR: 2.97, 95% CI: 1.01-9.02, p=0.04), AIS A or B (OR: 8.00, 95% CI: 1.92-54.7, p=0.003), presence of tracheostomy (OR: 13.8, 95% CI: 4.62-44.3, p<0.001) were significant risk factors (Table 1). Discussion: The incidence of dysphagia after acute CSCI was 7.0%. Old age, severe neurological impairment scale, and presence of tracheostomy may be at risk for dysphagia after acute CSCI. When treating CSCI, understanding the risk factors of dysphagia is important to prevent aspiration pneumonia. SIGNIFICANCE: By detecting the risk factors of dysphagia, the incidence of aspiration pneumonia would be decreased.
Keywords: adolescent; adult; aged; American Spinal Injury Association impairment scale; aspiration pneumonia; brain injury; cervical spinal cord injury; confidence interval; data base; dysphagia; eating; female; human; institutional review; major clinical study; male; model; multivariate logistic regression analysis; neurologic examination; nuclear magnetic resonance imaging; odds ratio; paresis; prevention; risk factor; tracheostomy

Safety of aquatic therapy for adults with complex medical conditions among chronic spinal cord injury - Recio A.C.; Cabahug P. (Journal of Spinal Cord Medicine, 2016)
Objective: Demonstrate safety within aquatic environment for patients with spinal cord injury (SCI) with pressure ulcers, colostomy or ileostomy bags, supra pubic catheters, indwelling catheters, and tracheostomy tube, Describe aquatic interventions and task modifications for pressure ulcers, colostomy or ileostomy bags, supra pubic catheters, indwelling catheters, and tracheostomy tubes in adult patients with chronic spinal cord injury, and assess the impact of aquatic therapy on family and caregiver-reported quality of life. Design: Retrospective chart review Participants/methods: Data collected were obtained from medical charts after the patient was discharged from aquatic-based therapies. Relevant demographic, clinical and outcomes information were retrieved for entry in to this research. Inclusion criteria include patients 18 years old and older, and have a chronic (>1 year) spinal cord injury (any neurological level, any AIS classification) who received treatment at Aquatics Department of Kennedy Krieger Institute. Results: Aquatic based restorative therapy in 100 patients with spinal cord injury was an effective therapy to produce significant improvements in a variety of endpoints including upper & lower extremity strength, Modified Ashworth Scale, 6-minute walking distance, and functional reach test. Patients with Stage 2 and 3 pressure ulcers required protection using OpSite. No maceration or worsening of the wound were noted. Those with colostomy bags required ostomy bag to be picture frame taped to clean, dry skin with waterproof dressing before pool session. Only one reported accident was noted which required the pool to be shocked with appropriate chemicals. No untoward medical complications were noted with the patient. Supra-pubic and indwelling catheters required them being secured to their body or floats. No incidence of catheter being pulled out was noted. Patients with tracheostomies required that they tolerate capping for a minimum of 4 hours. The water level was maintained at the nipple level. No desaturation or respiratory complications were noted among these patients. Conclusion: This study demonstrates the safety and feasibility of aquatic therapy for adult patients with spinal cord injuries and pressure ulcers, colostomy or ileostomy bags, supra pubic catheters, indwelling catheters, and tracheostomy tube. Aquatic therapy is a valuable therapeutic tool for patients with SCI with various complex comorbidities.
Keywords: water; accident; adult; classification; colostomy bag; comorbidity; complication; decubitus; dry skin; feasibility study; female; human; ileostomy bag; indwelling catheter; lower limb; major clinical study; male; medical record review; nipple; safety; spinal cord injury; suprapubic catheter; tracheostomy tube; walking; wound; young adult

Subclassification of GMFCS level-5 cerebral palsy as a predictor of complications and health-related quality of life after spinal arthrodesis - Jain A.; Sponseller P.D.; Shah S.A.; Samdani A.; Cahill P.J.; Yaszay B.; Njoku D.B.; Abel M.F.; Newton P.O.; Marks M.C.; Narayanan U.G. (Journal of Bone and Joint Surgery - American Volume, 2016)
Background: The Gross Motor Function Classification System (GMFCS) of cerebral palsy categorizes patients by mobility. Patients at GMFCS level 5 are considered the most disabled and at high risk of hip and spine problems, yet they represent a wide spectrum of function. Our aim was to subclassify patients at GMFCS level 5 who underwent spinal arthrodesis on the basis of central neuromotor impairments and to assess whether subclassification predicted postoperative complications and changes in health-related quality of life. Methods: Using a prospective cerebral palsy registry, we identified 199 patients at GMFCS level 5 who underwent spinal arthrodesis from 2008 to 2013. Patients were assigned to subgroups according to preoperative central neuromotor impairments: the presence of a gastrostomy tube, a tracheostomy, history of seizures, and nonverbal status. Nine percent of patients had 0 impairments (GMFCS level 5.0), 14% had 1 impairment (level 5.1), 26% had 2 impairments (level 5.2), and 51% had 3 or 4 impairments (level 5.3). The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire was used for preoperative and postoperative health-related quality-of-life outcome assessments, and major complications were recorded. Results: The rate of major complications increased significantly with higher GMFCS level-5 subtype (p = 0.002), with 12% at level 5.0, 21% at level 5.1, 31% at level 5.2, and 49% at level 5.3. Five of the 7 patients who died within the follow-upperiod were at level 5.3. No significant differences were found among subgroups with respect to the magnitude of correction of the major coronal curve or pelvic obliquity. Preoperative and final follo w-up CPCHILD total scores decreased significantly from GMFCS level 5.0 to level 5.3. However, no significant differences were found by subgroup with respect to the magnitude of improvement in CPCHILD total scores from the preoperative to t he final follow-up evaluation (p = 0.597). Conclusions: Stratification based on central neuromotor impairments can help to identify patients with cerebral palsy at GMFCS level 5 who are at higher risk for developing complications after spinal arthrodesis. Level of Evidence: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence
Keywords: adolescent; arthrodesis; article; Caregiver Priorities and Child Health Index of Life with Disabilities questionna; cerebral palsy; deep vein thrombosis; emotion; female; follow up; Gross Motor Function Classification System; health; human; interpersonal communication; lung embolism; major clinical study; male; mental deficiency; motor neuron disease; operative blood loss; outcome assessment; postoperative care; postoperative complication; postoperative infection; preoperative evaluation; priority journal; prospective study; quality of life; questionnaire; seizure; social interaction; spine; stomach tube; tracheostomy

Relationship Between Depressive State and Treatment Characteristics of Acute Cervical Spinal Cord Injury in Japan - Matsuda Y.; Kubo T.; Fujino Y.; Matsuda S.; Wada F.; Sugita A. (Journal of epidemiology, 2016)
BACKGROUND: Few studies have assessed whether treatment of acute cervical spinal cord injury (SCI) patients contributes to depression. METHODS: Using an administrative database, we assessed patients for whom the diagnosis was unspecified injuries of cervical spinal cord (International Classification of Diseases and Injuries-10th (ICD-10) code; S14.1). We categorized patients with codes for depressive episode (ICD-10 code; F32) or recurrent depressive disorder (F33), or those prescribed antidepressants (tricyclic, tetracyclic, Selective Serotonin Reuptake Inhibitors, Serotonin Noradrenaline Reuptake Inhibitors, Trazodone, Sulpiride, or Mirtazapine) as having a depressive state. We compared the rate of each acute treatment between the depressive state group and the non-depressive state group using chi-square tests, and a multiple logistic regression model was used to identify the association between the acute treatment and depressive state. RESULTS: There were 151 patients who were judged to be in a depressive state, and the other 2115 patients were categorized into the non-depressive state group. Intervention of intravenous anesthesia, tracheostomy, artificial respiration, and gastrostomy had a significant positive correlation with depressive state. Multiple logistic regression analysis showed that tracheostomy (odds ratio [OR] 2.18; 95% confidence interval [CI], 1.09-4.38) and artificial respiration (OR 2.28; 95% CI, 1.32-3.93) were significantly associated with depressive state, and men had a 36% reduction in the risk of depressive state compared with women (OR 0.64; 95% CI, 0.44-0.94), whereas age, wound-treatment, all of the orthopedic procedures, intravenous anesthesia, and gastrostomy were not associated with depressive state. CONCLUSIONS: These findings suggest that tracheostomy, artificial respiration and female gender in the acute phase after cervical SCI might be associated with the development of depression.
Keywords: acute disease; adolescent; adult; child; depression; factual database; female; human; infant; Japan; male; middle aged; newborn; preschool child; psychology; risk factor; spinal cord injury; young adult

Respiratory problems and management in people with spinal cord injury - Berlowitz D.J.; Wadsworth B.; Ross J. (Breathe, 2016)
Spinal cord injury (SCI) is characterised by profound respiratory compromise secondary to the level of loss of motor, sensory and autonomic control associated with the injury. This review aims to detail these anatomical and physiological changes after SCI, and outline their impact on respiratory function. Injury-related impairments in strength substantially alter pulmonary mechanics, which in turn affect respiratory management and care. Options for treatments must therefore be considered in light of these limitations.
Keywords: article; autonomic dysfunction; body position; breathing; cardiovascular disease; chest infection; comorbidity; coughing; diaphragm; electrotherapy; extubation; health status; hospital readmission; human; life satisfaction; long term care; lung edema; lung embolism; lung mechanics; lung volume; magnetic stimulation; motor performance; muscle training; positive end expiratory pressure; quality of life; respiratory tract disease; respiratory tract disease assessment; sleep disordered breathing; speech; spinal cord injury; tracheostomy; ventilator weaning

Functional and radiological outcome in patients undergoing three level corpectomy for multi-level cervical spondylotic myelopathy and ossified posterior longitudinal ligament - Gupta A.; Rajshekhar V. (Neurology India, 2016)
Background: To review our experience with patients undergoing 3 level cervical central corpectomy (CC) with un-instrumented fibular autograft fusion. Materials and Methods: This is a retrospective study, involving 33 patients with cervical spondylotic myelopathy (CSM) or ossified posterior longitudinal ligament (OPLL) who underwent a 3 level CC between 2002 and 2010. The patients were followed up clinically and radiologically. Their functional status was assessed using Nurick's grading system. Parameters such as intraoperative complications, segmental curvature of the cervical spine, graft subsidence, graft fusion and functional outcome of these patients were assessed. Results: There was transient morbidity in 28.6% of patients, with no permanent morbidity or mortality. We obtained follow up in 29 patients (87.9%) with a mean duration of follow up of 65.1 months (range, 12 to 138 months). The mean difference of segmental cervical curvature on follow up was 3.600 and the average graft subsidence was 5.70 mm. We achieved a fusion rate of 90%. There was no instance of graft extrusion in our series. There was a significant improvement in the functional status of our patients (from Nurick grade 3.55 to 2.42; P = 0.0001), with no clinical deterioration in any patient. Conclusions: Three level cervical corpectomy with un-instrumented fusion is a relatively safe surgery in experienced hands, and can achieve excellent clinical and radiological outcomes.
Keywords: steroid; adult; aged; article; cervical corpectomy; cervical spine radiography; cervical spondylotic myelopathy; clinical article; decompression surgery; dysphagia; female; follow up; functional status assessment; human; ligament calcinosis; liquorrhea; male; morbidity; Nurick grading system; peroperative complication; pneumonia; postoperative period; respiratory arrest; retrospective study; spine disease; spine fusion; spine surgery; steroid therapy; surgical mortality; surgical technique; tracheostomy

Delayed post trauma retropharyngeal hematoma with acute airway obstruction in a non cervical-spine injury - Vengathajalam S.; Rahim F.A.; Zakaria Z. (Rawal Medical Journal, 2016)
We report a case of a 10 year old boy who presented with delayed onset of retropharyngeal hematoma post trauma with acute airway obstruction. He had motor vehicle accident 2 weeks prior and sustained extensive subcutaneous emphysema with bilateral pneumothorax due to rib fractures. CT showed hypodense collection measuring 1cm x 1cm x 2cm at retropharyngeal space with compression at the posterior wall of the trachea. He was intubated and underwent tracheostomy for persistent upper airway obstruction.
Keywords: airway obstruction; article; breathing disorder; bronchoscopy; case report; child; computer assisted tomography; emphysema; endotracheal intubation; follow up; hematoma; human; laryngoscopy; male; retropharyngeal hematoma; school child; tracheostomy; traffic accident

A Novel Combined Hybrid Approach to Enable Revascularisation of a Trauma-Induced Subclavian Artery Injury - Sabbagh C.N.; Chowdhury M.M.; Durrani A.; Van Rensburg L.; Koo B.; Coughlin P.A. (EJVES Short Reports, 2016)
Introduction This case highlights the complexity of upper limb revascularization after a subclavian artery traumatic injury and strengthens the role of a hybrid/multi-disciplinary approach to such injuries. Report A 45-year-old male patient presented with an acute right upper limb following a traumatic injury to the right subclavian artery due to a motor vehicle accident (MVA). Associated injuries included an unstable cervical spine injury, a large open right clavicular injury, and a brain injury, which limited the potential revascularisation options available. The arm was revascularised using a hybrid endovascular/open surgical approach, namely embolization of the proximal subclavian artery (just distal to vertebral artery) and a right common femoral artery to distal axillary artery bypass using prosthetic material. Discussion Blunt injuries to the subclavian artery are often high impact, complex and associated with multiple injuries to surrounding structures, which limit the role of standard procedures used in the elective setting. This case highlights the role of multidisciplinary team involvement, using a hybrid approach and a novel distal inflow site to restore upper limb perfusion.
Keywords: collagen sponge; gentamicin; hemostatic agent; heparin; abdominal wall; adult; Amplatzer vascular plug; aortic arch; artery bypass; artery catheter; artery injury; article; artificial embolization; atelectasis; axillary artery; brachial plexus injury; brachiocephalic trunk; brain contusion; brain injury; breathing; case report; cervical collar; cervical spine fracture; cervical spine injury; chest tube; clavicle; clavicle fracture; clavicular injury; cold; computer assisted tomography; convulsion; coronary artery bypass surgery; cranial nerve paralysis; disease association; electric activity; endovascular surgery; fasciotomy; femoral artery; fibula fracture; general anesthesia; hematoma; Human alphaherpesvirus 1; hospital admission; hospital readmission; human; intensive care unit; laceration; leg revascularization; limb injury; liver injury; male; mediastinum disease; medical history; middle aged; neurorehabilitation; oculomotor nerve disease; paresis; physiotherapy; pneumonia; politef implant; priority journal; respiratory tract intubation; resuscitation; rhabdomyolysis; right subclavian artery; scapula fracture; shoulder injury; sixth cervical vertebra; skin incision; speech production aid; speech therapy; sternum; subarachnoid hemorrhage; subclavian artery; subclavian artery traumatic injury; surgical technique; tension pneumothorax; thorax drainage; tibia fracture; tracheostomy; traffic accident; vertebral artery; wound; Amplatzer; ePTFE graft

Bradycardia Associated with Steroid Use for Laryngeal Edema in an Adult: A Case Report and Literature Review - John P.R.; Khaladj-Ghom A.; Still K.L. (Case Reports in Cardiology, 2016)
Steroids are used for specific indications in the perioperative period to reduce laryngeal or spinal cord edema, or for prophylaxis and treatment of postoperative nausea and vomiting. Given the other potential causes for hemodynamic alterations in the perioperative setting, it is important for physicians to be aware of cardiovascular side effects of short term steroids. Changes in blood pressure and heart rate, cardiac dysrhythmias, and even death have been described in patients receiving short term intravenous steroids. Bradycardia has been reported following short term methylprednisolone and dexamethasone therapy in both adult and pediatric patients. There are only two case reports in the literature of bradycardia following short term intravenous dexamethasone use in adult patients. This is the first case report that describes bradycardia following the use of dexamethasone in the postoperative setting for management of laryngeal edema in an adult. Telemetry and twelve lead electrocardiograms revealed sinus bradycardia and correlated directly with administration of dexamethasone in our patient. Bradycardia resolved following discontinuation of dexamethasone. We advocate for hemodynamic monitoring in patients receiving more than one dose of intravenous steroid therapy in the perioperative period, especially those with known cardiac and hepatic comorbidities and those taking medications with negative chronotropic effects.
Keywords: surgical mesh; dexamethasone; midazolam; nafcillin; propofol; troponin; adult; antibiotic therapy; case report; corticosteroid therapy; device infection; disease association; drug withdrawal; electrocardiography; endotracheal intubation; general anesthesia; human; hypercapnia; laryngoscopy; larynx edema; male; middle aged; nonhuman; noninvasive ventilation; respiratory failure; review; sinus bradycardia; small intestine resection; sputum culture; Staphylococcus infection; stridor; surgical infection; surgical mesh; telemetry; tracheostomy; videolaryngoscope; work of breathing

To the editor - Zou M.-X.; Lv G.-H.; Wang X.-B.; Li J. (Spine, 2016)
Keywords: cervical spinal cord injury; hematoma; human; letter; nuclear magnetic resonance imaging; priority journal; risk factor; tracheostomy

Globular Glial Mixed Four Repeat Tau and TDP-43 Proteinopathy with Motor Neuron Disease and Frontotemporal Dementia - Takeuchi R.; Toyoshima Y.; Tada M.; Tanaka H.; Shimizu H.; Shiga A.; Miura T.; Aoki K.; Aikawa A.; Ishizawa S.; Ikeuchi T.; Nishizawa M.; et al. (Brain Pathology, 2016)
Amyotrophic lateral sclerosis (ALS) may be accompanied by frontotemporal dementia (FTD). We report a case of glial mixed tau and TDP-43 proteinopathies in a Japanese patient diagnosed clinically as having ALS-D. Autopsy revealed loss of lower motor neurons and degeneration of the pyramidal tracts in the spinal cord and brain stem. The brain showed frontotemporal lobar degeneration (FTLD), the most severe neuronal loss and gliosis being evident in the precentral gyrus. Although less severe, such changes were also observed in other brain regions, including the basal ganglia and substantia nigra. AT8 immunostaining revealed that predominant occurrence of astrocytic tau lesions termed globular astrocytic inclusions (GAIs) was a feature of the affected regions. These GAIs were Gallyas-Braak negative. Neuronal and oligodendrocytic tau lesions were comparatively scarce. pS409/410 immunostaining also revealed similar neuronal and glial TDP-43 lesions. Interestingly, occasional co-localization of tau and TDP-43 was evident in the GAIs. Immunoblot analyses revealed band patterns characteristic of a 4-repeat (4R) tauopathy, corticobasal degeneration and a TDP-43 proteinopathy, ALS/FTLD-TDP-Type-B. No mutations were found in the MAPT or TDP-43 genes. We consider that this patient harbored a distinct, sporadic globular glial mixed 4R tau and TDP-43 proteinopathy associated with motor neuron disease and FTD.
Keywords: amyloid beta protein; glial fibrillary acidic protein; sequestosome 1; Sarkosyl insoluble tau; TAR DNA binding protein; tau protein; ubiquitin; unclassified drug; acute cholecystitis; aged; amygdala; article; assisted ventilation; brain atrophy; brain stem; capsula interna; case report; cause of death; cell inclusion; cerebellum cortex; cerebral peduncle; coiled body; dentate gyrus; dentate nucleus; facial nerve nucleus; female; frontotemporal dementia; gene mutation; globus pallidus; human; human tissue; hypoglossal nucleus; immunoblotting; immunofluorescence; immunohistochemistry; inferior olivary nucleus; locus ceruleus; Meynert basal nucleus; motoneuron; motor cortex; motor neuron disease; neuropil thread; oculomotor nucleus; pontine nucleus; putamen; pyramidal tract; red nucleus; respiratory distress; senile plaque; spinal cord dorsal horn; spinal cord ventral horn; substantia nigra; subthalamic nucleus; tauopathy; TDP 43 proteinopathy; tectum; thalamus; tracheotomy; trigeminal motor nucleus; very elderly; white matter

Acute flaccid paralysis following spinal anaesthesia: A diagnostic dilemma - Dassanayake A. (Sri Lankan Journal of Anaesthesiology, 2016)
We report on a patient who developed an acute flaccid paralysis following spinal anaesthesia for a right sided inguinal hernia repair and orchidopexy, while on post exposure prophylaxis with anti-rabies vaccination following a dog bite.
Keywords: aciclovir; cefuroxime; rabies vaccine; adult; article; artificial ventilation; autopsy; blood analysis; bulbar paralysis; case report; death; diaphragm paralysis; dog bite; flaccid paralysis; Glasgow coma scale; hernioplasty; hospital admission; human; inguinal hernia; intensive care unit; lumbar puncture; male; middle aged; muscle weakness; nerve conduction; neurologic examination; orchidopexy; paresthesia; post exposure prophylaxis; postoperative period; rabies; restlessness; spinal anesthesia; tracheostomy

Globular glial mixed four repeat tau and TDP-43 proteinopathy with motor neuron disease and frontotemporal dementia - Takeuchi R.; Toyoshima Y.; Tada M.; Shiga A.; Miura T.; Aoki K.; Ikeuchi T.; Nishizawa M.; Kakita A.; Takahashi H. (Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2015)
Case Study: Amyotrophic lateral sclerosis (ALS) may be accompanied by frontotemporal dementia (FTD). We report a case of glial mixed tau and TDP-43 proteinopathies in a patient diagnosed clinically as having ALS-D. A 76-year-old Japanese woman became aware of gait disturbance, and subsequently developed dysarthria. On examination, she showed atrophy and fasciculation in the tongue, a hypoactive gag reflex, and muscle weakness in the upper extremities. Increased deep tendon reflexes were also present in the upper and lower extremities, with positive Babinski sign in both legs. About 10 months after onset, at the age of 77, she was diagnosed as having ALS. Her mental performance deteriorated rapidly and a state of apathy ensued; at this stage, the clinical diagnosis of ALS-D was made. She also suffered from progressive respiratory distress and underwent tracheotomy for artificial respiratory support. At the age of 78 years, she eventually became bedridden in a totally locked-in state. Brain CT scan performed at the age of 81 years revealed frontotemporal atrophy. At the age of 85 years, the patient died of septic acute cholecystitis, about 9 years after onset of the disease. There were no parkinsonian features during the disease course. There had been no family history of ALS, dementia or other neurological disease. A general autopsy was performed, at which time the brain weighed 910 g. Histologically, loss of lower motor neurons and degeneration of the pyramidal tracts were evident in the spinal cord and brainstem. The brain showed frontotemporal lobar degeneration (FTLD); the most severe neuronal loss and gliosis being evident in the precentral gyrus. Although less severe, such changes were also observed in other brain regions, including the basal ganglia and substantia nigra. AT8 immunostaining revealed that predominant occurrence of astrocytic tau lesions termed globular astrocytic inclusions (GAIs) was a feature of the affected regions. These GAIs were Gallyas- Braak negative. Neuronal and oligodendrocytic tau lesions were comparatively scarce. pS409/410 immunostaining also revealed similar neuronal and glial TDP-43 lesions. Interestingly, occasional co-localization of tau and TDP- 43 was evident in the GAIs. Immunoblot analyses revealed band patterns characteristic of a 4-repeat (4R) tauopathy, corticobasal degeneration and a TDP-43 proteinopathy, ALS/FTLD-TDP Type B. No mutations were found in the MAPT or TDP-43 genes. We consider that this patient harbored a distinct, sporadic globular glial mixed 4R tau and TDP-43 proteinopathy associated with motor neuron disease and FTD.
Keywords: frontotemporal dementia; motor neuron disease; TDP 43 proteinopathy; human; patient; brain; leg; atrophy; immunohistochemistry; amyotrophic lateral sclerosis; arm; reflex; case study; tongue; muscle weakness; autopsy; motoneuron; tendon reflex; acute cholecystitis; fasciculation; examination; gene; dysarthria; computer assisted tomography; gait; immobility; mutation; assisted ventilation; Japanese (people); tracheotomy; disease course; tauopathy; family history; respiratory distress; neurologic disease; immunoblotting; degeneration; pyramidal tract; spinal cord; diagnosis; apathy; substantia nigra; basal ganglion; brain region; primary motor cortex; gliosis; brain stem; mental performance; Babinski reflex; corticobasal degeneration; female

Ruptured spinal arteriovenous malformation: Presenting as stunned myocardium and neurogenic shock - Mehesry T.; Shaikh N.; Malmstrom M.; Marcus M.; Khan A. (Surgical Neurology International, 2015)
Background: Neurogenic pulmonary edema (NPE) is a clinical syndrome usually defined as an acute pulmonary edema occurring shortly after a central neurologic insult. NPE was identified 100 years ago, but it is still underappreciated in the clinical setup. NPE usually appears within minutes to hours after the injury. It has a high mortality rate if not recognized early and treated appropriately. Similarly, neurogenic shock is a known complication of spinal cord injury reported incidence is more than 20% in isolated upper cervical spinal injury. But NPE is rare to occur, and stunned myocardium (SM) is not reported in spinal arteriovenous malformation (AVM) rupture. SM is a reversible cardiomyopathy resulting in transient left ventricular dysfunction which has been described to occur in the setting of catecholamine release during situations of physiologic stress. We report a case of high spinal AVM rupture presenting as SM, NPE, and neurogenic shock. Case Description: A 32-year-old male who presented with sudden onset of pain and weakness in upper limbs. Imaging studies showed AVM rupture by imaging techniques. Initially, the patient had severe hypertension, respiratory distress requiring intubation and ventilation, then he developed hypotension, bradycardia, and asystole, which required immediate cardiopulmonary resuscitation and atropine. He remained with quadriplegia and suffered from frequent episodes of bradycardia and asystole. Conclusions: Spinal AVM rupture can present as neurogenic shock, stunned myocardium, and pulmonary edema. Early recognition of AVM rupture and prompt surgical intervention, as well as aggressive treatment of shock, may enhance recovery and decrease the long-term morbidity.
Keywords: aminophylline; atropine; dobutamine; dopamine; meropenem; adult; article; artificial ventilation; bradycardia; brain arteriovenous malformation; brain ventricle; case report; central venous catheterization; cervical spine; clinical feature; computer assisted tomography; disease severity; echocardiography; fever; fluid resuscitation; Glasgow coma scale; Haemophilus infection; Haemophilus influenzae; heart arrest; cardiac index; heart output; Holter monitoring; human; hypertension; hypokinesia; hypotension; induced hypotension; intensive care; intubation; limb pain; limb weakness; lung edema; male; neurogenic shoxk; neurologic disease; nuclear magnetic resonance imaging; oxygen desaturation; priority journal; quadriplegia; respiratory distress; resuscitation; shock; sinus bradycardia; spinal arteriovenous malformation rupture; stunned heart muscle; suction; thorax radiography; tracheostomy; transcutaneous pacemaker; treatment response

Current results of open repair of descending thoracic and thoracoabdominal aortic aneurysms - Gaudino M.; Lau C.; Munjal M.; Elsayed M.; Benedetto U.; Girardi L.N. (Circulation, 2015)
Introduction: Open repair of thoracic and thoracoabdominal aortic aneurysms (DTA and TAAA respectively) constitutes a benchmark for comparison with endovascular techniques. Hypothesis. To evaluate the current results of the open repair of DTA and TAAA. Methods. Open DTA and TAAA repair was performed on 675 consecutive patients (210 TAA and 465 TAAA). Three surgical techniques were used based on the type of the aneurysm and the risk profile of the patient: clamp and saw, partial bypass or hypothermic circulatory arrest. Spinal drain was used in 82.7% of patients. Regression analysis was performed to identify independent determinants of in-hospital and late outcomes and individual major postoperative complications. Propensity matching (PPM) was used to compare results of the different surgical techniques Results. Operative mortality was 5.6% (38/675); incidence of postoperative spinal cord injury, stroke, need for tracheostomy, myocardial infarction and dialysis were 2.8%, 0.7%, 7.7%, 0.6% and 5.2% respectively. Kaplan Mayer survival at five-year was 57.8%. Female gender, urgent operation and preoperative dialysis were independent predictors of adverse in-hospital adverse events. Preoperative renal and respiratory failure were associated with follow-up death. Female gender and preoperative chronic pulmonary disease were associated with postoperative need for tracheostomy, whereas preoperative renal failure, urgent operation and peripheral vascular disease predicted the need for postoperative dialysis. Due to the low number of events, logistic regression analysis was not possible for predictors of spinal cord injury. In the PPM groups no difference was found in the early and late outcome between patients treated with different techniques (see table). Conclusion: In the current era open TAA and TAAA repair allows excellent outcomes.
Keywords: thoracic aorta; aneurysm; aortic aneurysm; medical society; resuscitation; human; patient; dialysis; surgical technique; tracheostomy; spinal cord injury; female; gender; hospital; regression analysis; cerebrovascular accident; heart arrest; clamp; logistic regression analysis; risk; hypothesis; surgical mortality; chronic lung disease; heart infarction; follow up; postoperative complication; respiratory failure; survival; death; kidney failure; peripheral vascular disease

Surgical Therapy of Cervical Spine Fracture in Patients with Ankylosing Spondylitis - Ma J.; Wang C.; Zhou X.; Zhou S.; Jia L. (Medicine (United States), 2015)
The present study aimed to explore surgical treatments and assess the effects based on the features of cervical spine fracture in patients with ankylosing spondylitis (AS) and to summarize the experiences in perioperative management. Retrospective analysis was performed in 25 AS patients with cervical spine fracture treated in our hospital from January 2011 to December 2013. The patients were divided according to fracture segments, including 4 cases at C4 to C5, 8 cases at C5 to C6, and 13 cases at C6 to C7. Among them, 12 belonged to I type, 5 to II type, and 8 to III type based on the improved classification method for AS cervical spine fracture. The Subaxial Cervical Spine Injury Classification score for these patients was 7.2 ± 1.3, and the assessment of their neurological function states showed 6 patients (24%) were in American Spinal Injury Association (ASIA) A grade, 1 (4%) in ASIA B grade, 3 (12%) in ASIA C grade, 12 (48%) in ASIA D grade, and 3 (12%) in ASIA E grade. Surgical methods contained simple anterior approach alone, posterior approach alone, and combined posterior-anterior or anterior-posterior approach. The average duration of patients' hospital stay was 38.6 ± 37.6, and the first surgical methods were as follows: anterior approach alone on 6 cases, posterior surgery alone on 9 cases, and combined posterior-anterior or anterior-posterior approach on 10 patients. The median segments of fixation and fusion were 4.1 ± 1.4 sections. Thirteen patients developed complications. During 2 to 36 months of postoperative follow-up, 1 patient died of respiratory failure caused by pulmonary infections 2 months after leaving hospital. At the end of the follow-up, bone graft fusion was achieved in the rest of patients, and obvious looseness or migration of internal fixation was not observed. In addition, the preoperative neurological injury in 12 patients (54.5%) was also alleviated in different levels. AS cervical spine fracture, an unstable fracture, should be treated with operation, and satisfactory effects will be achieved after the individualized surgical treatment according to the improved classification method for AS cervical spine fracture.
Keywords: adult; ankylosing spondylitis; article; bone transplantation; cause of death; cervical spine fracture; clinical article; cognition; conservative treatment; decubitus; disease association; esophagus injury; female; hospitalization; human; intervertebral disk; length of stay; lung infection; male; middle aged; operation duration; osteosynthesis; perioperative period; postoperative complication; priority journal; respiratory failure; screw loosening; tracheotomy; urinary tract infection; vertebra body

Historical Vignette of Infamous Gunshot Injury to Spine: "an Ailment not to be Treated"? - Maiti T.K.; Konar S.; Bir S.C.; Bollam P.; Nanda A. (World Neurosurgery, 2015)
The increasing prevalence and gloomy socioeconomic consequence of spine injury remain a concern in modern medicine. In this article, we highlight the infamous gunshot spinal injuries of a few eminent personalities across multiple centuries and their sociopolitical impact in context with the evolution of modern medicine. The role of available medicine in these victims was not more than a mere watcher, thus substantiating an infamous quote from ancient literature that describes spine injury as "an ailment not to be treated."
Keywords: rose oil; turpentine; anesthesia; article; blood transfusion; bullet; computer assisted tomography; egg yolk; gunshot injury; gunshot spinal injury; homicide; human; medical history; mortality; nuclear magnetic resonance imaging; politics; psychosurgery; socioeconomics; spine fracture; spine injury; suicide attempt; surgical glove; tracheostomy; vertebra dislocation; wound drainage

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with a significant elevation of β-2 microglobulin levels - Fujisawa N.; Oya S.; Mori H.; Matsui T. (Journal of Korean Neurosurgical Society, 2015)
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a relapsing-remitting disorder for which steroid administration is a key to control the progression. CLIPPERS can exhibit radiological features similar to malignant lymphoma, whose diagnosis is confounded by prior steroid administration. We report a case of CLIPPERS accompanied by abnormal elevation of β-2 microglobulin in the cerebrospinal fluid (CSF). A 62-year-old man started to experience numbness in all fingers of his left hand one year ago, which gradually extended to his body trunk and legs on both sides. Magnetic resonance imaging demonstrated numerous small enhancing spots scattered in his brain and spinal cord. CSF levels of β-2 microglobulin were elevated; although this often indicates central nervous system involvement in leukemia and lymphoma, the lesions were diagnosed as CLIPPERS based on the pathological findings from a biopsy specimen. We emphasize the importance of biopsy to differentiate between CLIPPERS and malignant lymphoma because the temporary radiological response to steroid might be the same in both diseases but the treatment strategies regarding the use of steroid are quite different.
Keywords: beta 2 microglobulin; lactate dehydrogenase; methylprednisolone; prednisone; steroid; adult; article; brain damage; case report; central nervous system disease; cerebellar ataxia; cerebrospinal fluid analysis; chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; drug dose reduction; dysarthria; dysphagia; human; human tissue; immunohistochemistry; male; middle aged; neurologic examination; nuclear magnetic resonance imaging; paresthesia; respiratory distress; tracheostomy

Perspective from spinal muscular atrophy families: Care of a child with tracheostomy and home mechanical ventilatory support - Tanyildiz M.; Topaloʇlu H.; Oncel I.; Bayrakci B.; Haliloglu G. (Neuromuscular Disorders, 2015)
Management of pulmonary disease and respiratory failure in spinal muscular atrophy (SMA) is challenging depending on social and cultural issues. Exploring options with the individual family in terms of the patients' potential, quality of life and family's desires is essential. We retrospectively analyzed 30 SMA patients (Girls: 15, Boys: 15) followed due to acute/chronic respiratory failure in the Pediatric Intensive Care Unit (PICU) between 2002 and 2014. As a first step, medical records of all patients, and reflections from 21 of the families by phone call were reviewed. As a second step, we evaluated pediatric quality of life (PedsQLTM) inventory and Beck depression inventory from 8 families of the already alive 10 SMA patients. Twenty-nine patients had a genetically confirmed diagnosis including SMA Type I (n = 26), SMA Type II (n = 2), and SMARD (n = 1). Mean age at the time of diagnosis was 4 months (intrauterine - 36 months). 22 out of 30 patients had tracheostomy. To date, 10 out of 21 reached patients are alive with a mean age of 3.4 years (20 months-8 years). All of the surviving patients are on home-mechanical ventilation. Feeding was via nasogastric tube (n = 3) and gastrostomy (n = 5). All of the patients receive regular chest and physical therapy. Eight of 10 surviving patients use home care health services provided by Ministry of Health. PedsQL inventory and Beck depression inventory in 8 families showed that despite the poor parental quality of life status, families have no depressive symptoms except one. All the parents expressed that living with a child with SMA is difficult, but they learned to cope with it in time. Invasive procedures for SMA type I is a challenging decision. Our results show that families develop a complex coping mechanism including a multilevel medical care. Familial characteristics further deserve attention in the era of up-coming potential treatment options.
Keywords: spinal muscular atrophy; child; human; tracheostomy; muscle; society; patient; quality of life; diagnosis; Beck Depression Inventory; respiratory failure; nasogastric tube; girl; artificial ventilation; male; medical record; coping behavior; intensive care unit; feeding; health; boy; gastrostomy; health service; home care; physiotherapy; thorax; depression; parent; invasive procedure; medical care; female; lung disease

Prolonged orotracheal intubation - Rajagopal A.; Hirschowitz E.A.; Wallace T. (American Journal of Respiratory and Critical Care Medicine, 2015)
While timely exubation or conversion to tracheostomy are often early goals, data on prolonged orotracheal intubation is limited. We report and present a case of prolonged orotracheal intubation, greater than 73 days, without change in endotracheal tube. 64-year-old male patient with multiple medical problems including end-stage-COPD and ischemic systolic and diastolic heart failure (EF<20%) presented in acute hypoxic hypercapnic respiratory failure secondary to pulmonary edema. He had marked and fixed cervical kyphosis and had suffered traumatic injury to c-spine in a logging accident. As a result, he cannot extend his neck. He has very limited lateral bending and rotation to just a few degrees. He was successfully intubated, however multiple attempts at extubation were unsuccessful due to reaccumulation of fluid in his lungs secondary to heart failure. He was subsequently evaluated for a tracheostomy by ear-nose-throat, cardiothoracic surgery, and general surgery teams who were in agreement that he was not a candidate for tracheostomy secondary to his fixed cervical spine and intrathoracic trachea. On physical exam, the trachea was displaced posteriorly and inferiorly, the thyroid notch was palpable in his sternal notch and the cricothyroid membrane palapable with swallowing. However, in order to place a tracheostomy he would need a portion of his sternum and clavicular heads removed to gain access to the trachea. The risks/morbidity of this procedure outweighed the benefit. It was determined that the patient would remain intubated with the size 8 endotracheal tube. Approximately 30 days into the intubation an air leak developed and the patient declined any tube changes. There are episodes of periodic mucous plugging with possible anoxic brain injury. He continues to be supported by orotracheal intubation at day 73. Complications of prolonged orotracheal intubation are numerable and can include infection, tracheal stenosis, esophageal erosion, and innominate artery fistulization. While the risk of complication is present, keeping the tube in place may prolong life when tracheostomy is not an option. While early extubation is preferable it is not always feasible. As was the case in our patient, some cases are not ammenable to tracheostomy. In these cases, terminal extubation may not be the only option.
Keywords: endotracheal intubation; American; society; tracheostomy; human; patient; extubation; trachea; tube; endotracheal tube; diastolic heart failure; ear; male; lung; brain injury; liquid; throat; neck; infection; trachea stenosis; heart failure; brachiocephalic trunk; nose; lung edema; respiratory failure; accident; intubation; sternum; logging; spine; swallowing; membrane; procedures; thyroid gland; injury; cervical spine; general surgery; thorax surgery; kyphosis; risk

Outcomes of children with type 1 spinal muscular atrophy who were ventilator dependent at home - Cristea A.; Harris J.M.; Shelley D.; Ackerman V. (American Journal of Respiratory and Critical Care Medicine, 2015)
Introduction Data describing health outcomes of children with Type 1 Spinal Muscular Atrophy (SMA) originates from countries where tracheostomy and/or chronic ventilation are not routinely offered. There is significant debate in the medical literature regarding the ethics of the decision to offer chronic ventilation to these patients. To our knowledge there is limited information regarding long term outcomes of these children who receive care within the US healthcare system. Methods We retrospectively reviewed charts of patients with type 1 SMA who were ventilator dependent at home and who were enrolled in a university-affiliated home ventilator program between 1985 and 2014. Results During this interval, eleven children with type 1 SMA were cared for in our home ventilator program. Of these, six were males, seven were Caucasian and three were African American. All patients were in their biological parents' care. One patient was later placed in an extended care skilled facility after the death of his primary caregiver. All families were offered the choice of palliative care at time of diagnosis with one exception, when the tracheostomy was placed before the diagnosis was made. There were 63 readmissions among the group (range: 3 - 16 admissions/patient) with a median length of stay of 19 days (range: 6 - 116). Comorbidities encountered in this group include kyphoscoliosis (11), hypertension (2), inflammatory bowel disease (2), depression (4) and hirisutism (1). Three patients survived cardiopulmonary arrest at home after a tracheostomy catastrophic event or ventilator disconnect. Five patients died, at a median age of 7.9 years (range: 3.1 - 14.6). The remaining six are alive, with a median age of 22.1 years (range: 1.19 - 27.3). Conclusions Children with type 1 SMA who are electively placed on home ventilation via tracheostomy may survive for a long time. Families and caregivers who are considering long term chronic ventilation for a child with Type 1 SMA need better information regarding the potential long life expectancy and multiple complications encountered in this population in order to make better quality of life decisions for their child.
Keywords: human; spinal muscular atrophy; ventilator; American; society; child; patient; air conditioning; tracheostomy; caregiver; diagnosis; hospital readmission; palliative therapy; death; parent; African American; population; Caucasian; life expectancy; cardiopulmonary arrest; university; health care system; length of stay; inflammatory bowel disease; male; hypertension; kyphoscoliosis; ethics; medical literature; quality of life; health

Cardiovascular collapse during spinal anesthesia - Zakhary B.; Coritsidis G. (American Journal of Respiratory and Critical Care Medicine, 2015)
INTRODUCTION Cardiac arrest during spinal anesthesia is a relatively rare event. The pathogenesis is incompletely understood but likely involves the loss of sympathetic tone. We report a case of spinal anesthesia complicated by cardiac arrest. CASE PRESENTATION A 32 year old healthy male was brought in after sustaining a gun-shot wound to the right lower extremity. In the trauma bay, the patient was awake and interactive with a Glasgow Coma Scale score of fifteen. On examination, he had an entry wound in the right posterior knee. Imaging demonstrated a fractured right patella with multiple bullet fragments. The patient was scheduled for surgical debridement. In the operating room, the patient received spinal anesthesia at the lumbar level. Within ten minutes, he was noted to be hypotensive then quickly apneic and unresponsive. Rhythm monitor demonstrated pulseless electrical activity. The patient was intubated with initiation of advanced cardiac life support. He required two rounds of epinephrine with subsequent return of spontaneous circulation. The patient was transferred to the surgical intensive care unit. Over the next several days, the patient had a poor neurological recovery, ultimately requiring percutaneous tracheostomy and gastrostomy tubes. DISCUSSION Cardiac arrest during spinal anesthesia is a relatively rare event with an incidence of about 0.07%. Although the mechanism is not clearly defined, it is believed that there is superior migration of the anesthetic with subsequent loss of sympathetic tone. Risk factors associated with subsequent circulatory arrest include high basal parasympathetic tone, low blood volume prior to anesthesia, and rapid progression of spinal blockade. The Bezold-Jarisch reflex (a cardio-inhibitory reflex in response to an underfilled ventricle leading to bradycardia, hypotension, and apnea) may also play a role. Clinical findings include hypotension (secondary to vasodilation and reduced venous return) and bradycardia (secondary to loss of the cardio-accelerator fibers arising from T1-4 and to the Bezold-Jarish reflex) resulting in reduced cardiac output and possibly cardiac arrest. Respiratory arrest may also be seen. Treatment is primarily supportive and includes aggressive volume resuscitation with pressor and inotropic support as needed. Ventilatory support is also often required. CONCLUSION Spinal anesthesia can be complicated by circulatory collapse and respiratory failure. Although rare, this case highlights that the clinical presentation can be dramatic and requires immediate recognition and aggressive support.
Keywords: antihypertensive agent; hypertensive factor; anesthetic agent; epinephrine; spinal anesthesia; American; society; shock; patient; human; heart arrest; reflex; sympathetic tone; resuscitation; hypotension; bradycardia; Glasgow coma scale; gunshot injury; operating room; male; debridement; blood volume; apnea; vasodilatation; rhythm; electric activity; respiratory failure; bullet; patella; stomach tube; imaging; bay; tracheostomy; knee; intensive care unit; risk factor; parasympathetic tone; return of spontaneous circulation; anesthesia; wound; venous return; fiber; heart output; respiratory arrest; examination; inotropism; injury; leg; pathogenesis

Cervical anterior spinal cord syndrome following pulmonary artery rupture during a left and right heart catheterization: A rare but significant complication - Niccum D.; Dincer H.; Arndt P. (American Journal of Respiratory and Critical Care Medicine, 2015)
Introduction: Anterior spinal cord infarction is a rare procedural complication, particularly after cardiac catheterization. Previous reports of anterior spinal cord infarction after heart catheterization involved only patients undergoing left heart catherization and affected the lower spinal cord levels of L1, L2 , or conus medularis. The pathophysiology of ischemia was attributed to the rupture and embolism of pre-existing atherosclerotic plaques upon manipulation of the aorta during endovascular procedures. A much rarer presentation is anterior spinal cord infarction after right heart catheterization (RHC). Case: A 74-year-old female with history of OSA and spinal stenosis underwent a right and left cardiac catheterization via right femoral approach for evaluation of pulmonary hypertension. Coronary angiography demonstrated clear coronary arteries. On RHC, pulmonary artery systolic pressure (68 mm Hg) and wedge pressure (25 mm Hg) were found to be elevated. Upon inflation of the balloon in wedge position, she developed coughing with frank hemoptysis requiring intubation. Given the copious amounts of blood suctioned from her airway and unilateral circumscribed alveolar opacification on chest x-ray, she was diagnosed with a ruptured pulmonary artery. Two days later she was found to have paralysis of all four extremities though she was able to lift her head to command. She had intact sensation throughout but flaccid paralysis below the neck. MRI of the head and cervical spine revealed severe cervical spinal stenosis but no evidence of compression. Electromyography ruled out peripheral neuropathy. Lumbar puncture revealed no evidence of Guillain-Barre syndrome. Given these findings, the diagnosis of anterior spinal cord infarction was made. Over the ensuing month she had no neurological improvement and subsequently required tracheostomy before transferring to a rehab/long term care center. Discussion: This case is unique due to its involvement of the cervical spinal cord and onset after RHC; likely due to pulmonary artery rupture, in itself a rare complication of RHC. The association between these two complications can most easily be explained by the embolism of air or thrombus via collateral circulation between the pulmonary artery and bronchial (systemic) circulation at the time of the pulmonary rupture. Less likely mechanisms include an independent complication due to arterial plaque rupture on left heart catheterization or watershed infarct due to transient hypotension as previously described following coronary artery bypass surgeries. While one prior case of anterior spinal cord infarct has been described post pulmonary artery injury, this is the first report following right heart catheterization.
Keywords: pulmonary artery; artery rupture; heart catheterization; American; society; spinal cord; spinal cord infarction; rupture; infarction; vertebral canal stenosis; embolism; human; pulmonary hypertension; endovascular surgery; flaccid paralysis; aorta; cervical spine; electromyography; angiocardiography; sensation; paralysis; blood; airway; pathophysiology; female; intubation; hypotension; hemoptysis; coronary artery bypass surgery; artery injury; coughing; patient; atherosclerotic plaque; systolic blood pressure; neck; thrombus; compression; peripheral neuropathy; lumbar puncture; Guillain Barre syndrome; tracheostomy; coronary artery; ischemia; diagnosis; cervical spinal cord; collateral circulation; watershed; nuclear magnetic resonance imaging; thorax radiography

Bronchial artery embolization as a cause of myocardial infarction - Dhungana S.; Ali M.M.; Maskey A. (American Journal of Respiratory and Critical Care Medicine, 2015)
A 26 year old male with cystic fibrosis presented to the ED with massive hemoptysis following a coughing spell. He was emergently intubated for airway protection. A CT angiogram didn't reveal any extravasation of dye. Bronchoscopy revealed bleeding from the left lower lobe. After epinephrine and thrombin injection failed, the left lobe was isolated using an Arndt endobronchial blocker. Pt was transferred to the interventional radiology (IR) suite where he underwent embolization of the superior and inferior divisions of the left bronchial artery using microspheres. During the procedure, he developed visible ST elevation on the monitor. A stat EKG was concerning for acute inferior ST elevation MI. An emergent left heart catheterization revealed lack of flow in the distal posterolateral branch of the left circumflex vessel. The patient underwent unsuccessful balloon angioplasty of the culprit vessel. He continued to bleed and was taken back to the IR suite, where successful embolization of the left superior bronchial artery was done, with resolution of hemoptysis. The patient however went into profound septic shock and ARDS. He subsequently underwent tracheostomy in anticipation of prolonged mechanical ventilation. After two weeks, the patient started bleeding profusely again, raising the specter of trachea-innominate fistula. A CT angiogram of neck and chest confirmed the absence of a fistula, but revealed a third bronchial artery arising from the pericardiophrenic branch of the right internal mammary artery, as well as a 6 mm pseudoaneurysm involving the posterobasal segment of the left lower lobe. The patient was taken back to the IR suite, and embolization was performed on the right pericardiophrenic artery as well as the posterior basal segment of the left pulmonary artery, which was feeding the pseudoaneusym. The patient subsequently stopped bleeding. He had a prolonged hospital course due to inability to wean, and was transferred to a long term acute care hospital. stemi with bronchial artery embolization.jpg DISCUSSION Bronchial artery embolization (BA) is an established therapy for massive hemoptysis1. Complications of the procedure include chest pain, dysphagia, posterior circulation stroke, spinal cord infarction2, systemic embolization, etc. Collaterals between the bronchial artery and coronary arteries have been described3. Caution is advised to rule out such collaterals before embolization. We describe the first case of MI following BAE in US.
Keywords: thrombin; epinephrine; dye; microsphere; bronchial artery; artificial embolization; heart infarction; American; society; human; patient; bleeding; hospital; hemoptysis; procedures; fistula; cystic fibrosis; bronchoscopy; heart catheterization; septic shock; artificial ventilation; injection; ST segment elevation myocardial infarction; ST segment elevation; extravasation; protection; dysphagia; cerebrovascular accident; male; weaning; interventional radiology; airway; percutaneous transluminal angioplasty; tracheostomy; false aneurysm; trachea; endobronchial blocker; internal mammary artery; thorax; neck; artery; pulmonary artery; feeding; coughing; emergency care; therapy; thorax pain; spinal cord; coronary artery; adult respiratory distress syndrome

'Survey of clinicians' experience and attitudes regarding spinal muscular atrophy with respiratory distress syndrome type 1 (SMARD1) in the UK - Shah S.; Jerrom T.; Fraser J.; Majumdar A. (Neuromuscular Disorders, 2015)
SMARD1 is a rare progressive neurological condition seen in infants and caused by an autosomal recessive mutation of the IGHMBP2 gene. The major clinical features include respiratory distress progressing to ventilatory failure, diaphragmatic eventration or paralysis and a progressive distal muscular weakness. Due to the rarity of SMARD1 there is currently little consensus regarding appropriate treatment. To help provide more standardised care for children with this condition we carried out an online survey in April 2012 to investigate clinician's experiences and attitudes regarding treatment of SMARD1. We distributed a survey to 601 Paediatric Consultants via four specialist interest groups (intensivists, neurologists, respiratory and generalists) based in the UK. The survey had eight questions, and posed a series of ethical and clinical dilemmas. In total we received 80 responses (13.3%, n = 80/601) of which 36.3% were Neurologists (n = 29/80), 25% Intensivists (n = 20/80), 6.3% Respiratory (n = 5/80) and 32.4% were General Paediatrics (n = 26/80). When asked about long term treatment, 47.9% would offer extubation followed by palliative care, 14.1% would advocate Long Term Tracheostomy Home Ventilation (LTTHV), and 38% would offer both. Consultants with less than 10 years of experience and those who had not previously cared for a child with SMARD1 were more likely to recommend extubation followed by palliative care. Reasons for treatment: 45.1% (n = 32/71) cited parental wishes, and 35.2% (n = 25/71) cited the expectation that the child might have several years of good quality life. Reasons for not offering children with SMARD1 LTTHV were: unacceptably poor quality of life on LTTHV (54.9%, n = 39/71), the burden of care placed upon carers (42.3%, n = 30/71) and assumed short life expectancy despite LTTHV (35.2%, n = 25/71). Our study shows that a minority of clinicians would advocate LTTHV for children with SMARD1. Motivating factors for decision making was different by speciality.
Keywords: spinal muscular atrophy; respiratory distress syndrome; United Kingdom; muscle; society; human; child; palliative therapy; intensivist; neurologist; extubation; consultation; respiratory distress; clinical feature; diaphragm eventration; gene; mutation; decision making; consensus; autosomal recessive inheritance; medical specialist; muscle weakness; paralysis; pediatrics; long term care; infant; tracheostomy; air conditioning; quality of life; caregiver; life expectancy

Mycobacterium avium complex (MAC) sepsis in an immunocompetent patient isolated from routine blood culture - Khan F.; Naab T.; Geleta M. (American Journal of Clinical Pathology, 2015)
Mycobacterium avium complex (MAC) includes Mycobacterium avium and Mycobacterium intracellulare. Disseminated MAC characteristically involves multiple nodes, spleen, liver, bone marrow, and blood, and is an opportunistic infection almost uniquely associated with patients with advanced AIDS with CD4 lymphocyte count < 50 cells/mm3 and/or high plasma HIV RNA > 100,000 copies/mL. In HIV negative patients without immunosuppression, pulmonary MAC occurs in smokers, cystic fibrosis patients, and elderly women with nodular bronchiectasis. We report a case of a 60-year-old African American HIV-negative man with a past medical history of quadriplegia secondary to C3-C4 spinal cord injury, status post tracheostomy and PEG tube placement, ventilatordependent chronic respiratory failure, presenting to the emergency department for dislodged PEG tube. At admission, the patient was hypertensive with blood pressure of 163/123 mm of Hg and was given one dose of labetalol. Subsequently, he became hypotensive with blood pressure of 60/30 mm of Hg and was treated with IV fluids and pressors. The patient had leukocytosis (31.3 TH/cu mm) with 90% neutrophils, mild anemia (Hb 9.3 g/dL), and thrombocytopenia (77 TH/mm3). Routine peripheral blood cultures were positive for acid-fast bacilli 6 days after inoculation. MAC was identified using DNA probe 3 weeks later. The patient was started on broadspectrum antibiotics (vancomycin and zosyn). The patient continued to have worsening hypotension despite increasing doses of pressors and ultimately died on the fifth day of admission despite aggressive life support. Disseminated MAC is a potentially fatal infection and the most common cause of death in AIDS patients. This case highlights the unusual presentation of MAC in an immunocompetent patient with multiple comorbidities. It is important to screen for MAC irrespective of HIV status so that prompt treatment of this potentially lethal infection can be initiated.
Keywords: hypertensive factor; CD4 antigen; labetalol; antihypertensive agent; antibiotic agent; vancomycin; piperacillin plus tazobactam; DNA; RNA; sepsis; patient; human; blood culture; American; society; pathology; Mycobacterium avium complex; blood pressure; tube; blood; Human immunodeficiency virus; infection; spleen; lymphocyte count; Mycobacterium intracellulare; chronic respiratory failure; opportunistic infection; Mycobacterium avium; leukocytosis; plasma; immunosuppressive treatment; tracheostomy; spinal cord injury; male; acquired immune deficiency syndrome; bone marrow; female; African American; bronchiectasis; aged; cystic fibrosis; hypotension; emergency ward; liver; liquid; neutrophil; anemia; thrombocytopenia; DNA probe; inoculation; smoking; acid fast bacterium; cause of death; AIDS patient; quadriplegia; medical history

The effects of landiolol on pial microvessels during normal state period in rabbits - Hishiyama S.; Nobumasa A.; Ishiyama T.; Matsukawa T. (Journal of Neurosurgical Anesthesiology, 2015)
Background: Landiolol is ultra-short-acting b1-selective blocker and widely used for perioperative tachyarrhythmia. Landiolol has nerve protective functions for spinal cord and brain ischemia in rats. However vascular effect of landiolol has not been investigated. The aim of this study was to evaluate the direct effect of landiolol on cerebral pial arterial diameter change in the normal state rabbits. Methods: Experiments were performed on Japanese white rabbits weighing 2.8-3.6 kg. After obtaining IV access in an ear vein, the animals were anesthetized with pentobarbital sodium (20mg. kg-1 IV and 5mg kg-1 . h-1 CIV). The animals were tracheostomized and their lungs were mechanically ventilated. A closed cranial window was implanted over the cerebral hemisphere. Topical application of 4 increasing concentrations of landiolol (10-10, 10-8, 10-6, and 10-4 mol . L-1) dissolved in artificial cerebrospinal fluid was superfused for 5 minutes each. We measured the diameters of pial vessels before (control) and 5 minutes after the application of each concentration. The window was then flushed with a CSF for 30 minutes before the next concentration was administered. Values are represented as mean ± SD. Cerebral pial vascular changes in diameter from the control were examined via paired ttest. A P value less than 0.05 was considered statistically significant. Results: There were no significant differences in hemodynamic and physiologic variables during the experimental period. When landiolol was superfused at 10-10 mol . L-1, pial arteriolar diameter changed by 14.3±20.5%. While landiolol at 10-6, 10-4 mol . L-1 also dilated pial arterioles, 10-10 mol . L-1 significantly dilated pial arterioles. Conclusions: Landiolol dilated normal pial arterioles in rabbits. Landiolol may be a potent cerebrovascular dilator. Cerebral vasodilation caused by landiolol may partly contribute to nerve protection.
Keywords: landiolol; pentobarbital; microvasculature; Leporidae; human; Japanese (people); society; anesthesia; intensive care; Japanese White (rabbit); arteriole; nerve; cerebrospinal fluid; brain ischemia; lung; statistical significance; vein; spinal cord; ear; hemisphere; topical drug administration; artery diameter; protection; vasodilatation; rat; tachycardia

Open repair of ruptured descending thoracic and thoracoabdominal aortic aneurysms Read at the 95th Annual Meeting of the American Association for Thoracic Surgery, Seattle, Washington, April 25-29, 2015. - Gaudino M.; Lau C.; Munjal M.; Girardi L.N. (Journal of Thoracic and Cardiovascular Surgery, 2015)
Objective To evaluate the results of the open repair of ruptured thoracic and thoracoabdominal aortic aneurysms. Methods From January 1997, a total of 100 consecutive open repairs of ruptured thoracic or thoracoabdominal aortic aneurysms were performed (43 thoracic and 57 thoracoabdominal). These patients were compared with contemporary cases that underwent repair of corresponding intact aneurysms. Propensity matching analysis was used to neutralize the differences in baseline characteristics. Results Patients with ruptured aneurysm had a significantly worse baseline clinical profile. The surgical strategy adopted was similar in intact and ruptured aneurysms, with the exception of lower use of spinal drainage, intercostal reimplantation, and associated procedures in those with rupture (P <.001 for all comparisons). In the unmatched population, in-hospital mortality was 14% in the rupture group, and 4.2% in the intact group (P =.01). The incidence of postoperative myocardial infarction, need for tracheostomy, and need for dialysis was 3%, 19%, and 11% in the rupture, and 0.8%, 5.7%, and 4.2% in the intact series (P <.01 for all variables). Five-year survival was 47.5% for the rupture, and 59.5% for the intact series (P <.001). In the matched population, no differences in postoperative and long-term outcome were found between the rupture and intact cases. Logistic regression analysis showed that female gender, urgent/emergent operation, and preoperative hemodialysis, but not ruptured aneurysm, were predictive of in-hospital major adverse events. Conclusions Open repair of ruptured thoracic and thoracoabdominal aortic aneurysms can be performed with a gratifying rate of success. For patients with similar preoperative comorbidities, postoperative survival is not affected by the presence of a ruptured aneurysm.
Keywords: aged; aneurysm rupture; artery diameter; cardiopulmonary bypass; cerebrovascular accident; conference paper; controlled study; deep hypothermic circulatory arrest; endovascular aneurysm repair; female; follow up; heart arrhythmia; heart infarction; human; kidney failure; long term survival; major clinical study; male; multiple organ failure; postoperative complication; postoperative hemorrhage; priority journal; recurrent laryngeal nerve palsy; reimplantation; ruptured descending thoracic aortic aneurysm; sepsis; spinal cord injury; surgical mortality; thoracic aorta aneurysm; thoracoabdominal aorta aneurysm; thoracotomy; tracheostomy; treatment outcome

American Spinal Injury Association Impairment Scale Predicts the Need for Tracheostomy after Cervical Spinal Cord Injury - Childs B.R.; Moore T.A.; Como J.J.; Vallier H.A. (Spine, 2015)
Study design. Retrospective review. Objective. The objective of this study was to evaluate the ability of the American Spinal Injury Association (ASIA) Impairment Scale and neurological level of injury to predict the need for mechanical ventilation as well as tracheostomy. Summary of Background Data. High-level cervical spinal cord injuries, high Injury Severity Score, and low Glasgow Coma Scale have been shown to predict tracheostomy. Methods. A total of 383 patients with fractures, dislocations, or ligamentous injury of the cervical spine were included in the study. Charts were reviewed to determine demographics, Injury Severity Score, Glasgow Coma Scale, presence and severity of chest injuries, length of hospital stay, intensive care unit stay, mechanical ventilation time, and mortality. Results. Fifty-nine patients (15.4%) underwent tracheostomy. An ASIA Impairment Scale of A had a specificity of 98.8% and sensitivity of 32.2% for predicting the need for tracheostomy. This yielded a 1.2% false-positive rate. The ASIA Impairment Scale remained the most significant predictor after regression for Injury Severity Score, Glasgow Coma Scale, and Chest Abbreviated Injury Scale. Neurological level of injury was not a significant predictor of tracheostomy. Conclusion. An ASIA Impairment Scale of A at any level of injury is a specific predictor of the need for tracheostomy with a low false-positive rate. Given the relatively low risk of early tracheostomy and the potential benefits, an ASIA Impairment Scale of A would be a sensible early criterion to determine the need for tracheostomy. Level of Evidence: 3.
Keywords: adult; American Spinal Injury Association impairment scale; artificial ventilation; cervical spinal cord injury; cervical spine dislocation; cervical spine fracture; cervical spine injury; Chest Abbreviated Injury Scale; falling; false positive result; female; Glasgow coma scale; hospitalization; human; injury scale; injury severity; major clinical study; male; middle aged; mortality; priority journal; retrospective study; review; sensitivity and specificity; skull fracture; subarachnoid hemorrhage; subdural hematoma; thorax injury; tracheostomy; traffic accident; traumatic brain injury

Developing a comprehensive assessment system: A case report - Sliwa J.A.; Moore J. (PM and R, 2015)
Case Description: A 23-year-old man admitted for rehabilitation following high speed motor vehicle accident resulting in C3eC4 fracture and C4 ASIA B spinal cord injury. The patient required a posterior spinal reduction and fixation with HALO placement, prolonged ventilation with tracheostomy and feeding tube placement. During rehabilitation the patient participated fully and tolerated therapy well. At the time of discharge the patient had been decannulated, his spasticity was controlled with medication, feeding tube was removed, bowels were regulated and an indwelling catheter was used for bladder management. The patient demonstrated improved breath support, cough strength and vocal intensity. He was independent in power wheelchair mobility with sip and puff controls and the use of a mouthstick for iPad use and TV controls. The caregiver (patient's father) was independent in providing and the patient independent in directing all self care, transfers, skin care, pressure reliefs, positioning and stretching. The patient was discharged home with his father. Despite attainment of rehabilitation goals and safe discharge home the total FIM gain was only 4 points. Setting: Inpatient rehabilitation facility. Results or Clinical Course: FIM does not capture progress made by this patient and for many other patients during rehabilitation. Discussion: To address this issue we are developing an assessment tool that can reliably capture and reflect progress in a comprehensive manner. Multi disciplinary expert clinician panels first identified 60 new clinically relevant domains and 40 assessment tools suitable for patients with stroke, TBI, SCI, neurologic and general rehabilitation needs. We are now pilot testing these tools on inpatient units for clinical relevance and operational feasibility. Based on pilot data a final battery of assessment tools will be fully implemented. We will then use item response theory (IRT) models to reduce assessment burden without decreasing measurement precision. Conclusion: This project will identify and evaluate a set of standardized assessment tools that minimizes clinician and patient assessment burden while assessing areas of function in which patient progress is not adequately documented.
Keywords: human; case report; American; physical medicine; rehabilitation; patient; male; hospital patient; feeding apparatus; father; spinal cord injury; fracture; bladder; velocity; cerebrovascular accident; disease course; measurement precision; therapy; spasticity; intestine; indwelling catheter; tracheostomy; breathing; coughing; wheelchair; traffic accident; air conditioning; skin care; self care; stretching; caregiver; model; patient assessment; drug therapy

Development of a hybrid tracheostomy management skills workshop for physical medicine and rehabilitation residents: A case study - Pineda C. (PM and R, 2015)
Case Description: To describe the development of a hybrid formative instruction and evaluation of tracheostomy management skills focused on responding to accidental decannulation in a supervised simulated setting. Program Description: 18 PM&R Residents participated in a hybrid formative workshop composed of a didactic session and hands-on simulation. The didactic session led by an ENT specialist focused on: 1) anatomy and physiology of the upper respiratory tract; 2) tracheostomy tube types; 3) management of accidental decannulation; 4) complications of tracheostomy re-insertion; 5) post-tracheostomy reinsertion assessment ; 6) documentation of hand-off care and 7) tracheostomy practice guidelines per hospital standards. The simulation session focused on: 1) rapid assessment and rescue of a patient with a dislodged tracheostomy using a mannequin simulator and 2) basic airway skills training. A checklist was used to assess competencies. Setting: Free-standing academic rehabilitation hospital. Results or Clinical Course: Instructional techniques were experiential and learner-centered. Instructors assessed resident performance through return demonstrations structured to apply competencies. Participants received additional instruction and practice as needed. Resident post-activity evaluations using a 5-point Likert scale: overall resident evaluation (62%=excellent, 37%=good); a new skill/ patient management approach was learned (50%=strongly agree; 50%=agree). Discussion: Tracheostomy management is an essential skill for the practicing physiatrist especially those involved in the care of patients with brain injury, spinal cord injury and stroke. PM&R residents are likely to encounter clinical situations that require re-insertion of tracheostomy tubes in the acute rehabilitation setting. Conclusion: Accidental tracheostomy decannulation is a condition that may be encountered in an acute rehabilitation setting. It is imperative that rehabilitation care providers, such as PM&R residents, who care for patients with tracheostomies be knowledgeable on how to assess and manage this medical emergency to ensure patient safety.
Keywords: rehabilitation; physical medicine; skill; workshop; case study; American; tracheostomy; human; patient; tracheostomy tube; spinal cord injury; hospital; simulation; disease course; airway; upper respiratory tract; simulator; patient safety; practice guideline; physiology; checklist; Likert scale; cerebrovascular accident; documentation; medical specialist; brain injury; rehabilitation care; emergency; patient care; otorhinolaryngology

Airway complications in traumatic lower cervical spinal cord injury: A retrospective study - Liebscher T.; Niedeggen A.; Estel B.; Seidl R.O. (Journal of Spinal Cord Medicine, 2015)
Objective: To investigate risk factors for pneumonia in patients with traumatic lower cervical spinal cord injury. Design: Observational study, retrospective study. Setting: Spinal cord unit in a maximum care hospital. Methods: Thirty-seven patients with acute isolated traumatic spinal cord injury at levels C4-C8 and complete motor function injury (AIS A, B) treated from 2004 to 2010 met the criteria for inclusion in our retrospective analysis. The following parameters were considered: ventilation-specific parameters, re-intubation, creation of a tracheostomy, pneumonia, antibiotic treatment, and length of intensive care unit (ICU) stay and total hospitalization. Results: Among the patients, 81% had primary invasive ventilation. In 78% of cases a tracheostomy was created; 3% of these cases were discharged with invasive ventilation and 28% with a tracheostomy without ventilation. Pneumonia according to Centers for Disease Control criteria occurred in 51% of cases within 21 ± 32 days of injury, and in 3% at a later date. The number of pre-existing conditions was significantly associated with pneumonia. Length of ICU stay was 25 ± 34 days, and average total hospital duration was 230 ± 144 days. Significant factors affecting the duration of ventilation were the number of pre-existing conditions and tetraplegia-specific complications. Conclusions: Our results confirm that patients with traumatic lower cervical spinal cord injuries defined by lesion level and AIS constitute a homogeneous group. This group is characterized by a high rate of pneumonia during the first 4 weeks after injury. The number of pre-existing general conditions and spinal injury-specific comorbidities are the only risk factors identified for the development of pneumonia and/or duration of ventilation.
Keywords: adolescent; adult; aged; antibiotic therapy; article; artificial ventilation; cervical spinal cord injury; clinical article; comorbidity; controlled study; extubation; female; hospital discharge; hospital subdivisions and components; hospitalization; human; intensive care unit; length of stay; male; motor dysfunction; pneumonia; quadriplegia; respiratory tract disease; retrospective study; risk factor; tracheostomy

A clinical study of laryngotracheal separation in spinal muscular atrophy type i patients - Fujioka T.; Nakano K.; Maniwa S. (No To Hattatsu, 2015)
Artificial respiration by tracheostomy is necessary for long-term survival in spinal muscular atrophy (SMA) Type I patients. There are two types of tracheostomy: i) simple tracheostomy and ii) tracheostomy plus aspiration prevention surgery, including laryngotracheal separation. We experienced three cases of SMA type I patients that had good outcomes after undergoing laryngotracheal separation. The patients' ages at onset were 14 days, two months and one and a half months. Laryngotracheal separation was performed at five months, seven months, and 15 years and five months respectively, and the times from diagnosis to surgery were a month, three months and 15 years. The aspiration pneumonia disappeared in all three cases. This study suggests that laryngotracheal separation is an effective surgery to prevent aspiration in SMA type I cases, and also contributes to the improved quality of life of patients and their families.
Keywords: article; artificial ventilation; aspiration pneumonia; human; laryngotracheal separation; long term survival; outcome assessment; quality of life; spinal muscular atrophy; surgical technique; trachea surgery; tracheostomy

Cough augmentation techniques for extubation and weaning critically ill patients from mechanical ventilation - Rose L.; Adhikari N.K.J.; Leasa D.; Fergusson D.A.; Mckim D. (Cochrane Database of Systematic Reviews, 2015)
This is the protocol for a review and there is no abstract. The objectives are as follows: The primary objective of this systematic review is to determine extubation success rates of cough augmentation techniques compared to no cough augmentation for critically ill adult and paediatric patients with acute respiratory failure admitted to a high intensity care setting capable of providing care to patients requiring invasive mechanical ventilation (such as ICU, specialized weaning centre, respiratory intermediate care unit, or high dependency unit). Secondary objectives will be to determine the following. The effect of cough augmentation techniques on reintubation; weaning success; duration of mechanical ventilation; duration of weaning; high intensity care setting and hospital length of stay; high intensity care setting and hospital mortality, as well as mortality after hospital discharge; pneumonia; tracheostomy; and tracheostomy decannulation rates. The harms associated with use of cough augmentation techniques when applied via an artificial airway (or non-invasive mask once extubated/decannulated) such as haemodynamic compromise, arrhythmias, pneumothorax, haemoptysis, and mucus plugging requiring change of the endotracheal or tracheostomy tube. The type of patients (such as those with neuromuscular disorders or weakness and spinal cord injury) for whom these techniques are most likely to be efficacious.
Keywords: endotracheal tube; tracheostomy tube; acute respiratory failure; article; artificial ventilation; coughing; critically ill patient; extubation; heart arrhythmia; hemodynamics; hemoptysis; hospital discharge; hospital mortality; hospitalization; human; length of stay; patient care; pneumonia; pneumothorax; priority journal; risk assessment; systematic review; tracheostomy; weaning

Strategy by stages for preventing respiratory complications of acute cervical spinal cord injury - Lai B.; Wu J.; Gao Z.; Lian W. (Zhongguo gu shang = China journal of orthopaedics and traumatology, 2015)
OBJECTIVE: To investigate clinical effects of strategy by stages for preventing respiratory complications of patients with acute cervical spinal cord injury (ACSCI). METHODS: From September 2009 to May 2013,the clinical data of 91 patients with ACSCI underwent surgery were retrospectively analyzed. Among the patients, 42 patients were divided into in-stages group, including 30 males and 12 females with an average age of 50 years old (ranged 28 to 76) which were treated with strategy by stages for preventing respiratory complications; others 49 patients which were not treated with the strategy regarded as control group, including 38 males and 11 females with an average age of 47 years old (ranged 30 to 77). All of them had definite history of trauma, and were admitted to orthopaedics within 48 h after trauma. In in-stages group, respiratory muscle strength training, high-dose ambroxol using and other treatment were performed to prevent respiratory complilcations according to preoperative, intraoperative and postoperative stage. While in control group, there were no systematic and effective measures utilized. Chi-square test was used to evaluate the difference for respiratory complications rate, the rate of tracheostomy or intubation and mortality caused by the respiratory complications between two groups. RESULTS: Ten patients developed with respiratory complications in in-stages group (7 patients with pneumonia, 1 with atelectasis and 2 with respiratory failure), among which 3 patients underwent tracheostomy or intubation. In control group, 24 patients developed with respiratory complilcations (15 with pneumonia,3 with atelectasis and 6 with respiratory failure), among which 11 patients underwent tracheostomy or intubation. There was significant difference between two groups (χ2 = 6.12, 4.07; P = 0.013, 0.044). Five patients died because of respiratory complications, one case were in in-stages group and 4 in control group. There was significant difference between two groups (χ2 = l.39, P = 0.238). CONCLUSION: The strategy by stages is an effective method for preventing respiratory complications of ACSCI and can reduce the respiratory complications rate and improve the prognosis of respiratory complications.
Keywords: acute disease; adult; aged; cervical spinal cord; complication; female; human; injuries; injuries; male; middle aged; pneumonia; atelectasis; respiratory failure; retrospective study; spinal cord injury; tracheostomy

Oesophageal perforation following anterior cervical spine fixation: Management and results - Daddi N.; Perrone O.; Lugaresi M.; Borghesi I.; Belloni G.P.; Uneddu M.; Mattioli S. (Interactive Cardiovascular and Thoracic Surgery, 2015)
Objectives: Delayed pharyngooesophageal perforation is a rare and fairly unknown complication of anterior cervical spine fixation (ACDF). A survey of the Cervical Spine Research Society reported an overall perforation rate of 0.25%. The optimal treatment remains debatable. A retrospective review of our personal case series was conducted. Methods: Thirteen cases of oesophageal perforation related to ACDF procedure were observed in the period between 2004 and 2014. ACDF had been carried out at C4-C7 level. Common symptoms were facial, neck swelling or persistent pain, dysphagia hyperpyrexia, clinical evidence of abscess or cutaneous salivary fistula. The mean delay in symptoms acme was 24 days (range 12 days-9 years). Computed tomography scan with or without ± gastrografin was the preferred preoperative strategy. Test to investigate the neck soft tissues and the fixation devices status. Results: All patients had tracheostomy. The primary treatment was lateral neck drainage and percutaneous gastrostomy and broad-spectrum antibiotics. Three patients with minor infection and small fistulas not adjacent to fixation devices were managed conservatively. Ten patients (in 2 cases perforation was intraoperative, in 8 cases it was due to screw decubitus) were surgically managed: 1) adequate preoperative spine stabilization, 2) lateral cervicotomy, 3) wide pharynx and proximal oesophagus mobilization, 4) clearing of the mucosal tear margins, 4) double-layer suture of the fistula, 5) muscular flap reinforcement (with sternocleidomastoid muscle in 8 cases, with pectoralis major muscle in 2. Mean length of stay was 24.1 days (range 10-61). No operative mortality occurred. Major comorbidities were chronic respiratory failure (n = 7), infective status (n = 6) and arterial hypertension (n = 2). Conclusions: Neck drainage to protect tracheostomy and treat infection are mandatory. Enteral nutrition promotes a favourable outcome. For large fistulas, spine stabilization, screw device removal and repair with blanket muscular flap are recommended.
Keywords: diatrizoate; antibiotic agent; esophagus perforation; cervical spine; spine stabilization; European; thorax surgery; fistula; neck; human; perforation; patient; infection; tracheostomy; orthopedic fixation device; enteric feeding; soft tissue; chronic respiratory failure; computer assisted tomography; surgical mortality; pectoralis major muscle; case study; reinforcement; lacrimal fluid; abscess; mobilization; esophagus; hyperpyrexia; dysphagia; pharynx; suture; decubitus; sternocleidomastoid muscle; length of stay; pain; hypertension; gastrostomy; device removal; swelling; procedures; society

Respiratory care in neuromuscular disease - Khurshid I. (Neurorehabilitation and Neural Repair, 2015)
Respiratory muscles including diaphragm constitute the ventilatory pump on which the act of breathing depends. In most neuromuscular disorders, there is simultaneous respiratory muscle weakness of similar or even greater extent than to other skeletal muscles. Causes of neuromuscular weakness: cerebral cortex-stroke, seizure disorders, degeneration, neoplasm; brainstem/basal ganglia-stroke, postpolio syndrome, progressive bulbar palsy, Parkinson's disease, multiple sclerosis; spinal cord-trauma, demyelinating disease, disk compression, multiple sclerosis; anterior horn cells-motor neuron disease, postpolio syndrome, amyotrophic lateral; neuromuscular junction myasthenia gravis, Lambert-Eaton syndrome, drugs (steroids, AChE sclerosis, spinal muscular atrophy, primary lateral sclerosis inhibitors); myopathies muscular dystrophies, glycogen storage disease, polymyositis, dernatomyositis, other myopathies. Major respirator problems in patients with neuromuscular disease: pneumonia-aspiration; diurnal ventilation failure; sleep disordered breathing. Aspiration pneumonia-Caused by oropharyngeal muscle weakness, gastric reflux, and cough insufficiency. Aspiration risk factors: Head-end elevation <30°, use of sedatives/opioids, low Glasgow Coma Scale score, that is, <9, gastric residual volume (>200 cm3), gastroesophageal reflux/vomiting. Diurnal ventilatory failure: Respiratory symptoms are often initially absent or minimal because of the large reserve of the respiratory system. Respiratory muscle involvement may also be masked because patients with skeletal muscle weakness spontaneously decrease their overall activity level, thereby reducing the daily physiologic challenge faced by the respiratory system. For all these reasons, it is not unusual for respiratory muscle weakness to go undetected until overt respiratory failure is precipitated by an acute episode of pulmonary aspiration or infection. Evaluation. Symptoms and signs-unexplained dyspnea on exertion, orthopnea, recurrent cough, choking; objective tests; nocturnal pulse oximetry; forced vital capacity (FVC); sniff nasal inspiratory pressure (SNIP); arterial blood gases. Treatment. Noninvasive positive pressure ventilation (NIPPV)-indications: acute respiratory failure secondary to chest infection, perioperative support/ peg tube placement, hypercapnic chronic respiratory failure, sleep-disordered breathing, during pregnancy, palliate symptoms. Long-term ventilation-tracheostomy care (TRACHE): tape-keep tube position secured; resuscitation- know the resuscitation process; airway clear-use correct suction technique; care of the site-care of stoma and neck; humidity-essential to keep tube clear; emergency kit-have the kit ready. Obstructive sleep apnea (OSA) in patients with neuromuscular disorders: Higher incidence of OSA in patients with neuromuscular disease; need high index of suspicion for OSA in these patients; polysomnography (PSG) is the test of choice for diagnosis of OSA; NIPPV (CPAP/bilevel) is recommended if PSG is positive for OSA.
Keywords: acetylcholinesterase; endogenous compound; steroid; acute respiratory failure; airway obstruction; anterior horn cell; apnea monitoring; arterial blood; aspiration pneumonia; basal ganglion; blood gas; brain cortex; brain stem; breathing muscle; bulbar paralysis; cerebrovascular accident; chest infection; chronic respiratory failure; clinical study; coughing; diagnosis; diaphragm; dyspnea; Eaton Lambert syndrome; emergency; epilepsy; female; forced vital capacity; gastroesophageal reflux; Glasgow coma scale; glycogen storage disease; head; human; humidity; hypercapnia; hypoventilation; multiple sclerosis; muscular dystrophy; myasthenia gravis; neck; neoplasm; neuromuscular junction; Parkinson disease; polymyositis; polysomnography; postpoliomyelitis syndrome; pregnancy; primary lateral sclerosis; pulse oximetry; residual volume; respiratory care; resuscitation; risk factor; sleep disordered breathing; spinal cord injury; stoma; stomach; suction; surgery; tracheostomy; ventilator; vomiting

Purely endoscopic endonasal surgery of the craniovertebral junction: A systematic review - Fang C.H.; Friedman R.; Schild S.D.; Goldstein I.M.; Baredes S.; Liu J.K.; Eloy J.A. (International Forum of Allergy and Rhinology, 2015)
Background: Endoscopic endonasal surgery (EES) is a relatively novel approach to the craniovertebral junction (CVJ). The purpose of this analysis is to determine the surgical outcomes of patients who undergo purely EES of the CVJ. Methods: A search for articles related to EES of the CVJ was performed using the MEDLINE/PubMed database. A bibliographic search was done for additional articles. Demographics, presenting symptoms, imaging findings, complications, follow-up, and patient outcomes were analyzed. Results: Eighty-five patients from 30 articles were included. The mean patient age was 47.9 ± 24.8 years (range, 3 to 96 years), with 44.7% being male. The most common presenting symptom was myelopathy (n = 64, 75.3%). The most common indications for surgery were brainstem compression secondary to basilar invagination (n = 41, 48.2%) and odontoid pannus (n = 20, 23.5%). Odontoidectomy was performed in 97.6% of cases. Intraoperative complications occurred in 16 patients (18.8%) and postoperative complications occurred in 18 patients (21.2%). Six patients developed postoperative respiratory failure necessitating a tracheostomy. Neurologic improvement was seen in 89.4% of patients at a mean follow-up of 22.2 months. Conclusion: Our analysis found that EES of the CVJ results in a high rate of neurologic improvement with acceptable complication rates. Given its minimally invasive nature and high success rate, this approach appears to be a reasonable alternative to the traditional transoral approach in select cases. This study represents the largest pooled sample size of EES of the CVJ to date. Increasing use of the endoscopic endonasal approach will allow for further studies with greater statistical power.
Keywords: Arnold Chiari malformation; bleeding; cause of death; craniovertebral junction; craniovertebral junction compression; demography; disease association; dysphagia; endoscopic endonasal surgery; epistaxis; follow up; human; image analysis; liquorrhea; meningitis; neck pain; nerve compression; nuclear magnetic resonance imaging; odontoid process fracture; outcome assessment; palatopharyngeal incompetence; peroperative complication; postoperative complication; priority journal; respiratory failure; review; rheumatoid arthritis; spinal cord disease; spine; systematic review; wound infection

Difficult airway response team: A novel quality improvement program for managing hospital-wide airway emergencies - Mark L.J.; Herzer K.R.; Cover R.; Pandian V.; Bhatti N.I.; Berkow L.C.; Haut E.R.; Hillel A.T.; Miller C.R.; Feller-Kopman D.J.; Schiavi A.J.; Xie Y.J.; et al. (Anesthesia and Analgesia, 2015)
BACKGROUND: Difficult airway cases can quickly become emergencies, increasing the risk of life-threatening complications or death. Emergency airway management outside the operating room is particularly challenging. METHODS: We developed a quality improvement program - the Difficult Airway Response Team (DART) - to improve emergency airway management outside the operating room. DART was implemented by a team of anesthesiologists, otolaryngologists, trauma surgeons, emergency medicine physicians, and risk managers in 2005 at The Johns Hopkins Hospital in Baltimore, Maryland. The DART program had 3 core components: operations, safety, and education. The operations component focused on developing a multidisciplinary difficult airway response team, standardizing the emergency response process, and deploying difficult airway equipment carts throughout the hospital. The safety component focused on real-time monitoring of DART activations and learning from past DART events to continuously improve system-level performance. This objective entailed monitoring the paging system, reporting difficult airway events and DART activations to a Web-based registry, and using in situ simulations to identify and mitigate defects in the emergency airway management process. The educational component included development of a multispecialty difficult airway curriculum encompassing case-based lectures, simulation, and team building/communication to ensure consistency of care. Educational materials were also developed for non-DART staff and patients to inform them about the needs of patients with difficult airways and ensure continuity of care with other providers after discharge. RESULTS: Between July 2008 and June 2013, DART managed 360 adult difficult airway events comprising 8% of all code activations. Predisposing patient factors included body mass index >40, history of head and neck tumor, prior difficult intubation, cervical spine injury, airway edema, airway bleeding, and previous or current tracheostomy. Twenty-three patients (6%) required emergent surgical airways. Sixty-two patients (17%) were stabilized and transported to the operating room for definitive airway management. There were no airway management-related deaths, sentinel events, or malpractice claims in adult patients managed by DART. Five in situ simulations conducted in the first program year improved DART's teamwork, communication, and response times and increased the functionality of the difficult airway carts. Over the 5-year period, we conducted 18 airway courses, through which >200 providers were trained. CONCLUSIONS: DART is a comprehensive program for improving difficult airway management. Future studies will examine the comparative effectiveness of the DART program and evaluate how DART has impacted patient outcomes, operational efficiency, and costs of care.
Keywords: local anesthetic agent; adult; article; body mass; cervical spine injury; edema; emergency care; emergency physician; female; head and neck tumor; human; major clinical study; male; operating room; patient safety; priority journal; rapid response team; respiration control; reaction time; simulation; total quality management

Evolution in the Management of Aberrant Subclavian Arteries and Related Kommerell Diverticulum - Van Bogerijen G.H.W.; Patel H.J.; Eliason J.L.; Criado E.; Williams D.M.; Knepper J.; Yang B.; Deeb G.M. (Annals of Thoracic Surgery, 2015)
Background Various options have been described to treat aberrant subclavian arteries and associated Kommerell diverticulum. We describe our experience with the management of this entity over a 15-year period. Methods Twenty-two patients underwent repair of aberrant subclavian arteries and associated Kommerell diverticulum. Indications for intervention included a large Kommerell diverticulum (n = 18), dysphagia lusoria (n = 12), rupture (n = 4), type B aortic dissection (n = 4), thoracic aortic aneurysm (n = 2), and coarctation (n = 1). Patients were treated with either open surgery (n = 9) or an endovascular approach (n = 13). For the open surgical patients, hypothermic circulatory arrest (n = 7) or left heart bypass (n = 2) was used. For those patients undergoing an endovascular approach (n = 13), carotid to subclavian arterial bypasses were performed preoperatively in 11 patients and intraoperatively in 2 patients. Bilateral revascularization was more frequently performed with endovascular repair compared with open surgery (69% vs 22%, p = 0.01). Results Early outcomes included in-hospital mortality (n = 1), stroke (n = 1), and permanent spinal cord ischemia (n = 1) after endovascular approaches, and renal failure requiring dialysis (n = 1) and need for tracheostomy (n = 1) after open repair. The frequency of endovascular repair increased after the commercialization of thoracic endovascular aortic repair in 2005 from 33% to 63%. Four patients developed type I (n = 1) or type II (n = 3) endoleaks, of which 1 required reintervention. Median hospital stay was 7 days (interquartile range 4 to 17). Five-year survival was 81.8%. No late aortic ruptures occurred and 3 patients required late reintervention; 1 after an open and 2 after an endovascular approach. Dysphagia lusoria was relieved in all patients except for 1 in the open repair and 1 in the endovascular group. Conclusions Aberrant subclavian arteries and associated Kommerell diverticulum can be treated with acceptable rates of mortality and morbidity. The evolution toward an endovascular approach did not appear to affect late outcomes, suggesting that the choice of treatment should be based on patient-specific anatomy and associated comorbidities.
Keywords: aberrant subclavian artery; adult; aged; aortic coarctation; aortic dissection; aortic rupture; article; cardiovascular mortality; cerebrovascular accident; clinical article; congenital blood vessel malformation; diverticulosis; dysphagia; endoleak; endovascular aneurysm repair; endovascular surgery; extracorporeal circulation; female; frequency; hospitalization; human; kidney failure; male; middle aged; morbidity; open heart surgery; postoperative complication; priority journal; retrospective study; spinal cord ischemia; subclavian artery; survival; thoracic aorta aneurysm; treatment outcome

The influence factors and meanings of tracheotomy after cervical spinal cord injury - Zhang P.; Zhang X. (Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology, head, and neck surgery, 2015)
Cervical spinal cord injury is a common and serious disease in clinic, and tracheotomy combined with mechanical ventilation is an effective way to prevent respiratory complications. Although tracheotomy is used widely, there are not unified indications of tracheotomy after cervical spinal cord injury in the practical application. At the same time, the advantages and disadvantages of the application of tracheotomy in patients with cervical spinal cord injury are still on dispute. Based on the recent literature, we summarize the influence factors and meanings of tracheotomy after cervical spinal cord injury.
Keywords: artificial ventilation; cervical spinal cord; human; injuries; neck injury; spinal cord injury; tracheotomy

Caesarean section in a parturient with preexisting Transverse myelitis: An anaesthetist's dilemma - Iyer S.J. (Acta Anaesthesiologica Scandinavica, 2015)
Introduction: Transverse myelitis is an inflammatory, spinal cord disorder causing motor, sensory and autonomic dysfunction. Pregnancy in patients with pre-existing transverse myelitis is extremely uncommon. There are several anaesthetic challenges to consider when these patients present for surgery. These include autonomic dysreflexia, hyperkalemia following use of succinylcholine, poor respiratory reserve and possible diaphragmatic paralysis in patients with high lesions. In addition, there is potential for both general and neuraxial anaesthesia to aggravate symptoms of pre-existing transverse myelitis. Case description: We discuss the management of a 35-year-old Malay primigravida presenting for elective Caesarean section at term pregnancy. She was diagnosed with transverse myelitis 20 years ago, associated with tetraplegia and neurogenic bladder. Cervical spine MRI revealed a high lesion at the C5-C6 interval. She also had a previous history of tracheostomy secondary to prolonged ventilation at time of her diagnosis. Given these considerations and the concern of autonomic dysreflexia, decision was made to proceed with spinal anaesthesia instead of general anaesthesia. Intra-operative course was uneventful with stable hemodynamics and delivery of a healthy newborn. Post-operatively, patient had delayed recovery of both sensory and motor components, taking 30 h to return to baseline levels, but she was subsequently well and discharged home. Discussion: We discuss the merits of neuraxial anaesthesia vs general anaesthesia in this rare group of patients, for who there is limited evidence in literature for management. Treatment should be should be individualised, along with multi-disciplinary approach and careful planning for safe provision of anaesthesia in these mothers.
Keywords: suxamethonium; adult; adverse drug reaction; anesthesist; autonomic dysreflexia; case report; cervical spine; cesarean section; clinical article; conference abstract; diagnosis; diaphragm paralysis; female; general anesthesia; hemodynamics; human; hyperkalemia; mother; neurogenic bladder; newborn; nuclear magnetic resonance imaging; primigravida; quadriplegia; remission; sensory dysfunction; side effect; spinal anesthesia; spinal cord disease; surgery; tracheostomy

A rare case of medulloblastoma coexisting with multiple spinal intramedullary tumors - Rege S.; Gupta R.; Awasthi R. (Pediatric Neurosurgery, 2015)
Medulloblastoma is the most common primary central nervous system tumor of childhood. Medulloblastoma can metastasize along the neuraxis and to extraneural locations, but multiple intramedullary spinal metastases are very rare. The usual presenting clinical features are related to posterior fossa syndrome and/or hydrocephalus. We describe a unique case of medulloblastoma with multiple intramedullary lesions at the D-7 and D-12 levels.
Keywords: antibiotic agent; antibiotic therapy; article; ataxia; backache; bradycardia; brain ventricle peritoneum shunt; breathing exercise; cancer surgery; case report; cause of death; chest infection; child; childhood cancer; contrast enhancement; craniectomy; cytoreductive surgery; deterioration; eye examination; fever; headache; histopathology; human; hydrocephalus; intracranial pressure; leg pain; male; medical history; medulloblastoma; multiple cancer; nuclear magnetic resonance imaging; papilledema; pediatric surgery; pneumonia; posterior fossa; postoperative complication; postoperative infection; priority journal; recurrent disease; school child; sitting; spinal cord tumor; spinal intramedullary tumor; supine position; thorax radiography; tracheostomy; unsteady gait; ventriculostomy catheter; vomiting; walking

Respiratory dysfunction in multiple sclerosis - Tzelepis G.E.; McCool F.D. (Respiratory Medicine, 2015)
Respiratory dysfunction frequently occurs in patients with advanced multiple sclerosis (MS), and may manifest as acute or chronic respiratory failure, disordered control of breathing, respiratory muscle weakness, sleep disordered breathing, or neurogenic pulmonary edema. The underlying pathophysiology is related to demyelinating plaques involving the brain stem or spinal cord. Respiratory complications such as aspiration, lung infections and respiratory failure are typically seen in patients with long-standing MS. Acute respiratory failure is uncommon and due to newly appearing demyelinating plaques extensively involving areas of the brain stem or spinal cord. Early recognition of MS patients at risk for respiratory complications allows for the timely implementation of care and measures to decrease disease associated morbidity and mortality.
Keywords: methylprednisolone; steroid; acute respiratory failure; anamnesis; apnea; arm weakness; artificial ventilation; aspiration pneumonia; atelectasis; brain stem; breathing disorder; breathing exercise; breathing muscle; breathing pattern; bulbar paralysis; chronic respiratory failure; cognitive defect; coughing; daytime somnolence; exercise; Expanded Disability Status Scale; fatigue; forced vital capacity; human; hypoventilation; immobility; insomnia; libido disorder; limited mobility; lung edema; lung function; lung function test; lung infection; lung minute volume; lung ventilation; mood change; motor dysfunction; multiple sclerosis; muscle strength; muscle training; muscle weakness; noninvasive ventilation; pathophysiology; physical examination; plasmapheresis; polysomnography; priority journal; quadriplegia; residual volume; respiration center; respiration control; respiratory arrest; respiratory failure; restlessness; review; risk assessment; sleep disordered breathing; spastic paraplegia; spinal cord; standing; supine position; thorax pressure; tidal volume; total lung capacity; tracheostomy; vital capacity

Diffuse leptomeningeal carcinomatosis mimicking brain death - Kung N.H.; Dhar R.; Keyrouz S.G. (Journal of the Neurological Sciences, 2015)
Keywords: alkaline phosphatase; antineoplastic agent; technetium 99m; adult; alkaline phosphatase blood level; apnea; areflexia; article; bone metastasis; bone pain; brain death; breast carcinoma; cancer chemotherapy; cancer diagnosis; carcinomatous meningitis; case report; cauda equina; cerebrospinal fluid analysis; cerebrospinal fluid cytology; lack of drug effect; electrodiagnosis; electroencephalography; epileptic discharge; facial nerve paralysis; female; hospital admission; hospital readmission; human; human cell; hypercalcemia; hypercapnia; intensive care unit; limb weakness; mental deterioration; metabolic acidosis; metastatic breast carcinoma; middle aged; neurologic examination; nuclear magnetic resonance imaging; ophthalmoplegia; paraplegia; pressure support ventilation; priority journal; radiculopathy; respiratory failure; spinal cord compression; systemic therapy; tracheostomy; uremia

Spinal muscular atrophy: Review of clinical characteristics and treatment in the last 25 years - Calvo Medina R.; Madrid Rodríguez A.; Peláez Cantero M.J.; Martínez-Martínez P.L.; Ramos Fernández J.M.; Moreno Medinilla E.; Mora Ramírez M.D.; Martínez Antón J.; Urda Cardona A. (European Journal of Paediatric Neurology, 2015)
Objective: Spinal muscular atrophy (SMA) include a group of disorders that produce disabilities in all patients. Our aim is to review the epidemiological and clinical characteristics, genetic distribution and to analyze the care and development of the SMA in our area. Methods: Retrospective descriptive study of patients diagnosed in our hospital during the past 25 years (1987-2013), with clinical and neurophysiological diagnosis of SMA. Results: 37 patients were found, representing an incidence of 1 case per 10,000 live births in our reference population. The male/female ratio was 1.6/1. The type of SMA diagnosed more frequently was type I (26 cases), followed by type II with 9 cases, 1 case of SMA type III, and 1 case of SMA with respiratory distress (SMARD1). The most frequent genetic alteration was homozygous deletion of exons 7 and 8 of the SMN1 gene (31 cases). 25 patients were followed in our centre, 7 by the Pediatric Home Hospitalisation Unit. Most of them received motor rehabilitation (15), breathing physiotherapy in 8 cases or basic respiratory care in 5. 2 cases received oxygen by low-flow nasal cannula, non-invasive ventilation in 1 case and invasive ventilation with tracheostomy in 2 cases. In 4 cases a nasogastric tube and in 1 case a gastrostomy was prescribed. The median survival for type I was 8.0 months and 15.8 years for type II. Conclusion: The incidence in our population remains stable at around 1/10.000. Most cases presented a typical genetic alteration and were predominantly male. In approximately 1/10 patients the genetic alteration was different from the classical to the SMN gene. The caring level has increased in line with social and welfare demands in recent years. We improve our work with consensus statement for standard of care in spinal muscular atrophy.
Keywords: oxygen; spinal muscular atrophy; European; neurology; society; human; patient; population; gene; hospital; respiratory care; nasal cannula; diagnosis; breathing; genetic trait; rehabilitation; consensus; survival; health care quality; exon; physiotherapy; male; gastrostomy; noninvasive ventilation; nasogastric tube; respiratory distress; tracheostomy; air conditioning; live birth; welfare; disability; diseases

Amyotrophic lateral sclerosis with juvenile onset. Case report - Nechay A.; Stetsenko T.; Savchenko O. (European Journal of Paediatric Neurology, 2015)
Objective: To present clinical symptoms of ALS in 16 year adolescent and approaches to differential diagnosis. Methods: Case notes review. Results: A boy 16 years old at presentation, born from non-consanguineous parents, without evidence of weakness in relatives, had normal development. Aged 15 years he gradually developed weakness in legs, arms, more prominent on the left side. During 9 month he lost ability to walk, stand, seat, lift arms and control head. On neurological examination he presented with pronounced tetraparesis more prominent on the left proximally. Muscle strength was decreased. Muscle hypotonus, hypotrophy of limbs and neck, mild fibrillations of tongue and proximal arm muscles were observed. Tendon reflexes were high and asymmetric but absent ankle jerk reflexes. Patient had swallowing difficulties due to mild bulbar paresis, had pyramid signs, mosaic impairment of sensitivity of neuronal type and superficial breathing. Traumatic spine injury, tick-borne encephalitis, SMAIII were excluded. CSF analysis: protein - 0.93 g/l, glucose - 4.08 mmol/l, cytosis-2 lymphocytes, Pandy's reaction +. PCR to EBV, HSV1/2, CMV - negative. HIV-test - negative. Antibodies to Flavivirus were negative. Intramuscular EMG showed denervation and potentials of fasciculation, NCS revealed decreasing of velocity on left peroneal and both femoral nerves to 20%. First brain and spine MRI were normal. MRI in 10 month after debut of illness revealed foci of increased signal on TIRM in T1- 33, T2-33, T2-TIRM, D-33, SWI in posterior parts of the internal capsule 5-6 mm symmetrically. ALS had been diagnosed. Management mainly consisted of breathing support. The boy suffered from recurrent pneumonia, had tracheotomy. 1 year follow-up demonstrated steady course of the disease. The boy is normal intellectually, but has profound tetraparesis, fibrillations, breathing disturbances. Conclusion: The diagnosis has been based on El Escorial Criteria for the Diagnosis of ALS (1998). Clinical symptoms still stay the main part of the diagnosis of ALS.
Keywords: glucose; antibody; amyotrophic lateral sclerosis; juvenile; human; case report; European; neurology; society; male; breathing; boy; diagnosis; quadriplegia; arm; weakness; parent; hypotrophy; neck; arm muscle; muscle strength; spine injury; neurologic examination; tick borne encephalitis; nuclear magnetic resonance imaging; muscle hypotonia; limb; lymphocyte; tongue; HIV test; adolescent; mosaicism; paresis; swallowing; leg; capsula interna; pneumonia; follow up; differential diagnosis; protein analysis; patient; velocity; reflex; fasciculation; achilles reflex; denervation; femoral nerve; brain; spine; diseases; Flavivirus; tendon reflex; tracheotomy; development; electromyogram; Human immunodeficiency virus; cerebrospinal fluid

An anterior cervical spinal cord infarct in a first trimester - Patel A.; Hassan S.; Richardson K.; Davies R. (Cerebrovascular Diseases, 2015)
Background: Spinal cord infarcts (SCI) are most often seen in the presence of atherosclerotic disease or as a complication of aortic surgery. There are few reported cases of SCI's during pregnancy, and unfortunately, mortality and morbidity are high. We report a case of an anterior SCI in young woman whose only risk factor was pregnancy; detection was swift, management timely, and - following rehabilitation- a near complete recovery was achieved. Case: A 27 year old woman, 12 weeks gestation, presented to our hospital with a rapid onset weakness following 8 hours of paraesthesia in all of her limbs. She required intubation and ventilation following respiratory compromise and was subsequently transferred to the Critical Care Unit. Magnetic resonance imaging of her brain and spinal cord showed increased signal in the cervical spine extending into the medulla -consistent with a SCI. Infective, thrombophilia, and vasculitic screens were negative; as were transthoracic bubble echocardiogram and lupus ratio. The patient was extubated and had an early tracheostomy performed to help monitor her neurological recovery. After several days she regained motor and sensory function and was weaned from ventilatory support. Early rehabilitation was commenced on transferal to the acute stroke unit; she was discharged with early supported discharge team input. 2 months later the patient was independently mobile -swimming 3 times a week; sensation had returned to normal. Her Modified Clinical Outcomes Variable Scale score had improved to 91/91 -from 83 at the start of rehabilitation. The patient remained on low molecular weight heparin; 20 week growth scan was normal and the baby was healthy and of good size. Conclusion: A SCI is a rare and life threatening condition that must be considered as a differential in all patients with rapidly progressing neurology. Although unusual it can present in the young; here early diagnosis and support is essential to enable recovery.
Keywords: low molecular weight heparin; infarction; first trimester pregnancy; European; cerebrovascular accident; cervical spinal cord; female; human; patient; pregnancy; rehabilitation; spinal cord; nuclear magnetic resonance imaging; morbidity; air conditioning; aortic surgery; intubation; limb; sensation; intensive care; mortality; paresthesia; tracheostomy; brain; weakness; echocardiography; thrombophilia; hospital; cervical spine; risk factor; stroke unit; swimming; baby; neurology; early diagnosis

Chronic spinal cord injury treated with transplanted autologous bone marrow-derived mesenchymal stem cells tracked by magnetic resonance imaging: A case report - Chotivichit A.; Ruangchainikom M.; Chiewvit P.; Wongkajornsilp A.; Sujirattanawimol K. (Journal of Medical Case Reports, 2015)
Introduction: Intrathecal transplantation is a minimally invasive method for the delivery of stem cells, however, whether the cells migrate from the lumbar to the injured cervical spinal cord has not been proved in humans. We describe an attempt to track bone marrow-derived mesenchymal stem cells in a patient with a chronic cervical spinal cord injury. Case presentation: A 33-year-old Thai man who sustained an incomplete spinal cord injury from the atlanto-axial subluxation was enrolled into a pilot study aiming to track bone marrow-derived mesenchymal stem cells, labeled with superparamagnetic iron oxide nanoparticles, from intrathecal transplantation in chronic cervical spinal cord injury. He had been dependent on respiratory support since 2005. There had been no improvement in his neurological function for the past 54 months. Bone marrow-derived mesenchymal stem cells were retrieved from his iliac crest and repopulated to the target number. One half of the total cells were labeled with superparamagnetic iron oxide nanoparticles before transplantation to the intrathecal space between L4 and L5. Magnetic resonance imaging studies were performed immediately after the transplantation and at 48 hours, two weeks, one month and seven months after the transplantation. His magnetic resonance imaging scan performed immediately after the transplantation showed hyposignal intensity of paramagnetic substance tagged stem cells in the subarachnoid space at the lumbar spine area. This phenomenon was observed at the surface around his cervical spinal cord at 48 hours. A focal hyposignal intensity of tagged bone marrow-derived stem cells was detected at his cervical spinal cord with magnetic resonance imaging at 48 hours, which faded after two weeks, and then disappeared after one month. No clinical improvement of the neurological function had occurred at the end of this study. However, at 48 hours after the transplantation, he presented with a fever, headache, myalgia and worsening of his motor function (by one grade of all key muscles by the American Spinal Injury Association impairment scale), which lasted for 48 hours. Conclusion: Intrathecal injection of bone marrow-derived stem cells at the lumbar spine level could deliver the cells to the injured cervical spinal cord. Transient complications should be observed closely in the first 48 hours after transplantation. Further study should be carried out to evaluate the result of the treatment.
Keywords: dexamethasone; phosphate buffered saline; superparamagnetic iron oxide nanoparticle; adult; American Spinal Injury Association impairment scale; article; assisted ventilation; atlantoaxial subluxation; bone marrow biopsy; bone marrow derived mesenchymal stem cell; case report; cauda equina; cervical spinal cord; computer assisted tomography; culture medium; fever; headache; human; iliac crest; immobilization; lumbar spine; male; mesenchymal stem cell transplantation; motor performance; myalgia; neuropathic pain; nuclear magnetic resonance imaging; pilot study; priority journal; quadriplegia; respiratory function; spinal cord injury; subarachnoid space; tracheostomy

Palliative care in children with spinal muscular atrophy type I: What do they need? - García-Salido A.; de Paso-Mora M.G.; Monleón-Luque M.; Martino-Alba R. (Palliative & supportive care, 2015)
OBJECTIVE: Our aim was to describe the clinical evolution and needs of children with spinal muscular atrophy type I treated in a domiciliary palliative care program. METHOD: We undertook a retrospective chart review of nine consecutive patients. Descriptions of the clinical and demographic profile of children with spinal muscular atrophy (SMA) type I were referred to a pediatric palliative care team (PPCT). RESULTS: Six males and three females were admitted to the PPCT, all before six months of age, except for one afflicted with SMA type I with respiratory distress. The median time of attention was 57 days (range 1-150). The domiciliary attention mainly consisted of respiratory care. The patient with SMA type I with respiratory distress required domiciliary mechanical ventilation by tracheotomy. In all cases, a nasogastric tube (NT) was indicated. As end-of-life care, eight required morphine to manage the dyspnea, four received it only by enteral (oral or NT) administration, and four received it first by enteral administration with continuous subcutaneous infusion (CSI) later. Three of the four patients with CSI also received benzodiazepines. While they were attended by the PPCT, none required hospital admission. All the patients died at home except for the one attended to for just one day. SIGNIFICANCE OF RESULTS: Domiciliary care for these patients is possible. The respiratory morbidity and its management are the main issues. Application of an NT is useful to maintain nutritional balance. Morphine administration is necessary to manage the dyspnea. Palliative sedation is not always necessary.
Keywords: female; health service; hereditary spinal muscular atrophy; human; infant; male; nursing; palliative therapy; procedures; retrospective study; terminal care

Eroding through: An uncommon cause of dysphagia - Goodwin A.K.; Moore K.M.; Rawl R.; Collins D. (Journal of General Internal Medicine, 2015)
LEARNING OBJECTIVE #1: Surgically implanted cervical hardware may erode into the esophagus months or even years after initial implantation. LEARNING OBJECTIVE #2: Early diagnosis of esophageal perforation may require multiple diagnostic modalities with prompt surgical management crucial for minimizing morbidity and mortality. CASE: A 44 year-old man presented to the emergency department with an 8-12 month history of progressive dysphagia to solids and liquids, 80 lb weight loss, odynophagia, and significant dyspepsia. Review of systems was significant for episodic productive cough and orthopnea. Medical history included type 2 diabetes mellitus and a cerebral vascular accident 3 years prior with residual left-sided extremity motor weakness but no resultant speech or swallowing dysfunction. Surgical history was significant for remote traumatic right arm amputation and multiple cervical spine surgeries, with most recent anterior fixation of C3-C7 2 years prior for critical cervical stenosis. Physical examination showed a thin-appearingmale in no acute distress with stable vital signs. Neurologic examshowed no cranial nerve dysfunction and stable weakness in left sided extremities. Head and neck exam were unremarkable without palpable abnormality or pain on movement of the neck. Auscultation of the chest was remarkable for mild inspiratory stridor. Initial plain films and computed tomography imaging of head and neck showed surgical fixation of C3-C7 with anterior displacement of C6/C7 hardware and screws into the prevertebral soft tissue without frank intrusion in the esophagus. Barium esophagram with fluoroscopic evaluation showed no esophageal abnormalities. Flexible laryngoscopy was unremarkable for significant findings of the nasopharynx, hypopharynx, and larynx. Gastroenterology was consulted for upper endoscopy which found surgical hardware visualized at the level of the cricopharyngeus. Six days after presentation, the patient underwent removal of cervical hardware and repair of esophageal perforation with placement of percutaneous endoscopic gastrostomy, tracheostomy, and salivary bypass tube. Purulent fluid was found surrounding implanted hardware, and cultures isolated viridans streptococci, Candida albicans and Methicillin-resistant Staphylococcus aureus. He was treated with a total antibiotic course of four weeks with IV vancomycin and oral fluconazole. Post-operative course was relatively unremarkable, and after an 18 day hospitalization he was transferred to a rehabilitation facility. He has now returned home and denies dysphagia with oral intake. DISCUSSION: Anterior cervical spinal fixation has become a common management modality for multiple spinal pathologies including fracture, degenerative disc disease and neoplasm. Esophageal erosion and perforation by surgical hardware is uncommon with rates reported as low as 0.15 %. While most often occurring in the early post-operative phase, late-onset cases have been reported with erosion and perforation occurring more than 10 years after surgery. The most common symptoms include neck and throat pain, odynophagia, dysphagia, hoarseness, and aspiration. Less-common presenting symptoms include recurrent pneumonia, fever, cough, subcutaneous emphysema, and neurologic deficits. Our patient presented with progressive dysphagia, odynophagia, and weight loss with recurrent episodes of productive cough. Physician awareness is vital for identifying pertinent presenting symptoms which, in the setting of previous anterior cervical instrumentation, should raise concern for esophageal compression, erosion, and/or perforation. Prompt detection and intervention is crucial as mortality and complication rates rise significantly with delayed detection and intervention. Multiple modalities including CT imaging, endoscopy, and even surgical exploration may be required for diagnosis. In one study of patients with esophageal injury only 72.7 % were accurately diagnosed by imaging. Consequently, negative imaging should not be used as the sole means to rule out esophageal erosion or perforation. Esophageal perforation, as with our patient, is most common at the C5/C6 or C6/C7 level. Underlying causes which commonly lead to erosion include hardware migration, new trauma and chronic compression. Irrespective of cause, essential components of therapy include surgical removal of hardware, abscess drainage with culture-directed parental antibiotics, and salivary fluid diversion. Reports have noted normal swallowing function within 10-14 days after the surgical repair. Often temporary placement of gastrostomy tube is warranted for nutritional support. Esophageal erosion of surgical hardware, though typically occurring in the early post-operative course, may present years later with progressive dysphagia, cough, voice changes, neck pain or recurrent pneumonias among some of the many presenting symptoms. Clinician awareness with prompt diagnosis and surgical intervention are crucial for minimizing morbidity and mortality.
Keywords: antibiotic agent; fluconazole; vancomycin; barium; society; internal medicine; dysphagia; computer; human; coughing; imaging; patient; neck; perforation; surgery; mortality; esophagus perforation; odynophagia; liquid; diagnosis; morbidity; pneumonia; endoscopy; body weight loss; compression; esophagus; swallowing; weakness; computer assisted tomography; stridor; thorax; auscultation; dyspepsia; pain; cranial nerve; vital sign; aspiration; fever; subcutaneous emphysema; physician; esophagus injury; therapy; stomach tube; nutritional support; voice change; physical examination; hoarseness; sore throat; stenosis; spine surgery; solid; early diagnosis; neoplasm; fracture; arm amputation; pathology; rehabilitation; speech; methicillin resistant Staphylococcus aureus; hospitalization; emergency ward; Candida albicans; alpha hemolytic Streptococcus; tube; cerebrovascular accident; tracheostomy; percutaneous endoscopic gastrostomy; non insulin dependent diabetes mellitus; gastroenterology; larynx; hypopharynx; devices; nasopharynx; injury; laryngoscopy; abscess drainage; soft tissue; medical history; neck pain; male; implantation

What are the perspectives for ventilated tetraplegics? A French retrospective study of 108 patients with cervical spinal cord injury - Quesnel A.; Veber B.; Proust F.; Agasse E.; Beuret Blanquart F.; Verin E. (Annals of Physical and Rehabilitation Medicine, 2015)
Objective: Tetraplegic patients are often difficult to manage in intensive care units (ICU). The aim of our study was to calculate the incidence of patients in ICU with cervical spinal cord injury with special focus on tetraplegic patients on ventilation support and their future perspectives. Materials and methods: This retrospective study included patients with cervical spinal cord injury in Upper Normandy, between 2002 and 2012. Data analyzed included age, sex, past medical history, date of onset of quadriplegia, level of neurological involvement, AIS grade, and ventilatory status. Results: One hundred and eight patients were included (49.0 ± 21.1 years). The most common etiology was fall (50 patients [46.3%]). Incidence was calculated at 12.7 per one million inhabitants. Tracheotomy was performed in 40.7% of patients. Long-term mechanical ventilation was required for 6.5%. At the end of the study, 9 patients (9.6%) were permanently hospitalized, 61 patients (64.9%) had returned home but none of the four ventilated patients had been discharged. Conclusion: The conclusion of this work is that the future of highly tetraplegic patients is compromised, especially for those who remain reliant on mechanical ventilation.
Keywords: adult; article; artificial ventilation; cervical spinal cord injury; falling; female; home care; hospital patient; human; intensive care unit; long term care; major clinical study; male; outcome assessment; quadriplegia; retrospective study; sport injury; tracheotomy; traffic accident; ventilated patient

Radiotherapy QA of the DAHANCA 19 protocol - Samsøe E.; Andersen E.; Hansen C.R.; Johansen J.; Sand H.M.B.; Andersen L.J.; Petersen J.B.B.; Jensen K.; Smulders B.; Kristensen C.A.; Grau C. (Radiotherapy and Oncology, 2015)
Purpose/Objective: It has been demonstrated that nonadherence to protocol-specified radiotherapy (RT) requirements is associated with reduced survival, local control and potentially increased toxicity [1]. Thus, quality assurance (QA) of RT is important when evaluating the results of clinical trials. RT-QA of large multicentre-trials, however, requires substantial effort and resources. Recently, we presented a digital QA platform, the CIRRO dose plan bank, which allows for central review of such trials. Here, we present our RT-QA results from the latest completed clinical protocol from the Danish Head and Neck Cancer Group (DAHANCA). Materials and Methods: The clinical results of the DAHANCA 19 randomized phase III trial evaluated the effect of concurrent EGFR-inhibition during primary curative (chemo) radiotherapy in patients with head and neck squamous cell carcinoma (HNSCC). A total of 504 Danish patients entered the protocol in 2007-2012. Patients received RT at 5 different oncology centers to a total dose of 66-68Gy, 2Gy/fx, 6 fx/week. For the current QA analysis, total treatment time, CTV coverage, and near-max doses to the spinal cord and brainstem including the corresponding planning risk volumes (PRVs) were evaluated according to 2004 DAHANCA guidelines. Each QA parameter was scored within three categories: 1) Full compliance to protocol guidelines, 2) Minor deviations: Not according to guidelines, but without clinical relevance, and 3) Major deviations: Clinical significant deviation. Categories 1) and 2) are clinical acceptable, whereas category 3) is clinical unacceptable. Results: The complete digital RT dataset was uploaded for all 504 patients and QA parameters were extracted. The results are shown in table 1. Not all patients contribute to the PRV data, since the PRV concept was introduced in the Danish clinics shortly after 2007. Furthermore, 13 patients (2.6%) were eliminated from the CTV QA due to challenges to extract dose summations (e.g. re-scans and primary/ boost dose plans) from the database. A total of 11 major deviations were recorded. Four major deviations in CTV1 dose coverage were due to clinical considerations of the tolerance dose to the spinal cord, thus compromising target dose. Five of the major deviations in total treatment time were related to comorbidities, such as alcohol- or cardiac related matters and hospitalization. The remaining two cases were due to tracheotomy procedures and subsequent re-scans, which delayed the RT course. Table 1: Summary of results from RT-QA of the DAHANCA protocol. Conclusions: RT dose plans in DAHANCA 19 adhere well to national clinical guidelines. A total of 11 major deviations were found in 504 patients. All majors were clinically well accounted for.
Keywords: alcohol; radiotherapy; human; patient; spinal cord; quality control; survival; multicenter study; clinical protocol; head and neck cancer; toxicity; head and neck squamous cell carcinoma; hospitalization; oncology; brain stem; planning; risk; protocol compliance; parameters; hospital; data base; clinical trial (topic); tracheotomy; procedures

Unmasking and provoking severe disease activity in a patient with NMO spectrum disorder - Kornberg M.D.; Newsome S.D. (Neurology: Neuroimmunology and NeuroInflammation, 2015)
Keywords: aquaporin 4 antibody; fingolimod; methylprednisolone; natalizumab; rituximab; adult; brain biopsy; case report; cervical spinal cord injury; corticosteroid therapy; disease severity; drug substitution; lack of drug effect; drug withdrawal; dysarthria; dysphagia; enzyme linked immunosorbent assay; female; follow up; human; human tissue; immunotherapy; middle cerebellar peduncle; multiple sclerosis; myelooptic neuropathy; neuroimaging; myelooptic neuropathy; note; nuclear magnetic resonance imaging; paraplegia; patient transport; plasma exchange; priority journal; quadriplegia; relapse; repeat procedure; respiratory tract intubation; speech disorder; stomach intubation; stomach tube; tracheostomy; trismus; white matter lesion

Global health care use by patients with type-2 diabetes: Does the type of comorbidity matter? - Calderón-Larrañaga A.; Abad-Díez J.M.; Gimeno-Feliu L.A.; Marta-Moreno J.; González-Rubio F.; Clerencia-Sierra M.; Poblador-Plou B.; Poncel-Falcó A.; Prados-Torres A. (European Journal of Internal Medicine, 2015)
To identify patterns of health care use among diabetic patients with multimorbidity across primary, specialised, hospital and emergency care, depending on their type of chronic comorbidity. Methods Longitudinal study of a population-based retrospective cohort conformed by adult patients with type-2 diabetes assigned to any of the primary care centres in Aragon during 2010 and 2011 (n = 65,716). Negative binomial regressions were run to model the effect of the type of comorbidity on the number of visits to each level of care. Comorbidities were classified as concordant, discordant or mental based on expert consensus and depending on whether they shared the same overall pathophysiologic risk profile and disease management plan designed for type-2 diabetes. Results Mental comorbidity was independently associated with total and unplanned admissions (incidence rate ratio [IRR]:1.25; 95% confidence interval [CI]:1.12-1.39, IRR:1.21; 95% CI:1.06-1.39), average length of stay (IRR:1.47; 95% CI:1.25-1.73), and total and priority emergency room visits (IRR:1.26; 95% CI:1.17-1.35, IRR:1.30; 95% CI:1.18-1.42). Patients with discordant comorbidities showed the strongest associations with the number of visits to specialists (IRR:1.38; 95% CI:1.33-1.43) and to different specialties (IRR:1.36; 95% CI:1.32-1.39). Differences regarding GP visits were lower but still significant for patients with discordant comorbidity (IRR:1.08; 95% CI:1.06-1.11), but especially for those with mental comorbidity (IRR:1.17; 95% CI:1.14-1.21). Conclusion In patients with type-2 diabetes, the coexistence of mental comorbidity significantly increases the use of unplanned hospital services, and discordant comorbidities have an important effect on specialised care use. Differences with respect to primary care use are not as prominent.
Keywords: acquired immune deficiency syndrome; acute heart infarction; acute leukemia; adult; affective psychosis; anxiety disorder; aortic aneurysm; aphakia; aplastic anemia; arthropathy; article; asthma; atherosclerosis; attention deficit disorder; autoimmune disease; behavior disorder; bladder cancer; blindness; blood clotting disorder; breast cancer; cardiomyopathy; cardiovascular disease; cataract; cerebral palsy; cerebrovascular disease; chromosome aberration; chronic kidney failure; chronic liver disease; chronic obstructive lung disease; chronic pancreatitis; cleft lip; clinical assessment; colorectal cancer; comorbidity; congenital heart disease; congenital hip dislocation; congenital malformation; congestive heart failure; connective tissue disease; cystic fibrosis; deep vein thrombosis; delirium; dementia; depression; dermatitis; developmental disorder; diabetic patient; diabetic retinopathy; disorders of lipid metabolism; diverticulosis; eczema; emergency ward; emphysema; endocrine disease; endometriosis; esophagus cancer; female; gallstone formation; gastroesophageal reflux; glaucoma; gout; hair disease; health care utilization; hearing impairment; heart arrhythmia; hematologic disease; hemolytic anemia; hemophilia; hospital service; human; Human immunodeficiency virus infection; hypertension; hypospadias; hypothyroidism; immunopathology; iron deficiency anemia; irritable colon; ischemic heart disease; kidney disease; kyphoscoliosis; lactose intolerance; leg varicosis; length of stay; longitudinal study; low back pain; lung embolism; lymphoma; major clinical study; male; malignant neoplasm; medical specialist; metabolic disorder; multiple sclerosis; muscular dystrophy; nephritis; neuritis; neurologic disease; neurosis; non insulin dependent diabetes mellitus; obesity

Course of near-hanging victims succumbed to death: A seven year study - Ramchandra Sane M.; Mugadlimath A.B.; Zine K.U.; Farooqui J.M.; Phalke B.J. (Journal of Clinical and Diagnostic Research, 2015)
Introduction: Near hanging refers to victims who survive a hanging injury following attempted hanging, long enough to reach hospital. Delayed deaths in near hanging patients are mostly due to complication of hanging. The purpose of this study was to evaluate the demographics, mortality patterns and cause of delayed deaths in near hanging victims. Materials and Methods: In this study autopsy files over a seven year period from 2007 to 2013 were reviewed, and data of near hanging deaths (attempted hanging cases who succumbed to death and subjected for medicolegal autopsy) was extracted. Records of 14,000 autopsies was reviewed, and 10 deceased having died delayed deaths after near hanging episode were identified. In each case, the patients’ details, including gender, age, type of suspension, type of ligature material used for hanging and subsequent hanging mark produced were reviewed using autopsy reports and photographs taken during autopsy. Results: Demographic and pathological aspects of the each case discussed to throw light on autopsy findings in victims who died following near hanging. Complete suspension was present in 3 cases, while partial suspension was present in 7 cases. Survivals in delayed death after near hanging episode have ranged from 9 h to 72 d. Hypoxic encephalopathy was the most common cause of death, followed by pneumonia. Conclusion: Most of the near hanging patients did succumb to hypoxic encephalopathy; however, consolidation of lungs (pneumonia) was the next common cause of death reflecting need for aggressive oxygen therapy and selective resuscitation should be performed in all such cases.
Keywords: adolescent; adult; article; autopsy; cause of death; cervical spinal cord injury; death; delayed death; demography; female; hanging; human; male; mortality; near hanging; pathology; pneumonia; resuscitation; retrospective study; tracheostomy; young adult

A study of the clinical and radiological features in a cohort of 93 patients with a COL2A1 mutation causing spondyloepiphyseal dysplasia congenita or a related phenotype - Terhal P.A.; Nievelstein R.J.A.J.; Verver E.J.J.; Topsakal V.; van Dommelen P.; Hoornaert K.; Le Merrer M.; Zankl A.; Simon M.E.H.; Smithson S.F.; Marcelis C.; Kerr B.; et al. (American Journal of Medical Genetics, Part A, 2015)
Type 2 collagen disorders encompass a diverse group of skeletal dysplasias that are commonly associated with orthopedic, ocular, and hearing problems. However, the frequency of many clinical features has never been determined. We retrospectively investigated the clinical, radiological, and genotypic data in a group of 93 patients with molecularly confirmed SEDC or a related disorder. The majority of the patients (80/93) had short stature, with radiological features of SEDC (n=64), others having SEMD (n=5), Kniest dysplasia (n=7), spondyloperipheral dysplasia (n=2), or Torrance-like dysplasia (n=2). The remaining 13 patients had normal stature with mild SED, Stickler-like syndrome or multiple epiphyseal dysplasia. Over 50% of the patients had undergone orthopedic surgery, usually for scoliosis, femoral osteotomy or hip replacement. Odontoid hypoplasia was present in 56% (95% CI 38-74) and a correlation between odontoid hypoplasia and short stature was observed. Atlanto-axial instability, was observed in 5 of the 18 patients (28%, 95% CI 10-54) in whom flexion-extension films of the cervical spine were available; however, it was rarely accompanied by myelopathy. Myopia was found in 45% (95% CI 35-56), and retinal detachment had occurred in 12% (95% CI 6-21; median age 14 years; youngest age 3.5 years). Thirty-two patients complained of hearing loss (37%, 95% CI 27-48) of whom 17 required hearing aids. The ophthalmological features and possibly also hearing loss are often relatively frequent and severe in patients with splicing mutations. Based on clinical findings, age at onset and genotype-phenotype correlations in this cohort, we propose guidelines for the management and follow-up in this group of disorders.
Keywords: aspartic acid; glycine; procollagen; serine; adolescent; amino acid substitution; article; atlantoaxial dislocation; bronchomalacia; child; cleft palate; clinical feature; clubfoot; COL2A1 gene; coxa vara; disease severity; female; gene; gene mutation; genotype phenotype correlation; gestational age; hearing impairment; heterozygosity; hip arthroplasty; human; hypermetropia; major clinical study; male; missense mutation; mutational analysis; myopia; osteotomy; Pierre Robin syndrome; population research; respiratory distress; retina detachment; school child; scoliosis; spondyloepiphyseal dysplasia; tracheomalacia; tracheostomy

Complications and survival after long posterior instrumentation of cervical and cervicothoracic fractures related to ankylosing spondylitis or diffuse idiopathic skeletal hyperostosis - Robinson Y.; Robinson A.-L.; Olerud C. (Spine, 2015)
Objective. This study investigates the results of long posterior instrumentation with regard to complications and survival. Summary of Background Data. Fractures of the cervical spine and the cervicothoracic junction related to ankylosing spinal disease (ASD) endanger both sagittal profi le and spinal cord. Both anterior and posterior stabilization methods are well established, and clear treatment guidelines are missing. Methods. Forty-one consecutive patients with fractures of the cervicothoracic junction related to ASD were treated by posterior instrumentation. All patients were followed prospectively for 2 years using a standardized protocol. Results. Five patients experienced postoperative infections, 3 patients experienced postoperative pneumonia, 2 patients required postoperative tracheostomy, and 1 patient had postoperative cerebrospinal fl uid leakage due to accidental durotomy. No patient required reoperation due to implant failure or nonunion. Mean survival was 52 months (95% confi dence interval: 42-62 mo). Survival was affected by patient age, sex, smoking, and spinal cord injury. Conclusion. Patients with ASD experiencing a fracture of the cervicothoracic region are at high risk of developing complications. The posterior instrumentation of cervical spinal fractures related to ASD is recommended due to biomechanical superiority.
Keywords: bone screw; antibiotic agent; titanium; aged; ankylosing hyperostosis; ankylosing spondylitis; arm pain; article; bone screw; cervical spine fracture; cervicothoracic fracture; clinical article; cohort analysis; controlled study; epidural hematoma; female; follow up; human; laminectomy; liquorrhea; lung insufficiency; male; medical device complication; neck pain; nuclear magnetic resonance imaging; pneumonia; postoperative complication; postoperative infection; postoperative pain; priority journal; prospective study; reoperation; skull clamp

Pharyngoesophageal perforation 3 years after anterior cervical spine surgery: a rare case report and literature review - Yin D.-H.; Yang X.-M.; Huang Q.; Yang M.; Tang Q.-L.; Wang S.-H.; Wang S.; Liu J.-J.; Yang T.; Li S.-S. (European Archives of Oto-Rhino-Laryngology, 2015)
Pharyngoesophageal perforation after anterior cervical spine surgery is rare and the delayed cases were more rarely reported but potentially life-threatening. We report a case of pharyngoesophageal perforation 3 years after anterior cervical spine surgery. The patient presented with dysphagia, fever, left cervical mass and developing dyspnea 3 years after cervical spine surgery for trauma. After careful examinations, he underwent an emergency tracheostomy, neck exploration, hardware removal, abscess drainage and infected tissue debridement. 14 days after surgery, CT of the neck with oral contrast demonstrated no contrast extravasation from the esophagus. Upon review of literature, only 14 cases of pharyngoesophageal perforation more than 1 year after anterior cervical spine surgery were found. We discussed possible etiology, diagnosis and management and concluded that in cases of dysphagia, dyspnea, cervical pain, swelling and edema of the cervical area even long time after anterior cervical spine surgery, potential pharyngoesophageal damage should be considered.
Keywords: abscess drainage; adult; anterior cervical spine surgery; article; case report; cervical plate; computer assisted tomography; debridement; dysphagia; dyspnea; esophagography; esophagus perforation; fever; human; male; neck pain; pharyngoesophageal perforation; priority journal; spine surgery; spine tumor; tracheostomy

Asia impairment scale predicts the need for tracheostomy after cervical spinal cord injury - Childs B.R.; Moore T.A.; Como J.J.; Vallier H.A. (Spine, 2015)
Introduction: High neurologic level of injury, high Injury Severity Score (ISS), and low Glasgow Coma Scale (GCS) have been shown to predict tracheostomy in patients with cervical spinal cord injury. The objective of this study was to evaluate the ability of the American Spinal Injury Association (ASIA) impairment scale and neurological level of injury to predict the need for mechanical ventilation as well as tracheostomy. Methods: Three hundred eighty-three patients with fractures, dislocations, or ligamentous injury of the cervical spine were included in this retrospective study. Charts were reviewed to determine demographics, ISS, GCS, presence and severity of chest injuries, length of hospital stay (LOS), ICU stay, mechanical ventilation time, and mortality. Results: Fifty-nine patients (15.4%) underwent tracheostomy. An ASIA impairment scale of A had a specificity of 98.8% and sensitivity of 32.2% for predicting the need for tracheostomy. This yielded a 1.2% false positive rate. The ASIA impairment Scale remained the most significant predictor for tracheostomy after regression for ISS, GCS, and Chest Abbreviated Injury Scale. Neurological level of injury was not a significant predictor of tracheostomy. Conclusions: An ASIA impairment scale of A at any level of injury is a specific predictor of the need for tracheostomy with a low false positive rate. Given the relatively low risk of early tracheostomy and the potential benefits, an ASIA impairment scale of A would be a sensible early criterion to determine the need for tracheostomy.
Keywords: adult; American Spinal Injury Association impairment scale; artificial ventilation; cervical spinal cord injury; conference abstract; diagnostic test accuracy study; dislocation; female; fracture; Glasgow coma scale; hospitalization; human; ligament injury; major clinical study; male; mortality; retrospective study; sensitivity and specificity; thorax injury; tracheostomy

Predictors of the necessity for early tracheostomy in patients with acute cervical spinal cord injury: A 15-year experience - Jones T.S.; Burlew C.C.; Johnson J.L.; Jones E.; Kornblith L.Z.; Biffl W.L.; Stovall R.T.; Pieracci F.M.; Stahel P.F.; Moore E.E. (American Journal of Surgery, 2015)
Background The need for mechanical ventilation (MV) after spinal cord injury (SCI) is a risk factor for prolonged critical care. The "purpose" of this study was to identify the level of cervical SCI that requires MV, thereby defining candidates for tracheostomy. Methods Patients with cervical SCI over a 15-year period were reviewed. Results One hundred sixty-three patients sustained cervical SCI. Of 76 complete injuries, 91% required MV for greater than 48 hours. By injury level, MV incidence was 100% for C2-4, 91% for C5, 79% for C6, and 80% for C7. Only one quarter of patients with incomplete SCI required MV for greater than 48 hours; Glascow Coma Score and Injury Severity Score were significantly worse compared with patients not requiring MV. Conclusions Factors influencing the decision for tracheostomy in cervical SCI patients include the presence of a complete SCI, anatomic level of injury, Glascow Coma Score, Injury Severity Score, and associated thoracic injury. Patients with complete cervical SCI often require prolonged MV. Conversely, the minority of incomplete SCI required MV; the need for tracheostomy was likely performed for associated injuries. Utilizing identified factors permits a thoughtful approach to tracheostomy in this patient population.
Keywords: acute disease; adult; article; artificial ventilation; cervical spinal cord injury; outpatient department; female; Glasgow coma scale; hospital discharge; hospitalization; human; injury scale; major clinical study; male; prediction; priority journal; spine stabilization; tracheostomy

Spinal muscular atrophy type 1: Impact of NIV on survival and gastrostomy decision in the UK setting - Sframeli M.; Abel F.; Craig F.; Curry J.; Scoto M.; Muntoni F.; Manzur A.Y. (Developmental Medicine and Child Neurology, 2015)
Objective: The current figures on NIV use in SMA1 in the UK are not available. We aim to evaluate the current clinical practice of NIV, nasogastric tube (NGT), gastrostomy feeding and survival in a cohort of SMAI. Methods: Retrospectively case note review of SMAI patients at Great Ormond Street Hospital over the last 5 years. Patients were subdivided in three groups determined by the age of onset of symptoms (before 3mo, 3- 6mo, after 6mo). The prevailing practice entailed NIV support discussion with all families, and gastrostomy in infants with anticipated longer survival. Results: Forty infants with SMA1 were identified. 21 families chose NIV support. 5 infants did not tolerate NIV. 16 infants used night-time NIVt (mean age of starting NIV: 10 m) and 5 went on to require NIV 16 hours/day. No infant underwent tracheostomy. 29/40 infants received NGT, and 7 underwent gastrostomy. The median of age at gastrostomy was 18 months. 30 infants died at the median age of 7.5 months (mean 10.7 m). Median age at death in 8 NIV supported infants was 21.5 months, and 5.5 months in 5 infants who did not tolerate NIV. 8 infants on NIV are still alive at a median age 35.7 months (range 12-87m). Median age at death, stratified by age at onset: onset <3 months=6 months, onset 3-6 months=9.5 months, onset >6 months=17.8 months. Of the infants who survived >2 years, the age of onset was 3-6 months in 1 and >6 months in 4. Conclusions: NIV support increased survival in this SMA1 cohort in UK with important implications for service models and resource allocation. The optimal timing of gastrostomy insertion needs clarification. There is a range of severity SMA1 and key management decisions should take account of age of onset/severity and prognosis.
Keywords: spinal muscular atrophy; survival; gastrostomy; United Kingdom; neurology; infant; onset age; death; patient; human; prognosis; feeding; model; nasogastric tube; tracheostomy; night; clinical practice; resource allocation; hospital

Recent thoraco-abdominal aortic repair outcomes using moderate-to-deep hypothermia combined with targeted reconstruction of the Adamkiewicz artery - Tanaka H.; Minatoya K.; Sasaki H.; Seike Y.; Itonaga T.; Oda T.; Kobayashi J. (Interactive Cardiovascular and Thoracic Surgery, 2015)
OBJECTIVES We retrospectively reviewed the surgical results of thoraco-abdominal aortic repair using moderate-to-deep hypothermia combined with targeted reconstruction of the Adamkiewicz artery (AKA). METHODS Between 2006 and 2014, 100 patients underwent thoraco-abdominal aortic aneurysm repair using moderate-to-deep hypothermia. Their mean age was 58 ± 15 years and 76 (76%) were men. Their aortic pathologies included acute dissection (5), chronic dissection (74), degeneration (20) and infection (1). Thirty-four had connective tissue disorders and 5 had emergency operations. The degrees of repair were Crawford extent I for 11, II for 76 and III for 13. Seven had concomitant arch repair. Preoperative magnetic resonance angiography or computed tomographic angiography was performed to detect the AKA in 95. We used deep hypothermia (18°C) for those requiring open proximal aortic anastomosis for cerebral protection and moderate hypothermia (25°C) for those not requiring open proximal aortic anastomosis. RESULTS Two patients had spinal cord injuries and 4 had a stroke. For those in whom the AKA was identified (90%), all had targeted artery reconstruction. The mean pairs of reconstructed intercostal arteries were 1.5 ± 0.7. There were 5 in-hospital deaths for which the causes were lung bleeding (2), infection (2) and iliac aneurysm rupture (1). Temporary dialysis for new-onset renal failure was required for 9. The mean postoperative mechanical ventilation period was 1.7 ± 1.9 days. Six required a tracheostomy due to respiratory failure. CONCLUSIONS Moderate-to-deep hypothermia combined with targeted reconstruction of the AKA provided satisfactory outcomes with thoraco-abdominal aortic repair, particularly for spinal cord protection.
Keywords: Adamkiewicz artery; adult; aneurysm rupture; aortic reconstruction; artery; artery reconstruction; article; artificial ventilation; cause of death; cerebrovascular accident; controlled study; dialysis; female; human; iliac aneurysm rupture; induced hypothermia; infection; intercostal artery; kidney failure; lung hemorrhage; major clinical study; male; middle aged; moderate to deep hypothermia; onset age; outcome assessment; postoperative care; priority journal; respiratory failure; spinal cord injury; surgical anatomy; surgical mortality; targeted artery reconstruction; thoraco abdominal aortic repair; tracheostomy; treatment duration; treatment response

Modified total arch replacement using a four-branched arch graft for acute type a aortic dissection with minimal brain and spinal cord ischemic time - Lu S.; Sun X.; Hong T.; Yang S.; Song K.; Lai H.; Wang C. (Journal of Cardiovascular Surgery, 2015)
Aim: This study aimed to evaluate the results of modified surgical strategies of total arch replacement using a four-branched arch graft, stented elephant trunk, and innovative organ protection method for acute type A aortic dissection. Methods: Between August 2011 and December 2011, 21 patients with acute type A aortic dissection underwent modified total arch replacement using the four-branched arch graft technique. All 21 patients had emergency surgery. Five patients had undergone previous aortic or cardiac surgery. The operations were stented elephant trunk implantation in 17 patients, ascending aorta replacement in 21 patients, coronary artery bypass grafting in four patients, Bentall operation in two patients, and aortic valve replacement in one patient. Twenty-one operations were performed under deep hypothermic extracorporeal circulation, modified selective cerebral perfusion, and end-organ and spinal cord protection for arch reconstruction. Results: There was two in-hospital deaths (9.5%). No persisting neurologic deficits or paraplegia occurred in 21 patients. Cardiopulmonary bypass time was 177.9±37.8 minutes. Myocardial ischemic time was 110.3±29.3 minutes. ICU time was 8.8±6.9 days and in-hospital duration was 28.7±13.7 days. Ventilation time varied from 9 hours to 21 days. A tracheotomy was necessary in four patients. Mean follow-up was 7.3±1.7 months and all patients are still alive. Conclusion: Modified total arch replacement using a four-branched arch graft with stented elephant trunk and innovative organ protection is a useful and safe alternative technique for the treatment of acute type A aortic dissection and the results are encouraging.
Keywords: creatinine; adult; aged; aortic dissection; aortic graft; aortic prosthesis; aortic arch surgery; article; brain ischemia; cardiopulmonary bypass; cause of death; clinical article; creatinine blood level; female; follow up; heart muscle ischemia; human; hypoxemia; intensive care; kidney failure; male; multiple organ failure; operation duration; paraplegia; postoperative hemorrhage; reoperation; spinal cord ischemia; sternotomy; surgical mortality; total arch replacement; tracheotomy

Outcomes of open repair of mycotic descending thoracic and thoracoabdominal aortic aneurysms - Lau C.; Gaudino M.; De Biasi A.R.; Munjal M.; Girardi L.N. (Annals of Thoracic Surgery, 2015)
Background The purpose of this study was to evaluate the short- and intermediate-term outcomes of open repair of mycotic thoracic and thoracoabdominal aneurysms. Contemporary surgical and perioperative techniques were utilized. Methods From November 1997 to May 2014, 14 consecutive patients underwent open repair of descending thoracic (n = 9, 64.3%) and thoracoabdominal (n = 5, 35.7%) mycotic aortic aneurysms. All procedures were performed through the left side of the chest. Infected tissue was completely debrided and excised. Aortic continuity was restored in situ with a Dacron prosthesis (Macquet Corp, Oakland, NJ). Soft tissue coverage of the prosthesis was performed when anatomy and patient condition permitted. Perioperative outcomes, intermediate-term survival, and reinfection rates were examined. Results All patients presented with either aneurysm-related symptoms or a clinical picture of sepsis. Diagnosis was confirmed utilizing computed tomography imaging. Mean age was 66 ± 13 years, 8 patients (57.1%) were male, and mean aneurysm size was 5.9 ± 1.3 cm. All patients were hypertensive, 3 (21.4%) had prior coronary revascularization, 7 (50%) had chronic pulmonary disease, 5 (35.7%) had diabetes mellitus, and 2 (14.3%) had end-stage renal disease requiring dialysis. Twelve patients (85.7%) had aneurysm-related pain, and 9 (64.3%) of them had contained rupture. Mean time from onset of illness to surgery was 36 days (range, 0 to 153). On preoperative blood cultures, 4 (28.6%) grew Staphylococcus aureus, 4 (28.6%) grew gram negative organisms, 2 (14.3%) grew mycobacterium, and 4 cultures (28.6%) had negative results. Empiric broad-spectrum antibiotics were initiated on all patients and adjusted based on final cultures. A majority of patients underwent repair utilizing a clamp-and-sew technique (n = 10, 71.4%); the remainder (n = 4, 28.6%) required repair under profound hypothermic circulatory arrest. After radical debridement of the infected tissue, grafts were placed in the normal anatomic position; 6 (42.9%) patients had additional soft tissue coverage, 5 (35.7%) utilizing an omental flap and 1 (7.1%), a serratus muscle flap. There was 1 in-hospital death (7.1%) secondary to ischemic bowel. Four patients (28.6%) required tracheostomy, and 1 (7.1%) had recurrent nerve injury. None of the patients incurred spinal cord injury, stroke, or new onset renal failure requiring dialysis. After surgery, all patients were given 6 weeks of intravenous antibiotics. Lifelong suppression therapy was maintained with oral antibiotics. There were no episodes of prosthetic graft infection on follow-up. Univariate analysis revealed that New York Heart Association functional class, diabetes, and preoperative renal dysfunction were preoperative risk factors for major adverse events. Mean follow-up time was 26.5 months (median 8.2; range, 1 to 142). Actuarial 5-year survival was 71%. Conclusions Open repair of mycotic descending thoracic and thoracoabdominal aortic aneurysms remains the gold standard of therapy. Aggressive intraoperative debridement with in situ prosthetic reconstruction permits a high rate of success in this very high risk cohort of patients. Lifelong antibiotic suppression therapy may prevent late prosthetic graft infection.
Keywords: nafcillin; oxacillin; piperacillin plus tazobactam; sultamicillin; vancomycin; adult; aged; article; blood culture; cardiac patient; cardiovascular risk; cerebrovascular accident; chronic lung disease; clinical article; cohort analysis; computer assisted tomography; deep hypothermic circulatory arrest; diabetes mellitus; end stage renal disease; female; follow up; gold standard; heart muscle revascularization; human; hypertension; male; mean arterial pressure; mycotic aneurysm; mycotic descending thoracic aortic aneurysm; New York Heart Association class; outcome assessment; peritoneal dialysis; positron emission tomography; priority journal; prospective study; salmonellosis; spinal cord injury; Staphylococcus aureus; sternotomy; surgical patient; thoracoabdominal aorta aneurysm; tracheostomy; treatment duration; tuberculosis

An autopsied case of adult-onset bulbospinalform Alexander disease with a novel S393R mutation in the GFAP gene - Iwasaki Y.; Saito Y.; Mori K.; Ito M.; Mimuro M.; Aiba I.; Saito K.; Mizuta I.; Yoshida T.; Nakagawa M.; Yoshida M. (Clinical Neuropathology, 2015)
A 50-year-old Japanese man with no apparent family history noticed diplopia. He gradually showed gait disturbance and dysuria. Abducens disorder of eye movement with nystagmus, tongue atrophy with fasciculation, spastic tetraparesis, and sensory disturbance were also observed. MRI showed severe atrophy of the medulla oblongata to the cervical cord ("tadpole appearance"). Tracheotomy and gastrostomy were performed 7 years after onset due to the development of bulbar palsy. Death occurred following respiratory failure after 11 years total disease duration. The brain weighed 1,380 g. The cerebrum, cerebellum, midbrain, and upper pons were preserved from atrophy, but the medulla oblongata to the cervical cord showed severe atrophy. A few Rosenthal fibers were observed in the cerebral white matter, basal ganglia, and cerebellum, whereas numerous Rosenthal fibers were observed in the medulla oblongata to the cervical cord. Myelin loss with relatively preserved axons was extensively observed from the middle of the pons to the spinal cord. The clinicopathological diagnosis was adult-onset bulbospinal-form Alexander disease. Glial fibrillary acidic protein (GFAP) gene analysis revealed a novel mutation of S393R. Expression patterns of S393R mutant GFAP using adrenal carcinoma-derived cells (SW13 cells) showed a decreased number of filamentous structures and abnormal aggregates.
Keywords: glial fibrillary acidic protein; adrenal cortex carcinoma; adult; Alexander disease; article; basal ganglion; bulbar paralysis; case report; cerebrospinal fluid; cervical spinal cord; disease duration; electroencephalography; eye movement; family history; fasciculation; gastrostomy; gene mutation; human; joint contracture; lung alveolus hypoventilation; male; medulla oblongata; middle aged; neurologic examination; nystagmus; priority journal; respiratory failure; tracheotomy; white matter

Clinical Evaluation and Airway Management for Adults with Cervical Spine Instability - Martini R.P.; Larson D.M. (Anesthesiology Clinics, 2015)
Airway management of patients with cervical spine instability may be difficult as a result of immobilization, and may be associated with secondary neurologic injury related to cervical spine motion. Spinal cord instability is most common in patients with trauma, but there are additional congenital and acquired conditions that predispose to subacute cervical spine instability. Patients with suspected instability should receive immobilization during airway management with manual in-line stabilization. The best strategy for airway management is one that applies the technique with the highest likelihood of success on the first attempt and the lowest biomechanical influence on a potentially unstable spine.
Keywords: ankylosing spondylitis; assisted ventilation; awake flexible fiberoptic intubation; cervical collar; cervical spine injury; cervical spine instability; cervical spine radiography; clinical evaluation; extubation; Glasgow coma scale; human; intervertebral disk; laryngoscopy; ligamentum flavum; low risk patient; neck pain; nuclear magnetic resonance imaging; odontoid process; pathogenesis; posterior longitudinal ligament; respiration control; respiratory tract intubation; review; rheumatoid arthritis; second cervical vertebra; spinal cord injury; spine instability; spiral computer assisted tomography; spondyloarthropathy; supraglottic airway device; tracheostomy; vertebra dislocation; videolaryngoscope

The impact of a multidisciplinary safety checklist on adverse procedural events during bedside bronchoscopy-guided percutaneous tracheostomy - Hazelton J.P.; Orfe E.C.; Colacino A.M.; Hunter K.; Capano-Wehrle L.M.; Lachant M.T.; Ross S.E.; Seamon M.J. (Journal of Trauma and Acute Care Surgery, 2015)
BACKGROUND: Bedside procedures are seldom subject to the same safety precautions as operating room (OR) procedures. Since July 2013, we have performed a multidisciplinary checklist before all bedside bronchoscopy-guided percutaneous tracheostomy insertions (BPTIs). We hypothesized that the implementation of this checklist before BPTI would decrease adverse procedural events. METHODS: A prospective study of all patients who underwent BPTI after checklist implementation (PostCL, 2013-2014, n = 63) at our Level I trauma center were compared to all patients (retrospectively reviewed historical controls) who underwent BPTI without the checklist (PreCL, 2010-2013, n = 184). Exclusion criteria included age less than 16 years, OR, and open tracheostomy. The checklist included both a procedural and timeout component with the trauma technician, respiratory therapist, nurse, and surgeon. Demographics and variables focusing on BPTI risk factors were compared. Variables associated with the primary end point, adverse procedural events, during univariate analysis were used in the multiple variable logistic regression model. A p ≤ 0.05 was significant. RESULTS: Of 247 study sample patients, no difference existed in body mass index, baseline mean arterial pressure, duration or mode of mechanical ventilation, cervical spine or maxillofacial injury, or previous neck surgery between PreCL and PostCL BPTI patients. PreCL patients were younger (48 [20] years vs. 57 [21] years, p < 0.01) but more often had adverse procedural events compared with PostCL patients (PreCL,14.1% vs. PostCL,3.2%, p = 0.020). After adjusting for age, vitals, BPTI risk factors, and intensive care unit duration after BPTI, multiple variable logistic regression determined that performing the safety checklist alone was independently associated with a 580% reduction in adverse procedural events (odds ratio, 5.8; p = 0.022). CONCLUSION: Our results suggest that the implementation of a multidisciplinary safety checklist similar to those used in the OR would benefit patients during invasive bedside procedures. LEVEL OF EVIDENCE: Therapeutic/care management study, level IV.
Keywords: adult; article; artificial ventilation; body mass; bronchoscopy; bronchoscopy guided percutaneous tracheostomy; cervical spine injury; checklist; female; human; intensive care unit; major clinical study; male; maxillofacial injury; mean arterial pressure; middle aged; nurse; patient safety; priority journal; prospective study; respiratory therapist; retrospective study; risk factor; surgeon; tracheostomy

Risk factors for dysphagia in acute cervical spinal cord injury - Hayashi T.; Maeda T.; Sakai H.; Morishita Y.; Shiba K. (Spine, 2015)
Introduction: Dysphagia following traumatic cervical spinal cord injury (CSCI) is an under-recognized complication that can lead to aspiration pneumonia, which is a significant cause of morbidity and mortality. Several authors have investigated dysphagia associated with CSCI, however, risk factors for dysphagia are still not well understood. The objective of this study was to elucidate the incidence and risk factors of dysphagia in patients with acute CSCI. Methods: A total of 464 consecutive patients with traumatic cervical spinal injury with and without spinal cord damage were treated at our institute and were registered in a database from January 2007 to December 2014. All patients underwent CT, MRI, and neurological examination on admission. We retrospectively selected 298 patients based on following criteria: (1) admission within 3 days following injury, (2) patients with paresis or paralysis, (3) patients without brain injury. Neurological impairment scale was evaluated according to ASIA impairment scale (AIS), and level of injury was identified using CT and MRI. We analyzed the factors postulated to increase the risk for dysphagia, including the patient's age, neurological impairment scale grade, level of injury, tracheostomy, and operative treatment, using a multiple logistic regression model to compute odds ratios (ORs) and 95% confidence intervals (95% CI). Results: 298 eligible patients (256 males and 42 females) with an average age 61.4 ± 17.3 (range, 14-91 yr.) were identified during 8-year study period. 21 of 298 patients appeared to be suffering from dysphagia after CSCI (7.0%). All of them experienced evident aspiration and had to stop eating their meals due to aspiration. The neurological status revealed that 13 of those patients were AIS A, 6 patients were AIS B, and 2 patients were AIS C. 12 of 21 patients (57.1%) received tracheostomy (Table 1). Multivariable logistic regression analysis revealed that age > 72 years (OR: 2.97, 95% CI: 1.01-9.02, p = 0.04), AIS A or B (OR: 8.00, 95% CI: 1.92-54.7, p = 0.003), presence of tracheostomy (OR: 13.8, 95% CI: 4.62-44.3, p < 0.001) were significant risk factors (Table 2). Conclusions: The incidence of dysphagia after acute CSCI was 7.0%. Old age, severe neurological impairment scale, and presence of tracheostomy may be at risk for dysphagia after acute CSCI. When treating CSCI, understanding the risk factors of dysphagia is important to prevent aspiration pneumonia. [Table Presented].
Keywords: adult; American Spinal Injury Association impairment scale; aspiration pneumonia; brain injury; cervical spinal cord injury; conference abstract; dysphagia; eating; female; human; incidence; major clinical study; male; middle aged; multivariate logistic regression analysis; neurologic examination; nuclear magnetic resonance imaging; paresis; prevention; retrospective study; risk assessment; risk factor; tracheostomy

Highly unstable cervical spine injury in an infant: a case report - young Ha S.; Lee S.-H.; Kim E.-S.; Shin H.J.; Eoh W. (Child's Nervous System, 2015)
Summary: Although the rate of surgical intervention for pediatric cervical spine injuries has risen, none of these instrumentation techniques has been reported in children less than 1 year of age. Additional consideration with placement of wires or cables is not safe because of the presence of cartilaginous bone and poor bone strength. The authors report a technique of internal fixation without fusion using nonabsorbable synthetic suture in an infant with unstable cervical injury. Methods: A 5-month-old girl was transported to emergency department and computed tomography showed severe distraction injury at the C6-7 level with total disruption of the facet joints bilaterally. To improve her general condition, she was placed in a customized occipito-cervical brace until surgical stabilization could be performed. After 1 month, surgical fixation was performed. After removing all soft tissues at the appropriate level, a hole was made in the center of the superior articular process of C6. A nonabsorbable suture (2–0 Ethibond; Ethicon, Somerville, NJ, USA) was passed from the superior articular process to the facet joint. The suture was passed into the spinous process of the lower level, and it was progressively tightened and radiographs were taken until anatomic reduction was achieved. Results: After surgery, solid fusion was achieved. She was able to sit up in a stroller after 6  months. Conclusion: As surgical treatment of pediatric cervical spine injuries is unsuitable for infants, the Ethibond sutures are strong enough to hold and stabilize the spine. This suturing technique can be used as an alternative treatment for cervical injury in infants.
Keywords: article; artificial ventilation; case report; cervical collar; cervical spine injury; cervical spine radiography; computer assisted tomography; conservative treatment; disease severity; emergency surgery; emergency ward; endotracheal intubation; female; fluoroscopy; general anesthesia; human; infant; neurologic examination; nuclear magnetic resonance imaging; ossification; osteosynthesis; polyester suture; poor general condition; priority journal; quadriplegia; rehabilitation care; soft tissue; spinal cord disease; spinal hematoma; spine surgery; surgical technique; suture technique; tracheostomy; traffic accident; vertebra body; Ethibond

Pressure ulcer surveillance in neurotrauma patients at a level one trauma centre in India - Babu A.; Madhavan K.; Singhal M.; Sagar S.; Ranjan P. (Oman Medical Journal, 2015)
Objectives: Pressure ulcers are a multifactorial, prevalent, and preventable morbidity. They cause a burden both financially and emotionally, to the individual, their family and doctor, and to society as a whole. Pressure ulcers are extremely difficult to treat; therefore, prevention is key. Methods: We started a Wound Care Surveillance Program in 2012 involving nurses, physiotherapists, and doctors. We intended to prevent the occurrence of pressure ulcers, ensure early detection, and facilitate the healing process. The Braden scale was used to stratify patients’ risk. The number of patients observed in our study was 2,974 over a one-year period. Results: The pressure sore prevalence was 3.1%. Younger and middle-aged patients were most commonly affected; 27% of these patients did not survive. Mortality was not attributed to the pressure ulcer directly. The most common mode of injury was road traffic accidents. Most of our patients had just a single pressure area affected, most commonly the sacrum. Most patients were managed with debridement and dressings while 12% received surgical treatment. Of those with stage one ulcers, 29% healed completely at two months. In stage two and three patients, 17% and 6% healed in two months, respectively, and this number was zero in stage four patients. Conclusion: The Wound Care Surveillance Program has been a very effective strategy for the prevention and management of pressure ulcers. Stage two ulcers were the most common in our setup. Braden scoring, traditionally used to screen these ulcers, can be used as a predictive and prognostic tool to predict healing of pressure ulcers. Poor healing is expected in higher staged ulcers and patients with spinal injury and major solid organ injury and those who need a tracheostomy. Home-based care is not up to mark in our society and accounts for most of the cases in the follow-up.
Keywords: article; bandages and dressings; Braden Scale; debridement; decubitus; disease surveillance; falling; female; Glasgow coma scale; health program; human; India; length of stay; major clinical study; male; mortality; nervous system injury; observational study; paraplegia; prospective study; quadriplegia; self care; spine injury; split thickness skin graft; tracheostomy; traffic accident; ulcer healing; Wound Care Surveillance Program

Pediatric intramedullary spinal cord lesions: Pathological spectrum and outcome of surgery - Sahu R.K.; Das K.K.; Bhaisora K.S.; Singh A.K.; Mehrotra A.; Srivastava A.K.; Sahu R.N.; Jaiswal A.K.; Behari S. (Journal of Pediatric Neurosciences, 2015)
Background: Pediatric intramedullary spinal cord lesions are not only rare but also different from adults in a number of aspects. We aimed to study the incidence and the frequencies of various pediatric intramedullary mass lesions, their outcome to treatment and the factors determining their outcome of treatment. Materials and Methods: Thirty-one consecutive children (aged 1-18 years, mean 11.1 years, male: female = 1.8:1) with pathologically proven intramedullary spinal cord lesions treated at our center were studied. Clinico-radiological, histopathological, operative, and outcome data were reviewed retrospectively. The functional status was assessed using the modified McCormick grading system. Results: Gross total tumor excision was performed in 19 patients (61.3%), subtotal in 9 patients (29%), partial excision was performed in 2 (6.5%) patient, and only biopsy was performed in 1 patient (6.5%). There was one peroperative death, 2 patients died at follow-up. Complications included wound related complications (n = 4), transient deterioration in the motor power, and respiratory complication requiring a tracheostomy. Six patients showed recurrence at a mean follow-up of 16.4 months. Developmental tumors, high-grade ependymomas, and incompletely excised grade 2 ependymomas showed a tendency to recur. Conclusions: Children constituted nearly 1/5 th (17.4%) of intramedullary spinal cord tumors. Astrocytomas and ependymomas taken together constituted the most common intramedullary spinal lesions in children; however, developmental tumors predominated in the first decade. Children usually presented in good functional grades preoperatively and maintained good grades after surgery. Functional outcome was dependent on the preoperative neurological status and histopathology of the lesions.
Keywords: lomustine; procarbazine; vincristine; adolescent; adult; article; astrocytoma; backache; biopsy; bladder disease; cancer combination chemotherapy; cancer radiotherapy; cancer surgery; child; childhood disease; clinical article; clinical feature; ependymoma; epidermoid tumor; female; follow up; functional status; hemangioblastoma; histopathology; human; human tissue; incidence; lipoma; male; motor power transient deterioration; multiple cycle treatment; neck pain; outcome assessment; pediatric intramedullary spinal cord lesion; postoperative complication; respiratory tract disease; retrospective study; spasticity; spinal cord lesion; teratoma; tracheostomy; trigeminal nerve; tuberculoma; weakness; wound complication

Tetraplegia after thyroidectomy in a patient with cervical spondylosis : A case report and literature review - Xiong W.; Li F.; Guan H. (Medicine (United States), 2015)
Cervical spondylosis is degeneration of the cervical spine that occurs during the normal course of aging, and may progress into compression of the spinal cord, or cervical spondylotic myelopathy (CSM), which can cause neurologic dysfunction. Cervical spondylosis can be identified in the majority of people older than 50 years. Many people with cervical spondylosis or CSM are asymptomatic. However, patients with CSM are at higher risk of spinal cord injury (SCI) following minor injury. A 60-year-old woman with asymptomatic cervical spondylosis underwent an elective subtotal thyroidectomy for thyroid nodules. After the surgery, she developed tetraplegia. MRI revealed spinal cord compression and injury. Main diagnoses, therapeutics interventions, and outcomes: Acute cervical SCI was diagnosed. After an emergency anterior cervical corpectomy and fusion surgery, she almost completely recovered. Iatrogenic cervical SCI after nonspinal surgeries that requires neck hyperextension is rarely reported, probably due to underdiagnosis and underreport. Among the 14 cases (including ours) published in the literature, most patients had cervical spondylosis and were senior men. Five patients had diabetes. Four patients had long-term hemodialysis. Seven patients had undergone coronary artery bypass surgery that requires prolonged operative time. Only 3 patients had almost complete recovery. Most patients were disabled. Two patients required tracheostomy for long-term ventilator support. Two patients died. These cases reiterate the potential risk of iatrogenic SCI in people with predisposing conditions such as cervical spondylosis, especially considering the rising prevalence and severity of cervical spondylosis caused by the aging of the population and modern sedentary lifestyle. Surgeries requiring prolonged neck hyperextension put patients with cervical spondylosis at risk for SCI. Failure to recognize the potential occurrence of iatrogenic SCI might endanger patients' lives.
Keywords: adult; anterior cervical corpectomy and fusion; article; asymptomatic disease; brain dysfunction; case report; cervical spondylosis; cervical spondylotic myelopathy; disease course; elective surgery; emergency surgery; female; high risk patient; human; middle aged; nuclear magnetic resonance imaging; priority journal; quadriplegia; spinal cord compression; spinal cord injury; spine fusion; subtotal thyroidectomy; thyroid nodule; treatment outcome; whiplash injury

Open thoracoabdominal aortic repair for chronic type B dissection - Kouchoukos N.T.; Kulik A.; Castner C.F. (Journal of Thoracic and Cardiovascular Surgery, 2015)
Objectives Advances in endovascular surgery have brought into question the role of open operative treatment of chronic thoracoabdominal aortic dissection. In this context, we evaluated our experience with open repair of this condition using a single operative technique. Methods From January 1986 to January 2014, 69 patients with chronic thoracoabdominal aortic dissection underwent open repair using total cardiopulmonary bypass (CPB) and hypothermic circulatory arrest (HCA). The degree of repair was as follows: Crawford extent I, 13 patients (19%), Crawford extent II, 41 patients (59%), and Crawford extent III, 15 patients (22%). Thirty patients (43%) had Marfan or Loeys-Dietz syndrome. Fifty-three patients (77%) had previous operations on the thoracic or abdominal aorta. Results The 30-day mortality rate was 5.8% (4 patients). Stroke occurred in 2 (3%) of 66 operative survivors, and spinal cord ischemic injury in 4 (6%). Temporary dialysis for new-onset renal failure was required in 4.5% of hospital survivors and tracheostomy in 10.6%. Survival after 1, 5, and 10 years was 87%, 65%, and 40%, respectively. Eighteen patients (26%) required a total of 20 subsequent operations on the thoracic or abdominal aorta of whom 15 had Marfan or Loeys-Dietz syndrome. Three of these procedures were for contiguous distal aortic disease and 10 were for patch aneurysms of the intercostal or visceral/renal arteries. Conclusions Open thoracoabdominal aortic repair for chronic dissection using CPB and HCA can be accomplished with mortality and morbidity rates that are comparable with those reported for endovascular or hybrid techniques. Open repair should remain a viable and primary option for the management of this condition until the long-term effectiveness of alternative methods of treatment is clearly established.
Keywords: adult; aged; aortic dissection; aortic reconstruction; article; cardiopulmonary bypass; cerebrovascular accident; human; Loeys Dietz syndrome; major clinical study; Marfan syndrome; mortality; priority journal; survival rate; survival time; thoracoabdominal aortic dissection; thoracoabdominal aortic repair; tracheostomy

Beam Attenuators and the Risk of Unrecognized Large-Fraction Irradiation of Critical Tissues - Luka S.; Marks J.E. (Medical Dosimetry, 2015)
The use of radiation beam attenuators led to radiation injury of the spinal cord in one patient and of the peripheral nerve in another due to unsuspected large-fraction irradiation. The anatomic distribution of radiation dose was reconstructed in the sagittal plane for the patient who developed radiation myelopathy and in the axial plane for the patient who developed peripheral neuropathy. The actual dose delivered to the injured structure in each patient was taken from the dose distribution and recorded along with the time, number of fractions, and dose per fraction. The patient who developed radiation myelopathy received a total of 46.5 Gy in twenty-three 2.1 Gy fractions in 31 days to the upper cervical spinal cord where the thickness of the neck was less than the central axis thickness due to cervical lordosis and absence of a posterior compensating filter. The patient who developed peripheral neuropathy received 55 Gy in twenty-five 2.2 Gy fractions in 50 days to the femoral nerve using bolus over the groins and an anterior one-half value layer Cerrobend pelvic block to bias the dose anteriorly. Compensating filters and other beam attenuators should be used with caution because they may result in unsuspected large-fraction irradiation and total doses of radiation that exceed the tolerance of critical structures.
Keywords: antibiotic agent; carbamazepine; fluorouracil; ibuprofen; mitomycin; morphine; prednisone; abdominal pain; aged; anus carcinoma; areflexia; arytenoid cartilage; Brown Sequard syndrome; cancer staging; case report; cervical spinal cord; cervical spinal cord injury; Clostridium difficile infection; computer assisted tomography; conference paper; lack of drug effect; dysphagia; dyspnea; electromyography; erythema; esophageal squamous cell carcinoma; external beam radiotherapy; female; femoral nerve; fever; hoarseness; human; inguinal region; kyphosis; larynx edema; leukocytosis; lordosis; lumbar spine; male; muscle weakness; myelography; nerve root compression; neuroimaging; neuromuscular transmission; nuclear magnetic resonance imaging; pain; pain severity; paresthesia; peripheral nerve injury; peripheral neuropathy; pinprick test; priority journal; radiation attenuation; radiation beam; radiation dose; radiation dose distribution; radiation dose fractionation; radiation field; radiation hazard; radiation injury; radiation myelopathy; radiation neuropathy; radiation response; radiation tolerance; sensorimotor neuropathy; sensory dysfunction; skin exfoliation; sore throat; spondylolisthesis; stomach tube; tendon reflex; tracheoesophageal fistula; tracheostomy; treatment duration; vocal cord paralysis; walking difficulty; body weight loss; motrin; tegretol

Sleep onset hypoventilation in chronic spinal cord injury - Bascom A.T.; Sankari A.; Goshgarian H.G.; Badr M.S. (Physiological Reports, 2015)
A high prevalence of sleep-disordered breathing (SDB) after spinal cord injury (SCI) has been reported in the literature; however, the underlying mechanisms are not well understood. We sought to determine the effect of the withdrawal of the wakefulness drive to breathe on the degree of hypoventilation in SCI patients and able-bodied controls. We studied 18 subjects with chronic cervical and thoracic SCI (10 cervical, 8 thoracic SCI; 11 males; age 42.4 ± 17.1 years; body mass index 26.3 ± 4.8 kg/m2) and 17 matched ablebodied subjects. Subjects underwent polysomnography, which included quantitative measurement of ventilation, timing, and upper airway resistance (RUA) on a breath-by-breath basis during transitions from wake to stage N1 sleep. Compared to able-bodied controls, SCI subjects had a significantly greater reduction in tidal volume during the transition from wake to N1 sleep (from 0.51 ± 0.21 to 0.32 ± 0.10 L vs. 0.47 ± 0.13 to 0.43 ± 0.12 L; respectively, P < 0.05). Moreover, end-tidal CO2 and end-tidal O2 were significantly altered from wake to sleep in SCI (38.9 ± 2.7 mmHg vs. 40.6 ± 3.4 mmHg; 94.1 ± 7.1 mmHg vs. 91.2 ± 8.3 mmHg; respectively, P < 0.05), but not in able-bodied controls (39.5 ± 3.2 mmHg vs. 39.9 ± 3.2 mmHg; 99.4 ± 5.4 mmHg vs. 98.9 ± 6.1 mmHg; respectively, P = ns). RUA was not significantly altered in either group. In conclusion, individuals with SCI experience hypoventilation at sleep onset, which cannot be explained by upper airway mechanics. Sleep onset hypoventilation may contribute to the development SDB in the SCI population.
Keywords: adult; airway resistance; apnea hypopnea index; article; assisted ventilation; body mass; clinical article; controlled study; electroencephalography; end tidal carbon dioxide tension; female; forced expiratory volume; forced vital capacity; human; human experiment; hypoventilation; male; maximal expiratory pressure; peripheral vascular disease; polysomnography; quadriplegia; respiratory function; sleep disordered breathing; spinal cord injury; spirometry; tracheostomy; upper airway resistance syndrome

A case of acute pancreatitis secondary to spinal cord injury - Cao L.; Sun Y.; Lu Z.; Zhang P.; Yin L.; Li H.; Hua T.; Zheng Y. (Neuroendocrinology Letters, 2015)
Acute pancreatitis (AP) is a frequent and potentially life-threatening disease with high morbidity and mortality. The overall mortality of AP is approximately 5%. Alcohol consumption and gallstones are the main etiology of AP. Hypertriglyceridemia, idiosyncratic reactions to drugs, anatomic alterations and ascaris lumbricoides can also give rise to AP. Although spinal cord injury (SCI) can cause AP, however, the case of induced by cervical spine surgery has not been reported. A 61-year-old man with quadriplegic and respiratory distress received cervical spine surgery for spinal cervical spondylosis and multi-stage longitudinal ligament. He was admitted to intensive care unit (ICU) after tracheotomy for progressive dyspnea, one day after the cervical spine surgery. The patient was diagnosed with AP, in the absence of any identifiable causes of pancreatitis. He was treated with intravenous fluids, no oral feeding, enteral and parenteral nutrition, antibiotic and mechanical ventilation. The patient s condition gradually improved after the treatment. This case describes a case of postoperative cervical spondylosis that led to AP. In this report, we highlight the importance of early diagnosis and subsequent appropriate treatment. We conclude that the outcome can be favorable, if the treatment is appropriate.
Keywords: amylase; antibiotic agent; hemoglobin; infusion fluid; triacylglycerol lipase; abdominal distension; abdominal radiography; acute pancreatitis; adult; amylase blood level; antibiotic therapy; article; artificial ventilation; Babinski reflex; blood pressure measurement; body temperature; breathing rate; case report; cervical spondylosis; dyspnea; enteric feeding; erythrocyte count; follow up; hemoglobin blood level; hospital admission; human; intensive care unit; leukocyte count; male; middle aged; nuclear magnetic resonance imaging; operative blood loss; parenteral nutrition; pulse rate; quadriplegia; respiratory distress; sensory dysfunction; spinal cord injury; spine surgery; tracheotomy; triacylglycerol lipase blood level

Recurrent aneurysmal bone cyst of the cervical spine in childhood treated with doxycycline injection - Doyle A.; Field A.; Graydon A. (Skeletal Radiology, 2015)
A 12-year-old female was treated surgically for an aneurysmal bone cyst of the cervical spine involving the C2 and C3 vertebrae. The patient underwent surgery twice and further surgery was assessed as unfeasible. Treatment by intraosseous injection of doxycycline was uncomplicated and has resulted in resolution of the multifocal recurrent disease.
Keywords: doxycycline; iohexol; aneurysmal bone cyst; article; bone graft; bone radiography; bone transplantation; cancer radiotherapy; cancer surgery; case report; cervical spine; child; childhood disease; clinical assessment; computer assisted tomography; disease course; dura mater; dysphagia; dyspnea; female; general anesthesia; human; iliac crest; lymphedema; nasogastric tube; neck pain; nuclear magnetic resonance; ossification; postoperative care; postoperative complication; priority journal; rectum anterior resection; recurrent disease; school child; spine disease; spine surgery; tracheostomy; tumor recurrence; validation study; vertebral artery

Neuromuscular Disorders and Sleep in Critically Ill Patients - Irfan M.; Selim B.; Rabinstein A.A.; St Louis E.K. (Critical Care Clinics, 2015)
Sleep-disordered breathing (SDB) is a frequent presenting manifestation of neuromuscular disorders and can lead to significant morbidity and mortality. If not recognized and addressed early in the clinical course, SDB can lead to clinical deterioration with respiratory failure. The pathophysiologic basis of SDB in neuromuscular disorders, clinical features encountered in specific neuromuscular diseases, and diagnostic and management strategies for SDB in neuromuscular patients in the critical care setting are reviewed. Noninvasive positive pressure ventilation has been a crucial advance in critical care management, improving sleep quality and often preventing or delaying mechanical ventilation and improving survival in neuromuscular patients.
Keywords: amyotrophic lateral sclerosis; botulism; central sleep apnea syndrome; comorbidity; critical illness; critically ill patient; Duchenne muscular dystrophy; Eaton Lambert syndrome; endotracheal intubation; fascioscapulohumeral dystrophy; Guillain Barre syndrome; human; hypoventilation; Kennedy disease; limb girdle muscular dystrophy; lung function test; metabolic myopathy; mitochondrial encephalomyopathy; morbidity; mortality; muscular dystrophy; myasthenia gravis; myopathy; myositis; myotonic dystrophy; neuromuscular disease; noninvasive ventilation; polysomnography; positive end expiratory pressure; postpoliomyelitis syndrome; priority journal; REM sleep; review; sleep disordered breathing; spinal cord injury; symptom; tracheostomy

Feeding patients with tracheostomies - McRae J.; Fox R.; Morgan S. (Journal of the Intensive Care Society, 2015)
Keywords: cervical spinal cord injury; clinical decision making; critically ill patient; dysphagia; human; larynx; letter; nasogastric tube; speech therapy; swallowing; tracheostomy

An instrument approach to airway management - Thomson D.P.; Braude D.; High K.; Miller-Tester R. (Air Medical Journal, 2015)
Aviation terminology and thought processes are commonly applied to medicine. We further propose the adaptation of instrument flight terminology to emergency airway management including the aviation approach plate visual aid and replacement of the term "failed airway" with "missed airway,"
Keywords: airway creation device; airway obstruction; article; assisted ventilation; aviation; emergency airway management; emergency care; emergency health service; endotracheal intubation; head injury; health care personnel; human; hypercapnia; hypocapnia; hypoxemia; laryngoscopy; manual emergency ventilator; morbidity; mortality; oxygenation; patient positioning; positive end expiratory pressure; practice guideline; priority journal; respiration control; resuscitation; spinal cord injury; survival; tracheotomy

Influences of chronic obstructive pulmonary disease on outcomes of total arch replacement - Miyahara S.; Nakai H.; Izawa N.; Yamanaka K.; Sakamoto T.; Nomura Y.; Inoue T.; Matsumori M.; Okada K.; Okita Y. (Annals of Thoracic Surgery, 2015)
Background Although an association between chronic obstructive pulmonary disease (COPD) and adverse surgical outcomes has been proposed, the impact of COPD severity on postoperative outcomes remains unclear. Our objective was to analyze the prognostic implication of COPD severity on outcomes after total aortic arch replacement. Methods Between October 1999 and December 2012, 269 patients undergoing total arch replacement through median sternotomy, who were elective cases with preoperative spirometry records, were retrospectively reviewed. Patients were divided into four groups: control group, with ratio of forced expiratory volume of air in 1 second (FEV1) to forced vital capacity (FVC) of 70% or greater; mild airflow obstruction, with FEV1/FVC ratio less than 70% and FEV1 80% or greater of predicted; moderate airflow obstruction, FEV1/FVC ratio less than 70% and FEV1 50% to 79% of predicted; severe airflow obstruction, FEV1/FVC ratio less than 70% and FEV1 less than 50% of predicted. Symptoms of functional dyspnea and disability were also assessed. Multivariate logistic and Cox regression methods were used to determine if there was an independent association between COPD and short-term and long-term outcomes, respectively. Results The in-hospital mortality rate was 2.2% (6 of 269). A consistent trend of increasing frequency of postoperative respiratory complications with advanced airflow obstruction was noted. In multivariate analysis, in-hospital mortality (p = 0.022), incidence of respiratory complications (p = 0.021) and overall mortality (p = 0.025) was significantly associated with the symptoms of COPD, respectively. Conclusions The severity of COPD as defined by spirometry and symptoms of functional dyspnea may be an important prognostic marker of patients undergoing total arch replacement.
Keywords: aged; airway obstruction; aortic reconstruction; aortic arch surgery; article; brain hemorrhage; breathing disorder; cerebrovascular accident; chronic obstructive lung disease; controlled study; death; disability; disease severity; dyspnea; female; forced expiratory volume; forced vital capacity; heart arrest; atrial fibrillation; heart muscle ischemia; hemodialysis; hoarseness; human; incidence; intestine necrosis; Japan; kidney failure; major clinical study; male; mediastinitis; morbidity; mortality; multiple organ failure; neurological complication; outcome assessment; pneumonia; postoperative complication; prevalence; priority journal; prognosis; recurrent laryngeal nerve palsy; respiratory function; retrospective study; sepsis; smoking; spinal cord injury; spirometry; sternotomy; surgical mortality; thoracoabdominal aortic replacement; trachea stoma; tracheostomy; transient neurologic deficit; tympanic temperature; vocal cord paralysis; wound infection

Spinal muscular atrophy associated with progressive myoclonic epilepsy: A rare condition caused by mutations in ASAH1 - Rubboli G.; Veggiotti P.; Pini A.; Berardinelli A.; Cantalupo G.; Bertini E.; Tiziano F.D.; D'Amico A.; Piazza E.; Abiusi E.; Fiori S.; Pasini E.; et al. (Epilepsia, 2015)
Objective To present the clinical features and the results of laboratory investigations in three patients with spinal muscular atrophy associated with progressive myoclonic epilepsy (SMA-PME), a rare condition caused by mutations in the N-acylsphingosine amidohydrosilase 1 (ASAH1) gene. Methods The patients were submitted to clinical evaluation, neurophysiologic investigations (that included wakefulness and sleep electroencephalography [EEG], video-polygraphic recording with jerk-locked back-averaging, multimodal evoked potentials, and electromyography), brain magnetic resonance imaging (MRI), biochemical screening, muscle and skin biopsies, and molecular genetic analysis. Results The main clinical features were onset in childhood with proximal muscular weakness, generalized epilepsy with absences and myoclonic seizures, cognitive impairment of variable degree; the course was progressive with muscle wasting and uncontrolled epileptic seizures. In one patient, earlier onset before the age of 2 years was associated with a more complex clinical picture, with abnormal eye movements, progressive cognitive impairment, and a more rapid and severe course. EEG/polygraphic data were consistent with PME, demonstrating generalized spike-and-wave discharges, evidence of positive and negative myoclonia, and prominent photosensitivity. In one patient, transcranial magnetic stimulation showed a hyperexcitable motor cortex, whereas somatosensory evoked potentials were unaffected. Possible involvement of the central acoustic and visual pathways was suggested by abnormal auditory and visual evoked potentials. Muscle biopsies showed typical signs of neurogenic damage. Molecular genetic analysis showed mutations of the ASAH1 gene. Significance Our data indicate that SMA-PME associated with ASAH1 mutations is a genetically distinct condition with specific clinical and neurophysiologic features. Further studies are warranted to explore the role of the ASAH1 gene in muscle and brain function.
Keywords: barbituric acid derivative; ceramide; clonazepam; creatine kinase; ethosuximide; etiracetam; felbamate; glucosaminidase; lamotrigine; lorazepam; lysosome enzyme; mitochondrial DNA; n acylsphingosine amidohydrosilase 1; piracetam; unclassified drug; valproic acid; zonisamide; absence; adult; anticonvulsant therapy; article; ataxic gait; biochemical analysis; brain function; bronchus secretion; case report; child; clinical evaluation; clinical feature; cognitive defect; disease association; drug megadose; dyskinesia; dysphagia; electroencephalogram; electroencephalography; electromyography; epileptic discharge; auditory evoked potential; somatosensory evoked potential; visual evoked potential; eye movement disorder; fasciculation; female; frameshift mutation; gait disorder; gene expression assay; gene mutation; generalized epilepsy; genetic analysis; Griffiths Mental Development scale; human; hypotrophy; intelligence quotient; lung infection; mental development; mental development assessment; missense mutation; molecular genetics; motor cortex; motor performance; multichannel recorder; muscle atrophy; muscle biopsy; muscle function; muscle weakness; myoclonus; myoclonus epilepsy; myoclonus seizure; neuroimaging; neurologic disease; neurologic examination; neurophysiological monitoring; neuropsychological test; nonsense mutation; nuclear magnetic resonance imaging; onset age; percutaneous endoscopic gastrostomy; photosensitivity; preschool child; priority journal; real time polymerase chain reaction; school child; scoliosis; seizure; skin biopsy; spike wave; spinal muscular atrophy; tongue disease; tongue fasciculation; tonic clonic seizure; tracheostomy; transcranial magnetic stimulation; uncontrolled epileptic seizure; visual system; wakefulness

Severe transient pharyngeal paralysis following C2 fracture repair - Cumpston E.C.; Bock J.M. (Annals of Otology, Rhinology and Laryngology, 2015)
Objectives: We report a case of isolated pharyngeal plexus injury following posterior plating of a C2 fracture in an 84-year-old white male. Methods: Methods include a case report with literature review. Results: The patient presented with dense pharyngeal paralysis and inability to initiate swallowing but maintained true vocal cord movement. The patient required gastrostomy tube feeding for 3 months but eventually recovered his swallowing function. Prior literature on acute pharyngeal paralysis following upper cervical spine fracture repairs is reviewed with recommendations for care of this unique patient population. Conclusions: Traumatic C2 fracture repair can lead to isolated dense pharyngeal paralysis due to pharyngeal plexus injury with subsequent severe dysphagia. Observation may be appropriate in this population, with consideration of tracheotomy for recurrent aspiration.
Keywords: bone screw; aged; article; aspiration pneumonia; bone screw; case report; cervical collar; computer assisted tomography; coughing; dysphagia; dysphonia; falling; fever; hospital readmission; human; male; nuclear magnetic resonance imaging; odontoid process fracture; paralysis; percutaneous endoscopic gastrostomy; pharyngeal paralysis; pharynx disease; posterior spine fusion; priority journal; recurrent aspiration; swallowing; thorax radiography; very elderly; vocal cord; vocal cord paralysis; voice disorder

International guidelines for the management and treatment of Morquio a syndrome - Hendriksz C.J.; Berger K.I.; Giugliani R.; Harmatz P.; Kampmann C.; Mackenzie W.G.; Raiman J.; Villarreal M.S.; Savarirayan R. (American Journal of Medical Genetics, Part A, 2015)
Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder associated with skeletal and joint abnormalities and significant non-skeletal manifestations including respiratory disease, spinal cord compression, cardiac disease, impaired vision, hearing loss, and dental problems. The clinical presentation, onset, severity and progression rate of clinical manifestations of Morquio A syndrome vary widely between patients. Because of the heterogeneous and progressive nature of the disease, the management of patients with Morquio A syndrome is challenging and requires a multidisciplinary approach, involving an array of specialists. The current paper presents international guidelines for the evaluation, treatment and symptom-based management of Morquio A syndrome. These guidelines were developed during two expert meetings by an international panel of specialists in pediatrics, genetics, orthopedics, pulmonology, cardiology, and anesthesia with extensive experience in managing Morquio A syndrome.
Keywords: bronchodilating agent; dipeptidyl carboxypeptidase inhibitor; elosulfase alfa; fluoride; influenza vaccine; placebo; Pneumococcus vaccine; abdominal disease; anesthesia; body growth; cardiovascular disease; clinical classification; dental procedure; disease association; drug efficacy; endurance; enzyme replacement; eye disease; eye surgery; health care delivery; health care personnel; health care planning; hearing aid; hearing impairment; heart valve replacement; human; Morquio syndrome; musculoskeletal disease; neurologic disease; orthopedic surgery; pain; patient care; patient monitoring; perioperative period; phase 1 clinical trial (topic); phase 2 clinical trial (topic); phase 3 clinical trial (topic); physical examination; physiotherapy; positive end expiratory pressure; practice guideline; priority journal; quality of life; respiratory tract disease; review; risk assessment; spinal cord decompression; stomach surgery; tooth disease; tracheostomy; treatment duration; treatment indication; vaccination

A novel role for otolaryngologists in the multidisciplinary difficult airway response team - Hillel A.T.; Pandian V.; Mark L.J.; Clark J.; Miller C.R.; Haut E.R.; Cover R.; Berkow L.C.; Agrawal Y.; Bhatti N. (Laryngoscope, 2015)
Objectives/Hypothesis: The Difficult Airway Response Team (DART) was implemented in July 2008 to address emergent difficult airway situations. The main objective of this study was to highlight the unique role and skill set that otolaryngologists bring and their impact on patient outcomes. Study Design: Retrospective review of prospectively collected data from the hospital's airway registry. Methods: We collected data on demographics, airway characteristics, airway management techniques used by each specialty, and clinical outcomes (such as cricothyrotomies) for patients for whom a code was activated between July 2006 and June 2010. We compared data between pre- and post-DART cohorts and between DART and non-DART patients using a matched case-control approach. Results: Of the 2,826 codes, 90 patients required DART management between July 2008 and June 2010. Body mass index, cervical spine injury/fixation, history of difficult airway, head and neck mass, and oropharyngeal and/or supraglottic angioedema were identified as significant predictors for DART activation. Forty-nine (60%) patients' airways were secured by anesthesiologists, 30 (36%) by otolaryngologists, and three (4%) by trauma surgeons. Otolaryngologists were able to use specialized techniques such as Holinger and Dedo laryngoscopes to significantly decrease the number of cricothyrotomies from seven (0.73%) pre-DART implementation to four (0.21%) post-DART implementation. Conclusions: Otolaryngologists were able to decrease the need for cricothyrotomies using specialized techniques for patients with difficult airways. Otolaryngologists bring a special skill set to the DART that is beyond the scope of anesthesiologists and trauma surgeons and that can improve patient outcomes by preventing unnecessary emergency surgical airways.
Keywords: adult; airway constriction; ambulatory care; anesthesist; angioneurotic edema; article; body mass; cervical spine injury; cohort analysis; comparative study; controlled study; female; general condition; head and neck tumor; human; intensive care unit; laryngoscope; major clinical study; male; medical history; middle aged; morbid obesity; nasotracheal intubation; oropharyngeal angioedema; otolaryngologist; priority journal; professional competence; professional practice; rapid response team; respiration control; retrospective study; supraglottic angioedema; tracheotomy; videolaryngoscope

Intubations and airway management: An overview of Hassles through third millennium - Alanazi A. (Journal of Emergencies, Trauma and Shock, 2015)
Background: The placement of a tube into a patient's trachea "the intubation" as we call is not as simple as it looks. It is a very tricky and tedious maneuver that entails skills to assess and perform. Nevertheless, often this is left to the chores of inefficient hands due to a paucity of the availability of experts. They seldom are able to complete the task and often wind up calling the attention of the unit. The present review is an attempt to describe the need to undertake intubation, the procedures and techniques, the complications, including morbidity and mortality and airway management. This overview includes explicit descriptions of the difficult airway which represents multifaceted interface amid patient factors, clinical setting, and skills of the practitioner. Materials and Methods: To accomplish the target, peer-reviewed English language articles published during third millennium up to 2013 were selected from Pub Med, Pub Med Central, Science Direct, Up-to-date, Med Line, comprehensive databases, Cochrane library, and the Internet (Google, Yahoo). Review of Literature: The review constituted a systematic search of literature on the requirements that necessitate the practice of intubation, different techniques that facilitate easy conduct of procedure, the complications, including, morbidity and mortality, and the airway management. Conclusion: Recording every single detail has been beyond the scope of this review, however; some aspects have been wrapped up in nutshell. Some areas of the review are too basic which the medics are well aware of and knowledgeable. Nevertheless, these are difficult to be dispensed with in consideration of their source to the awareness of a common man and a great majority of the patients.
Keywords: 2 propanol; epinephrine; anesthetic agent; chloroxylenol; ketamine; neurotoxin; phenol derivative; phenytoin; quetiapine; salbutamol; topiramate; vegetable oil; ziprasidone; acute heart infarction; airway obstruction; article; artificial ventilation; asthma; brain injury; bronchoscopy; cerebrovascular accident; cervical spinal cord injury; childhood cancer; chronic obstructive lung disease; coma; congenital diaphragm hernia; croup; double lumen tube; drug exposure; drug intoxication; early intervention; echography; emergency; endotracheal intubation; endotracheal stent; endotracheal tube; esophagus cancer; esophagus obturator; Glasgow coma scale; hemoptysis; human; intubation; laryngeal mask; laryngeal tube; laryngoscopy; lymph node metastasis; myxedema; newborn disease; obesity; physician; priority journal; respiration control; respiratory tract injury; skill; subglottic stenosis; therapy delay; thoracotomy; thorax drainage; thyroid carcinoma; tracheostomy; tracheotomy; ventilator associated pneumonia

Conflicts of interest in orthopaedic surgery: The intertwining of orthopaedic surgery, peer review publications and corporate sponsorship - Dhillon K.S. (Malaysian Orthopaedic Journal, 2015)
Conflicts of interest in medicine has created deep concerns about the integrity of medicine and raised doubts about the trustworthiness of the medical professional. New stories of conflict of interest in medicine have become a commonplace. The interactions between the medical professional and the biomedical device as well as the pharmaceutical industry has become so pervasive that the primary interest of the medical professional in protecting and promoting the welfare of the patient has been compromised. The professional judgement and actions have been influenced by secondary interests, the major fungible and quantifiable being financial interest. The industry influence not only affects the way we practice orthopaedics but also affects medical education and peer review publications. Peer review publications have been shown to exaggerate benefits of the industry products while at the same time downplaying the risks. These conflicts of interest in orthopaedic surgery are particularly common in spinal and joint replacement surgery where joint replacement has been described as a ‘fashion trade’. The introduction of new products appears to be an uncontrolled experiment which has been hijacked by large corporations. This article explores the unhealthy pervasive interaction between the orthopaedic surgeon and the medical devices as well as the pharmaceutical industry. It highlights how the biomedical and the pharmaceutical industry dominate all aspects of the healthcare system. With its wealth and political clout, its influence is present everywhere, from the use of devices and drugs, research, publications, trials, education and even formulation of CGPs.
Keywords: bone morphogenetic protein 2; celecoxib; cyclooxygenase 2; cyclooxygenase 2 inhibitor; nonsteroid antiinflammatory agent; prostacyclin; rofecoxib; arthroplasty; article; bone development; controlled clinical trial (topic); drug industry; dysphagia; health economics; hoarseness; human; joint prosthesis; medical device; multicenter study (topic); orthopedic surgery; osteoarthritis; osteolysis; practice guideline; spinal cord injury; spine surgery; systematic review (topic); tracheotomy; urine retention; celebrex; ketorolac; oxycodone; pregablin; vioxx

Acute Onset of Guillain-Barré Syndrome after Elective Spinal Surgery - Boghani Z.; Livingston A.D.; Simpson E.P.; Holman P.J.; Grossman R.G. (World Neurosurgery, 2015)
Background Guillain-Barré syndrome (GBS) is an acute peripheral neuropathy caused by an autoimmune response against myelin of peripheral nerves. GBS has been reported after surgery, in general, and after spinal surgery, in particular. In most cases, GBS developed 1-3 weeks after surgery. Methods Report of 2 cases of GBS after elective spine surgery that developed in the immediate postoperative period. Results Within 1 and 3 hours after surgery, respectively, both patients developed ascending loss of motor and sensory function. They were taken back urgently to the operating room for wound exploration to ensure that an epidural hematoma had not developed. Cerebrospinal fluid studies and electromyography/nerve conduction velocity were then rapidly obtained and were compatible with acute inflammatory demyelinating polyradiculoneuropathy. Therapy was initiated with administration of intravenous immunoglobulin and plasmapheresis. Both patients made substantial motor recovery during the course of 1-2 years but have residual sensory abnormalities. Conclusions GBS developing acutely after spinal surgery is a rare occurrence but should be considered in the differential diagnosis of neurological deterioration after surgery. Rapid diagnosis and treatment are essential for recovery of neurological function.
Keywords: ganglioside GM1 antibody; immunoglobulin; acute disease; acute inflammatory demyelinating polyradiculoneuropathy; adult; antibody titer; article; case report; cerebrospinal fluid; cervical spine; echography; elective surgery; electromyography; electrophoresis; epidural hematoma; foraminotomy; Guillain Barre syndrome; hospital admission; hospitalization; human; immunotherapy; discectomy; laminectomy; leg pain; lumbar puncture; lumbar spine; male; middle aged; motor performance; nerve conduction; nerve decompression; neurologic examination; nuclear magnetic resonance imaging; operating room; peripheral neuropathy; peroneus nerve paralysis; personal experience; plasmapheresis; polyradiculoneuropathy; postoperative period; prostatectomy; protein cerebrospinal fluid level; radicular pain; sedation; spine surgery; subdural hematoma; tracheostomy; urine incontinence; wound closure

Hyperacute onset of guillain barre syndrome in the immediate postpartum period following Caesarean section under spinal anaesthesia - Vinay B.; Sonia B.; Bhadrinarayan V. (Indian Journal of Anaesthesia, 2015)
Keywords: adult; albuminocytological dissociation; case report; cerebrospinal fluid examination; cesarean section; clinical feature; dissociation; epidural hematoma; female; Guillain Barre syndrome; human; hyperreflexia; letter; limited mobility; muscle strength; myelitis; poliomyelitis; puerperium; quadriplegia; sensory nerve conduction; spinal anesthesia; tracheostomy; young adult

A cluster of acute flaccid paralysis and cranial nerve dysfunction temporally associated with an outbreak of enterovirus D68 in children in Colorado, USA - Messacar K.; Schreiner T.L.; Maloney J.A.; Wallace A.; Ludke J.; Oberste M.S.; Nix W.A.; Robinson C.C.; Glodé M.P.; Abzug M.J.; Dominguez S.R. (The Lancet, 2015)
Background: Clusters of acute flaccid paralysis or cranial nerve dysfunction in children are uncommon. We aimed to assess a cluster of children with acute flaccid paralysis and cranial nerve dysfunction geographically and temporally associated with an outbreak of enterovirus-D68 respiratory disease. Methods: We defined a case of neurological disease as any child admitted to Children's Hospital Colorado (Aurora, CO, USA) with acute flaccid paralysis with spinal-cord lesions involving mainly grey matter on imaging, or acute cranial nerve dysfunction with brainstem lesions on imaging, who had onset of neurological symptoms between Aug 1, 2014, and Oct 31, 2014. We used Poisson regression to assess whether the numbers of cases during the outbreak period were significantly greater than baseline case numbers from a historical control period (July 31, 2010, to July 31, 2014). Findings: 12 children met the case definition (median age 11.5 years [IQR 6.75-15]). All had a prodromal febrile illness preceding neurological symptoms by a median of 7 days (IQR 5.75-8). Neurological deficits included flaccid limb weakness (n=10; asymmetric n=7), bulbar weakness (n=6), and cranial nerve VI (n=3) and VII (n=2) dysfunction. Ten (83%) children had confluent, longitudinally extensive spinal-cord lesions of the central grey matter, with predominant anterior horn-cell involvement, and nine (75%) children had brainstem lesions. Ten (91%) of 11 children had cerebrospinal fluid pleocytosis. Nasopharyngeal specimens from eight (73%) of 11 children were positive for rhinovirus or enterovirus. Viruses from five (45%) of 11 children were typed as enterovirus D68. Enterovirus PCR of cerebrospinal fluid, blood, and rectal swabs, and tests for other causes, were negative. Improvement of cranial nerve dysfunction has been noted in three (30%) of ten children. All ten children with limb weakness have residual deficits. Interpretation: We report the first geographically and temporally defined cluster of acute flaccid paralysis and cranial nerve dysfunction in children associated with an outbreak of enterovirus-D68 respiratory illness. Our findings suggest the possibility of an association between enterovirus D68 and neurological disease in children. If enterovirus-D68 infections continue to happen in an endemic or epidemic pattern, development of effective antiviral or immunomodulatory therapies and vaccines should become scientific priorities.
Keywords: immunoglobulin G1; methylprednisolone; anterior horn cell; article; central gray matter; child; childhood disease; clinical article; cranial nerve paralysis; disease association; Enterovirus; enterovirus D68 infection; Enterovirus infection; epidemic; flaccid paralysis; gastrostomy; human; International Classification of Diseases; medical record review; patient history of heart transplantation; plasmapheresis; pleocytosis; positive end expiratory pressure; priority journal; school child; tracheostomy; treatment duration; United States

Chromobacterium haemolyticum pneumonia possibly due to the aspiration of runoff water - Takenaka R.; Nureki S.-I.; Ueno T.; Shigemitsu O.; Miyazaki E.; Kadota J.-I.; Miki T.; Okada N. (Japanese Journal of Infectious Diseases, 2015)
Here, we report a case of pneumonia possibly caused by the aspiration of runoff water containing Chromobacterium haemolyticum. A 69-year-old man became intoxicatedwithalcohol,fell into a ditch, and lost consciousness after suffering a blow to the head. The lower half of his body was completely paralyzed because of damage to his spinal cord, and he aspirated runoff water from the ditch. Chest computed tomography scans revealed consolidation in the right upper lobe and bilateral lower lobes. A sputum culture detected gram-negative bacteria that was identified as C. haemolyticum. Antibacterial chemotherapy was initiated, and the clinical course was favorable. To the best of our knowledge, this is the first report of C. haemolyticum pneumonia in the literature.
Keywords: meropenem; piperacillin plus tazobactam; runoff; sultamicillin; aged; alcohol intoxication; article; artificial ventilation; aspiration pneumonia; bacterium identification; cardiopulmonary arrest; case report; Chromobacterium haemolyticum pneumonia; Chromobacterium violaceum; computer assisted tomography; consciousness disorder; coughing; drug eruption; fever; human; male; minimum inhibitory concentration; paralysis; pneumonia; polymerase chain reaction; pulsed field gel electrophoresis; sputum culture; thorax radiography; tracheotomy

A patient with refractory shock induced by several factors, including obstruction because of a posterior mediastinal hematoma - Obinata M.; Ishikawa K.; Osaka H.; Mishima K.; Omori K.; Oode Y.; Yanagawa Y. (American Journal of Emergency Medicine, 2015)
A 44-year-old man who drove a motorcycle experienced a collision with the side of another motorcycle. Because he had sustained a highenergy injury to the spinal cord, he was transferred to our hospital. His circulation was unstable, and received tracheal intubation in addition to thoracostomy for the hemothorax. Whole-body computed tomography (CT) revealed multiple fractures, right hemopneumothorax with pulmonary contusion, and minor liver injury. After infusing 5000 mL of lactated Ringer's solution and 10 units of blood, his circulation remained unstable. On a repeat CT examination, the left atriumwas found to be compressed by a posterior mediastinal hematoma induced by the fracture of the thoracic spine, and a diagnosis of shock induced by multiple factors, including hemorrhagic, neurogenic, and obstructive mechanisms, was made. After obtaining stable circulation and respirations, internal fixation of the extremities and extubation were performed on the 12th hospital day. Chest CT performed on the 27th day showed the disappearance of compression of the left atrium by the hematoma.
Keywords: contrast medium; dopamine; noradrenalin; Ringer lactate solution; vasopressin; adult; article; aspiration; atelectasis; blood transfusion; case report; clavicle fracture; compression; computer assisted tomography; conservative treatment; elevated blood pressure; endotracheal intubation; exercise tolerance; extubation; fibula fracture; forward heart failure; heart tamponade; hematoma; hematopneumothorax; hematothorax; human; hypovolemia; intensive care unit; liver injury; lung contusion; male; mediastinum disease; multiple trauma; osteosynthesis; positive end expiratory pressure; posterior mediastinal hematoma; priority journal; quadriplegia; refractory shock; respiratory function; rib fracture; shock; shoulder fracture; spinal cord injury; spine injury; systolic blood pressure; tension pneumothorax; thorax drainage; tracheostomy; traffic accident; whole body CT

Mechanical insufflation-exsufflation: Practice patterns among respiratory therapists in Ontario - Prevost S.; Brooks D.; Bwititi P.T. (Canadian Journal of Respiratory Therapy, 2015)
BACKGROUND: The mechanical insufflator-exsufflator (MIE) is effective in assisting cough and in helping to avoid unplanned hospitalizations, tracheostomy and long-term ventilation in patients with neuromuscular disease or spinal cord injury. Despite this, the availability and usage of the device in Canada is unknown. OBJECTIVE: To investigate practice patterns and availability of the MIE in Ontario hospitals. METHODS: A cross-sectional, self-administered mail survey was sent to a random sample of 400 respiratory therapists practicing in 96 Ontario hospitals. RESULTS: A total of 114 (28%) completed surveys were returned from 62 (65%) hospitals. Twenty (32%) hospitals had a MIE. The respiratory therapist was the predominant health care provider using the MIE. The device was most commonly used in the intensive care unit, and medical/ surgical units in patients with neuromuscular diseases or spinal cord injuries. Optimal pressure spans of 35 cmH2O to 40 cmH2O were used by 54% of respondents. Fourteen of the 20 hospitals with an MIE had policies or guidelines in place, and four of these hospitals had established staff competencies. Measurements of peak cough flow, maximal inspiratory/expiratory pressure and vital capacity were reported to be infrequently performed. CONCLUSIONS: The present study demonstrated that the MIE device is not widely available in Ontario hospitals and there are variations in how the devices are applied, possibly resulting in suboptimal therapy. A comprehensive educational program about MIE devices that incorporates best practices and a practical component is recommended for current providers as well as for inclusion in student curricula.
Keywords: adult; aeration; amyotrophic lateral sclerosis; article; artificial ventilation; atelectasis; bronchiectasis; Canada; chronic obstructive lung disease; coughing; cross-sectional study; cystic fibrosis; female; Guillain Barre syndrome; health care policy; human; intensive care unit; lung fibrosis; male; mechanical insufflation exsufflation; medical practice; middle aged; multicenter study; muscular dystrophy; neuromuscular disease; practice guideline; random sample; respiratory therapist; spinal cord injury; vital capacity

Development and validation of a risk prediction model for tracheostomy in acute traumatic cervical spinal cord injury patients - Hou Y.F.; Lv Y.; Zhou F.; Tian Y.; Ji H.Q.; Zhang Z.S.; Guo Y. (European Spine Journal, 2014)
Purpose: Tracheostomy may become indispensable for patients with acute traumatic cervical spinal cord injuries. However, the early prediction of a tracheostomy is often difficult. Previous prediction models using the pulmonary function test (PFT) have limitations because some severely injured patients could not provide acceptable PFT results. We aim to develop an alternative model for predicting tracheostomy using accessible data obtained from the bedside. Method: Clinical, neurological and radiological data from 345 consecutive patients with acute tetraplegia were retrospectively reviewed. We applied multiple logistic regression analysis (MLRA) and classification and regression tree (CART) analysis to develop the prediction model for tracheostomy. By train-test cross-validation, we used the sensitivity, specificity, area under the receiver operating characteristics curve (AUC) and correction rate to evaluate the performance of these models. Results: According to the American Spinal Injury Association (ASIA) standards, an admission ASIA motor score (AAMS) ≤ 22, ASIA grade A and presence of respiratory complications were identified as independent predictors of tracheostomy by both models. The model derived by CART suggested that the highest signal change (HSC) in the spinal cord on magnetic resonance imaging (MRI) also affected a patient’s requirement for a tracheostomy, while MLRA demonstrated that tracheostomy was also influenced by the presence of an ASIA grade B injury. The CART model had a sensitivity of 73.7 %, specificity of 89.7 %, AUC of 0.909 and overall correction rate of 87.3 %. The sensitivity, specificity, AUC and correction rate of the MLRA model were 81.8, 86.4, 0.889 and 85.7 %, respectively. Conclusions: We suggest using the CART model in clinical applications. Patients with AAMS ≤ 1 exhibit an increased likelihood of requiring a tracheostomy. For patients with an AAMS in the range of 2–22, surgeons should consider giving these patients a tracheostomy once respiratory complications occur. Surgeons should be cautious to give a tracheostomy to patients with an AAMS ≥ 23, if the patient experiences an incomplete spinal cord injury and the HSC in the spinal cord is at C3 level or lower based on MRI. For other patients, close observation is necessary; generally, patients with complete SCI might require a tracheostomy more frequently.
Keywords: American; American Spinal Injury Association impairment scale; cervical spinal cord injury; classification; human; injury; logistic regression analysis; lung function test; model; multivariate logistic regression analysis; nuclear magnetic resonance imaging; patient; prediction; quadriplegia; receiver operating characteristic; risk; spinal cord; spinal cord injury; spine injury; surgeon; tracheostomy; tree

Endoscopic transnasal odontoidectomy without resection of nasal turbinates: Clinical outcomes of 13 patients - Yen Y.-S.; Chang P.-Y.; Huang W.-C.; Wu J.-C.; Liang M.-L.; Tu T.-H.; Cheng H. (Journal of Neurosurgery: Spine, 2014)
Object: The goal of the study was to report a series of consecutive patients who underwent endoscopic transnasal odontoidectomy (ETO) without resection of nasal turbinates. The techniques for this minimally invasive approach are described in detail. Methods: The authors conducted a retrospective review of consecutive patients who underwent ETO for basilar invagination. All the patients had myelopathy caused by compression at the cervicomedullary junction, which required surgical decompression. Preoperative and postoperative data, including those from radiographic and clinical evaluations, were compared. Morbidity and mortality rates for the procedure are also reported in detail. Results: Thirteen patients (6 men and 7 women) with a mean age of 52.7 years (range 24-72 years) were enrolled. The basilar invagination etiologies were rheumatoid arthritis (n = 5), trauma (n = 4), os odontoideum (n = 2), ankylosing spondylitis (n = 1), and postinfectious deformity (n = 1). The average follow-up duration was 51.2 months (range 0.3-105 months). One patient died 10 days after the operation as a result of meningitis caused by CSF leakage. Among the other 12 patients, the average postoperative Nurick grade (3.2) was significantly improved over that before the operation (4.1, p = 0.004). The mean (± SD) duration of postoperative intubation was 1.5 ± 2.1 days, and there was no need for perioperative tracheostomy or nasogastric tube feeding. There also was no postoperative velopharyngeal insufficiency. There were 6 (46%) intraoperative and 2 (15%) postoperative CSF leaks in the 13 patients in this series. Conclusions: ETO is a viable and effective option for decompression at the ventral cervicomedullary junction. This approach is minimally invasive and causes little velopharyngeal insufficiency. The pitfall of this approach is the difficulty in repairing dural defects and subsequent CSF leakage.
Keywords: adult; aged; ankylosing spondylitis; article; bone malformation; cerebrospinal fluid rhinorrhea; clinical article; clinical evaluation; controlled study; drainage catheter; endoscopic surgery; endoscopic transnasal odontoidectomy; female; follow up; human; male; medical record review; meningitis; middle aged; minimally invasive surgery; morbidity; multiple organ failure; nasogastric tube; nose feeding; Nurick (grade); odontoid process; odontoid process fracture; outcome assessment; palatopharyngeal incompetence; peroperative complication; postoperative complication; postoperative period; preoperative evaluation; respiratory tract intubation; retrospective study; rheumatoid arthritis; sepsis; spinal cord decompression; spinal cord disease; spine surgery; Streptococcus pneumonia; surgical mortality; tracheostomy; treatment outcome; vertebra dislocation

Five-level cervical corpectomy for neurofibromatosis-associated spinal deformity: case report - Parker S.L.; Wolinsky J.P.; Tufaro A.P.; Gokaslan Z.L.; Witham T.F. (European Spine Journal, 2014)
Purpose: To describe a successful five-level cervical corpectomy and circumferential reconstruction in a patient with a plexiform neurofibroma causing a severe kyphotic deformity. Methods: Case report. Results: 43-year-old man with history of Neurofibromatosis presented with signs and symptoms of myelopathy with spastic lower extremities and gait difficulties. Imaging studies demonstrated a severe kyphotic deformity of the cervical spine with associated cord compression secondary to an anteriorly positioned plexiform neurofibroma. Two-stage surgical procedure was designed to treat this lesion. Stage I consisted of tracheostomy placement, transmandibular, circumglossal approach to the anterior cervical spine, C2–C6 corpectomies, and C1–C7 reconstruction with a custom titanium cage/plate. Stage II consisted of suboccipital craniectomy, C1–C2 laminectomies, and occipital-cervical thoracic instrumented fusion (O-T8). There were no operative complications, but the patient did develop a small pulmonary embolism post-operatively treated with anticoagulation. Patient required two-weeks of inpatient rehabilitation following surgery. Gastrostomy tube and tracheostomy were successfully discontinued with preserved swallowing and respiratory function. Patient-reported outcome measurements revealed significant and sustained improvement post-operatively. Conclusions: Five-level cervical corpectomy including C2 can be safely and successfully performed via a transmandibular, circumglossal approach. Circumferential reconstruction utilizing a custom anterior titanium cage and plate system manufactured from a pre-operative CT scan was utilized in this case. Long segment occipital-cervical-thoracic reconstruction is recommended in such a case. Using such a technique, improvement in myelopathy, correction of deformity, and improved quality of life can be achieved.
Keywords: case report; deformity; human; neurofibromatosis; spine malformation

Decanulation of patients with severe respiratory muscle insufficiency: efficacy of mechanical insufflation-exsufflation - Bach J.R.; Saporito L.R.; Shah H.R.; Sinquee D. (Journal of rehabilitation medicine, 2014)
OBJECTIVE: Ventilator dependent patients with neuromuscular disorders and high level spinal cord injury have been extubated and decanulated to continuous noninvasive intermittent positive pressure ventilatory support after mechanical insufflation-exsufflation was used to achieve specific criteria for tube removal. The purpose of this study is to report changes in extent of need for ventilator use and in vital capacity related to mechanical insufflation-exsufflation used via tracheostomy tubes and post-decanulation via oronasal interfaces. METHODS: Upon presentation patients were placed on fiO2 21% and CO2 was normalized by adjusting ventilator settings as needed. The vital capacity (1st data point) and h/day of ventilator dependence were noted. Then mechanical insufflation-exsufflation was used via the tubes up to every 2 h until ambient air oxyhemoglobin saturation (SpO2) baseline remained ≥ 95% and other decanulation criteria were achieved. The vital capacity was re-measured (2nd data point) and the patient decanulated to continuous noninvasive intermittent positive pressure ventilatory support in ambient air as care providers used mechanical insufflation-exsufflation up to every 30 min to maintain SpO2 ≥ 95%. The vital capacity (3rd data point) and minimum hours/day of noninvasive intermittent positive pressure ventilatory support requirement during the next 3 weeks were recorded. RESULTS: The vital capacities of 61 tracheostomized ventilator users, 36 of whom were continuously dependent, increased significantly (p < 0.001) from presentation to immediately pre-decanulation and in the 3 weeks post-decanulation and all except one were successfully decanulated. CONCLUSION: Many ventilator users can be decanulated in outpatient clinics to continuous noninvasive intermittent positive pressure ventilatory support with mechanical insufflation-exsufflation used to increase vital capacity, SpO2, and autonomous ability to breathe.
Keywords: adult; aeration; artificial ventilation; artificial ventilation; breathing muscle; devices; female; human; intermittent positive pressure ventilation; male; middle aged; neuromuscular disease; pathophysiology; procedures; respiratory failure; spinal cord injury; tracheostomy; vital capacity

Respiratory-aided, long-term survival cases of amyotrophic lateral sclerosis (ALS) with communication abilities, motoneuron system-confined degeneration, and scanty TDP-43 aggregation-a subgroup of ALS? - Mochizuki Y.; Hayashi K.; Nakayama Y.; Shimizu T.; Kamide M.; Ogino M.; Komori T.; Isozaki E.; Nakano I. (Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2014)
Background: About 13% of patients (1) with ALS surviving on long-term mechanical ventilation (LTMV) develop into a totally locked-in state (TLS) (2), which refers to a state in which all voluntary movements are lost and communication by any means is impossible. However, it is known that some respirator-aided, long-term survival patients maintain communication abilities. The position that the latter group occupies along the spectrum of ALS cases has not been described. Objective: To clarify, along the ALS cases, the position of the long-surviving ALS subgroup with good communication abilities. Methods: Three autopsied ALS cases with an approximately 30-year survival and good communication abilities were clinicopathologically investigated in direct comparison with previously reported ALS cases suffering from a TLS (2, 3). Case reports: Clinical course: Age of onset and disease duration of cases 1, 2, and 3 were 48, 55, and 31, and 28, 29, and 33 years, respectively. Case 1 underwent a tracheostomy 8 years after disease onset, and required mechanical ventilation regularly 26 years after onset. Case 2 and case 3 underwent mechanical ventilation 14 and 6 years after disease onset, respectively. Case 1 and 3 were in a state of complete tetraplegia and case 2 was able to move only his knee joint a little, and the three cases were in a state of bulbar palsy. However they could communicate well via eye movements. Neuropathological findings: In all the cases, both upper and lower motor neurons were markedly degenerated while the brainstem tegmentum was preserved. Multiple system degeneration, a characteristic pathology of ALS in the TLS (2), was not seen. A few normal-looking motor neurons remained in the anterior horn of the spinal cord. Neither hypertrophic astrocytes nor macrophages were observed. There were no TDP-43-immunoreactive inclusions in the lower motor neurons of any case and only occasional ones in the cerebral cortex of case 3. Bunina bodies were not seen. Discussion and conclusion: Each of the present cases displayed a much slower disease progression into required respiratory assistance compared with typical ALS patients and also maintained good communication in spite of survival on LTMV. Neuropathological findings of remaining good-shaped motor neurons, motoneuron system-confined degeneration, and a few TDP-43-immunoreactive inclusions indicate a significant difference from findings of ALS in the TLS. Thus, our clinicopathological investigations of these three cases give rise to the possibility that there is a distinct subgroup characterized by the above-mentioned features.
Keywords: artificial ventilation; long term survival; amyotrophic lateral sclerosis; interpersonal communication; motoneuron; degeneration; human; patient; disease course; survival; quadriplegia; disease duration; onset age; brain cortex; case report; macrophage; astrocyte; spinal cord; pathology; tracheostomy; ventilator; knee; eye movement; bulbar paralysis; brain stem; tegmentum; spinal cord ventral horn; voluntary movement

Surgical strategies and outcomes of spinal ependymomas of different lengths: Analysis of 210 patients - Wang G. (Neuro-Oncology, 2014)
OBJECTIVE: The aim of this study was to investigate the surgical strategies and outcomes for spinal ependymomas of different lengths. METHODS: The authors used data from 210 patients with spinal ependymomas (WHO Grades II and III) in this 10-year retrospective study (January 1999 to December 2008), dividing them into 3 different groups according to length (spinal ependymomas < 5 cm, 5-10 cm, and > 10 cm). All patients underwent tumor resection. The basic characteristics of the patients were reviewed and the functional status was assessed using the McCormick classification. RESULTS: There were 89, 81, and 40 patients, respectively, in the 3 groups (< 5 cm, 5-10 cm, and > 10 cm). Grosstotal resections (GTRs) were performed in 172 patients (81.9% overall, or S6.5%, 79.0%, and 77.5% in the 3 groups, respectively). Subtotal and partial resections were achieved in 38 patients (18.1%). Eight patients with medulla oblongata or upper cervical cord tumors received a tracheotomy postoperatively. The follow-up period ranged from 56 to 176 months. One hundred thirty-five patients (76.7%) experienced improvement, (88.2%, 83.8%, and 34.4% in the < 5 cm, 5-10 cm, and > 10 cm groups, respectively). Thirty-three patients (18.8%) maintained their pretreatment status, and 8 patients (4.5%) showed deterioration following tumor resection at 6 months. CONCLUSIONS: Radical resection of spinal ependymomas could be performed in most patients, and the rate of GTR was significantly different in the different-length groups Patients with longer tumors had worse surgical results compared with those with small tumors and more postoperative neuropathic pain and proprioceptive deficits could usually be observed in patients harboring larger tumors. Early diagnosis and timely operation are critical to achieving better neurological outcomes. For tumors with dense adhesions, complete removal should be performed cautiously because of the significant incidence of neurological deterioration.
Keywords: human; society; oncology; patient; neoplasm; deterioration; cancer surgery; surgery; functional status; early diagnosis; cervical spinal cord; tracheotomy; follow up; medulla oblongata; radical resection; neuropathic pain; adhesion; classification; retrospective study

Institutional care for long-term mechanical ventilation in Canada: A national survey - Rose L.; McKim D.; Katz S.; Leasa D.; Nonoyama M.; Pedersen C.; Avendano M.; Goldstein R. (Canadian Respiratory Journal, 2014)
INTRODUCTION: No national Canadian data define resource require mcnts and care delivery for ventilator-assisted individuals (VAIs) requiring long-term institutional care. Such data will assist in planning health care services to this population. OBJECTIVE: To describe institutional and patient characteristics, prevalence, equipment used, care elements and admission barriers for VAIs requiring long-term institutional care. METHODS: Centres were identified from a national inventory and snow-ball referrals. The survey weblink was provided from December 2012 to April 2013. Weekly reminders were sent for six weeks. RESULTS: The response rate was 84% (54 of 64), with 44 adult and 10 pediatric centres providing data for 428 VAIs (301 invasive ventilation; 127 noninvasive ventilation [NIV]), equivalent to 1.3 VAIs per 100,000 population. An additional 106 VAIs were on wait lists in 18 centres. More VAIs with progressive neuromuscular disease received invasive ventilation than NIV (P<0.001); more VAIs with chronic obstructive pulmonary disease (P<0.001), obesity hypoventilation syndrome (P<0.001) and central hypoventilation syndrome (P=0.02) required NIV. All centres used positive pressure ventilators, 21% diaphragmatic pacing, 15% negative pressure and 13% phrenic nerve stimulation. Most centres used lung volume recruitment (55%), manually (71%) and mechanically assisted cough (55%). Lack of beds and provincial funding were common admission barriers. CONCLUSIONS: Variable models and care practices exist for institutionalized care of Canadian VAIs. Patient prevalence was 1.3 per 100,000 Canadians. © 2014 Pulsus Group Inc. All rights reserved.
Keywords: mechanical ventilator; adult; adult respiratory distress syndrome; aged; airway obstruction; Arnold Chiari malformation; article; artificial ventilation; Canada; cerebral palsy; cerebrovascular accident; chronic obstructive lung disease; congestive heart failure; coughing; Guillain Barre syndrome; health care survey; human; hydrocephalus; hypoventilation; injury; institutional care; invasive procedure; long term care; lung volume; major clinical study; mechanical ventilator; metabolic disorder; myasthenia gravis; negative pressure ventilator; nerve stimulation; neuromuscular disease; noninvasive ventilation; obesity hypoventilation syndrome; pediatric hospital; phrenic nerve; postpoliomyelitis syndrome; seizure; spinal cord injury; spinal dysraphism; trachea stenosis; tracheostomy; ventilator; ventilator associated pneumonia

Non invasive assessment of the effect of implantable phrenic nerve stimulation in two paediatric patients using structured light plethysmography - Morley S.; Khalid A.; Kimber K.; De Boer W.; Iles R. (American Journal of Respiratory and Critical Care Medicine, 2014)
Introduction: Patient A is a 17 year old girl who sustained a C1 complete spinal cord injury at the age of 9 years. Patient B is a 10 year old boy who sustained a complete C1 spinal injury at the age of 6 years. Both children show no respiratory drive and have been invasively ventilated via tracheostomy since the time of injury. Neither has any other significant pathology. Following assessment both patients underwent insertion of bilateral Atrostim Implantable Phrenic Nerve Stimulators (IPNS) in 2013. Methodology: The children were studied using Structured Light Plethysmography (SLP) (Thora3Di TM PneumaCare Ltd, UK). SLP enables non-invasive, non-contact assessment of the compartmental change of Rib Cage (RC) v Abdominal (AB) and Right (RH) v left (LH) hemi-thorax. The movement of a projected grid of light is analysed to derive a Konno-Mead loop (KM) from which KM Principal Angle (Phi), KM Spread, Overall phase (OPhi), Windowed phase (W phase), Phase breath (B phase) and a measure of variation of per-breath phase (B phase Ent) can be calculated. Each child was monitored in a sitting and supine position both on invasive ventilation and on IPNS. Patient B was reassessed at 75% and 100% IPNS. Results: RC v AB: In both patients, in both positions, Phi became more negative with IPNS, indicating a shift from RC to an AC / diaphragmatic volume recruitment. There was an increase in KM spread in the supine position, but either no change or a negative change in the sitting position. KM Spread and Phase measurements became positive indicating a more complex multi phased mode of volume recruitment. Measurements changed when Patient B was paced at 75% and 100% IPNS; Phi becoming more negative and the KM spread, decreased. B Phase Ent decreased in both positions indicating more respiratory stability. RH v LH: Patient A showed an increase in right sided ventilation in the sitting position following IPNS. There was a change in Phi between sitting and lying positions for both patients, however these changes were not the same for each patient. There was no difference in KM spread before or after IPNS in either patient. Conclusion: IPNS offers significant quality of life improvements for the patient, but effectiveness is often subjective. SLP offers a non-invasive method to objectively quantify and record the regional, idiosyncratic and positional effectiveness of IPNS, when compared to conventional ventilation and varying % IPNS support.
Keywords: phrenic nerve; nerve stimulation; patient; human; plethysmography; American; society; sitting; child; air conditioning; breathing; supine position; thorax; spinal cord injury; United Kingdom; injury; girl; male; tracheostomy; respiratory drive; pathology; nerve stimulator; methodology; spine injury; female; non invasive procedure; quality of life; boy

Stentgraft first staged repair of type 2 thoraco-abdominal aneurysm: The endovascular elephant trunk - Vivacqua A.; Idrees J.; Johnston D.R.; Soltesz E.; Svensson L.; Roselli E. (Interactive Cardiovascular and Thoracic Surgery, 2014)
Abstract Objectives: Repair of extensive thoraco-abdominal aneurysm carries high risk of death and morbidity, especially spinal cord ischaemia. Objectives were to investigate outcomes with a staged hybrid approach using a thoracic endovascular aortic repair (TEVAR) first in combination with later open abdominal repair for type 2 thoraco-abdominal aneurysm. Methods: From to 2001 to 2013, 22 patients underwent TEVAR first staged type 2 thoraco-abdominal repair for degenerative aneurysm and dissection. The first stage included descending aortic endovascular repair (endovascular elephant trunk), followed by completion repair with open replacement of the visceral and abdominal segment. At initial repair, all patients were deemed high risk for conventional open repair. Median interval between stages was 6.5 months. Mean age was 56 ± 15 years, 5 patients had connective tissue disorder and the mean maximum aortic diameter was 58 ± 16°mm preoperatively. Results: There was no death or major complication after initial TEVAR, but the operative mortality was 9% (n = 2) after open procedure. One patient underwent emergency repair and died due to intraoperative myocardial infarction and the other had disseminated intravascular coagulation. Other complications included paralysis (n = 1, 4.5%), tracheostomy (n = 2, 9%), dialysis (n = 1, 4.5%), and there was 1 reoperation for bleeding (4.5%). Median follow-up was 37 months and there were no late deaths, but 1 late reoperation for a new ascending dissection. Conclusion: Use of TEVAR as endovascular elephant trunk in combination with staged open repair is a safe and feasible approach for repair of extensive type 2 thoraco-abdominal aneurysm. Staged approach with TEVAR first may reduce morbidity in patients at high risk for extensive single stage aortic repair.
Keywords: elephant; thorax surgery; aneurysm; human; patient; endovascular aneurysm repair; risk; death; morbidity; dissection; reoperation; paralysis; disseminated intravascular clotting; heart infarction; emergency; procedures; surgical mortality; aorta; follow up; tracheostomy; bleeding; dialysis; connective tissue disease; ischemia; aortic reconstruction; spinal cord

Intrathecal baclofen withdrawal: A rare cause of high morbidity and mortality - Logue L.; Saini P.; Babinski K.; Russi T. (Chest, 2014)
INTRODUCTION: Baclofen withdrawal rarely occurs in patients receiving intrathecal baclofen (ITB). We report a patient with spasticity that quickly declined to circulatory shock requiring prolonged hospitalization. CASE PRESENTATION: 44 year-old man with T1 paraplegia and lower extremity spasticity, from prior gunshot needing an ITB pump, presented with episodic dyspnea, confusion, and lower extremity cramps for 2 days. He admitted to regular pump interrogations and refills. Exam revealed an anxious man with tachycardia, tachypnea, and hypertensive emergency. He had myoclonus, intact deep tendon reflexes, and absent voluntary motor function of his lower extremities. Labs showed leucocytosis, elevated creatinine kinase, and toxicology positive for cocaine. Oral baclofen and intravenous diazepam was futile. Rapid respiratory failure requiring intubation and mechanical ventilation, shock and hyperthermia (108°F) ensued. He was cooled, volume resuscitated, started on vasopressors, broad-spectrum antibiotics, benzodiazepines, and supra-therapeutic oral doses of baclofen. Injection of baclofen into the pump and trial of cyproheptadine were ineffective. Imaging and electroencephalography ruled out stroke and seizures. Renal failure requiring dialysis, limb ischemia requiring leg amputation, myocardial infarction, deep vein thrombosis and prolonged mechanical ventilation requiring tracheostomy complicated his hospitalization. Sedation was slowly withdrawn correlating with a decline in spasms. After 2 months, he followed simple commands and was discharged to rehabilitation. DISCUSSION: Spasticity occurs from alpha or gamma motor neuron inhibition after spinal injury. Baclofen is a gamma-aminobutyric acid type-B receptor agonist that binds presynaptically, decreasing calcium release resulting in decreased muscle tone1. ITB doses can be multi-fold higher than oral doses. ITB withdrawal from human or device error can be life-threatening. Patients may develop rigidity, seizures, hyperthermia, and shock. Multi-organ dysfunction, disseminated intravascular coagulation, and death can result. Intensivists must be cognizant of confounding etiologies such as cocaine use and neuroleptic malignant syndrome. Electroencephalogram aids in differentiating status dystonicus from status epilepticus. Treatment is baclofen replacement and supportive. In severe cases, early intubation, mechanical ventilation, benzodiazepines, anesthesia, and paralytics have reduced complications2. Patients refractory to replacement require slow down-titration of sedation and prolonged hospitalization. CONCLUSIONS: Severe cases are rare but need a heightened awareness in patients with myalgias and ITB pumps. Intensivists must have a low threshold for early intubation and intravenous sedation before the development of complications in such cases.
Keywords: baclofen; cocaine; benzodiazepine derivative; cyproheptadine; 4 aminobutyric acid; diazepam; receptor; hypertensive factor; antibiotic agent; creatine kinase; intrathecal drug administration; morbidity; mortality; human; patient; pump; intubation; leg; artificial ventilation; sedation; hospitalization; spasticity; intensivist; seizure; male; hyperthermia; myoclonus; shock; injection; emergency; tachypnea; calcium transport; spine injury; tachycardia; muscle cramp; respiratory failure; agonist; muscle; devices; etiology; epileptic state; imaging; tendon reflex; dyspnea; muscle spasm; tracheostomy; deep vein thrombosis; gunshot injury; heart infarction; leg amputation; paraplegia; limb ischemia; dialysis; rehabilitation; gamma motoneuron; toxicology; kidney failure; cerebrovascular accident; electroencephalography; rigidity; disseminated intravascular clotting; death; leukocytosis; motor performance; neuroleptic malignant syndrome; electroencephalogram; anesthesia; titrimetry; myalgia

Contemporary approach of thoraco-abdominal aortic repair using moderate to deep hypothermia combined with target reconstruction of the adamkiewicz artery - Tanaka H.; Minatoya K.; Sasaki H.; Oda T.; Itonaga T.; Kobayashi J. (Interactive Cardiovascular and Thoracic Surgery, 2014)
Objectives: We reviewed the surgical results of thoraco-abdominal aortic repair using moderate to deep hypothermia combined with target reconstruction of the Adamkiewicz artery Methods: Between 2006 and 2014, 100 patients underwent thoraco-abdominal aortic aneurysm repair using moderate to deep hypothermia. Mean age was 58 ± 15 years. Seventy-three (73%) were men. Aortic pathology was acute dissection in 5, chronic dissection in 74, degenerative in 20, and infection in 1. Thirty-four had connective tissue disorders. Five had emergent operation. The degree of repair was Crawford extent I in 8, II in 79, and III in 13. Four had concomitant arch repair. Preoperative magnetic resonance angiography or computed tomographic angiography was performed to detect the Adamkiewicz artery. We used deep hypothermia (18°C) requiring open proximal aortic anastomosis for cerebral protection, and moderate hypothermia (25°C) in patients not requiring cerebral protection Results: There were 2 patients with spinal cord injury and 2 with stroke. Of the patients with the identified Adamkiewicz artery (90%), all had target reconstruction of the artery. Mean number of pairs of reconstructed intercostal arteries was 1.5 ± 0.7. In-hospital mortality was 5%. The cause of death was lung bleeding in 2, infection in 2, and rupture of iliac aneurysm in 1. Temporary dialysis for new-onset renal failure was required in 9%. Mean duration of postoperative mechanical ventilation was 1.7 ± 1.9 days. Six had tracheostomy due to respiratory failure Conclusion: Moderate to deep hypothermia combined with target reconstruction of the Adamkiewicz artery brings satisfactory outcomes in thoraco-abdominal aortic repair, especially for spinal cord protection.
Keywords: hypothermia; artery; thorax surgery; descending aortic surgery; human; patient; protection; dissection; infection; connective tissue disease; anastomosis; spinal cord injury; computed tomographic angiography; pathology; abdominal aortic aneurysm; aneurysm; magnetic resonance angiography; lung hemorrhage; cerebrovascular accident; cause of death; mortality; rupture; dialysis; kidney failure; artificial ventilation; tracheostomy; respiratory failure; spinal cord; male

Open thoracoabdominal aortic repair for chronic type B dissection - Kouchoukos N.T.; Kulik A.; Castner C.F. (Journal of Thoracic and Cardiovascular Surgery, 2014)
Objectives: Advances in endovascular surgery have brought into question the role of open operative treatment of chronic thoracoabdominal aortic dissection. In this context, we evaluated our experience with open repair of this condition using a single operative technique. Methods: From January 1986 to January 2014, 69 patients with chronic thoracoabdominal aortic dissection underwent open repair using total cardiopulmonary bypass (CPB) and hypothermic circulatory arrest (HCA). The degree of repair was as follows: Crawford extent I, 13 patients (19%), Crawford extent II, 41 patients (59%), and Crawford extent III, 15 patients (22%). Thirty patients (43%) had Marfan or Loeys-Dietz syndrome. Fifty-three patients (77%) had previous operations on the thoracic or abdominal aorta. Results: The 30-day mortality rate was 5.8% (4 patients). Stroke occurred in 2 (3%) of 66 operative survivors, and spinal cord ischemic injury in 4 (6%). Temporary dialysis for new-onset renal failure was required in 4.5% of hospital survivors and tracheostomy in 10.6%. Survival after 1, 5, and 10 years was 87%, 65%, and 40%, respectively. Eighteen patients (26%) required a total of 20 subsequent operations on the thoracic or abdominal aorta of whom 15 had Marfan or Loeys-Dietz syndrome. Three of these procedures were for contiguous distal aortic disease and 10 were for patch aneurysms of the intercostal or visceral/renal arteries. Conclusions: Open thoracoabdominal aortic repair for chronic dissection using CPB and HCA can be accomplished with mortality and morbidity rates that are comparable with those reported for endovascular or hybrid techniques. Open repair should remain a viable and primary option for the management of this condition until the long-term effectiveness of alternative methods of treatment is clearly established. © 2014 The American Association for Thoracic Surgery.
Keywords: dissection; aortic reconstruction; human; patient; aortic dissection; survivor; mortality; Loeys Dietz syndrome; abdominal aorta; procedures; survival; hospital; kidney failure; dialysis; surgical technique; injury; spinal cord; heart arrest; cerebrovascular accident; tracheostomy; cardiopulmonary bypass; thorax surgery; morbidity; aortic disease; artery; aneurysm; surgery; endovascular surgery

Respiratory nursing interventions following tracheostomy in acute traumatic cervical spinal cord injury - Luo C.; Yang H.; Chen Y.; Zhang Z.; Gong Z. (Cell biochemistry and biophysics, 2014)
Tracheostomy is frequently performed in severe cervical spinal cord injury (SCI) patients with the pulmonary dysfunction. A series of respiratory nursing interventions are required to plan tracheostomy removal. Tracheostomy was performed in 29 patients after acute traumatic cervical SCI. A series of respiratory nursing interventions were introduced in these patients after closed tracheostomy and decannulation, including closed tracheostomy tube training, manually assisted cough. Chacheostomy was successfully removed in 21 patients after the respiratory nursing interventions. In contrast, eight patients died from associated injuries. The average time from tracheostomy to decannulation was 40 days (14-104 days), the average time from closed tracheostomy to decannulation was 18.80 ± 13.50 days. Second tracheostomy was performed in one patient after 29 days' removal due to pulmonary infection. One patient presented with delayed incision healing for 29 days. Closed tracheostomy tube training and manually assisted cough are key factors for tracheostomy removal, although intensive nursing are also needed. The time from tracheostomy to decannulation and from closed tracheostomy to decannulation is increased in case of "late" (>24 h) tracheostomy and longer mechanical ventilation.
Keywords: acute disease; adolescent; adult; aged; cervical spinal cord; child; cohort analysis; female; human; injuries; male; middle aged; nursing; respiratory system; retrospective study; spinal cord injury; tracheostomy; young adult

Bickerstaff-s brainstem encephalitis variant of guillain-barre syndrome causing complete spinal cord injury: A case report - Solinsky R.; Smith C.H. (PM and R, 2014)
Case Description: A 46-year-old woman presented with 6 days of diarrhea, fever, altered mentation, and ataxia to an outside hospital. She quickly developed urinary retention, severe headache, diplopia, general tonic-clonic seizures, and flaccid paralysis requiring intubation. Extensive infectious workup was negative and she was treated with 5 days of intravenous immunoglobulin followed by 11 days of high dose steroids and an extended prednisone taper. Serum and CSF samples were sent to the Center for Disease Control who confirmed past Campylobacter jejuni infection and findings consistent with Bickerstaff-s brainstem encephalitis. She presented to acute inpatient rehabilitation 36 days after initial presentation with flaccid tetraplegia, dysphagia with PEG tube in place and on a ventilator with tracheostomy. Setting: Acute Inpatient Rehabilitation Unit Results or Clinical Course: Admission ASIA examination demonstrated C5 ASIA A tetraplegia with a zone of partial preservation (ZPP) bilaterally to sensory/motor level T4. She was weaned off the ventilator on day 6 and decannulated on day 15. Oropharyngeal dysphagia slowly resolved and tube feeds were discontinued on day 17. Patient developed a broad band of constrictive transitional zone pain extending from T6 to T12. Lower extremities remained largely flaccid, though discharge examination showed T6 ASIA A tetraplegia with ZPP to L2 motor bilaterally. Total motor FIM score improved from 20 at admission to 66 at discharge and patient was able to return home, 95 days after initial presentation to outside hospital. Discussion: Bickerstaff brainstem encephalitis is a rare Guillain- Barre variant that lies on a continuous spectrum with Miller-Fisher syndrome and has typically been associated with opthalmoplegia, ataxia, altered mentation, hyperreflexia or areflexia and occasionally paralysis. Recovery time, FIM improvement, or mention of significant transitional zone pain has not been previously described to our knowledge. Conclusions: This case describes how acute inpatient rehabilitation can be effective in facilitating transition back to independence following tetraplegia from Bickerstaff-s brainstem encephalitis.
Keywords: prednisone; steroid; immunoglobulin; encephalitis; Guillain Barre syndrome; spinal cord injury; human; case report; physical medicine; rehabilitation; brain stem; quadriplegia; hospital patient; patient; dysphagia; examination; hospital; thinking; tube; ventilator; pain; diplopia; headache; Campylobacter enteritis; disease control; urine retention; serum; fever; diarrhea; paralysis; drug megadose; Functional Independence Measure; leg; preservation; intubation; disease course; flaccid paralysis; tracheostomy; hyperreflexia; areflexia; tonic clonic seizure; independence; female; cerebrospinal fluid

Neuromuscular electrical stimulation is safe in children with intramuscular diaphragmatic pacers: A case series - MacDonald K.; Cagney J.; Martin R.; Sadowsky C. (PM and R, 2014)
Objective: Our objective is to present the first 3 known documented cases of successful use of Neuromuscular Electrical Stimulation (NMES) as an intervention with patients concurrently using Intramuscular Diaphragmatic Pacing Systems (IMDP) systems. Design: Retrospective Chart Review. Setting: Inpatient Pediatric Rehabilitation, Outpatient Rehabilitation. Participants: This case report discusses 3 patients with cervical level spinal cord injuries resulting in tetraplegia requiring respiratory support: male, age 16 years C2 AIS A; female, age 10 years C2 AIS A; male, 4 years C2 AIS not confirmed secondary to age (presented as complete). All patients were implanted with IMDP for respiratory support; they utilized a tracheostomy and intermittently utilized positive pressure ventilation as a supplement to IMDP. Interventions: All 3 patients successfully participated in intensive Activity-Based Rehabilitation (ABR). Each patient received 4 to 6 hours a day of combined occupational and physical therapy. Interventions included, but were not limited to: Neuromuscular Electrical Stimulation (NMES) and neuro re-education, functional electrical stimulation assisted cycling, functional mobility, activities of daily living skills training, and range of motion and strengthening. NMES parameters were optimized for function and customized to patients' tolerance to achieve a muscle contraction. Patients were informed of the risks, benefits and alternatives for each intervention, including those involving NMES. Main Outcome Measures: Vital signs including pulse rate, respiratory rate, and oxygen saturation levels were monitored to track patient's responses during interventions. Results or Clinical Course: All sessions in which NMES was utilized resulted with no adverse response, no acute change, no clinical or subjective respiratory function changes, and no interference with the IMDP device. Therefore it is determined that NMES is safe to utilize in patients with an implanted IMDP. Conclusions: It is safe to utilize NMES for rehabilitation with patients who use IMDP for respiratory support as it does not impact the functionality of the IMDP systems.
Keywords: neuromuscular electrical stimulation; child; human; case study; physical medicine; rehabilitation; patient; assisted ventilation; male; female; pediatric rehabilitation; positive end expiratory pressure; outpatient; functional electrical stimulation; tracheostomy; quadriplegia; pulse rate; spinal cord injury; oxygen saturation; disease course; devices; physiotherapy; education; hospital patient; daily life activity; muscle contraction; parameters; range of motion; risk; vital sign; skill; breathing rate; case report; respiratory function; medical record review

Nurse with acute onset ophthalmoplegia, ataxia and dysphagia: A case report of bickerstaff brainstem encephalitis - Williams L.S.; Mhatre P.V. (PM and R, 2014)
Case Description: The patient initially presented with acutely worsening headache, neck stiffness and “jumping vision.” Physical examination demonstrated bilateral cranial nerve III and VI palsies, nuchal rigidity, tachycardia, and progressive loss of airway requiring tracheostomy and percutaneous endoscopic gastrostomy (PEG). After stabilization, the patient was admitted for acute inpatient rehabilitation with significant impairments in gait and self care due to visual field deficits. Her course was complicated by urinary retention and dysautonomia. Program Description: A 28-year-old woman with acute onset ophthalmoplegia, ataxia, dysphagia and fatigue. Setting: Acute Inpatient rehabilitation. Results or Clinical Course: Lumbar puncture studies were inconclusive and MRI brain/spine revealed hyperintensities throughout the brainstem, particularly the medulla, and upper cervical spinal cord. She was treated for delirium/mood disturbance and noted to have transient hemisensory deficits. The constellation of acute onset ataxia and ophthalmoplegia were consistent with a possible diagnosis of Bickerstaff Brainstem Encephalitis (BBE). Discussion: This rare autoimmune disease is thought to be an unusual variant of acute idiopathic polyneuritis. The predominant central nervous system findings distinguish this condition from Fisher Syndrome, a subset of Guillain Barre Syndrome. It is often triggered by a preceding infection and has spontaneous recovery. The patient progressed from initial functional independence measures of total assistance to modified and complete independence after 4 weeks of intensive physical, occupational and speech therapies. Her central vestibular dysfunction improved with vestibular therapies. Prior to transition to outpatient, the patient was decannulated, tolerating oral nutrition and Foley decatheterization, with plans for full neuropsychiatric testing. Conclusions: BBE is a rare transient autoimmune disease with unique presentation that responds well to aggressive inpatient rehabilitation and vestibular therapies. Although known for spontaneous recovery, no studies exist on rehabilitation outcomes for these patients.
Keywords: human; ophthalmoplegia; encephalitis; dysphagia; brain stem; case report; nurse; physical medicine; rehabilitation; patient; hospital patient; Guillain Barre syndrome; therapy; autoimmune disease; rigidity; infection; percutaneous endoscopic gastrostomy; tracheostomy; headache; Functional Independence Measure; airway; central nervous system; tachycardia; neuritis; paralysis; cranial nerve; female; disease course; physical examination; jumping; dysautonomia; lumbar puncture; cervical spinal cord; diagnosis; laryngeal mask; urine retention; vestibular disorder; visual field; speech therapy; self care; gait; neck; independence; outpatient; nutrition; fatigue; nuclear magnetic resonance imaging

Craniocervical junction dural arteriovenous fistula misdiagnosed as transverse myelitis: A case report - Raverty M.K.; Boyle S.; Beck L. (PM and R, 2014)
Case Description: A 67-year-old man was admitted to inpatient rehabilitation following 3-4 months of progressive quadriparesis. He was diagnosed with transverse myelitis and treated with intravenous steroids and plasma exchange at an outside facility prior to rehabilitation admission. After approximately 4 months of rehabilitation, he had an acute exacerbation of weakness. He was treated with additional intravenous steroids and plasma exchange, but did not achieve the same level of function prior to exacerbation. After discharge home, the patient experienced a second exacerbation of weakness, prompting additional investigation at a tertiary care hospital. At that time, approximately 15 months after initial diagnosis, an arteriovenous dural fistula was noted at the craniocervical junction. He subsequently underwent craniotomy for disconnection of the fistula, as well as tracheostomy and gastrostomy tube placement, and was ultimately readmitted to acute inpatient rehabilitation. Setting: Spinal cord injury rehabilitation unit at Veteran's Affairs hospital Results or Clinical Course: Following readmission to acute rehabilitation, the patient continued to improve functionally with ongoing progress toward weaning ventilatory support and was transitioning to oral diet. Unfortunately, he did not achieve the level of functional independence attained prior to the first recurrence of symptoms. Discussion: Transverse myelitis is a heterogenous group of inflammatory disorders of the spinal cord that respond well to intravenous steroids in the majority of cases. Recurrence is rare and when it occurs, alternative diagnoses must be explored. Other autoimmune disorders with similar presentations were excluded in this case. Arteriovenous dural fistulas are a known cause of progressive myelopathy. A fistula was not identified in this case until the patient sustained further functional impairments and increased weakness. Thus, earlier detection of such vascular malformations can improve prognosis and minimize functional impairments. Conclusions: In cases of recurrent or progressive transverse myelitis, or those that do not respond well to steroids, it is important to pursue alternate diagnoses in order to initiate appropriate treatment and minimize progression of functional impairments and morbidity.
Keywords: steroid; dural arteriovenous fistula; myelitis; human; case report; physical medicine; rehabilitation; fistula; patient; diagnosis; functional disease; weakness; plasmapheresis; hospital patient; veteran; spinal cord injury; stomach tube; prognosis; tracheostomy; morbidity; quadriplegia; congenital blood vessel malformation; craniotomy; hospital; spinal cord disease; autoimmune disease; spinal cord; tertiary care center; diseases; independence; diet; weaning; male; hospital readmission; disease course

Adolescent diagnosed with spinal muscular atrophy with respiratory distress type 1: A case report - Khromouchkine N.; Mayer M.P. (PM and R, 2014)
Case Description: A 16-year-old diagnosed with Spinal Muscular Atrophy with Respiratory Distress Type 1 (SMARD1) at 15 years of age, who is presently modified-independent in the classroom and active in the community. During childhood, this patient achieved independent ambulation by 3 years of age. Despite tracheostomy and ventilator dependence, language development was essentially normal. Fine motor limitations progressed and by 9 years of age the patient was using dictation software to remain independent in the classroom. Progressive weakness resulted in the loss of ambulation by 13 years of age, and the patient now requires minimal assistance with transfers and a power wheelchair for independent mobility. Setting: Tertiary care pediatric hospital. Results or Clinical Course: This patient's diagnostic odyssey consisted of years of extensive testing including multiple electromyograms, a liver biopsy, nerve biopsy, two muscle biopsies and multiple genetic panels. At 15 years of age, whole exome genetic testing identified disease-causing compound heterozygous mutations of the IGHMBP2 gene. Discussion: SMARD1 is a rare genetic disease manifested in early infancy primarily by severe diaphragmatic weakness, respiratory distress, and distal neuromuscular weakness due to mutations in the IGHMBP2 gene. Respiratory failure occurs prior to six months of age and few patients survive beyond childhood. This 16-year-old patient represents one of the oldest reported living patients with SMARD1 and has well-preserved independent function. The patient was diagnosed with next-generation genetic testing that was not available at the time of the patient's birth. Conclusions: With advanced respiratory care, survival beyond childhood is possible for patients with SMARD1. For patients with rare genetic conditions, whole exome testing has the potential to avoid extensive workups involving tests which may be invasive, expensive and delay the speed of diagnosis.
Keywords: human; spinal muscular atrophy; rehabilitation; case report; respiratory distress; physical medicine; adolescent; patient; weakness; childhood; mutation; genetic screening; diagnosis; mobilization; gene; exome; pediatric hospital; tertiary health care; wheelchair; survival; software; respiratory care; infancy; disease course; language development; tracheostomy; community; muscle biopsy; ventilator; nerve biopsy; genetic disorder; liver biopsy; respiratory failure; velocity

A review of respiratory management in spinal cord injuries - Haylett R.; Gustafson O.; Fox V.; Barker G. (Intensive Care Medicine, 2014)
INTRODUCTION. No UK guidelines currently exist to inform best practice in the management of patients with a traumatic cervical spinal cord injury (SCI), particularly regarding ventilatory management in the acute stage. There is limited data available concerning the outcomes of specific management pathways or adverse events encountered, leading to individual centres adopting local strategies. OBJECTIVES. To review the respiratory management of cervical SCI patients admitted to a single general ICU in the UK. METHODS. Retrospective data collection and analysis of all 21 patients admitted to the intensive care unit following a SCI, between October 2010 and April 2014. Clinical data was collated from the electronic notes system, including the time from admission to tracheostomy, number of failed extubations, and number of adverse events associated with failed extubations. The cost of respiratory physiotherapy associated with periods of selfventilation, post re-intubation and post tracheostomy was also analysed. RESULTS. 16 patients admitted during the review period suffered a cervical SCI. Of those surviving to ICU discharge, 11 (85 %) received a tracheostomy. Of those not receiving a tracheostomy, one patient was never intubated and transferred to a spinal injury centre within one week. One suffered an incomplete lower cervical SCI that showed rapid neurological recovery post spinal stabilisation. Three patients died. There were 12 failed extubations, an average of 1.7 per patient. Patients that were extubated spent on average 1.4 days self ventilating, and received an average 8.9 h of physiotherapy input in this time (6.4 h per day). During periods post re-intubation, patients spent on average 5.5 days receiving mechanical ventilation, with an average 14.7 h of physiotherapy input during this time (2.7 h per day). There were complications associated with failed extubations and re-intubations. These include frequent episodes of physiotherapy and lung volume recruitment/airway clearance, one emergency tracheostomy to manage a difficult airway, one pneumothorax, and two bronchoscopies. There were no adverse events post tracheostomy. The average time from admission to receiving a tracheostomy was nine days. Patients then spent on average 22.4 days with the tracheostomy, and received an average of 35 h of therapy in this time (1.6 h per day). CONCLUSIONS. This limited review suggests that the majority of patients admitted to this general ICU with a cervical SCI will benefit from a tracheostomy to facilitate weaning from mechanical ventilation and physiotherapy management. A delay in tracheostomy and subsequent failed extubations are associated with several adverse events. Those that receive a tracheostomy experience fewer respiratory complications and the cost related to respiratory physiotherapy is reduced.
Keywords: spinal cord injury; society; intensive care; human; patient; tracheostomy; extubation; physiotherapy; intubation; artificial ventilation; United Kingdom; breathing exercise; airway; lung volume; clinical study; intensive care unit; information processing; spine injury; cervical spinal cord injury; weaning; emergency; pneumothorax; therapy; bronchoscopy

Traumatic spinal cord injury in a military hospital in Saudi Arabia - Al Jadid M. (PM and R, 2014)
Objective: To determine the causes, age and gender differences, hospital length of stay (HLoS), and prevalence of traumatic spinal cord injury (TSCI) for patients admitted to a military hospital in Saudi Arabia. Design: This was a retrospective study. All patients who completed the TSCI rehabilitation program at the Rehabilitation Unit at Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia from August 1982 to November 2010 were included. The admission criteria includes: stable medical condition, no pressure sore, no tracheostomy, and not more than 3 patients with quadriplegia in the unit at any time due to the loads on patients. Setting: Saudi referral trauma center. Participants: 466 patients (398 males and 68 females). Interventions: All patients were involved in total of 3 hours of physical and occupational therapy daily during the working days. Main Outcome Measures: Type and mechanism of trauma, type and severity of neurologic deficits, causes, frequency and HLoS. Results or Clinical Course: Compared to females, the frequency of TSCI was higher in males. The higher frequency of TSCI was found in the 16-30 age group, and a lower frequency was found in the 0-15 and >45 age groups. Out of the 466 TSCI patients 377 (80.1%) sustained their injuries as a result from motor vehicle accidents. Cervical cord was the most common site of injury accounting for 34% (n=137) of cases in male population, and in females, the higher frequency was the upper thoracic (n=31 [45.6%]) There were 250 TSCI patients that stayed in the hospital for 1-70 days, and only 12 patients stayed in hospital for >280 days. Conclusions: Compared to females, the frequency of TSCI was higher in males, and younger group sustained more TSCI. Road traffic accident is the most common cause of injury, and more than 50% of the TSCI patients stayed in the hospital for <70 days.
Keywords: spinal cord injury; public hospital; Saudi Arabia; physical medicine; rehabilitation; human; patient; injury; male; hospital; female; traffic accident; groups by age; occupational therapy; emergency health service; quadriplegia; decubitus; prevalence; tracheostomy; city; traffic; army; population; cervical spinal cord; retrospective study; disease course; length of stay; sex difference

Screening for proteinuria in 'at-risk' patients with spinal cord injuries: Lessons learnt from failure - Vaidyanathan S.; Abraham K.A.; Singh G.; Soni B.; Hughes P. (Patient Safety in Surgery, 2014)
Spinal cord injury patients may develop proteinuria as a result of glomerulosclerosis due to urosepsis, hydronephrosis, vesicoureteric reflux, and renal calculi. Proteinuria in turn contributes to progression of kidney disease. We report one paraplegic and two tetraplegic patients, who developed recurrent urine infections, urinary calculi, and hydronephrosis. These patients required several urological procedures (nephrostomy, cystoscopy and ureteric stenting, ureteroscopy and lithotripsy, extracorporeal shock wave lithotripsy). These patients had not received antimuscarinic drugs nor had they undergone video-urodynamics. Proteinuria was detected only at a late stage, as testing for proteinuria was not performed during follow-up visits. Urine electrophoresis showed no monoclonal bands in any; Serum glomerular basement membrane antibody screen was negative. Serum neutrophil cytoplasmic antibodies screen by fluorescence was negative. All patients were prescribed Ramipril 2.5 mg daily and there was no further deterioration of renal function.Spinal cord injury patients, who did not receive antimuscarinic drugs to reduce intravesical pressure, are at high risk for developing reflux nephropathy. When such patients develop glomerulosclerosis due to recurrent urosepsis, renal calculi, or hydronephrosis, risk of proteinuria is increased further. Take home message: (1) Screening for proteinuria should be performed regularly in the 'at-risk' patients. (2) In the absence of other renal diseases causing proteinuria, spinal cord injury patients with significant proteinuria may be prescribed angiotensin-converting enzyme inhibitor or angiotensin-II receptor antagonist to slow progression of chronic renal disease and reduce the risk of cardiovascular mortality. © 2014 Vaidyanathan et al.; licensee BioMed Central Ltd.
Keywords: amikacin; ampicillin; atorvastatin; creatinine; distigmine; gentamicin; hemoglobin; hemoglobin A1c; metronidazole; neutrophil cytoplasmic antibody; phenoxybenzamine; piperacillin plus tazobactam; prazosin; ramipril; urea; absence of side effects; adult; antibiotic therapy; article; balloon dilatation; cervical spine dislocation; clinical article; computer assisted tomography; cystography; cystoscopy; disease course; Enterococcus faecalis; extracorporeal shock wave lithotripsy; glomerulosclerosis; glomerulus basement membrane; glomerulus filtration rate; high risk patient; human; hydronephrosis; indwelling catheter; kidney abscess; kidney cyst; kidney disease; kidney function; lung infiltrate; male; nephrolithiasis; nephrostomy tube; orchitis; proteinuria; quadriplegia; rigor; screening test; spinal cord injury; Streptococcus milleri; tracheostomy; ureter stent; ureter stone; ureteropelvic junction obstruction; ureteroscopy; urinary tract infection; urosepsis; vesicoureteral reflux; vomiting

Tetraplegia incidence in Normandy - Quesnel A.; Beuret Blanquart F.; Proust F.; Verin E. (Annals of Physical and Rehabilitation Medicine, 2014)
We conducted a retrospective study on patients over 18 years with traumatic cervical spinal cord injury level in Upper Normandy, between 2002 and 2012. One hundred and eight patients were included (49.0±21.1 years), with an increase in the proportion of falls. The male/female ratio is 3.9 males to 1 female, with the level most frequently found the C5 level, then the C6 level, C4 level, the C7 level, the level C8 and C3 level; 27.8% of patients were admitted in ICU, and 40.7% had a tracheotomy, which 56.8% were able to be weaned from their tracheotomy; 6.5% required permanent mechanical ventilatory support. The incidence of admissions in ICU of post-traumatic tetraplegia was calculated at 12.7 and 13.4 cases per million inhabitants, stable for the years 2009, 2010, 2011 and 2012. This study shows that taking into account the ventilated quadriplegic remains difficult, and the creation of a national health sector would serve as a dedicated use for patients and supporting the teams throughout the territory.
Keywords: rehabilitation medicine; quadriplegia; human; patient; tracheotomy; public health; male; cervical spinal cord injury; female; retrospective study

Continuous Positive Airway Pressure (CPAP) for prevention of recurrent pneumonia in the Neuromyelitis Optica patient - Welker J.A. (Respiratory Medicine Case Reports, 2014)
Introduction: Traumatic spinal cord injury patients with quadriplegia associated respiratory compromise are at an immediately increased risk of developing pneumonia, but the onset of pneumonia risk and use of prevention strategies in the patient with quadriplegia due to Neuromyelitis Optica has not been described. Case report: This is a case of a Neuromyelitis Optica patient with quadriplegia, dysphagia and tracheostomy that suffered recurrent fevers due to respiratory infections. The non-specific presentation and test results led to extensive testing, while the frequent recurrence resulted in the patient residing in the acute care hospital 201 days and outside of this hospital only 118 days during the period of August 2011 to June 2012. The initiation of CPAP 10cm while sleeping overnight for 8-10heliminated the recurrence of respiratory infections and thereby reduced both the frequency and duration of the patient's hospital stays. Conclusions: Patients with Neuromyelitis Optica differ from those with traumatic spinal cord injury as they have a chronic progressive systemic illness that causes continued deterioration of their nervous system resulting in the need for routine monitoring that ensures the timely addition of CPAP for the prevention of pneumonia and its associated medical expenses. © 2014 The Authors.
Keywords: antibiotic agent; adult; antibiotic therapy; article; atelectasis; bladder dysfunction; breathing muscle; case report; daily life activity; decubitus; deep vein thrombosis; drug fever; drug withdrawal; dysphagia; feeding apparatus; female; hemidiaphragm; hormone substitution; hospital; hospitalization; human; hypopituitarism; hypothalamus; hypoventilation; leg disease; leg paralysis; muscle strength; myelitis; myelooptic neuropathy; nursing home patient; osteomyelitis; pneumonia; positive end expiratory pressure; priority journal; quadriplegia; recurrent fever; recurrent infection; respiratory failure; respiratory tract infection; sleep; suprapubic catheter; thorax radiography; tracheostomy; urinary tract infection

Difficult-to-wean: High index of suspicion - Gelaye A.; Patel B.; Farra W.; Kole B. (American Journal of Case Reports, 2014)
Objective: Rare disease Background: Failed planned extubation occurs in a minority of patients with acute respiratory failure requiring invasive mechanical ventilation. In patients presenting with acute respiratory failure with no identifiable cardiopulmonary causes, uncommon conditions, such as cervical spondylotic myelopathy, should be considered. In very rare instances, when cervical spondylotic myelopathy and syringomyelia present concomitantly, they can be devastating. Case Report: A 57-year-old woman visited the emergency room (ER) after feeling unwell for several days. She was brought to the ER with acute respiratory distress and obtunded state with GCS of 6/ 15. She was hypotensive and agonally breathing. Her initial neurologic evaluation was unrevealing. Based on these findings, she was intubated. Over the next several days, she was difficult to wean from the ventilator and had persistent respiratory acidosis. After a short-lived extubation, the patient was again re-intubated. This time the neurologic evaluation showed decreased movements of all muscle groups against gravity and forces, with generalized weakness. An MRI of the brain and cervical spine demonstrated moderate degenerative disc disease and syringomyelia extending from C2 to C7 level. The patient underwent de-compression laminectomy. After failing several weaning trials, she underwent bronchoscopically-assisted tracheotomy. Conclusions: Acute cardiopulmonary and intensive care unit-acquired neuromuscular conditions have been attributed as a major cause of difficult weaning and extubation. Failure to identify and correct other rare combinations (such as cervical degenerative disc disease and syringomyelia) may cause acute respiratory failure and subsequent failure to wean and extubation, resulting in high rates of mortality and morbidity. © Am J Case Rep, 2014.
Keywords: adult; arterial gas; article; artificial ventilation; breathing rate; bronchoscopy; case report; cervical spondylotic myelopathy; clinical feature; decompression surgery; diagnostic imaging; disease association; disease course; extubation; female; Glasgow coma scale; heart rate; human; image analysis; intervertebral disk disease; laminectomy; mechanical ventilator; medical history; middle aged; muscle weakness; neuroimaging; neurologic examination; nuclear magnetic resonance imaging; outcome assessment; patient assessment; quadriplegia; respiratory acidosis; respiratory distress; syringomyelia; tracheostomy; treatment failure

Cervico-dorsal intramedullary hematoma in a patient with pompe disease - Nadile D.; Toledo M.G.; Tamargo A.; Cassara F.P.; Thomson A.; Muñoz F.; Klein F. (Neurology, 2014)
OBJECTIVE: To suggest that Pompe Disease can cause microvascular alterations that may also affect the spinal cord.BACKGROUND: Pompe disease (PD) or acid maltase deficiency leads to accumulation of glycogen in different tissues, particularly in skeletal muscle. Late-onset forms have a progressive evolution in elderly patients. An alteration in the cerebral microvasculature has been described in this group, leading to cerebral bleeding.DESIGN/METHODS: 83-year-old woman with diagnosis of PD. She pesented with a history of 20 years of progressive generalized weakness predominantly in proximal muscles of lower limbs, and diaphragmatic paralysis, leading to respiratory failure requiring tracheostomy and chronic mechanical ventilation. She was admitted for a left moderate braquiocrural motordeficit and left upper limb sensitive deficit as well. A brain CT discarded hemorrhagic or ischemic lesions. 48 hours later she added left upper and lower limb plegia, right severe upper and lower limb paresis and a sensitive level at D 4.RESULTS: The neck vessel Doppler and Cardiac Doppler showed no significant findings. Cervical and dorsal MRI was performed showing an heterogeniclesion extending from the medulla to D6, that is hypointense in T1 and hyperintense on T2 , FLAIR andGrE, compatible with intramedullary spinal hematoma. A selective spinal angiography discarded any vascular malformation.CONCLUSIONS: Late onset Pompe disease may be associated with spontaneous cerebral hemorrhages even in the absence of typical cardiovascular risk factors. These patients may have glycogen deposits in the tunica media of arteriolar walls, with vacuolar degeneration and formation of microaneurysms that predisposes spontaneous cerebral hemorrhage . While basilar dolichoectasia and vascular malformations in the posterior territory are described as an association to hemorrhagic lesions in this population, in our case, this was ruled out by a digital angiography. There are numerous reports of brain intraparenchymal hematomas associated with PD but not in the topography above mentioned.
Keywords: glycogen; glucan 1,4 alpha glucosidase; patient; human; glycogen storage disease type 2; neurology; hematoma; leg; brain hemorrhage; brain; skeletal muscle; tissues; female; artificial ventilation; digital subtraction angiography; population; arm; paresis; neck; spinal hematoma; tracheostomy; respiratory failure; degeneration; diaphragm paralysis; tunica media; cardiovascular risk; muscle; weakness; diagnosis; spinal angiography; congenital blood vessel malformation; microvasculature; topography; aged; nuclear magnetic resonance imaging

Strengthened respiratory management for lower tracheostomy risks in patients with complete cervical spinal cord injury - Zhu W.; Kan L.; Sun Y.; Chen X.; Yuan W.; Jia L. (National Medical Journal of China, 2014)
Objective: To access the feasibility of reducing tracheostomy rates in patients with complete cervical spinal cord injury through a systemic respiratory management mode. Methods: A retrospective review was performed for 239 patients on a systemic respiratory management mode after complete cervical spinal cord injury in Shanghai Changzheng Hospital from 2006 to 2012. Their demographic and clinical data, including age, gender, cause of injury, level of cervical spinal cord injuries, surgical approaches, tracheostomy rates and mortality rates, were collected and analyzed. Tracheostomy rates were compared with those of patients with complete cervical spinal cord injury. Tracheostomy rates were compared with those patients with complete cervical spinal cord injury from 1991 to 2005. The new respiratory management mode for patients with severe cervical spinal cord injury included maintaining adequate energy and water for patients, relieving bronchospasm, loosening phlegm, strengthening body-turning and hackslapping, sputum aspiration with suction tube through nasal cavity or bronchofibroscope, strengthening deep breath and cough training, strict control of tracheostomy indications and short-term postoperative intubation reserving if necessary. Results: 32 patients were implemented tracheostomy. The tracheostomy rate was 13.4%. 10 patients died, the mortality rate was 4.18%. However, the tracheostomy rate was 19.1% and mortality rate 21.4% from 1991 to 2005. The tracheostomy rate was significantly lower than before and survival rate improved significantly. Conclusion: The systemic respiratory management mode can effectively reduce tracheostomy rate of patients with complete cervical spinal cord injury and improve their survival rate. Copyright © 2014 by the Chinese Medical Association.
Keywords: age; article; breathing; bronchospasm; cervical spine injury; demography; feasibility study; gender; human; major clinical study; medical record review; mortality; risk reduction; surgical technique; tracheostomy

Innominate artery injury: A catastrophic complication of tracheostomy, operative procedure revisited - Maruti Pol M.; Gupta A.; Kumar S.; Mishra B. (BMJ Case Reports, 2014)
A patient presented with profuse bleeding from the oronasal cavity following orofaciomaxillary trauma associated with tracheolaryngeal injury and suspected cervical-spine injury due to collapse of a wall on the face, neck and upper chest. The patient was gasping, coughing blood and was unable to speak. Threatened airway was diagnosed. Inability to maintain oxygenation on cricothyroidotomy, forced emergency department surgeons to shift the patient to the operating room for definitive airway. During tracheostomy a major vessel was injured. Application of vascular clamp in the event of achieving haemostasis resulted in disappearance of saturation and pulse in the right upper limb, thus we suspected innominate artery (IA) injury. High tracheostomy performed and endotracheal tube passed into the trachea after removing clot and overcoming compromised narrow tracheal lumen. The injured IA was repaired and the patient survived for 14 days. On postoperative day 14 he died following profound bleeding into the tracheobronchial tree and asphyxia/ apnoea. Tracheoinnominate artery fistula was detected at autopsy. Copyright 2014 BMJ Publishing Group. All rights reserved.
Keywords: adult; aortic aneurysm; artery injury; article; autopsy; blood transfusion; blood vessel injury; blunt trauma; case report; crystalloid; death; differential diagnosis; emphysema; extubation; false aneurysm; human; human tissue; hypoxia; intensive care unit; larynx injury; male; multiple trauma; nech vascular anomaly; oral bleeding; outcome assessment; peroperative care; postoperative care; priority journal; surgical approach; surgical technique; tracheostomy; tracheotomy

Chordoma: A rare cause of progressive neurologic symptoms and Horner's syndrome - Hovaida M.M. (Journal of General Internal Medicine, 2014)
LEARNING OBJECTIVE 1: Recognize chordomas as rare bone tumors that commonly present as chronic, progressive neurologic symptoms refractory to routine management LEARNING OBJECTIVE 2: Coordinate multidisciplinary care for complex patients CASE: A 59 year old female with history of morbid obesity, diabetes mellitus complicated by CKDIII, hypertension and OSA presented to her primary internist with persistent left arm and neck pain and tingling for 9 months refractory to physical therapy that prevented her from typing at work. She also complained of dry eyes and progressive shortness of breath, previously attributed to worsening OSA with uptitration of her CPAP settings. Physical exam was notable for an obese Hispanic female with left neck fullness and left miosis and ptosis consistent with Horner's syndrome. CT neck/thorax demonstrated a large, infiltrating pre-vertebral mass extending 20 cm from the lower clivus to the upper chest, displacing the trachea, posterior to the descending aorta and encasing the left subclavian, vertebral and common carotid artery. The patient was referred to ENT with laryngoscopy showing a patent but narrowed airway. Subsequent tracheostomy with exploration and biopsy identified metastatic adenocarcinoma on frozen section. However, formal pathology identified a classic type chordoma with cytokeratin and EMA positivity. Radiation Oncology recommended maximal resection with post-operative proton beam radiotherapy to residual disease and areas at risk for recurrence. Coordinated tumor de-bulking and spinal cord decompression was performed by Orthopedics, Neurosurgery and ENT, however tumor could only be partially removed due to high risk location. Repeat imaging demonstrated residual tumor within the spinal canal at the cervico-thoracic junction and mediastinum. She underwent 30 sessions of radiation, although repeat imaging showed increased tumor size and necrosis. Patient's course was also complicated by multiple ED visits and hospitalizations for pain control, neurologic complaints and infection requiring close follow-up with her primary. After multiple discussions, patient and family requested focus on comfort-oriented treatment and was subsequently enrolled in hospice, although she was discharged after 6 months given her stable clinical status. DISCUSSION: Chordomas are rare bone tumors characterized by slow growth, frequent local recurrence and rare systemic spread. They arise from embryonic remnants of the notochord and can be spheno-occipital (35 %), vertebral (15 %), or sacro-coccygeal (50 %). Approximately 7-10 % of chordomas are cervical and typically present in the 5th-6th decade without gender predilection. Nerve impingement, progressive pain and headaches are common symptoms. Radiographically, chordomas are usually solitary, lytic lesions ranging from 1 to 10 cm. In this case, the large size and considerable extension of the tumor were uncommon. Immunohistochemistry typically demonstrates positivity for EMA or cytokeratin. Initial treatment consists of wide, local excision, which is associated with longer survival and delayed recurrence, although as in this case, complete resection is often difficult secondary to involvement of vital structures and thus adjuvant radiation is employed. Limited literature reviews of molecular based therapy have also demonstrated symptomatic, and rarely, radiological response. Ultimately, chordomas are challenging to manage and often require a multi-disciplinary approach, demonstrating the importance of the primary provider in care coordination, advocacy and ongoing assessment of patient symptoms and goals of care through this complicated course.
Keywords: cytokeratin; adjuvant; internal medicine; society; chordoma; neurologic disease; Horner syndrome; human; patient; neoplasm; bone tumor; imaging; radiation; female; risk; pain; surgery; internist; clivus; hypertension; miosis; neck; diabetes mellitus; minimal residual disease; spinal cord decompression; ptosis (eyelid); vertebral canal; thorax; trachea; morbid obesity; oncology; dyspnea; wide excision; biopsy; dry eye; tracheostomy; survival; adenocarcinoma; frozen section; pathology; Hispanic; otorhinolaryngology; airway; physiotherapy; patent; orthopedics; neurosurgery; hospice; laryngoscopy; mediastinum; neck pain; tumor volume; necrosis; hospitalization; common carotid artery; follow up; infection; comfort; descending aorta; notochord; gender; nerve; headache; immunohistochemistry; therapy; arm; proton therapy; positive end expiratory pressure

Mortality and morbidity after high-dose methylprednisolone treatment in patients with acute cervical spinal cord injury: A propensity-matched analysis using a nationwide administrative database - Chikuda H.; Yasunaga H.; Takeshita K.; Horiguchi H.; Kawaguchi H.; Ohe K.; Fushimi K.; Tanaka S. (Emergency Medicine Journal, 2014)
Objective To examine the magnitude of the adverse impact of high-dose methylprednisolone treatment in patients with acute cervical spinal cord injury (SCI). Methods We examined the abstracted data from the Japanese Diagnosis Procedure Combination database, and included patients with ICD-10 code S141 who were admitted on an emergency basis between 1 July and 31 December in 2007-2009. The investigation evaluated the patients' sex, age, comorbidities, Japan Coma Scale, hospital volume and the amount of methylprednisolone administered. One-to-one propensity-score matching between high-dose methylprednisolone group (>5000 mg) and control group was performed to compare the rates of in-hospital death and major complications (sepsis; pneumonia; urinary tract infection; gastrointestinal ulcer/bleeding; and pulmonary embolism). Results We identified 3508 cervical SCI patients (2652 men and 856 women; mean age, 60.8±18.7 years) including 824 (23.5%) patients who received high-dose methylprednisolone. A propensity-matched analysis with 824 pairs of patients showed a significant increase in the occurrence of gastrointestinal ulcer/bleeding (68/812 vs 31/812; p<0.001) in the high-dose methylprednisolone group. Overall, the high-dose methylprednisolone group demonstrated a significantly higher risk of complications (144/812 vs 96/812;OR, 1.66; 95% CI 1.23 to 2.24; p=0.001) than the control group. There was no significant difference in in-hospital mortality between the high-dose methylprednisolone group and the control group ( p=0.884). Conclusions Patients receiving high-dose methylprednisolone had a significantly increased risk of major complications, in particular, gastrointestinal ulcer/bleeding. However, high-dose methylprednisolone treatment was not associated with any increase in mortality.
Keywords: methylprednisolone; adult; age; aged; article; cervical spinal cord injury; Charlson Comorbidity Index; comorbidity; controlled study; digestive system ulcer; drug megadose; emergency care; female; gastrointestinal hemorrhage; gender; hospital admission; human; ICD-10; Japan Coma Scale; length of stay; lung embolism; major clinical study; male; morbidity; mortality; pneumonia; priority journal; propensity score; rating scale; risk assessment; sepsis; spine surgery; steroid therapy; tracheostomy; urinary tract infection; very elderly

Brown-Vialetto-van Laere syndrome: A riboflavin responsive neuronopathy of infancy with singular features - Spagnoli C.; Pitt M.C.; Rahman S.; De Sousa C. (European Journal of Paediatric Neurology, 2014)
We report the case of a previously healthy child presenting at 6 months of age with mild feeding difficulties and then developing hypotonia, progressive bulbar palsy with respiratory compromise and lower motor neuron signs, causing her to spend 4 months in the Paediatric Intensive Care Unit. Neurophysiological studies demonstrated a motor neuronopathy involving anterior horn cells and cranial nerve nuclei and abnormal brainstem auditory evoked potentials, leading to a diagnosis of Brown-Vialetto-van Laere Syndrome, confirmed by genetic testing (SLC52A3). Magnetic Resonance Imaging showed signal changes in the dorsal column of the spinal cord. She developed a coarse face and abnormal hair pattern. Sustained clinical improvement has been observed during almost 4 years of high-dose riboflavin therapy. © 2013 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Keywords: acylcarnitine; carboxylic acid; citrate synthase; cytochrome c oxidase; immunoglobulin; riboflavin; ubidecarenone; anterior horn cell; article; assisted ventilation; atelectasis; Brown Vialetto van Laere syndrome; bulbar paralysis; case report; cervical spinal cord; chest infection; coarse face; cranial nerve; diaphragm paralysis; electromyography; enzyme activity; evoked brain stem auditory response; fasciculation; feeding difficulty; female; frameshift mutation; gastrostomy; genetic screening; hair disease; histochemistry; human; human tissue; infant; infant disease; lipid blood level; missense mutation; motoneuron; muscle action potential; muscle biopsy; muscle denervation; muscle hypotonia; muscle strength; muscle weakness; neuroimaging; neurologic examination; neurophysiological recruitment; neurophysiology; newborn intensive care; nuclear magnetic resonance imaging; ophthalmoplegia; perception deafness; priority journal; ptosis (eyelid); respiratory failure; riboflavin deficiency; seizure; tendon reflex; tracheostomy; urine level; walker

Recombinant human β-glucuronidase enzyme replacement therapy for mucopolysaccharidosis type VII: Report of the first patient treated - Fox J.E.; Kakkis E.D.; Sly W.S. (Molecular Genetics and Metabolism, 2014)
Mucopolysaccharidosis type VII (MPS VII, Sly syndrome), is a very rare lysosomal disease, caused by deficiency of beta-glucuronidase (GUS), required for the degradation of dermatan sulfate and heparan sulfate. Clinically, this multi-systemic disorder resembles MPS I and MPS II, howevermarked heterogeneity of presentation and progression has been noted. Two decades of animal research demonstrate effective treatment with enzyme replacement therapy (ERT) in MPS VII mouse models. Furthermore, there are extensive data on approved ERT in three other MPS disorders. However, there is no product approved for treatment of MPS VII patients. We present the clinical features of the first patient with MPS VII who was recently infused with recombinant human GUS (rhGUS) as ERT. The patient is an 12 year old boy diagnosed with MPS VII by fibroblast assay, consistent with the clinical features including hydrops fetalis, hepatosplenomegaly, heart valve disease, frequent sinopulmonary infections and upper airway obstruction, declining pulmonary function, dysostosis multiplex, and spinal cord compression requiring cervical fusion. His urinary glycosaminoglycan levels were significantly elevated, at >4x the upper limit of normal, at baseline. Despite a tracheostomy, nocturnal CPAP and oxygen therapy, significant pulmonary restriction and obstruction led to oxygen dependence, rising CO2 levels in the 60-80 range, approaching respiratory failure and the need for full-time ventilation. Since no additional medical measures could improve his function, we implemented experimental ERT by infusing rhGUS at 2 mg/kg over 4 hours every 2weeks, after pretreatment with an oral antihistamine. To evaluate his response to therapy, urinary GAG levels, pulmonary function, oxygen dependence, CO2 levels, cardiac valve function, liver and spleen size, and growth velocitywill be assessed. Safety will be evaluated by standard assessments and observance of any infusion-related reactions. Data on efficacy and safety in this first report of ERT in MPS VII will be reported.
Keywords: beta glucuronidase; oxygen; heparan sulfate; dermatan sulfate; antihistaminic agent; glycosaminoglycan; human; enzyme replacement; patient; mucopolysaccharidosis type 7; clinical feature; safety; lung function; mouse; systemic disease; Hurler syndrome; mouse model; upper respiratory tract obstruction; valvular heart disease; hepatosplenomegaly; fetus hydrops; spleen size; infection; male; assay; heart valve; fibroblast; spinal cord compression; air conditioning; boy; respiratory failure; obstruction; oxygen therapy; diseases; tracheostomy; therapy; liver; infusion related reaction; positive end expiratory pressure

Risk factors for prolonged duration of mechanical ventilation in acute traumatic tetraplegic patients-a retrospective cohort study - Roquilly A.; Seguin P.; Mimoz O.; Feuillet F.; Rosenczweig E.; Chevalier F.; Loutrel O.; Malledant Y.; Sebille V.; Asehnoune K. (Journal of Critical Care, 2014)
Purpose: Respiratory complications constitute an important determinant of length of stay in tetraplegic patients. In a population of tetraplegic patients, we investigated the factors involved in the duration of mechanical ventilation (MV) and whether the duration of MV was associated with the long-term neurologic status. Material and Methods: In a retrospective study in 3 intensive care units (ICUs) (January 2001 to December 2009), consecutive patients (≥ 18 years) hospitalized for acute (≤ 24 hours) traumatic tetraplegia were included in the study. Patients with severe brain injury or who died in the first 48 hours were excluded. The primary outcome was the duration of MV. The secondary outcomes were the American Spinal Injury Association (ASIA) motor score on ICU discharge and at 1 year. Results: A total of 164 consecutive adult patients with tetraplegia were analyzed. Median (interquartile range) ASIA motor scores were 15 (6-26) on admission, 22 (9-40) on ICU discharge (n = 145 survivors), and 37 (10-80) at 1 year (n = 52 complete follow-up). The median duration of MV was 11 (2-26) days. In multivariate analysis, MV duration increased with pneumonia (P < .0001), atelectasis (P = .0042), and tracheotomy (P < .0001). In exploratory analysis, an increased duration of MV was the only factor associated in multivariate analysis with a low ASIA motor score on ICU discharge (P = .0201) and at 1 year (P = .0003). Conclusions: Prevention of pneumonia and atelectasis is critical for the reduction of MV in tetraplegic patients. Prolonged MV was independently associated with poor neurologic status. © 2014 Elsevier Inc.
Keywords: adult; American Spinal Injury Association impairment scale; article; artificial ventilation; atelectasis; cohort analysis; female; hospital admission; hospital discharge; human; intensive care unit; major clinical study; male; middle aged; pneumonia; quadriplegia; retrospective study; risk factor; spinal cord injury; survivor; tracheotomy; treatment duration

Complete cervical spinal cord injury above C6 predicts the need for tracheostomy - McCully B.H.; Fabricant L.; Geraci T.; Greenbaum A.; Schreiber M.A.; Gordy S.D. (American Journal of Surgery, 2014)
Background: Failed extubation and delayed tracheostomy contribute to poor outcomes in patients with a traumatic spinal cord injury (SCI). We determined if the level and completeness of SCI predict the need for tracheostomy. Methods: Data from 256 patients with SCI between C1 and T3 with or without tracheostomy were retrospectively analyzed. Logistic regression identified predictors for tracheostomy. Data are presented as raw percentage or odds ratio (OR) with 95% confidence interval. P <.05 indicates significance. Results: Complete spinal cord injuries were common in patients requiring tracheostomy (55% vs 18%, P <.05), and predicted the need for tracheostomy (OR: 6.4 (3.1 to 13.5), P <.05). An injury above C6 predicted the need for tracheostomy in patients with complete injury (OR: 3.7 (1 to 11.9), P <.05), but not incomplete injury (OR:.7 (.3 to 1.9); P =.53). Conclusion: Tracheostomy is unlikely in patients with incomplete SCI, regardless of the level of injury. Patients with complete SCI above C6 are likely to require tracheostomy. © 2014 Elsevier Inc. All rights reserved.
Keywords: adult; article; cervical spinal cord injury; female; Glasgow coma scale; human; injury scale; injury severity; length of stay; major clinical study; male; penetrating trauma; prediction; priority journal; retrospective study; tracheostomy

Resection of an upper cervical aneurysmal bone cyst and spinal reconstruction using a midline mandibular osteotomy in a pediatric patient: Case report - McDowell M.M.; Hanft S.J.; Greenberg S.A.; Rahmati R.; Carrao V.; Eisig S.; Anderson R.C.E. (Journal of Neurosurgery: Pediatrics, 2014)
The authors report on the surgical management of an extensive lesion of the upper cervical spine that required an uncommon transmandibular approach to facilitate exposure, resection, and stabilization in a pediatric patient. A 6-year-old boy with a large aneurysmal bone cyst of the C-2 vertebra presented with progressive weakness and right-sided neck pain. The lesion extended laterally into the soft tissue of the neck, inferiorly to C-4, and posteriorly around the spinal cord. A transmandibular osteotomy was performed to provide adequate exposure for complete resection of the mass and anterior C1-3 instrumentation and fusion. Subsequently, the patient underwent occiput to C-4 posterior instrumentation and fusion. The patient tolerated the operation well and had regained all function at 3 and 11 months' follow-up. No neurological complications or problems of speech, swallowing, or respiration occurred. Even in pediatric patients, the transmandibular approach for the treatment of upper cervical spine lesions is an effective method of maximizing exposure for complex lesions requiring resection and stabilization. ©AANS, 2014.
Keywords: allograft; anesthesia induction; aneurysmal bone cyst; article; bone matrix; case report; cervical spine; child; computer assisted tomography; fluoroscopy; follow up; histology; human; incision; male; mandible osteotomy; neck pain; nuclear magnetic resonance imaging; patient positioning; preschool child; priority journal; spine surgery; submandibular gland; surgical approach; torticollis; tracheostomy; weakness; body weight loss

Risk factors relating to the need for mechanical ventilation in isolated cervical spinal cord injury patients - Lertudomphonwanit T.; Wattanaapisit T.; Chavasiri C.; Chotivichit A. (Journal of the Medical Association of Thailand, 2014)
Background: Cervical spinal cord injuries (SCI) are a major public health problem. Respiratory complications are among the most important causes of morbidity and mortality in patients with cervical SCI, especially respiratory failure. Based on our evaluation of the existing English language literature, few previous studies appear to have reported on risk factors associated with the need for mechanical ventilation in isolated cervical SCI patients who had no concomitant injuries or diseases at the time of admission. Objective: The purpose of this study was to determine incidence and risk factors relating to the need for mechanical ventilation in isolated cervical spinal cord injury (SCI) patients who had no concomitant injuries. Material and Method: This retrospective study was conducted by reviewing and analyzing the patient data of 66 isolated cervical-SCI patients who were admitted in our hospital between January 1995 and December 2009. Patient medical records were reviewed for demographic data, neurological injuries, need for mechanical ventilation, definitive treatment, complications, and outcomes. Univariate and multivariate analysis were used to identify predisposing risk factors relating to patient dependency on mechanical ventilation. Results: Of the 66 patients, 30.3% (20/66) required mechanical ventilation and 22.7% (15/66) were identified as complete cord injury, of which seven sustained injury above C5. Of the patients with complete SCI, 66.7% (10/15) were dependent on mechanical ventilation, as were 85% (6/7) with SCI above C5. All five of the patients with complete-SCI above C5 who received operative treatment were dependent upon mechanical ventilation, postoperatively. Only 19.6% (10/51) of the incomplete injury group required mechanical ventilation. Univariate analysis indicated the following factors as significantly increasing the risk of ventilator dependence: complete SCI (p = 0.001), SCI above C5 level (p = 0.011) and operative treatment (p = 0.008). Multivariate analysis identified the following factors as being predisposing risk factors relating to the need of mechanical ventilation: complete SCI (OR: 12.8; 95% CI 2.4-66.9; p = 0.003), SCI above C5 level (OR: 12.0; 95% CI 2.4-60.2; p = 0.002), and operative treatment (OR: 14.8; 95% CI 2.1-106.9; p = 0.008). Conclusion: Complete SCI, SCI above C5, and operative treatment were predisposing risk factors relating to the need for mechanical ventilation in isolated cervical SCI patients. The data and findings put forth in this study suggest that these factors may assist in predicting the need for mechanical ventilation as a long-term treatment for isolated cervical SCI patients.
Keywords: adult; aged; American Spinal Injury Association impairment scale; article; artificial ventilation; cervical spinal cord injury; child; female; hospital admission; human; major clinical study; male; medical record; mortality; pneumonia; respiratory failure; respiratory tract infection; retrospective study; risk factor; tracheostomy

A review article on the benefits of early mobilization following spinal surgery and other medical/surgical procedures - Epstein N.E. (Surgical Neurology International, 2014)
Background: The impact of early mobilization on perioperative comorbidities and length of stay (LOS) has shown benefits in other medical/surgical subspecialties. However, few spinal series have specifically focused on the «pros» of early mobilization for spinal surgery, other than in acute spinal cord injury. Here we reviewed how early mobilization and other adjunctive measures reduced morbidity and LOS in both medical and/or surgical series, and focused on how their treatment strategies could be applied to spinal patients. Methods: We reviewed studies citing protocols for early mobilization of hospitalized patients (day of surgery, first postoperative day/other) in various subspecialties, and correlated these with patients perioperative morbidity and LOS. As anticipated, multiple comorbid factors (e.g. hypertension, high cholesterol, diabetes, hypothyroidism, obesity/elevated body mass index hypothyroidism, osteoporosis, chronic obstructive pulmonary disease, coronary artery disease and other factors) contribute to the risks and complications of immobilization for any medical/surgical patient, including those undergoing spinal procedures. Some studies additionally offered useful suggestions specific for spinal patients, including prehabilitation (e.g. rehabilitation that starts prior to surgery), preoperative and postoperative high protein supplements/drinks, better preoperative pain control, and early tracheostomy, while others cited more generalized recommendations. Results: In many studies, early mobilization protocols reduced the rate of complications/morbidity (e.g. respiratory decompensation/pneumonias, deep venous thrombosis/pulmonary embolism, urinary tract infections, sepsis or infection), along with the average LOS. Conclusions: A review of multiple medical/surgical protocols promoting early mobilization of hospitalized patients including those undergoing spinal surgery reduced morbidity and LOS.
Keywords: body mass; chronic obstructive lung disease; clinical protocol; coronary artery disease; correlation analysis; deep vein thrombosis; diabetes mellitus; hospital patient; human; hypercholesterolemia; hypertension; hypothyroidism; length of stay; lung embolism; medical procedures; mobilization; morbidity; obesity; osteoporosis; patient; peroperative complication; pneumonia; postoperative infection; preoperative treatment; priority journal; protein intake; respiratory tract disease; review; sepsis; spine surgery; supplementation; surgical patient; surgical technique; tracheostomy; urinary tract infection

A review article on the benefits of early mobilization following spinal surgery and other medical/surgical procedures - Epstein N.E. (Surgical Neurology International, 2014)
Background: The impact of early mobilization on perioperative comorbidities and length of stay (LOS) has shown benefits in other medical/surgical subspecialties. However, few spinal series have specifically focused on the "pros" of early mobilization for spinal surgery, other than in acute spinal cord injury. Here we reviewed how early mobilization and other adjunctive measures reduced morbidity and LOS in both medical and/or surgical series, and focused on how their treatment strategies could be applied to spinal patients. Methods: We reviewed studies citing protocols for early mobilization of hospitalized patients (day of surgery, first postoperative day/other) in various subspecialties, and correlated these with patients′ perioperative morbidity and LOS. As anticipated, multiple comorbid factors (e.g. hypertension, high cholesterol, diabetes, hypothyroidism, obesity/elevated body mass index hypothyroidism, osteoporosis, chronic obstructive pulmonary disease, coronary artery disease and other factors) contribute to the risks and complications of immobilization for any medical/surgical patient, including those undergoing spinal procedures. Some studies additionally offered useful suggestions specific for spinal patients, including prehabilitation (e.g. rehabilitation that starts prior to surgery), preoperative and postoperative high protein supplements/drinks, better preoperative pain control, and early tracheostomy, while others cited more generalized recommendations. Results: In many studies, early mobilization protocols reduced the rate of complications/morbidity (e.g. respiratory decompensation/pneumonias, deep venous thrombosis/pulmonary embolism, urinary tract infections, sepsis or infection), along with the average LOS. Conclusions: A review of multiple medical/surgical protocols promoting early mobilization of hospitalized patients including those undergoing spinal surgery reduced morbidity and LOS. Copyright: © 2014 Epstein NE.
Keywords: cholesterol; anterior cervical diskectomy and fusion; article; artificial ventilation; bariatric surgery; body mass; cholesterol blood level; chronic obstructive lung disease; comorbidity; coronary artery disease; cost control; deep vein thrombosis; diabetes mellitus; diet supplementation; gastrostomy; head and neck cancer; heart surgery; hospital cost; hospital readmission; human; hypertension; hypothyroidism; immobilization; length of stay; lung embolism; lung resection; medical procedures; mobilization; obesity; osteoporosis; physiotherapy; pneumonia; postoperative care; postoperative complication; preoperative care; priority journal; rehabilitation care; respiratory failure; scoliosis; sepsis; spinal cord injury; spinal cord surgery; spine surgery; surgical technique; total hip prosthesis; total knee arthroplasty; tracheostomy; urinary tract infection

Embolic brain infarction related to posttraumatic occlusion of vertebral artery resulting fromcervical spine injury: A case report - Nakao Y.; Terai H. (Journal of Medical Case Reports, 2014)
Introduction: The frequency of vertebrobasilar ischemia in patients with cervical spine trauma had been regarded as low in many published papers. However, some case reports have described cervical spine injury associated with blunt vertebral artery injury. Many aspects of the management of vertebral artery injuries still remain controversial, including the screening criteria, the diagnostic modality, and the optimal treatment for various lesions. The case of a patient who had a brain infarction due to recanalization of his occluded vertebral artery following open reduction of cervical spinal dislocation is presented here. Case presentation: A 41-year-old Asian man presented with C4 to C5 distractive flexion injury manifesting with quadriplegia and anesthesia below his C3 cord level (including phrenic nerve paralysis), and bowel and bladder dysfunction. Magnetic resonance angiography and computed tomography angiography showed left extracranial vertebral artery occlusion and patent contralateral vertebral artery. He was observed without antiplatelet and/or anticoagulation therapy, and underwent surgery (open reduction and internal fusion of C4 to C5, and tracheostomy) 8 hours after the injury. After surgery, supraspinal symptoms such as left horizontal nystagmus and left homonymous hemianopsia led to cranial computed tomography and magnetic resonance imaging, which showed left-side cerebellar infarction in his posterior inferior cerebellar artery territory and right-side posterior cerebral artery infarction. Magnetic resonance angiography and computed tomography angiography demonstrated patent bilateral vertebral artery (but hypoplastic right vertebral artery) and occluded right posterior cerebral artery. His injured vertebral artery was treated conservatively, which did not cause any other ischemic complications. Conclusions: The management of asymptomatic vertebral artery injury is controversial with several treatment options available, including observation alone, antiplatelet therapy, anticoagulation therapy, or invasive intervention. Although there are some reports in which management with observation alone is described as safe, we should pay serious attention to the vertebral artery injury caused by cervical spine trauma.
Keywords: adult; article; artificial embolization; Asian; brain embolism; brain infarction; case report; cervical spinal cord injury; cervical spine injury; computer assisted tomography; coronary artery recanalization; deterioration; hemianopia; human; magnetic resonance angiography; male; posterior cerebral artery; priority journal; sensory dysfunction; treatment response; unconsciousness; vertebral artery stenosis; vomiting

Treatment of traumatic spondylolisthesis of the lower cervical spine with concomitant bilateral facet dislocations: Risk of respiratory deterioration - Ye Z.-W.; Yang S.-H.; Chen B.-J.; Xiong L.-M.; Xu J.-Z.; He Q.-Y. (Clinical Neurology and Neurosurgery, 2014)
This study aimed to retrospectively examine 36 cases of bilateral cervical facet dislocations (BCFD) of the lower cervical spine who were at risk for respiratory deterioration. Methods The cases of 36 subjects with BCFD of the lower cervical spine who failed to achieve closed reduction were retrospectively studied. The extents of neurological injuries included posterior neck pain without neurological deficit (n = 2), incomplete spinal cord injury (ISCI) (n = 21), and complete spinal cord injury (CSCI) (n = 13). Results Among the subjects, 26 (72.22%) had dyspnea, 6 required mechanical ventilation due to respiratory muscle paralysis, 11 required tracheostomy, and 9 required intubation. All patients received posterior approach reduction, stabilization, and fusion treatment for BCFD in one operative session. For the 26 quadriparetic patients with dyspnea, priority was given to treating their respiratory problems. For the other 10 patients without dyspnea, surgical treatment for irreducible lower cervical spine dislocation was given priority. After an average follow-up period of 63 months, 21 complications were found, but all patients exhibited fusion. Twenty-one patients with ISCI exhibited improvements in their conditions of 1 or 2 grades on the American Spinal Injury Association scale, whereas those with CSCI did not improve. All 26 apnea cases improved. The majority (26) of the 36 cases with BCFD of the lower cervical spine suffered dyspnea. Conclusions Although further study is required, our study suggests that the posterior surgical approach to the cervical spine is safe and effective for patients with traumatic spondylolisthesis of the lower cervical spine concomitant with BCFD who are at risk of respiratory deterioration. © 2014 Elsevier B.V.
Keywords: adolescent; adult; aged; article; artificial ventilation; bilateral cervical facet dislocation; cervical spine dislocation; clinical article; diaphragm paralysis; dyspnea; female; fracture reduction; human; intubation; male; neck pain; patient safety; quadriplegia; rating scale; retrospective study; risk factor; spinal cord injury; spine fusion; spine stabilization; spondylolisthesis; surgical approach; tracheostomy; treatment failure

Attitudes to the potential development and use of units providing weaning from mechanical ventilation away from intensive care in the UK - Parker R.J.; Bentley A.M. (Journal of the Intensive Care Society, 2014)
Long-term ventilation and weaning units (LTVWUs) are common in the USA and Europe. Their development was recommended in a NHS Modernisation Agency report in 20021 and recently by the NHS Commissioning Board.2 Despite this there are only a small number of units in existence in the UK. We have performed an internet based survey of intensive care professionals to assess potential need and particularly attitudes towards their development. We obtained 180 responses to our questionnaire, at least in part, from 97 doctors (94 consultants), and 73 allied health professionals, working in both teaching and district hospitals. Prolonged mechanical ventilation (PMV) was a significant workload for 84.1% of respondents, 90.4% have ≥1 patient/day fulfilling criteria for weaning delay and 76.8% ≥1 patient/day meeting criteria for weaning failure. A majority of respondents (70.8%) felt this impacts on providing acute care and 82.1% feel this workload will increase in future. LTVWUs for spinal injury patients are available to 54.1% of respondents compared with 40% of non-spinal injured patients (p<0.05). Only 16.1% said their hospital was capable of managing tracheostomy ventilated patients in a facility outside intensive care, most commonly in teaching hospitals (p<0.05). For those without access 93% would consider referring patients requiring PMV for ongoing care to a LTVWU. Benefits of LTVWU were felt to be, increasing the availability of acute ICU beds (92.6%) and focusing rehabilitation resources (85.8%). Most common barriers to referral were concerns about safety of patients away from ICU (51.9%), a lack of proven benefit (48.3%) and an unwillingness to refer until patient felt to be truly ventilator dependent and community placement needed (43.2%). Respondents believe future LTVWUs should be regionally based (84.6%), linked to local critical care networks (76.6%), and led by multidisciplinary teams (56.4%). 24.4% of respondents felt the clinical lead should be a intensive care physician. Respondents in teaching hospitals had access to more ICU beds, but were significantly more likely to identify patients with weaning delay and failure. They perceived a bigger future problem compared with district hospitals, 95.7% vs 78.2% respectively (p<0.05). In conclusion, this survey supports the view that patients requiring PMV are perceived as a significant workload and impact on the availability of acute ICU beds. LTVWUs are a potential but currently limited resource. We would recommend that if LTVWUs are to grow and ultimately be successful intensive care practitioners actively engage in the commissioning process for their development, their clinical leadership, they are linked to intensive care operational delivery networks and adhere to the same standards of care.
Keywords: weaning; artificial ventilation; intensive care; United Kingdom; human; patient; workload; physician; public hospital; teaching hospital; spine injury; questionnaire; emergency care; Internet; teaching; Europe; health practitioner; ventilator; air conditioning; safety; rehabilitation; ventilated patient; tracheostomy; hospital; consultation; community; intensivist; leadership

Reliability of the spine adverse events severity system (SAVES) for individuals with traumatic spinal cord injury - Glennie R.A.; Noonan V.K.; Fallah N.; Park S.E.; Thorogood N.P.; Cheung A.; Fisher C.G.; Dvorak M.F.; Street J.T. (Spinal Cord, 2014)
Study design:Test-retest analysis. Objectives:To determine the intra- and inter-rater reliability of the Spine Adverse Events Severity System for Spinal Cord Injury (SAVES-SCI) in patients with traumatic SCI. Setting:Quaternary care spine program in Vancouver, Canada. Methods:Ten hypothetical patient cases were developed. The cases were completed by 10 raters (seven physicians, one nurse, one physiotherapist and one researcher) who were asked to identify and grade the severity of adverse events using SAVES-SCI twice with 1-week interval. Intra- and inter-rater reliability were calculated using kappa statistics and intraclass correlation coefficients (ICC). Results:Intra-rater reliability for both identifying and grading AEs were high with all AEs (kappa greater than 0.6) except for bone implant, diathermy burn, massive blood loss, myocardial infarction, neurological deterioration, pressure ulcer, return to operating room and tracheostomy requirment. The inter-rater reliability measured with ICC were all above 0.6 for identifying and grading intraoperative, pre and postoperative AEs and consequences of SCI. Conclusions:The SAVES-SCI demonstrated acceptable intra-and inter-rater reliability for a majority of the AEs. Further clarification and definition of some of the AEs as well as provision of sample training cases for clincians would assist in reducing measurement errors. The SAVES-SCI is a useful tool to assess and capture AEs in patients with acute traumatic SCI.Sponsorship:Funded by Rick Hansen Institute and Health Canada.
Keywords: article; Canada; correlation coefficient; human; interrater reliability; intrarater reliability; kappa statistics; assessment of humans; nurse; peroperative complication; physician; physiotherapist; postoperative complication; preoperative complication; spinal cord injury; spine adverse events severity system

Repair of retrograde ascending dissection after descending stent grafting - Idrees J.; Arafat A.; Johnston D.R.; Svensson L.G.; Roselli E.E. (Journal of Thoracic and Cardiovascular Surgery, 2014)
Objective: Retrograde dissection is now recognized as an important complication after thoracic endovascular aortic repair (TEVAR), but its treatment is poorly understood. Our objectives were to investigate the risks, describe the repair methods, and assess the outcomes of this complication. Methods: From 2000 to 2012, 766 patients underwent TEVAR. Of these patients, 14 (1.8%), plus 1 who had undergone TEVAR elsewhere (n = 15), developed retrograde dissection after stent grafting. They had undergone TEVAR for distal aortic dissection in 7, intramural hematoma in 5, aneurysm in 2, and transection in 1. Their mean age was 65 ± 9 years. At the initial TEVAR, the left subclavian artery was covered in 9, the mean stent graft diameter was 34 ± 2 mm, and >1 device was used in 8 patients. The site of entry tear was at the greater curvature in 11 and lesser curvature in 4. One patient ruptured and died 12 days after TEVAR and never made it to the operating room. The other 14 underwent proximal aortic repair. The median interval between TEVAR and repair of retrograde dissection was 6 months; 3 patients presented within 1 month. The repair techniques included reverse frozen elephant trunk in 5, total arch repair in 4, ascending or hemiarch repair in 4, and ascending TEVAR in 1. Concomitant procedures included aortic valve repair in 4, replacement in 2, root remodeling in 1, and coronary bypass in 1. Results: No operative mortality occurred. One patient underwent reoperation for bleeding. Two required a tracheostomy for respiratory failure. However, no renal failure, stroke, or spinal injury occurred. At a median follow-up of 26 months, 4 aortic reoperations had occurred: 1 distal stent graft extension for type 1b endoleak, 2 hybrid thoracoabdominal completion repairs for growth of residual distal disease, and 1 emergency TEVAR for aortobronchial fistula. The latter patient died of septic complications, and 3 other late noncardiac deaths occurred. Conclusions: Retrograde ascending dissection can present as an early or a late complication after descending stent grafting because of aortic instability or disease progression and has usually been associated with descending dissection or intramural hematoma. It is a life-threatening complication that can be managed safely with early recognition and rapid delivery of open or hybrid repair. Copyright © 2014 by The American Association for Thoracic Surgery.
Keywords: adult; aged; aortic aneurysm; aortic dissection; aortic reconstruction; aortic valve replacement; aortic aneurysm endovascular graft; aortic arch surgery; aortic root surgery; aortic valve repair; bleeding; cardiovascular risk; conference paper; coronary artery bypass graft; descending aortic surgery; endoleak; endovascular aneurysm repair; follow up; hematoma; human; major clinical study; operating room; postoperative infection; priority journal; reoperation; respiratory failure; retrograde ascending dissection; reverse frozen elephant trunk; subclavian artery; surgical mortality; tracheostomy; Gore-Tex; zenith

Postoperative spine and VEPTR infections in children: A case-control study - Farley F.A.; Li Y.; Gilsdorf J.R.; VanderHave K.L.; Hensinger R.N.; Speers M.; Childers D.; Caird M.S. (Journal of Pediatric Orthopaedics, 2014)
Background: Surgical site infection (SSI) after pediatric scoliosis surgery is a major cause of morbidity. We compared the odds ratios of various potential risk factors for infection among patients who developed a deep SSI following spinal deformity surgery and those who remained infection free. Methods: This was a case-control study, not a matched study. More noninfection cases (50) than infection cases (20) were selected because more were available. Twenty children with a deep SSI after scoliosis surgery were compared with 50 similar children who did not develop a deep SSI. Fourteen perioperative factors were examined in both the groups. Results: Of the 20 patients who had a deep SSI, 14 had neuromuscular scoliosis. In the infected group, 6 patients had undergone vertical expandable prosthetic titanium rib placement, 2 had undergone growing rod insertion, and 12 had undergone posterior spinal fusion. Eighteen patients developed a SSI within 1 year of the operation and 2 patients presented with a SSI >1 year after surgery. Sixteen patients had positive cultures. Majority were skin flora: coagulase-negative Staphylococcus (8) and Propionibacterium acnes (4). Both patients with tracheostomies had Enterococcus faecalis infections. When comparing the 20 patients with deep SSI to the 50 controls, increased preoperative Cobb angle (P=0.011), increased postoperative Cobb angle (P=0.0043), nonambulatory status (P=0.0002), and increased length of stay (P=0.015) were associated with significantly increased odds of infection. Conclusions: Our study shows that patients with neuromuscular scoliosis are at higher risk of developing a deep SSI after spinal deformity surgery. Skin flora is a common cause of deep SSI. We have now instituted a standard skin preparation protocol to include alcohol and chlorhexidine washes the night before and the morning of surgery. We have altered our prophylactic antibiotic regimen to cover skin flora in all patients and gastrointestinal flora in patients with a tracheostomy. We have counseled the families of nonambulatory children with large neuromuscular curves regarding the significantly increased odds of postoperative deep SSI. Level of evidence: Level III. Copyright © 2013 by Lippincott Williams & Wilkins.
Keywords: alcohol; chlorhexidine; titanium; adolescent; article; bone prosthesis; case control study; child; clinical article; coagulase negative Staphylococcus; controlled study; Enterococcus faecalis; female; human; infection rate; infection risk; length of stay; male; preschool child; priority journal; Propionibacterium acnes; school child; scoliosis; skin flora; spine; spine fusion; spine malformation; spine surgery; surgical infection; tracheostomy; vertical expandable prosthetic titanium rib

Anesthesia management of a patient with lumbar spinal stenosis and tracheal stenting - Canpolat D.G.; Çelik U.; Aydin A.; Biçer C.; Madenoǧlu H. (Erciyes Tip Dergisi, 2014)
Tracheal stenosis may occur after congenital problems and long-term endotracheal tube compression. Spinal stenosis is one of the 3 most common reasons in patients undergoing surgery due to low back and leg pain. Both general and regional anesthesia are used for lumbar surgery. A 56-year-old woman was admitted to our hospital with low back pain and leg paresthesia. A permanent tracheal stent was placed 11 years ago due to tracheal stenosis developed after tracheostomy performed for anaphylaxis and respiratory depression. Herein, a case of regional anesthesia technique applied during spinal stenosis surgery is presented. © 2014 by Erciyes University School of Medicine.
Keywords: bupivacaine; adult; article; body position; case report; computer assisted tomography; endotracheal tube; female; forced expiratory volume; forced vital capacity; human; intensive care unit; discectomy; laminectomy; leg pain; low back pain; lumbar disk hernia; lumbar spinal stenosis; lung function test; metal stent; middle aged; nuclear magnetic resonance imaging; oxygen saturation; paresthesia; regional anesthesia; respiration depression; spinal anesthesia; spinal cord decompression; spinal needle; trachea stenosis; tracheobronchial stent; tracheostomy; marcaine; 22G Quincke

Surgical strategies and outcomes of spinal ependymomas of different lengths: Analysis of 210 patients: Clinical article - Li T.-Y.; Chu J.-S.; Xu Y.-L.; Yang J.; Wang J.; Huang Y.-H.; Kwan A.-L.; Wang G.-H. (Journal of Neurosurgery: Spine, 2014)
Object. The aim of this study was to investigate the surgical strategies and outcomes for spinal ependymomas of different lengths. Methods. The authors used data from 210 patients with spinal ependymomas (WHO Grades II and III) in this 10-year retrospective study (January 1999 to December 2008), dividing them into 3 different groups according to length (spinal ependymomas < 5 cm, 5-10 cm, and > 10 cm). All patients underwent tumor resection. The basic characteristics of the patients were reviewed and the functional status was assessed using the McCormick classification. Results. There were 89, 81, and 40 patients, respectively, in the 3 groups (< 5 cm, 5-10 cm, and > 10 cm). Gross-total resections (GTRs) were performed in 172 patients (81.9% overall, or 86.5%, 79.0%, and 77.5% in the 3 groups, respectively). Subtotal and partial resections were achieved in 38 patients (18.1%). Eight patients with medulla oblongata or upper cervical cord tumors received a tracheotomy postoperatively. The follow-up period ranged from 56 to 176 months. One hundred thirty-five patients (76.7%) experienced improvement, (88.2%, 83.8%, and 34.4% in the < 5 cm, 5-10 cm, and > 10 cm groups, respectively). Thirty-three patients (18.8%) maintained their pretreatment status, and 8 patients (4.5%) showed deterioration following tumor resection at 6 months. Tumor recurrence or progression was observed in 6 (2.9%) of the 210 patients. Among the 6 patients, recurrent tumors were located in the conus (n = 3), thoracic (n = 1), and medullocervical cord (n = 2). Conclusions. Radical resection of spinal ependymomas could be performed in most patients, and the rate of GTR was significantly different in the different-length groups (< 10 cm vs > 10 cm, p = 0.032). Patients with longer tumors had worse surgical results compared with those with small tumors (p < 0.001), and more postoperative neuropathic pain and proprioceptive deficits could usually be observed in patients harboring larger tumors. Early diagnosis and timely operation are critical to achieving better neurological outcomes. For tumors with dense adhesions, complete removal should be performed cautiously because of the significant incidence of neurological deterioration. ©AANS, 2014.
Keywords: adolescent; adult; aged; article; bladder dysfunction; cancer prognosis; cancer surgery; cervical spinal cord; child; conus medullaris; deterioration; ependymoma; motor evoked potential; somatosensory evoked potential; female; follow up; functional status; human; major clinical study; male; medulla oblongata; neuropathic pain; nuclear magnetic resonance imaging; pain; paresthesia; postoperative care; proprioception; radical resection; retrospective study; spinal ependymoma; surgical technique; tracheotomy; tumor bleeding; tumor recurrence

A 24-year-old with spinal cord injury and an unusual polysomnogram - Khosa S.; Ali M.E.; Krishnan V. (Annals of the American Thoracic Society, 2014)
One of the challenges in interpreting sleep studies is determining whether findings are real or artifacts of electromechanical phenomena. The artifact seen on this study was consistent with the patient's history, but was an unusual finding for typical patients undergoing sleep studies. The findings are reviewed in the context of the clinical presentation of the case patient.
Keywords: adult; airway obstruction; article; artificial ventilation; bronchoscopy; case report; electrostimulation; follow up; human; hypoventilation; male; muscle stimulator; polysomnography; signal transduction; sleep disordered breathing; spinal cord injury; tracheostomy; young adult

Three-column osteotomies of the lower cervical and upper thoracic spine: comparison of early outcomes, radiographic parameters, and peri-operative complications in 48 patients - Theologis A.A.; Tabaraee E.; Funao H.; Smith J.S.; Burch S.; Tay B.; Kebaish K.; Deviren V.; Ames C. (European Spine Journal, 2014)
Purpose: To evaluate and compare early radiographic and clinical outcomes of lower cervical and upper thoracic three-column osteotomies (3CO) for cervicothoracic kyphosis correction. Methods: Patients who underwent 3CO at the cervicothoracic junction at two institutions were retrospectively reviewed. Patients were divided into two groups: lower cervical osteotomy (LCO) and upper thoracic osteotomy (UTO: T1–T5). Operative data, radiographic alignment, peri-operative complications, and clinical outcomes were compared between the groups. Results: Forty-eight patients [male: 24; female: 24; average age 61 years (range 18–92 years); mean follow-up: 22 months] met inclusion criteria. A total of 24 pedicle subtraction osteotomies and 24 vertebral column resections were performed. Compared to UTO, LCO operative time was significantly shorter, average ICU and hospital stays were significantly longer, and the average pre-operative cervical sagittal vertical axis (SVA) and kyphosis were significantly greater (p < 0.05). For both groups, there was significant improvement in cervical SVA, cervical lordosis, segmental angle, Neck Disability Index (NDI), SRS Activity, and pain visual analog score (p < 0.05). Reoperation rates were similar between the groups (LCO: 33.3 %; UTO: 18 %, p = 0.28). Significantly, more patients required tracheostomy/gastrostomy tubes after LCO (3 vs. 0 in the UTO group, p = 0.03). Conclusions: Three-column posterior osteotomies at the cervicothoracic junction restored regional sagittal alignment and improved quality of life in this series of patients with rigid cervicothoracic deformity, albeit with high complication rates. Lower cervical osteotomies provided greater cervical SVA correction and were shorter operations, although they were associated with more complications and longer hospital and ICU stays compared to upper thoracic osteotomies.
Keywords: antibiotic agent; adult; aged; article; metastatic breast cancer; cerebral palsy; cervical lordosis; cervical plate; cervical sagittal vertical axis; cervical spine; cervicothoracic kyphosis; clinical article; controlled study; debridement; deep vein thrombosis; diffuse idiopathic skeletal hyperostosis; dysphagia; female; follow up; hospitalization; human; hyperostosis; intensive care unit; intermethod comparison; kyphosis; laminectomy; lordosis; lower cervical osteotomy; male; methicillin resistant Staphylococcus aureus infection; musculoskeletal system parameters; Neck Disability Index; neck pain; neurofibromatosis type 1; nutritional support; operation duration; osteotomy; outcome assessment; pathologic fracture; pedicle subtraction osteotomy; percutaneous endoscopic gastrostomy; peroperative complication; pneumonia; pneumothorax; postoperative period; priority journal; pseudarthrosis; radiculopathy; radiological parameters; reoperation; retrospective study; segmental angle; spine surgery; stomach tube; surgical infection; thoracic spine; three column osteotomy; tracheostomy; tracheostomy tube; upper thoracic osteotomy; vertebral column resection; very elderly; visual analog scale

Tracheostomy: Epidemiology, indications, timing, technique, and outcomes - Cheung N.H.; Napolitano L.M. (Respiratory Care, 2014)
Tracheostomy is a common procedure performed in critically ill patients requiring prolonged mechanical ventilation for acute respiratory failure and for airway issues. The ideal timing (early vs late) and techniques (percutaneous dilatational, other new percutaneous techniques, open surgical) for tracheostomy have been topics of considerable debate. In this review, we address general issues regarding tracheostomy (epidemiology, indications, and outcomes) and specifically review the literature regarding appropriate timing of tracheostomy tube placement. Based on evidence from 2 recent large randomized trials, it is reasonable to wait at least 10 d to be certain that a patient has an ongoing need for mechanical ventilation before consideration of tracheostomy. Percutaneous tracheostomy with flexible bronchoscopy guidance is recommended, and optimal percutaneous techniques, indications, and contraindications and results in high-risk patients (coagulopathy, thrombocytopenia, obesity) are reviewed. Additional issues related to tracheostomy diagnosis-related groups, charges, and procedural costs are reviewed. New advances regarding tracheostomy include the use of real-time ultrasound guidance for percutaneous tracheostomy in high-risk patients. New tracheostomy tubes (tapered with low-profile cuffs that fit better on the tapered dilators, longer percutaneous tracheostomy tubes) are discussed for optimal use with percutaneous dilatational tracheostomy. Two new percutaneous techniques, a balloon inflation technique (Dolphin) and the PercuTwist procedure, are reviewed. The efficacy of tracheostomy teams and tracheostomy hospital services with standardized protocols for tracheostomy insertion and care has been associated with improved outcomes. Finally, the UK National Tracheostomy Safety Project developed standardized resources for education of both health care providers and patients, including emergency algorithms for tracheostomy incidents, and serves as an excellent educational resource in this important area. © 2014 Daedalus Enterprises.
Keywords: artificial ventilation; bronchoscopy; chronic obstructive lung disease; clinical assessment; conference paper; hospitalization; human; injury; operation duration; patient satisfaction; postoperative complication; respiratory tract disease; risk assessment; spinal cord injury; surgical technique; tracheostomy; traumatic brain injury; treatment indication; treatment outcome

Endoscopic transnasal surgery as a replacement for maxillotomy techniques to approach the central skull base: Fewer complications and more acceptable to patients? - Choi D.; Subramanian A.; Elwell V.; Andrews P.; Roberts D.; Gleeson M. (Journal of Neurological Surgery, Part B: Skull Base, 2014)
Objectives To compare the complication rates of endoscopic transnasal and open maxillotomy approaches for the central skull base. Design Retrospective review. Setting Single-center study, London, United Kingdom. Participants From 1992 to 2012, 81 patients underwent surgery for skull base lesions, 59 by maxillotomy and 22 by endoscopy. Main Outcome Measures Total time of surgical anesthesia, blood loss, complications, duration of tracheal intubation, duration of hospital stay, myelopathy score, and mortality rate. Results The surgical time, blood loss, and duration of the postoperative intubation period were significantly less with endoscopy (p < 0.001). Requirements for intensive care, ward stay, and total hospital stay were also significantly less in the endoscopic group (p = 0.01, p < 0.001, and p < 0.001, respectively). The complication rate was lower with transnasal endoscopic surgery. Conclusion In patients for whom open maxillotomy or endoscopic surgery are both feasible, the preference should be to perform endoscopic surgery, with better visualization and fewer complications. © 2014 Georg Thieme Verlag KG Stuttgart New York.
Keywords: adult; anesthesia; artery injury; article; basilar impression; benign neoplasm; brain infarction; cancer surgery; chordoma; comparative study; controlled study; cranial nerve paralysis; dysphagia; endoscopic surgery; endoscopic transnasal surgery; endotracheal intubation; face; female; follow up; hematoma; human; intensive care; internal carotid artery; length of stay; liquorrhea; major clinical study; male; malignant neoplasm; maxillofacial surgery; maxillotomy; meningitis; nose surgery; nuclear magnetic resonance imaging; operation duration; operative blood loss; osteotomy; priority journal; retrospective study; sepsis; skull base; skull injury; spinal cord disease; surgical approach; surgical infection; surgical mortality; tracheostomy; tumor volume; United Kingdom

Esophageal, pharyngeal and hemorrhagic complications occurring in anterior cervical surgery: Three illustrative cases - Paradells V.R.; Pérez J.B.C.; Vicente F.J.D.; Florez L.B.; De La Viuda M.C.; Villagrasa F.J. (Surgical Neurology International, 2014)
Background: The number of esophageal and pharyngeal perforations occurring in anterior cervical surgeries ranges from 0.25% to 1% and 0.2% to 1.2%, respectively. Symptoms usually appear postoperatively and are attributed to: Local infection, fistula, sepsis, or mediastinitis. Acute postoperative hematoma, although very rare (<1%), is the first complication to rule out due to its life-threatening complications (e.g. acute respiratory failure). Case Description: Over a 36-year period, the author(s) described three severe esophageal/pharyngeal complications attributed to anterior cervical surgery. As these complications were appropriately recognized/treated, patients had favorable outcomes. Conclusions: Anterior cervical spine surgery is a safe approach and is associated with few major esophageal/pharyngeal complications, which most commonly include transient dysphagia and dysphonia. If symptoms persist, patients should be assessed for esophageal/pharyngeal defects utilizing appropriate imaging studies. Notably, even if the major complications listed above are adequately treated, optimal results are in no way guaranteed. Copyright: © 2014 Epstein NE.
Keywords: antibiotic agent; polyetheretherketone; tranexamic acid; adult; anterior cervical diskectomy and fusion; anterior corpectomy fusion; antibiotic therapy; article; aspiration pneumonia; bleeding; bone graft; bone plate; case report; cervical myelopathy; cervical spine; cervical spine fracture; device removal; disease severity; dysphagia; dysphonia; endotracheal intubation; esophagography; esophagus foreign body; female; hemostasis; hospital admission; hospital discharge; human; intervertebral disk hernia; joint swelling; laryngoscopy; ligament calcinosis; male; nasogastric tube; parenteral nutrition; postoperative complication; priority journal; respiratory distress; septic shock; spinal cord compression; spinal hematoma; spine fusion; spine scintiscanning; spine surgery; spondylolisthesis; surgical approach; thrombocyte aggregation; tracheotomy; traffic accident

Comparison of perioperative complications following staged versus one-day anterior and posterior cervical decompression and fusion crossing the cervico-thoracic junction - Siemionow K.; Tyrakowski M.; Patel K.; Neckrysh S. (Neurologia i Neurochirurgia Polska, 2014)
Introduction: Multilevel cervical pathology may be treated via combined anterior cervical decompression and fusion (ACDF) followed by posterior spinal instrumented fusion (PSIF) crossing the cervico-thoracic junction. The purpose of the study was to compare perioperative complication rates following staged versus same day ACDF combined with PSIF crossing the cervico-thoracic junction. Material and methods: A retrospective review of consecutive patients undergoing ACDF followed by PSIF crossing the cervico-thoracic junction at a single institution was performed. Patients underwent either same day (group A) or staged with one week interval surgeries (group B). The minimum follow-up was 12 months. Results: Thirty-five patients (14 females and 21 males) were analyzed. The average age was 60 years (37–82 years). There were 12 patients in group A and 23 in group B. Twenty-eight complications noted in 14 patients (40%) included: dysphagia in 13 (37%), dysphonia in 6 (17%), post-operative reintubation in 4 (11%), vocal cords paralysis, delirium, superficial incisional infection and cerebrospinal fluid leakage each in one case. Significant differences comparing group A vs. B were found in: the number of levels fused posteriorly (5 vs. 7; p = 0.002), total amount of intravenous fluids (3233 ml vs. 4683 ml; p = 0.03), length of hospital stay (10 vs. 18 days; p = 0.03) and transfusion of blood products (0 vs. 9 patients). Smoking and cervical myelopathy were the most important risk factors for perioperative complications regardless of the group. Conclusions: Staging anterior cervical decompression and fusion with posterior cervical instrumented fusion 1 week apart does not decrease the incidence of perioperative complications.
Keywords: adult; aged; anterior cervical decompression and fusion; anterior spine fusion; article; cervical myelopathy; clinical article; delirium; dysphagia; dysphonia; female; human; intermethod comparison; intubation; length of stay; liquorrhea; male; nuclear magnetic resonance imaging; operative blood loss; pneumonia; retrospective study; risk factor; surgical infection; tracheostomy; vocal cord paralysis

The process of prehospital airway management: Challenges and solutions during paramedic endotracheal intubation - Prekker M.E.; Kwok H.; Shin J.; Carlbom D.; Grabinsky A.; Rea T.D. (Critical Care Medicine, 2014)
OBJECTIVES: Endotracheal intubation success rates in the prehospital setting are variable. Our objective was to describe the challenges encountered and corrective actions taken during the process of endotracheal intubation by paramedics. DESIGN: Analysis of prehospital airway management using a prospective registry that was linked to an emergency medical services administrative database. SETTING: Emergency medical services system serving King County, Washington, 2006-2011. Paramedics in this system have the capability to administer neuromuscular blocking agents to facilitate intubation (i.e., rapid sequence intubation). PATIENTS: A total of 7,523 patients more than 12 years old in whom paramedics attempted prehospital endotracheal intubation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: An intubation attempt was defined as the introduction of the laryngoscope into the patient's mouth, and the attempt concluded when the laryngoscope was removed from the mouth. Endotracheal intubation was successful on the first attempt in 77% and ultimately successful in 99% of patients (7,433 of 7,523). Paramedics used a rapid sequence intubation strategy on 54% of first attempts. Among the subset with a failed first attempt (n = 1,715), bodily fluids obstructing the laryngeal view (50%), obesity (28%), patient positioning (17%), and facial or spinal trauma (6%) were identified as challenges to intubation. A variety of adjustments were made to achieve intubation success, including upper airway suctioning (used in 43% of attempts resulting in success), patient repositioning (38%), rescue bougie use (19%), operator change (16%), and rescue rapid sequence intubation (6%). Surgical cricothyrotomy (0.4%, n = 27) and bag-valve-mask ventilation (0.8%, n = 60) were rarely performed by paramedics as final rescue airway strategies. CONCLUSIONS: Airway management in the prehospital setting has substantial challenges. Success can require a collection of adjustments that involve equipment, personnel, and medication often in a simultaneous fashion. © 2014 by the Society of Critical Care Medicine and Lippincott Williams & Wilkins.
Keywords: neuromuscular blocking agent; article; assisted ventilation; body fluid; bougie; critically ill patient; emergency care; emergency health service; endotracheal intubation; heart arrest; human; jet ventilation; laryngoscope; larynx; major clinical study; manual emergency ventilator; obesity; paramedical personnel; patient positioning; priority journal; respiration control; spine injury; suction; total quality management; tracheotomy; upper respiratory tract

Development of a spinal epidural abscess masked by critical illness 2C01, C300 - Chandler B.; Francis J.; Harper D. (Journal of the Intensive Care Society, 2014)
We describe a case of severe neurological injury secondary to an epidural abscess that developed during the course of critical illness. It was caused by an oesophageal perforation that developed into an invasive abscess while the patient was being treated in the intensive care unit (ICU) for respiratory failure. We discuss spinal epidural abscesses, and the importance of searching for an alternative diagnosis in patients with weakness. © The Intensive Care Society 2014.
Keywords: antibiotic agent; C reactive protein; clavulanic acid; fluconazole; metronidazole; noradrenalin; penicillin G; ticarcillin; timentin; aged; article; artificial ventilation; aspiration pneumonia; atelectasis; bronchoscopy; case report; computer assisted tomography; confusion; epidural abscess; esophageal stent; esophagus perforation; female; fever; Glasgow coma scale; human; leukocyte count; lung edema; mediastinitis; Medical Research Council power score; oxygen desaturation; polyneuropathy; quadriplegia; respiratory failure; scoring system; shoulder pain; spinal epidural abscess; thorax radiography; tracheostomy

Experience with intraoperative navigation and imaging during endoscopic transnasal spinal approaches to the foramen magnum and odontoid. - Choudhri O.; Mindea S.A.; Feroze A.; Soudry E.; Chang S.D.; Nayak J.V. (Neurosurgical focus, 2014)
In this study the authors share their experience using intraoperative spinal navigation and imaging for endoscopic transnasal approaches to the odontoid in 5 patients undergoing C1-2 surgery for basilar invagination at Stanford Hospital and Clinics from 2010 to 2013. Of these 5 patients undergoing C1-2 surgery for basilar invagination, 4 underwent a 2-tiered anterior C1-2 resection with posterior occipitocervical fusion during a first stage surgery, followed by endoscopic endonasal odontoidectomy in a separate setting. Intraoperative stereotactic navigation was performed using a surgical navigation system in all cases. Navigation accuracy, characterized as target registration error, ranged between 0.8 mm and 2 mm, with an average of 1.2 mm. Intraoperative imaging using a CT scanner was also performed in 2 patients. Endoscopic decompression of the brainstem was achieved in all patients, and no intraoperative complications were encountered. All patients were extubated within 24 hours after surgery and were able to swallow within 48 hours. After appropriate initial reconstruction of the defect at the craniocervical junction, no postoperative CSF leakage, arterial injury, or need for reoperation was encountered; 1 patient developed mild postoperative velopharyngeal insufficiency that resolved by the 6-month follow-up evaluation. There were no deaths and no patients required tracheostomy placement. The average inpatient stay after surgery varied between 72 and 96 hours, without extended intensive care unit stays for any patient. Technologies such as intraoperative CT scanning and merged MRI/CT can provide the surgeon with detailed, virtual real-time information about the extent of complex endoscopic vertebral segment resection and brainstem decompression and lessens the prospect of revision or secondary procedures in this challenging surgical corridor. Moreover, patients experience limited morbidity and can tolerate early oral intake after transnasal endoscopic odontoidectomy. Essential to the successful undertaking of these endoscopic adventures is 1) an understanding of the endoscopic nasal, skull base, and neurovascular anatomy; 2) advanced and extended-length instrumentation including navigation; and 3) a team approach between experienced rhinologists and spine surgeons comfortable with endoscopic skull base techniques.
Keywords: aged; article; decompression surgery; endoscopy; female; foramen magnum; human; male; methodology; middle aged; neuronavigation; nuclear magnetic resonance imaging; odontoid process; patient monitoring; reoperation; spine fusion; very elderly

Procedure-based complications to guide informed consent: Analysis of society of thoracic surgeons-congenital heart surgery database - Mavroudis C.; Mavroudis C.D.; Jacobs J.P.; Siegel A.; Pasquali S.K.; Hill K.D.; Jacobs M.L. (Annals of Thoracic Surgery, 2014)
Background Informed consent refers to the process by which physicians and patients engage in a dialogue to explain and comprehend the nature, alternatives, and risks of a procedure or course of therapy. The goal of this study is to better "inform the process of informed consent" by offering empirically derived procedural complication lists that provide objective contemporary data that surgeons may share with patients and families. Methods The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for complications for 12 congenital heart operations (2010 to 2011) performed across all Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) risk categories. Results The 12 index procedures reviewed for rates of complications were repair of atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), tetralogy of Fallot (TOF), coarctation, and truncus arteriosus, as well as arterial switch operation (ASO), ASO-VSD, BiGlenn, Fontan, Norwood procedure, and systemic to pulmonary artery (S-P) shunt. Arrhythmia was the most frequent complication for VSD (5.8%), TOF (8.9%), and AVSD (14.7%) repairs. There was a high rate of sternum left open (planned, unplanned) for ASO (26%, 7%), ASO-VSD (29%, 10%), truncus repair (41%, 11%), and Norwood (63%, 7%). The most frequent complications for other procedures include ASD (unplanned readmission, 1.9%), BiGlenn (chylothorax, 7%), Fontan (pleural effusion, 16%), S-P shunt (reintubation, 10.6%), and coarctation (reintubation, 5.2%). Conclusions The informed consent process for congenital heart surgery may be served by accurate contemporary data on occurrence of complications. While a threshold rate of occurrence of individual complications may guide the physician, rare but important debilitating complications should also be discussed irrespective of frequency. We propose to better inform the process of informed consent by providing objective complications data. © 2014 by The Society of Thoracic Surgeons.
Keywords: acute kidney failure; aortic coarctation; aortic surgery; arterial switch operation; arterial trunk; article; artificial heart pacemaker; artificial ventilation; assisted circulation; atrioventricular septal defect; atrioventricular septal defect repair; bacterial endocarditis; BiGlenn procedure; Blalock Taussig shunt; cardiac surgeon; cardioversion; cavopulmonary connection; cerebrovascular accident; chylothorax; defibrillation; dialysis; diaphragm paralysis; Fallot tetralogy; Fallot tetralogy surgery; family; Fontan procedure; forward heart failure; heart arrest; heart arrhythmia; heart atrium septum defect; heart atrium septum defect repair; heart catheterization; heart failure; heart surgery; heart ventricle septum defect; heart ventricle septum defect repair; hospital discharge; hospital readmission; human; hypertensive crisis; information dissemination; informed consent; intubation; mediastinitis; medical society; multiple organ failure; neurologic disease; Norwood procedure; pericardial effusion; peripheral nerve injury; peroperative complication; pleura effusion; pneumonia; pneumothorax; postoperative complication; postoperative hemorrhage; priority journal; pulmonary hypertension; pulmonary vein obstruction; reoperation; respiratory failure; seizure; sepsis; spinal cord injury; sternotomy; sternum; surgical drainage; surgical infection; surgical mortality; surgical risk; thorax drainage; tracheostomy; vein occlusion; vocal cord disorder; wound dehiscence

Transient hyperckemia in the setting of neuromyelitis optica (NMO) - Malik R.; Lewis A.; Cree B.A.C.; Ratelade J.; Rossi A.; Verkman A.S.; Bollen A.W.; Ralph J.W. (Muscle and Nerve, 2014)
Introduction: Neuromyelitis optica (NMO) is characterized by inflammatory demyelinating lesions of the spinal cord and optic nerves from an autoimmune response against water channel aquaporin-4 (AQP4). We report 2 patients with transient hyperCKemia associated with NMO suggesting possible skeletal muscle damage. Methods: Patient 1 was a 72-year-old man who presented with muscle soreness and elevated serum creatine kinase (CK) preceding an initial attack of NMO. Patient 2 was a 25-year-old woman with an established diagnosis of NMO who presented with diffuse myalgias, proximal upper extremity weakness, and hyperCKemia. Muscle biopsies were obtained for histopathologic evaluation, protein gel electrophoresis, immunofluorescence, and complement staining. Results: In both patients the muscle showed only mild variation in fiber diameter. There were no inflammatory changes or muscle fiber necrosis, though there was reduced AQP4 expression and deposition of activated complement. Conclusions: Complement-mediated sarcolemmal injury may lead to hyperCKemia in NMO.
Keywords: alanine aminotransferase; aquaporin 4; aquaporin 4 antibody; aspartate aminotransferase; creatine kinase; prednisone; rituximab; steroid; adult; aged; alanine aminotransferase blood level; arm weakness; article; aspartate aminotransferase blood level; case report; creatine kinase blood level; lack of drug effect; endotracheal intubation; female; human; human tissue; hyperckemia; immunofluorescence; infectious complication; intensive care unit; male; muscle biopsy; muscle injury; muscle necrosis; myelooptic neuropathy; nuclear magnetic resonance imaging; paresthesia; plasmapheresis; priority journal; protein expression; respiratory distress; tracheostomy; young adult

Basic science and spine literature document bone morphogenetic protein increases cancer risk - Epstein N.E. (Surgical Neurology International, 2014)
Background: Increasingly, clinical articles document that bone morphogenetic protein (BMP/INFUSE: Medtronic, Memphis, TN, USA) and its derivatives utilized in spinal surgery increase the risk of developing cancer. However, there is also a large body of basic science articles that also document that various types of BMP and other members of the TGF-Beta (transforming growth factor beta) family promote the growth of different types of cancers. Methods: This review looks at many clinical articles citing BMP/INFUSE's role, largely "off-label", in contributing to complications encountered during spinal surgery. Next, however, specific attention is given to the clinical and basic science literature regarding how BMP and its derivatives (e.g. members of the TGF-beta family) may also impact the development of breast and other cancers. Results: Utilizing BMP/INFUSE in spine surgery increased the risk of cancers/new malignancy as documented in several studies. For example, Carragee et al. found that for single-level instrumented posterolateral fusions (PLF) using high-dose rhBMP-2 (239 patients) vs. autograft (control group; n = 224), the risks of new cancers at 2 and 5 years postoperatively were increased. In laboratory studies, BMP's along with other members of the TGF-Beta family also modulated/contributed to the proliferation/differentiation of breast cancer (e.g. bone formation/turnover, breast cancer-related solid tumors, and metastases), lung, adrenal, and colon cancer. Conclusions: BMP/INFUSE when utilized clinically in spinal fusion surgery appears to promote cancer at higher rates than observed in the overall population. Furthermore, BMP and TGF-beta are correlated with increased cancer growth both in the clinic and the laboratory.
Keywords: estrogen receptor alpha; osteopontin; recombinant bone morphogenetic protein 2; transforming growth factor beta; adenocarcinoma cell line; adrenal cancer; angiogenesis; anterior interbody lumbar fusion; arachnoiditis; article; autograft; bone graft; bone turnover; breast calcification; breast cancer; breast epithelium cell; cancer growth; cancer risk; carcinogenesis; cell activity; cell differentiation; cell migration; cell proliferation; colon cancer; degenerative disease; disease severity; drug efficacy; drug megadose; drug safety; dysphagia; edema; ejaculation disorder; fibroblast; gene expression; genetic variability; hematoma; heterotopic ossification; human; infection; intermethod comparison; intubation; lumbar spine degenerative disease; lumbar spine degenerative disease; lung cancer; medical literature; nerve root injury; neurologic disease; nonhuman; off label drug use; ossification; posterolateral fusion; postoperative complication; postoperative period; priority journal; protein expression; radiculitis; randomized controlled trial (topic); seroma; signal transduction; spine fusion; swelling; tracheostomy; tumor invasion; INFUSE

Surgical emergencies in oncology - Bosscher M.R.F.; van Leeuwen B.L.; Hoekstra H.J. (Cancer Treatment Reviews, 2014)
An oncologic emergency is defined as an acute, potentially life threatening condition in a cancer patient that has developed as a result of the malignant disease or its treatment. Many oncologic emergencies are signs of advanced, end-stage malignant disease. Oncologic emergencies can be divided into medical or surgical. The literature was reviewed to construct a summary of potential surgical emergencies in oncology that any surgeon can be confronted with in daily practice, and to offer insight into the current approach for these wide ranged emergencies.Cancer patients can experience symptoms of obstruction of different structures and various causes. Obstruction of the gastrointestinal tract is the most frequent condition seen in surgical practice. Further surgical emergencies include infections due to immune deficiency, perforation of the gastrointestinal tract, bleeding events, and pathological fractures.For the institution of the appropriate treatment for any emergency, it is important to determine the underlying cause, since emergencies can be either benign or malignant of origin. Some emergencies are well managed with conservative or non-invasive treatment, whereas others require emergency surgery. The patient's performance status, cancer stage and prognosis, type and severity of the emergency, and the patient's wishes regarding invasiveness of treatment are essential during the decision making process for optimal management. © 2014 Elsevier Ltd.
Keywords: airway obstruction; biliary tract drainage; bleeding; cancer hormone therapy; cancer patient; cholecystectomy; cholecystitis; cholelithiasis; cholestasis; colon perforation; colorectal cancer; digestive system perforation; diverticulitis; emergency; endoscopic sphincterotomy; esophagus cancer; esophagus obstruction; external beam radiotherapy; gallbladder perforation; gastrectomy; gastrointestinal obstruction; gastrojejunostomy; gastrostomy; human; immune deficiency; infection; intestine obstruction; intestine volvulus; intussusception; jejunostomy; laparotomy; malnutrition; melanoma; metastasis; ovary cancer; palliative therapy; patient selection; pelvis cancer; quality of life; radiation enteropathy; review; small intestine obstruction; spinal cord compression; stomach cancer; stomach obstruction; stomach paresis; tracheotomy; tumor perforation; urinary tract obstruction

Successful liver transplantation in a patient with quadriparesis: A case report - Taesombat W.; Nonthasoot B.; Sirichindakul B.; Supaphol J.; Nivatwongs S. (Transplantation Proceedings, 2014)
Major abdominal surgeries, including liver transplantation, are considered high-risk procedures for patients with respiratory muscle dysfunction, such as patients with quadriparesis, due to possible fatal postoperative pulmonary complications. We report on a 57-year-old male patient with longstanding quadriparesis due to fifth cervical spine injury from a traffic accident who suffered from decompensated liver cirrhosis related to hepatitis C infection and hepatocellular carcinoma. A preoperative pulmonary function test showed forced expiratory volume in 1 minute (FEV1) 1.06 L, which was a risk for pulmonary complications. The patient required respiratory training. Cadaveric liver transplantation was performed successfully without surgical complications. The patient was extubated on the fourth day after surgery and initially did well. However, on the eighth postoperative day, an episode of status epilepticus from metabolic derangement developed. After controlling seizure with anticonvulsive medication and sedation, the patient was reintubated due to hypoventilation. Chest radiograph showed upper lung atelectasis. Due to this complication, tracheostomy was performed. The patient's condition gradually improved. He was ultimately discharged on the 45th postoperative day. Two months after the transplantation, liver functions were normal and the patient could breathe spontaneously without tracheostomy and had good quality of life. In conclusion, this is, to our knowledge, the first report of liver transplantation in a patient with quadriparesis. It shows that even with a very high risk for postoperative pulmonary complications, liver transplantation can be performed successfully with careful patient selection and effective respiratory care. © 2014 by Elsevier Inc. All rights reserved.
Keywords: conference paper; liver transplantation; priority journal

Spinal cord injury (SCI) - Aspects of intensive medical care - Leyk G.; Hirschfeld S.; Böthig R.; Willenbrock U.; Thietje R.; Lönnecker S.; Stuhr M. (Anasthesiologie Intensivmedizin Notfallmedizin Schmerztherapie, 2014)
Keywords: article; cardiovascular system; human; incidence; intensive care; intubation; patient monitoring; thromboembolism; tracheotomy

Airway management of fixed cervical spine undergoing transoral odontoidectomy: A novel technique - Hariharan U.; Ganguly N.; Sood J.; Shastri P. (Current Medicine Research and Practice, 2014)
Difficult airway can present in many ways: difficult mask ventilation, difficult oral or nasal intubation and/or difficult surgical airway. Either an oral or a nasal tube may be required, depending on the surgical exposure required, in complex head and neck surgeries. We present a unique case of nasal to orotracheal tube conversion with the help of a Ryles tube and rail-roading technique, in a setting of difficult airway. This case highlights that we have to keep our options open to innovative techniques for securing the desired airway in different circumstances.
Keywords: fentanyl; lidocaine; midazolam; adult; airway edema; article; artificial ventilation; bronchoscope; case report; fiberoptic bronchoscopy; fibreoptic bronchoscope; fibreoptic intubation; human; intubation; male; nasal intubation; nasotracheal tube; odontoid process; quadriplegia; respiration control; ryles tube; spasticity; spine stabilization; tracheostomy; transoral odontoidectomy

Early patency rate and fate of reattached intercostal arteries after repair of thoracoabdominal aortic aneurysms - Omura A.; Yamanaka K.; Miyahara S.; Sakamoto T.; Inoue T.; Okada K.; Okita Y. (Journal of Thoracic and Cardiovascular Surgery, 2014)
Objectives The present study analyzes the early patency of intercostal artery reconstruction, using graft interposition and aortic patch anastomosis, and determines the fate of reattached intercostal arteries after repair of thoracoabdominal aortic aneurysms. Methods We selected 115 patients (mean age, 63 ± 15 years; range, 19-83 years; male, n = 83) treated by thoracoabdominal aortic aneurysm repair with 1 or more reconstructed intercostal arteries at the Kobe University Graduate School of Medicine between October 1999 and December 2012. The intercostal arteries were reconstructed using graft interposition (n = 66), aortic patch anastomosis (n = 42), or both (n = 7). Results The hospital mortality rate was 7.8% (n = 9). Eleven patients (9.6%) developed spinal cord ischemic injury (permanent, n = 6, transient, n = 5). The average number of reconstructed intercostal arteries per patient was 3.0 ± 1.5 (1-7), and 345 intercostal arteries were reattached. The overall patency rate was 74.2% (256/345) and that of aortic patch anastomosis was significantly better than that of graft interposition (90.8% [109/120] vs 65.3% [147/225], P < .01), but significantly worse for patients with than without spinal cord ischemic injury (51.9% [14/27] vs 76.1% [242/318], P = .01). There was no patch aneurysm in graft interposition during a mean of 49 ± 38 (range, 2-147) postoperative months, but aortic patch anastomosis including 4 intercostal arteries became dilated in 2 patients. Conclusions Aortic patch anastomosis might offer better patency rates and prevent spinal cord ischemic injury compared with graft interposition. Although aneurysmal changes in intercostal artery reconstructions are rare, large blocks of aortic wall reconstruction should be closely monitored.
Keywords: abdominal aorta; adult; aged; anastomosis; aortic root; aortic surgery; aortic patch anastomosis; artery; artery reconstruction; article; ascending aorta; brain hemorrhage; cardiovascular mortality; computer assisted tomography; dacron implant; descending aorta; endovascular aneurysm repair; female; follow up; graft interposition; graft patency; hemodialysis; human; intercostal artery; major clinical study; male; mesenteric ischemia; postoperative complication; postoperative hemorrhage; priority journal; respiratory failure; risk factor; sepsis; spinal cord ischemia; surgical mortality; surgical technique; survival rate; thoracic aorta; thoracoabdominal aorta aneurysm; time to treatment; tracheostomy; vascular patency

Expert’s comment concerning Grand Rounds case entitled: “Trans-oral approach for the management of a C2 neuroblastoma” (K. M. I. Salem, J. Visser, and N. A. Quraishi) - Choi D. (European Spine Journal, 2014)
Keywords: article; c2 neuroblastoma; cancer radiotherapy; cranial nerve paralysis; fatigue; human; nasotracheal tube; neuroblastoma; neuroimaging; nuclear magnetic resonance imaging; priority journal; second cervical vertebra; spinal cord compression; surgical technique; tracheostomy; transoral surgical technique; X ray

Aggresome-autophagy involvement in a sarcopenic patient with rigid spine syndrome and a p.C150R mutation in FHL1 gene - Sabatelli P.; Castagnaro S.; Tagliavini F.; Chrisam M.; Sardone F.; Demay L.; Richard P.; Santi S.; Maraldi N.M.; Merlini L.; Sandri M.; Bonaldo P. (Frontiers in Aging Neuroscience, 2014)
The four-and-half LIM domain protein 1 (FHL1) is highly expressed in skeletal and cardiac muscle. Mutations of the FHL1 gene have been associated with diverse chronic myopathies including reducing body myopathy, rigid spine syndrome (RSS), and Emery-Dreifuss muscular dystrophy. We investigated a family with a mutation (p.C150R) in the second LIM domain of FHL1. In this family, a brother and a sister were affected by RSS, and their mother had mild lower limbs weakness. The 34-year-old female had an early and progressive rigidity of the cervical spine and severe respiratory insufficiency. Muscle mass evaluated by DXA was markedly reduced, while fat mass was increased to 40%. CT scan showed an almost complete substitution of muscle by fibro-adipose tissue. Muscle biopsy showed accumulation of FHL1 throughout the cytoplasm and around myonuclei into multiprotein aggregates with aggresome/autophagy features as indicated by ubiquitin, p62, and LC3 labeling. DNA deposits, not associated with nuclear lamina components and histones, were also detected in the aggregates, suggesting nuclear degradation. Ultrastructural analysis showed the presence of dysmorphic nuclei, accumulation of tubulofilamentous and granular material, and perinuclear accumulation of autophagic vacuoles. These data point to involvement of the aggresome-autophagy pathway in the pathophysiological mechanism underlying the muscle pathology of FHL1 C150R mutation. © 2014 Sabatelli, Castagnaro, Tagliavini, Chrisam, Sardone, Demay, Richard, Santi, Maraldi, Merlini, Sandri and Bonaldo.
Keywords: alpha crystallin; beta crystallin; collagen type 6; desmin; four and half LIM domain protein 1; glucose regulated protein 78; LIM protein; sequestosome 1; ubiquitin; unclassified drug; adult; aggresome; amino acid substitution; article; artificial ventilation; autophagy; body composition; body fat; case report; computer assisted tomography; contracture; cytoplasm; endomysium; exon; fat mass; female; forced vital capacity; human; joint limitation; lean body weight; limb weakness; male; missense mutation; muscle atrophy; muscle biopsy; muscle mass; muscle rigidity; nuclear lamina; perimysium; phenotype; protein aggregation; respiratory failure; rigid spine syndrome; sarcopenia; sarcoplasmic reticulum; spine disease; tracheostomy; underweight; upregulation

Prevalence of difficult airway predictors in cases of failed prehospital endotracheal intubation - Gaither J.B.; Spaite D.W.; Stolz U.; Ennis J.; Mosier J.; Sakles J.J. (Journal of Emergency Medicine, 2014)
Background Difficult airway predictors (DAPs) are associated with failed endotracheal intubation (ETI) in the emergency department (ED). However, little is known about the relationship between DAPs and failed prehospital ETI. Objective Our aim was to determine the prevalence of common DAPs among failed prehospital intubations. Methods We reviewed a quality-improvement database, including all cases of ETI in a single ED, over 3 years. Failed prehospital (FP) ETI was defined as a case brought to the ED after attempted prehospital ETI, but bag-valve-mask ventilation, need for a rescue airway (supraglottic device, cricothyrotomy, etc.), or esophageal intubation was discovered at the ED. Physicians performing ETI evaluated each case for the presence of DAPs, including blood/emesis, facial/neck trauma, airway edema, spinal immobilization, short neck, and tongue enlargement. Results There were a total of 1377 ED ETIs and 161 had an FP-ETI (11.8%). Prevalence of DAPs in cases with FP-ETI was obesity 13.0%, large tongue 18.0%, short neck 13%, small mandible 4.3%, cervical immobility 49.7%, blood in airway 57.8%, vomitus in airway 23.0%, airway edema 12.4%, and facial or neck trauma 32.9%. The number of cases with FP-ETI and 0, 1, 2, 3, or 4 or more DAPs per case was 22 (13.6%), 43 (26.7%), 23 (24.3%), 42 (26.1%), and 31 (19.3%), respectively. Conclusions DAPs are common in cases of FP-ETI. Some of these factors may be associated with FP-ETI. Additional study is needed to determine if DAPs can be used to identify patients that are difficult to intubate in the field. © 2014 Elsevier Inc.
Keywords: adult; aged; article; controlled study; difficult airway predictor; emergency care; emergency ward; endotracheal intubation; esophagus intubation; face injury; failed prehospital endotracheal intubation; female; human; immobility; immobilization; lung edema; major clinical study; male; manual emergency ventilator; neck injury; obesity; prediction; prevalence; priority journal; respiration control; respiratory tract parameters; retrospective study; supraglottic airway device; survival prediction; total quality management; tracheotomy; treatment failure; vomiting