Radiological and clinical outcome after multilevel anterior cervical discectomy and/or corpectomy and fixation - Oni P.; Schultheiß R.; Scheufler K.-M.; Roberg J.; Harati A. (Journal of Clinical Medicine, 2018)
Background: Multilevel anterior cervical decompression and fixation of four and more levels is a common surgical procedure used for several diseases. Methods: We reviewed the radiological and clinical outcomes after anterior cervical discectomy or corpectomy and fixation of four and more levels in 85 patients (55 men and 30 women) with an average age of 59.6 years. Surgical indication was multilevel cervical degenerative myelopathy and radiculopathy in 72 (85%) patients, multilevel cervical spondylodiscitis in four (5%), complex traumatic cervical fractures in four (5%), metastatic cervical spine tumor in two (2%), and ossification of the posterior longitudinal ligament in three (3%) patients. Results: There were no severe intraoperative complications such as spinal cord or vertebral artery injury or dissection. Seventy-three patients had four, 10 patients had five, and two patients had six anterior cervical level fixations. The visual analog scale (VAS) and Japanese Orthopedic Association (mJOA) scale scores improved (6.9 to 1.3 (p < 0.001) and 13.9 to 16.5 (p < 0.001), respectively). The Cobb angle increased from 5.7° to 17.6° postoperatively (p < 0.001). Secondary posterior fixation was necessary in three cases due to pseudarthrosis. Conclusion: The anterior approach appears to be optimal for ventral compressive pathology and lordosis restoration to the cervical spine. Limitations of multiple level decompression and fixation included increasing pseudoarthrosis rates, especially after corpectomy, and increasing fused level numbers.
Keywords: Boker und Schultheib; hygo c Cage; locking screw; titanium mesh; adult; aged; article; biomechanics; cervical myelopathy; cervical spine fracture; Cobb angle; computer assisted tomography; controlled study; corpectomy; degenerative disease; discectomy; esophagus injury; evaluation and follow up; female; human; infection; intervertebral disk degeneration; Japanese Orthopaedic Association score; kyphosis; ligament calcinosis; lordosis; lung insufficiency; major clinical study; male; middle aged; motor function test; multilevel analysis; nuclear magnetic resonance imaging; osteomyelitis; osteosynthesis; pathology; peroperative care; platysma muscle; pseudarthrosis; quadriplegia; radiculopathy; radiodiagnosis; retrospective study; skin tingling; spinal cord compression; spinal hematoma; spondylosis; sternocleidomastoid muscle; Streptococcus anginosus; tracheostomy; upper limb; vertebral artery; visual analog scale; Boker und Schultheib; hygo c Cage

Axial chordoma of the cervical spine managed by a trans-mandibular trans-oral approach - Younus A.; Kelly A.; Lekgwara P. (Interdisciplinary Neurosurgery: Advanced Techniques and Case Management, 2018)
Chordomas are low-grade but locally aggressive malignant neoplasms derived from notochordal remnants. These lesions are best treated by en bloc however this is especially challenging in the upper cervical spine due to the proximity of vital structures. We present a 46 year old male whom presented to our unit complaining of progressive upper cervical axial neck pain aggravated by neck rotation, of 4 months duration. He had no symptoms of myelopathy. Examination revealed high posterior spinal tenderness and resistance to neck rotation however the patient's motor and sensory examinations were normal and he had normal gait. An inter-disciplinary team comprising Orthopedics, Neurosurgery, Maxillofacial surgery and Otorhinolaryngology took the patient for surgery. The first stage of the procedure comprised Otorhinolaryngology performing a tracheostomy and thereafter a combined Orthopedic Neurosurgical procedure involving a posterior instrumented C1–C3/C4 fusion and biopsy of the lesion. The second stage involved the maxillofacial surgeons dividing the patient's mandible to increase operative exposure. Thereafter the Orthopedic and Neurosurgical surgeons proceeded to perform a transmandibular transoral en bloc resection of the body and odontoid process of C2 which was replaced by a cage construct. The vertebral arteries were preserved and no dural breech occurred. Post operatively the patient showed no new neurology and was ambulant. He was maintained in a Philadelphia collar for 6 weeks until his fusion was complete. In conclusion this difficult case was managed successfully by thorough pre-operative planning and inter-departmental co-operation. Fortunately the lesion was isolated to the body and odontoid process of C2 which was in the patients favor lending the case towards a favorable outcome.
Keywords: adult; article; axial chordoma; cancer patient; cancer surgery; case report; chordoma; clinical article; dental surgeon; disease duration; human; laminectomy; male; mandible resection; medical history; middle aged; neck pain; nuclear magnetic resonance imaging; odontoid process; physical examination; priority journal; spine tumor; surgical approach; surgical patient; trans mandibular trans oral approach

Mechanical insufflation-exsufflation to improve secretion clearance during invasive ventilation - Terzi N.; Prigent H.; Lofaso F. (Respiratory Care, 2018)
Keywords: endotracheal tube; aeration; airway resistance; amyotrophic lateral sclerosis; artificial ventilation; aspiration; atelectasis; breathing exercise; bronchitis; coughing; editorial; human; hyperinflation; infection; intensive care unit; length of stay; lung clearance; mucus; noninvasive ventilation; pneumonia; respiratory care; spinal cord injury; total lung capacity; tracheostomy

Fatal innominate artery hemorrhage in a patient with tetraplegia: Case report and literature review - Qureshi A.Z. (Journal of Spinal Cord Medicine, 2018)
Context: Hemorrhage is one of the potentially fatal complications of tracheostomy. A rare but lethal cause of tracheostomy related bleeding is hemorrhage from the innominate artery. This occurs following tracheo-innominate artery fistula (TIF) formation, which is associated with a mortality rate of more than 85%. Here, we report the case of an individual with tetraplegia and a tracheostomy who died as a result of innominate artery hemorrhage. This case highlights the possible causes and interventions associated with this complication, and provides insight into tracheostomy related bleeding in patients with spinal cord injury (SCI). Findings: A 15-year-old boy with a diagnosis of incomplete SCI at the C5 level was admitted for rehabilitation 4 months after injury. He required a tracheostomy for ventilation, and underwent subglottic stenosis dilatation thrice. Multiple decannulation attempts were performed without success. He received intensive care on several occasions for respiratory failure. During the course of his rehabilitation, a minimal tracheostomy bleed was observed, which became profuse within a few hours and led to hypoxia with loss of consciousness. An urgent sternotomy identified bleeding from a TIF. He suffered severe brain damage following massive tracheal hemorrhage and died. Conclusion/clinical relevance: Given the morbidity of TIF-related hemorrhage, it is important to increase awareness of this rare condition among health-care providers, especially those in non-acute settings. Patients with SCI and a tracheostomy pose unique challenges related to respiratory compromise, which may accentuate TIF formation.
Keywords: tracheostomy tube; fluticasone propionate plus salmeterol; salbutamol; abrasion; adolescent; article; artificial ventilation; bleeding; blood vessel fistula; brachiocephalic trunk; brain damage; breathing exercise; case report; chest infection; clinical article; decubitus; do not resuscitate order; fatality; heart arrest; home care; human; hypotension; hypoxia; intensive care; male; neurorehabilitation; oxygen saturation; plastic surgery; quadriplegia; respiratory care; respiratory failure; spinal cord injury; sternotomy; subglottic stenosis; thorax surgery; tracheal hemorrhage; tracheostomy; unconsciousness

Ertapenem-associated neurotoxicity in the spinal cord injury (SCI) population: A case series - Patel U.C.; Fowler M.A. (Journal of Spinal Cord Medicine, 2018)
Context: Ertapenem, a broad spectrum carbapenem antibiotic, is used often in Spinal Cord Injury (SCI) patients due to increased risk factors for multi-drug resistant (MDR) infections in this population. Neurotoxicity, specifically seizures, due to ertapenem is a known adverse effect and has been described previously. Other manifestations such as delirium and visual hallucinations have rarely been reported, and no literature, to the best of our knowledge, specifically describes these effects solely in the SCI population. Findings: Four cases of mental status changes and hallucinations in SCI patients attributed to ertapenem therapy are described. Onset of symptoms began between one and six days following initiation of ertapenem and resolved between two to 42 days following discontinuation. Based on the Naranjo probability scale, a probable relationship exists between the adverse events and ertapenem for three out of the four cases. Possible overestimation of renal function and hypoalbuminemia may be contributing factors to the noted adverse reactions. Conclusion/Clinical Relevance: The cases described highlight the importance of recognizing ertapenem-associated hallucinations in SCI patients. The population is particularly vulnerable due to risk factors for MDR infections necessitating ertapenem use, possible overestimation of renal function, and a high prevalence of hypoalbuminemia.
Keywords: stomach tube; allopurinol; amlodipine; antibiotic agent; atorvastatin; baclofen; ceftazidime; cilastatin plus imipenem; creatinine; diltiazem; docusate sodium; ertapenem; furosemide; hydralazine; isosorbide mononitrate; lisinopril; loperamide; losartan; meropenem; metoprolol; omeprazole; oxybutynin; pregabalin; risperidone; sennoside; simvastatin; terazosin; tizanidine; tramadol; unindexed drug; vancomycin; adult; agitation; alcohol abuse; antibiotic therapy; article; bladder stone; body height; body weight; bone biopsy; brain atrophy; case report; cellulitis; chronic disease; chronic obstructive lung disease; chronic osteomyelitis; Citrobacter koseri; clinical article; cocaine dependence; confusion; creatinine blood level; decubitus; depression; diabetes mellitus; drug use; Enterobacter cloacae; essential tremor; fasciocutaneous flap; follow up; glaucoma; graft failure; hallucination; human; hyperlipidemia; hypertension; hypoalbuminemia; hyponatremia; hypothyroidism; iron deficiency anemia; kidney function; leukocytosis; male; medical history; mental health; middle aged; neurofibromatosis; neurogenic bladder; neurogenic bowel; neurotoxicity; osteomyelitis; paraplegia; peripheral neuropathy; peripheral vascular disease; quadriplegia; recurrent infection; respiratory failure; sleep disordered breathing; spinal cord injury; Staphylococcus epidermidis; tracheostomy; tremor; urinalysis; urinary tract infection; urine culture; vitamin D deficiency; wound care; x-ray computed tomography

AVXS-101 phase 1 gene replacement therapy clinical trial in spinal muscular atrophy type 1: Improvement in respiratory and swallowing function stabilizes the need for ventilatory and nutritional support, and reduces frequency and duration of hospitalizations compared with natural history - Shell R.; Al-Zaidy S.; Arnold W.D.; Rodino-Klapac L.; Prior T.; Kotha K.; Paul G.; Lowes L.; Alfano L.; Berry K.; Church K.; Kissel J.; et al. (Journal of Pediatric Gastroenterology and Nutrition, 2018)
Background: Spinal muscular atrophy (SMA), an autosomal recessive neurodegenerative disorder, is caused by bi-allelic loss or dysfunction of the survival motor neuron 1 (SMN1) gene. With an incidence of approximately 1 in 10,000 live births and a carrier frequency of approximately 1 in 54, SMA is classified into 4 subtypes (1-4) on the basis of age of onset of symptoms and motor milestone achievement. Insufficient levels of the survival motor neuron (SMN) protein result in loss of motor neurons of the brainstem and spinal cord, progressive muscular atrophy, weakness, and severe swallowing and breathing dysfunction that increases the likelihood of hospitalizations due to failure to thrive, aspiration pneumonia, and acute-on-chronic ventilatory failure. According to the natural history of SMA1, the majority of patients with SMA1 are not able to speak or swallow effectively. By 12 months of age, the majority of patients will require nutritional and ventilatory support, and by 20 months of age, 92% are expected to die or require permanent ventilation. We report data on bulbar function, requirements for nutritional and ventilatory support, hospitalizations, and survival in patients with SMA1 treated with onasemnogene abeparvovec (AVXS-101), a gene replacement therapy. METHODS: In this 2-year follow-up phase 1 trial (NCT02122952), 15 SMA1 patients received a one-time, intravenous dose of AVXS-101 at low dose (cohort 1, n=3) or proposed therapeutic dose (cohort 2, n=12). All patients had bi-allelic exon 7 deletions of SMN1, 2xSMN2 copies, and no SMN2 disease modifying mutation. Here, we report survival, hospitalizations, bulbar function, and nutritional/ventilatory support outcomes in the proposed therapeutic dose cohort (cohort 2). Survival was defined as avoidance of death/permanent ventilation; swallow integrity was determined using a video swallow test, and ventilatory support was parent-reported. RESULTS: At 24 months follow-up, 12/12 (100%) patients with SMA1 were alive and without need for permanent ventilation. AVXS-101 had a favorable safety profile and was generally well tolerated in patients with SMA1. Ten of 12 patients were able to safely swallow thin liquids compared with 4 at baseline, and 11/12 were able to safely swallow to allow oral feeding compared with 8 at baseline. No patient lost the ability to feed orally following AVXS-101. Six patients who were feeding orally were not using nutritional support. At baseline, 7/12 patients did not require nutritional support, and 10/12 did not require ventilatory support. At 24 months follow-up, 6/7 patients who did not require nutritional support pre-dosing continued to eat exclusively by mouth, and 7/10 who did not require ventilatory support pre-dosing continued without ventilatory support. Ten patients were hospitalized for respiratory infections, but all survived without need for tracheostomy or permanent ventilation. Patients treated with AVXS-101 spent a median of 2.3% (range=0-18.3) of the time hospitalized; 10/12 (83%) patients treated with AVXS-101 were hospitalized <10% of the time (0-36 days/year), and none were hospitalized ≥20% of the time (≥73 days/year). The ENDEAR study included a control group of untreated patients with SMA1, and reported hospitalization data for these untreated patients. The ENDEAR study reported that untreated patients with SMA1 were hospitalized a median of 13.9% (range=0-75) of the time; 11/27 (41%) untreated patients from the ENDEAR study were hospitalized <10% of the time (0-36 days/year), and 10/27 (37%) were hospitalized ≥20% of the time (≥73 days/year). The mean unadjusted annualized hospitalization rate (hospitalizations/number of subject-years followed) for patients treated with AVXS-101 was 2.1 (range=0-7.6), which was half that in the ENDEAR control group (4.3). The mean hospital stay was 6.7 days (range=3-12.1) in patients treated with AVXS-101, compared with 13 days reported in untreated patients. CONCLUSION: In contrast to natural history, patients with SMA1 treated with AVXS-101 showed improved survival, improved bulbar function, reduced need for nutritional and ventilatory support, and reduced frequency and duration of hospitalizations, which may result in a significant reduction in healthcare utilization and cost burden on healthcare systems.
Keywords: endogenous compound; onasemnogene abeparvovec; allele; artificial ventilation; assisted ventilation; avoidance behavior; cohort analysis; conference abstract; controlled study; death; drug safety; drug therapy; feeding; female; follow up; gene deletion; gene replacement therapy; health care system; health care utilization; history; hospitalization; human; human cell; infant; intravenous drug administration; low drug dose; major clinical study; male; mouth; nutritional support; pharmacokinetics; phase 1 clinical trial; respiratory function; respiratory tract infection; survival; swallowing; tracheostomy; videorecording; Werdnig Hoffmann disease

Use of diaphragm pacing in the management of acute cervical spinal cord injury - Kerwin A.J.; Yorkgitis B.K.; Ebler D.J.; Madbak F.G.; Hsu A.T.; Crandall M.L. (Journal of Trauma and Acute Care Surgery, 2018)
BACKGROUND Cervical spinal cord injury (CSCI) is devastating. Respiratory failure, ventilator-Associated pneumonia (VAP), sepsis, and death frequently occur. Case reports of diaphragm pacing system (DPS) have suggested earlier liberation from mechanical ventilation in acute CSCI patients. We hypothesized DPS implantation would decrease VAP and facilitate liberation from ventilation. METHODS We performed a retrospective review of patients with acute CSCI managed at a single Level 1 trauma center between January 2005 and May 2017. Routine demographics were collected. Patients underwent propensity matching based on age, injury severity score, ventilator days, hospital length of stay, and need for tracheostomy. Outcome measures included hospital length of stay, intensive care unit length of stay, ventilator days (vent days), incidence of VAP, and mortality. Bivariate and multivariate logistic and linear regression statistics were performed using STATA Version 10. RESULTS Between July 2011 and May 2017, all patients with acute CSCI were evaluated for DPS implantation. Forty patients who had laparoscopic DPS implantation (DPS) were matched to 61 who did not (NO DPS). Median time to liberation after DPS implantation was 7 days. Hospital length of stay and mortality were significantly lower on bivariate analysis in DPS patients. Diaphragm pacing system placement was not found to be associated with statistically significant differences in these outcomes on risk-Adjusted multivariate models that included admission year. CONCLUSIONS Diaphragm pacing system implantation in patients with acute CSCI can be one part of a comprehensive critical care program to improve outcomes. However, the association of DPS with the marked improved mortality seen on bivariate analysis may be due solely to improvements in critical care throughout the study period. Further studies to define the benefits of DPS implantation are needed. LEVEL OF EVIDENCE Therapeutic, level IV.
Keywords: adult; artificial ventilation; cervical spinal cord injury; cohort analysis; comparative study; conference paper; controlled study; disease association; emergency health service; female; health program; hospital admission; hospitalization; human; injury scale; intensive care unit; length of stay; major clinical study; male; middle aged; mortality; outcome assessment; priority journal; propensity score; respiratory failure; retrospective study; systolic blood pressure; tracheostomy; ventilator associated pneumonia

Reporting the largest long term experience with diaphragm pacing for traumatic spinal cord injury: Successfully decreasing mechanical ventilation - Onders R.; Elmo M.; Katirji B.; Schilz R. (Intensive Care Medicine Experimental, 2018)
INTRODUCTION. Cervical spine injury (SCI) can result in catastrophic respiratory failure requiring invasive mechanical ventilation (MV) which is a leading cause of mortality and cost. Diaphragm Pacing (DP) was developed to replace/decrease mechanical ventilation. OBJECTIVE. Report the largest long term analysis of DP in a sub-group of traumatic SCI. METHODS. A retrospective review of prospective IRB approved pro-tocols. All patients underwent laparoscopic diaphragm mapping and implantation of electrodes. DP electrodes were characterized and diaphragm strengthening with mechanical ventilator weaning was initiated immediately post op. RESULTS. March 2000 through June 2017 there have been a total of 486 DP implants at this single site. Within this group, 155 had spinal cord damage and 92 of those patients had SCI secondary to trauma. The manner of injury: MVA 44, Sports 22, Falls 12, GSW 7, Crush 3, Forceps Delivery 2, Assault 1, and electrocution 1. The age at time of injury ranged from birth to 74 years old with the average of 27.3 years and median age of 23 years. Time on mechanical ventilation prior to DP was an average of 47.5 months (6 days to 25 years with median of 1.58 years). Patients' highest level of injury: 27 C1, 36 C2, 14 C3, 7 C4, 5 C5 and 3 C6. Twelve patients had internal permanent cardiac pacemakers. There was no device to device interactions between DP and cardiac pacemakers. A total of 83% of patients achieved 4 consecutive hours of pacing with fifty-six patients (60.8%) being full time and an additional 13% using DP >12 hours. DP decreased the need and risk of cuffed tracheostomy with 60% of patients going to cuffless tracheostomy and 7 decannulations. One patient with early DP implant avoided tracheostomy. Five patients were not successful in weaning off MV. Five patients had full recovery of automatic breathing with subsequent DP removal. Two pediatric patients have had growth spurts of 10 inches or greater without need of electrode replacement. There have been 23 deaths with no primary respiratory origin. Subgroup analysis showed that earlier DP implantation leads to greater 24 hour use of DP and no need for any MV. This group also had the greater proportionate re-covery of breathing. CONCLUSIONS. DP can successfully decrease need for mechanical ventilation for a significant number of SCI patients. There is a correlation of early implantation to greater weaning success, recovery of independent breathing and has the potential to obviate need for tracheostomy. Liberation from MV can substantially improve SCI re-habilitation. To minimize MV days and complications, DP should be considered early after injury even in those who may re-gain independent breathing.
Keywords: adult; assault; child; complication; conference abstract; death; diaphragm; electrocution; female; forceps delivery; growth acceleration; human; implant; implanted heart pacemaker; major clinical study; male; mechanical ventilator; pacemaker electrode; pediatric patient; remission; retrospective study; spinal cord injury; spinal cord lesion; sport; tracheostomy; ventilator weaning; young adult

Safety of early tracheostomy in trauma patients after anterior cervical fusion - Lozano C.P.; Chen K.A.; Marks J.A.; Jenoff J.S.; Cohen M.J.; Weinstein M.S. (Journal of Trauma and Acute Care Surgery, 2018)
BACKGROUND Cervical spine injuries (CSIs) can have major effects on the respiratory system and carry a high incidence of pulmonary complications. Respiratory failure can be due to spinal cord injuries, concomitant facial fractures or chest injury, airway obstruction, or cognitive impairments. Early tracheostomy (ET) is often indicated in patients with CSI. However, in patients with anterior cervical fusion (ACF), concerns about cross-contamination often delay tracheostomy placement. This study aimed to demonstrate the safety of ET within 4 days of ACF. METHODS Retrospective chart review was performed for all trauma patients admitted to our institution between 2001 and 2015 with diagnosis of CSI who required both ACF and tracheostomy, with or without posterior cervical fusion, during the same hospitalization. Thirty-nine study patients with ET (within 4 days of ACF) were compared with 59 control patients with late tracheostomy (5-21 days after ACF). Univariate and logistic regression analyses were performed to compare risk of wound infection, length of intensive care unit and hospital stay, and mortality between both groups during initial hospitalization. RESULTS There was no difference in age, sex, preexisting pulmonary or cardiac conditions, Glasgow Coma Scale score, Injury Severity Score, Chest Abbreviated Injury Scale score, American Spinal Injury Association score, cervical spinal cord injury levels, and tracheostomy technique between both groups. There was no statistically significant difference in surgical site infection between both groups. There were no cases of cervical fusion wound infection in the ET group (0%), but there were five cases (8.47%) in the late tracheostomy group (p = 0.15). Four involved the posterior cervical fusion wound, and one involved the ACF wound. There was no statistically significant difference in intensive care unit stay (p = 0.09), hospital stay (p = 0.09), or mortality (p = 0.06) between groups. CONCLUSION Early tracheostomy within 4 days of ACF is safe without increased risk of infection compared with late tracheostomy.
Keywords: adolescent; adult; adult respiratory distress syndrome; aged; American Spinal Injury Association impairment scale; anterior cervical fusion; article; bradycardia; cause of death; cervical spine injury; comorbidity; early tracheostomy; electronic medical record; esophagocutaneous fistula; esophagus fistula; esophagus perforation; female; Glasgow coma scale; hospital mortality; hospitalization; human; hypotension; ICD-9-CM; injury scale; intensive care unit; length of stay; lung disease; major clinical study; male; medical record review; priority journal; retrospective study; risk factor; safety procedure; spine surgery; surgical infection; surgical technique; therapy delay; tracheostomy; very elderly; wound infection

Results of Early and Late Surgical Decompression and Stabilization for Acute Traumatic Cervical Spinal Cord Injury in Patients with Concomitant Chest Injuries - Sewell M.D.; Vachhani K.; Alrawi A.; Williams R. (World Neurosurgery, 2018)
Background: The benefits of early surgical decompression and stabilisation (within 24 hours of injury) for patients with acute traumatic spinal cord injury (SCI) is unclear. The study objective was to investigate the effects of early (<24 hours of injury) versus late (>24 hours of injury) decompressive and stabilisation surgery for traumatic cervical SCI in patients with concomitant chest injuries. Methods: This was a retrospective study including adults aged 16 years or over with traumatic cervical SCI, Glasgow Coma Scale score >13, and concomitant chest injuries (e.g. hemopneumothoraces, flail chest and pulmonary contusions) necessitating intensive care unit (ICU) admission. Forty patients who met the inclusion criteria and underwent decompressive surgery within 24 hours were compared with 55 patients who underwent decompressive surgery after 24 hours. Primary outcomes were ordinal change in the ASIA Impairment Scale (AIS) at 6 months and duration of ICU stay. Secondary outcomes included complications occurring within 30 days. Results: In the early surgery group, 21 patients (52.5%) showed no improvement in ASIA grade, 13 (32.5%) had a 1-grade improvement, and 6 (15%) had a 2-grade improvement. The median length of ICU stay was 14 days (range, 2–68). 42.5% of patients developed a complication and 45% required a tracheostomy. In the late surgery group, AIS grade improvement was as follows: 32 (58%) no improvement, 19 (34.5%) had a 1 grade improvement, 3 (5.5%) had a 2 grade improvement and 1 (2%) had a 3 grade improvement. Mean ICU stay was 23 days (4–68). 53% of patients developed a complication and 55% required a tracheostomy. There was one mortality in the late surgery group. Conclusions: For patients with acute traumatic cervical SCI and concomitant chest trauma, early surgical decompression and stabilisation was associated with reduced ICU stay and a lower complication rate. Neurological recovery was more likely in younger patients and those with an incomplete SCI.
Keywords: adolescent; adult; aged; American Spinal Injury Association impairment scale; article; assault; cervical spinal cord injury; clinical outcome; controlled study; decubitus; early intervention; falling; female; flail chest; Glasgow coma scale; heart disease; hematopneumothorax; human; intensive care unit; length of stay; lung contusion; lung embolism; major clinical study; male; observational study; pneumothorax; postoperative complication; respiratory tract disease; retrospective study; rib fracture; spinal cord decompression; spine stabilization; surgical infection; surgical mortality; therapy delay; thorax injury; tracheostomy; traffic accident; treatment failure; ventilator associated pneumonia

Combined spinal-epidural and local anesthetic infiltration for cesarean delivery in a patient with myotonic dystrophy and severe cardiopulmonary compromise - Cai Y.; Scott C.; Anwar A.; James P.; Warrick A.; Vanderhoef K.; Diachun C. (Regional Anesthesia and Pain Medicine, 2018)
Background and Aims: Myotonic dystrophy (MD) is a rare disorder characterized by progressive myopathy and myotonia. Systemic complications may include cardiomyopathy and difficulty weaning from mechanical ventilation (MV). Discussion of obstetrical cases in the context of these comorbidities are sparse, and to our knowledge, this is the first case of regional anesthesia for full-term delivery in a parturient with MD and concomitant cardiopulmonary compromise. Methods: Case report. Results: A 27-year-old G3P0020 at 35w2d with MD and a history of prolonged tracheostomy was admitted for progressive hypoxia requiring supplemental oxygen at 25 weeks gestation. Hospital workup revealed periodic episodes of non-sustained ventricular tachycardia and severe right ventricular enlargement with septal wall flattening during both systole and diastole. Caesarean section was scheduled for 36w2d. Prior to surgery, arterial line and defibrillation pads were placed. A combined spinal-epidural (CSE) was placed at L3-4 without intrathecal drug administration. Due to incomplete sensory deficit, local infiltration using lidocaine was used on skin and uterine incision regions. A viable male with APGAR scores of 3 at 1 minute and 7 at 5 minutes was delivered at 33 minutes after skin incision. Hemodynamic stability was maintained with intravenous epinephrine and vasopressin infusions, and spontaneous ventilation was preserved throughout. Conclusions: Given the risk of prolonged MV, neuraxial anesthesia was critical to maintaining spontaneous respiration. Epidural or intrathecal opioid was avoided due to risk of apnea, and intrathecal anesthetic avoided due to sympathectomy. A CSE without intrathecal dosing may provide inadequate anesthesia and can be supplemented with local infiltration of lidocaine.
Keywords: endogenous compound; epinephrine; lidocaine; opiate; oxygen; vasopressin; adult; Apgar score; apnea; arterial line; artificial ventilation; case report; cesarean section; clinical article; comorbidity; conference abstract; defibrillation; diastole; drug combination; drug therapy; female; heart contraction; heart right ventricle hypertrophy; heart ventricle tachycardia; hemodynamics; human; hypoxia; infusion; male; myotonic dystrophy; pregnancy; regional anesthesia; sensory dysfunction; skin incision; surgery; sympathectomy; tracheostomy

The economic burden of spinal muscular atrophy patients in a commercially insured population in the united states - Goble J.; Dai D.; Boulos F.; Weng A.; Johnson K. (Journal of Managed Care and Specialty Pharmacy, 2018)
BACKGROUND: A high level of burden exists for patients with Spinal Muscular Atrophy (SMA) and their caregivers in their journey. In addition to the psychosocial impact of the disease, SMA can place a financial burden on families and the healthcare system, due to complex care needs requiring a spectrum of specialized providers and services. Despite SMA's high clinical burden, little information on the economic burden of disease exists. OBJECTIVE: To assess the economic burden associated with the treatment of SMA patients. METHODS: A retrospective analysis of SMA patients was conducted using the MarketScan Commercial and Medicare Supplemental Databases (January 2012-November 2017). A cohort of SMA patients identified by at least two diagnosis codes (ICD-9: 335.0, 335.1x; ICD-10: G12.0, G12.1, G12.8, G12.9) > 30 days apart was assigned to age groups on the index date of first SMA claim (< 2 years, 2-18 years, > 18 years). All-cause and disease-specific direct healthcare costs and healthcare resource utilization [HCRU] (procedures, office visits, durable medical equipment [DME]) were derived from the claims databases. Patients were followed from index date until the end of continuous enrollment, or the end of the study period, whichever occurred earlier. Costs were annualized and adjusted to 2017 U.S. dollars. RESULTS: A total of 1,120 patients were identified that met the inclusion criteria. The majority of patients were male (51.3%) with a mean (SD) age of 33.7 (24.0) years. Mean [SD] all-cause annual direct healthcare costs were highest for SMA patients < 2 years old ($159,227 [$313,121]), followed by to those 2-18 years ($105,206 [$154,689]) and those > 18 years ($39,355 [$82,066]). Outpatient medical services were the major driver in total healthcare costs, accounting for 52.8% of costs across the entire cohort. Disease-specific costs accounted for 49.4% of total costs, which on average (SD) totaled $32,332 ($93,687). The majority (87.0%) 68.4%) of pediatric patients had home health services while only 68.4% had ≥ 1 office visit to a neurologist or pulmonologist. Nearly half (53.3%) of pediatric patients had ≥ 1 hospitalizations with a mean (SD) length of stay of 4.7 (11.3) days. Procedures with the highest utilization across the cohort were gastrostomy (21.5%) and tracheostomy (17.8%). DME with frequent use included CPAP/BiPAP (28.1%), oxygen machines (25.1%), and suction devices (23.0%). CONCLUSIONS: In a commercially insured population, SMA patients had high economic burden driven by outpatient medical spend.
Keywords: oxygen; adult; ambulatory care; child; cohort analysis; conference abstract; controlled study; diagnosis; driver; gastrostomy; groups by age; hospitalization; human; ICD-10; ICD-9; length of stay; machine; major clinical study; male; medical service; medicare; neurologist; outpatient; pediatric patient; positive end expiratory pressure; pulmonologist; retrospective study; spinal muscular atrophy; suction pump; tracheostomy; United States

AVXS-101 phase 1 gene therapy clinical trial in spinal muscular atrophy type 1 (SMA1): Improvement in respiratory and bulbar function reduces frequency and duration of hospitalizations compared to natural history - Shell R.; Al-Zaidy S.; Arnold W.; Rodino-Klapac L.; Prior T.; Kotha K.; Paul G.; Lowes L.; Alfano L.; Berry K.; Church K.; Kissel J.; et al. (Annals of Neurology, 2018)
Objective: We report data on requirements for nutritional and ventilatory support, and hospitalizations in SMA1 patients treated with AVXS-101, a gene replacement therapy. Methods: Twelve patients with SMA1 received a onetime, intravenous, proposed therapeutic dose of AVXS-101 (NCT02122952). Results: At baseline, 7/12 patients did not require nutritional support, and 10/12 did not require ventilatory support. As of August 7, 2017, 6/7 patients who did not require nutritional support pre-dosing continued to eat exclusively by mouth, 11/12 could eat orally, and 7/10 who did not require ventilatory support pre-dosing continued without ventilatory support. Ten patients were hospitalized for respiratory infections, but all survived without need for tracheostomy or permanent ventilation. Patients treated with AVXS-101 spent a smaller percentage of time hospitalized (median of 2% [range=0-18.3]) in contrast with untreated SMA1 patients in the ENDEAR study (13.9% [0-75]). Ten of 12 patients treated with AVXS-101 were hospitalized <10% of the time (in contrast with 11/27 untreated patients, ENDEAR), and none were hospitalized ≥20% of the time (10/27 untreated patients, ENDEAR). The mean unadjusted annualized hospitalization rate (hospitalizations/number of subject-years followed) for patients treated with AVXS-101 was 2.0 (standard deviation=2.26), which was half that in the ENDEAR control group (4.3). The mean hospital stay was 7.1 days (range=3-12.1) in patients treated with AVXS-101, compared with 13 days reported in untreated patients. Conclusions: In contrast to natural history, SMA1 patients treated with AVXS-101 showed a reduced need for nutritional and ventilatory support, and reduced frequency and duration of hospitalizations.
Keywords: onasemnogene abeparvovec; adult; artificial ventilation; assisted ventilation; clinical article; conference abstract; controlled study; drug therapy; female; gene replacement therapy; genetics; history; hospitalization; human; male; mouth; nutritional support; phase 1 clinical trial; rare disease; respiratory function; respiratory tract infection; tracheostomy; Werdnig Hoffmann disease

The four fixation points of the axis: Technique and case report - Siemionow K.; Janusz P.; Mardjetko S. (International Journal of Spine Surgery, 2018)
Background: Instrumentation of the axis can be accomplished through a variety of techniques including transarticular screw fixation, pars and pedicle screw fixation, translaminar screw fixation, and posterior wiring. We report on the evolution of the axial 4-screw technique. Methods: Retrospective case review. After exposure of posterior spinal elements, the medial and superior walls of the C2 pedicle were identified from within the spinal canal. A high-speed drill was then advanced under lateral fluoroscopy, which guided craniocaudal angulation. Medial angulation was based on anatomic landmarks and preoperative imaging. This was followed by placement of translaminar screws according to the technique described by Wright. When extending the construct into the subaxial spine or the occiput, lateral connectors are placed in translaminar screws, which are usually more offset. The rod is directly connected to the pedicle screws, which are usually more in alignment with the subaxial/occipital instrumentation. Results: Two male patients ages 56 and 58 underwent posterior instrumentation of the axis employing a combination of pedicle and laminar polyaxial screws. Indications included multilevel spinal cord compression and deformity in a patient with Down syndrome and cervical meningioma, respectively. Follow-up was 1 year and 5 years, respectively. Medical complications (N ¼ 2) occurred in the patient with Down syndrome resulting in prolonged intubation with tracheostomy placement. Reduction was maintained in both patients at last follow-up. There were no neurologic, vascular, or instrumentation related complications. Conclusions: The axis serves as a versatile anchor point and offers 4 potential points of fixation. Lateral connectors play a crucial role and allow for incorporation of the C2 screws with the rest of the construct. Local anatomy will dictate the necessity and ability to place instrumentation and detailed preoperative planning is of paramount importance.
Keywords: adult; anatomic landmark; anatomy; article; case report; case study; clinical article; complication; deformity; Down syndrome; female; fluoroscopy; follow up; human; intubation; male; meningioma; pedicle screw; preoperative evaluation; rest; retrospective study; spinal cord compression; tracheostomy; velocity; vertebral canal

Nusinersen in infants who initiate treatment in a presymptomatic stage of spinal muscular atrophy (SMA): Interim efficacy and safety results from the phase 2 nurture study - Crawford T.; De Vivo D.; Bertini E.; Hwu W.L.; Foster R.; Bhan I.; Farwell W.; Reyna S. (Annals of Neurology, 2018)
Objective: NURTURE (NCT02386553) is an ongoing phase 2, open-label, single-arm efficacy/safety study of intrathecal nusinersen in infants who initiate treatment in a presymptomatic stage of spinal muscular atrophy (SMA). Methods: Enrolled infants were age ≤6 weeks at first dose, clinically presymptomatic, had genetically diagnosed SMA, and 2 or 3 copies of the SMN2 gene. The primary endpoint is time to death or respiratory intervention (≥6 hours/day continuously for ≥7 days or tracheostomy). Results: As of July 5, 2017, 25 infants (2 copies SMN2, n=15; 3 copies, n=10) were enrolled. Median (range) age at last visit was 14.7 (2.8-23.3) months. All infants were alive. No infants required tracheostomy or permanent ventilation. Two infants had an acute, reversible viral infection requiring respiratory intervention and thus met the primary endpoint. Among infants with enough observation time, all 22 infants (2 SMN2 copies, n=14; 3 SMN2 copies, n=8) had achieved the WHO motor milestone of sitting without support and 8/13 (2 SMN2 copies, n=3/8; 3 SMN2 copies, n=5/5) had achieved walking alone. AEs were reported in 24/25 (96%) infants; most 20/25 (80%) had AEs that were mild/moderate in severity; 9 had serious AEs. Four infants had an AE possibly related to study drug, which resolved despite continued treatment. No new safety concerns were identified. Results from a new interim analysis, with an additional 7 months of follow-up, will be presented. Conclusions: Nusinersen continued to benefit infants who initiated treatment in a presymptomatic stage of SMA, and the updated analyses will provide further information.
Keywords: endogenous compound; nusinersen; survival motor neuron protein 2; artificial ventilation; case report; clinical article; conference abstract; death; diagnosis; drug safety; drug therapy; female; follow up; genetics; human; infant; male; neurology; pharmacokinetics; phase 2 clinical trial; spinal muscular atrophy type 2; tracheostomy; virus infection; walking

Development of acute promyelocytic leukemia in a patient with tetraplegia while in inpatient rehabilitation: A case report - Beal C.A.; Krouse M.C.; Tubbs J.T. (Journal of Spinal Cord Medicine, 2018)
Study Design: a single case report. Objectives: To report a case of a patient with tetraplegia who developed acute promyelocytic leukemia (APL) while in inpatient rehabilitation after 10.5 months. Setting: A VA Medical Center Spinal Cord Injury Service and Disorders Unit Case Report: A 47 year-old male with a stage IV sacral pressure ulcer and C4 AIS A complete tetraplegia secondary to a motor vehicle collision, developed fever, thrombocytopenia, and anemia 20 months after his injury while in inpatient rehabilitation and was found to have APL, confirmed following bone marrow biopsy. Conclusion: There is a wide differential for fever after a spinal cord injury. In this case report, the source of fever was APL. It is important as healthcare providers to not overlook fevers when otherwise common causes do not fit the clinical picture. Additionally, there has been no association found between traumatic spinal cord injury and the development of acute leukemia, however this is the first case report. Therefore, it is important to continue investigating to determine if an association exists.
Keywords: arsenic trioxide; enoxaparin; ertapenem; hemoglobin; piperacillin plus tazobactam; retinoic acid; adult; anemia; article; blast cell; blood cell count; blood culture; bone marrow biopsy; case report; Caucasian; cervical spine fracture; clinical article; decubitus; dysphagia; febrile neutropenia; fever; gluteus muscle; hemoglobin blood level; hospital patient; human; intensive care; Klebsiella infection; laboratory test; leukemia remission; leukocyte count; male; middle aged; muscle flap; percutaneous endoscopic gastrostomy; promyelocytic leukemia; Proteus infection; Proteus mirabilis; quadriplegia; rehabilitation care; sacral vertebra; thrombocytopenia; tracheostomy; traffic accident; urinary tract infection; wound dehiscence

Predictive values of magnetic resonance imaging features for tracheostomy in traumatic cervical spinal cord injury - Jeong T.S.; Lee S.G.; Kim W.K.; Ahn Y.; Son S. (Journal of Korean Neurosurgical Society, 2018)
Objective: To evaluate the magnetic resonance (MR) imaging features that have a statistically significant association with the need for a tracheostomy in patients with cervical spinal cord injury (SCI) during the acute stage of injury. Methods: This study retrospectively reviewed the clinical data of 130 patients with cervical SCI. We analyzed the factors believed to increase the risk of requiring a tracheostomy, including the severity of SCI, the level of injury as determined by radiological assessment, three quantitative MR imaging parameters, and eleven qualitative MR imaging parameters. Results: Significant differences between the non-tracheostomy and tracheostomy groups were determined by the following five factors on multivariate analysis: complete SCI (p=0.007), the radiological level of C5 and above (p=0.038), maximum canal compromise (MCC) (p=0.010), lesion length (p=0.022), and osteophyte formation (p=0.015). For the MCC, the cut-off value was 46%, and the risk of requiring a tracheostomy was three times higher at an interval between 50–60% and ten times higher between 60–70%. For lesion length, the cut-off value was 20 mm, and the risk of requiring a tracheostomy was two times higher at an interval between 20–30 mm and fourteen times higher between 40–50 mm. Conclusion: The American Spinal Injury Association grade A, a radiological injury level of C5 and above, an MCC ≥50%, a lesion length ≥20 mm, and osteophyte formation at the level of injury were considered to be predictive values for requiring tracheostomy intervention in patients with cervical SCI.
Keywords: nuclear magnetic resonance scanner; adult; article; cervical spinal cord injury; cervical spine dislocation; data analysis; female; human; intubation; major clinical study; male; middle aged; nuclear magnetic resonance imaging; osteophyte; predictive value; retrospective study; spinal cord; spinal cord compression; tracheostomy; vertebra dislocation

In Reply to "Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery: Is Early Tracheostomy Really Associated with Fewer Wound Infections?" - Galeiras R.; Mourelo-Fariña M.; Bouza M.T.; Seoane M.T.; Ferreiro M.E.; Montoto A.; Salvador S.; Seoane L. (World neurosurgery, 2018)
Keywords: cervical vertebra; human; injuries; retrospective study; spinal cord injury; spine injury; tracheostomy; wound infection

Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery: Is Early Tracheostomy Really Associated with Fewer Wound Infections? - Agarwal N.; Agrawal M.; Sawarkar D.P. (World Neurosurgery, 2018)
Keywords: human; infection risk; intensive care unit; lower respiratory tract; microbial colonization; note; patient positioning; spinal cord injury; spine surgery; surgical infection; tracheostomy; treatment outcome; weakness

In Reply to “Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery: Is Early Tracheostomy Really Associated with Fewer Wound Infections?” - Galeiras R.; Mourelo-Fariña M.; Bouza M.T.; Seoane M.T.; Ferreiro M.E.; Montoto A.; Salvador S.; Seoane L. (World Neurosurgery, 2018)
Keywords: antibiotic agent; antibiotic therapy; artificial ventilation; cervical spinal cord injury; early intervention; human; note; operation duration; postoperative infection; spine surgery; tracheostomy; wound infection

Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery: Is Early Tracheostomy Really Associated with Fewer Wound Infections? - Agarwal N.; Agrawal M.; Sawarkar D.P. (World neurosurgery, 2018)
Keywords: cervical vertebra; human; injuries; retrospective study; spinal cord injury; spine injury; tracheostomy; wound infection

Spinal cord injury-induced heterotopic calcification on a balloon of a Foley catheter - Inamasu J.; Hirose Y.; Nakatsukasa M. (Neurology and Clinical Neuroscience, 2018)
Keywords: Foley balloon catheter; calcium oxalate; adult; article; bone mineralization; case report; clinical article; cystoscopy; human; male; micturition; priority journal; spinal cord injury; tracheostomy; urinary tract obstruction; young adult

Urgent Endovascular Treatment for Non-traumatic Descending Thoracic Aortic Rupture - Thomas R.P.; Amin S.S.; Eldergash O.; Kowald T.; Bremer S.; Easo J.; Weymann A.; Book M.; Szczechowicz M.; Schmuck B.; Chavan A. (CardioVascular and Interventional Radiology, 2018)
Purpose: To evaluate the efficacy of endovascular repair of non-traumatic rupture of the descending thoracic aorta. Materials and Methods: From January 2011 to August 2017, 22 consecutive patients (16 men, mean age 73.7 years, range 63–92 years) with non-traumatic rupture of the descending thoracic aorta were treated endovascularly. Thirty-day mortality as well as procedure-related morbidity including stroke, renal, pulmonary and spinal complications were retrospectively assessed. Median follow-up was 29 months (range 6–65 months). Results: Nine patients had type B dissections, and 13 had atherosclerotic aortic aneurysms. The technical success was 100%; 86.3% of patients (19/22) were treated percutaneously. Thirteen patients (59%) received a single endograft, 8 (36%) received 2 endografts, and 1 received 3 endografts. The endograft diameters ranged between 28 and 46 mm. Two dissection patients received additional distal bare metal true lumen stents. Eleven patients required additional drainage of thoracic hematoma. Three primary percutaneous sites (15.7%) required surgical revision. No spinal cord ischemic or renal complications were observed. Stroke was observed in 2 patients (9%), both with full coverage of left subclavian artery origin. Four patients (18%) required tracheostomy with prolonged respiratory support (range 10–15 days). The median intensive care unit stay was 5 days (range 1–27 days). The 30-day mortality was 18.1% (4/22). Conclusion: Endoluminal repair of non-traumatic rupture of the descending thoracic aorta can be performed percutaneously. It showed a 30-day mortality of 18.1%, and no spinal cord injury or renal complications were observed in surviving patients at follow-up. Level of Evidence: Level 4, case series.
Keywords: angiography device; aortic aneurysm endovascular graft; X ray system; Back-up Meier; bare metal stent; catheter sheath; diagnostic catheter; drainage catheter; Evita E-XL; Lunderquist; Prostar XL; Valiant Captivia; vascular closure device; vascular guide wire; adult; aged; angiocardiography; aortic atherosclerosis; aortic dissection; article; assisted ventilation; bare metal stenting; cerebrovascular accident; clinical article; cohort analysis; controlled study; descending aorta; endovascular aneurysm repair; female; follow up; hematothorax; human; incidence; left subclavian artery; length of stay; male; morbidity; mortality rate; percutaneous drainage; priority journal; respiratory failure; retrospective study; surgical mortality; survival rate; thoracic aorta aneurysm; thorax drainage; tracheostomy; Axiom Artis; Back-up Meier; Evita E-XL; Lunderquist; Prostar XL; Valiant Captivia

Section 7: Spinal muscular atrophy and home ventilation - Thavagnanam S.; Chiang J.; Zielinski D.; Amin R. (Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2018)
Keywords: acute disease; article; artificial ventilation; home care; human; information retrieval; Medline; noninvasive ventilation; palliative therapy; priority journal; prophylaxis; publication; scoliosis; spinal muscular atrophy; spine surgery; tracheostomy

Risk Analysis Based on the Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery - Galeiras R.; Mourelo M.; Bouza M.T.; Seoane M.T.; Ferreiro M.E.; Montoto A.; Salvador S.; Seoane L.; Freire D. (World Neurosurgery, 2018)
Objective: To determine the optimal moment to perform tracheostomy in a patient requiring anterior cervical fixation. Methods: A retrospective observational study conducted over an 18-year period included 56 patients who had been admitted to the intensive care unit with acute spinal cord injury and underwent tracheostomy and surgical fixation. The sample was divided into 2 groups: at-risk group (31 patients who had undergone tracheostomy before cervical surgery or <4 days after surgery) and not-at-risk group (25 patients who had undergone tracheostomy >4 days after fixation surgery). Descriptive and comparative studies were carried out. Overall trend of the collected data was analyzed using cubic splines (graphic methods). Results: The only infectious complications diagnosed as related to the surgical procedure were infection of the surgical wound in 2 patients in the not-at-risk group (12%) and deep tissue infection in 1 patient in the at-risk group (3.2%). During the study period, we identified a tendency toward performance of early tracheostomies. Conclusions: Our results suggest that the presence of a tracheostomy stoma before or immediately after surgery is associated with a low risk of infection of the cervical surgical wound in instrumented spinal fusion.
Keywords: vancomycin; accidental injury; adult; American Spinal Injury Association impairment scale; anterior spine fusion; antibiotic prophylaxis; APACHE; article; assisted ventilation; cervical spinal cord injury; Charlson Comorbidity Index; corticosteroid therapy; crush trauma; deep tissue infection; falling; female; Glasgow coma scale; granuloma; human; injury scale; length of stay; major clinical study; male; medical record review; middle aged; observational study; postoperative hemorrhage; respiratory failure; retrospective study; risk assessment; Sequential Organ Failure Assessment Score; spine stabilization; stenosis; surgical infection; time factor; tracheostomy

Risk Analysis Based on the Timing of Tracheostomy Procedures in Patients with Spinal Cord Injury Requiring Cervical Spine Surgery - Galeiras R.; Mourelo M.; Bouza M.T.; Seoane M.T.; Ferreiro M.E.; Montoto A.; Salvador S.; Seoane L.; Freire D. (World neurosurgery, 2018)
OBJECTIVE: To determine the optimal moment to perform tracheostomy in a patient requiring anterior cervical fixation. METHODS: A retrospective observational study conducted over an 18-year period included 56 patients who had been admitted to the intensive care unit with acute spinal cord injury and underwent tracheostomy and surgical fixation. The sample was divided into 2 groups: at-risk group (31 patients who had undergone tracheostomy before cervical surgery or <4 days after surgery) and not-at-risk group (25 patients who had undergone tracheostomy >4 days after fixation surgery). Descriptive and comparative studies were carried out. Overall trend of the collected data was analyzed using cubic splines (graphic methods). RESULTS: The only infectious complications diagnosed as related to the surgical procedure were infection of the surgical wound in 2 patients in the not-at-risk group (12%) and deep tissue infection in 1 patient in the at-risk group (3.2%). During the study period, we identified a tendency toward performance of early tracheostomies. CONCLUSIONS: Our results suggest that the presence of a tracheostomy stoma before or immediately after surgery is associated with a low risk of infection of the cervical surgical wound in instrumented spinal fusion.
Keywords: corticosteroid; adolescent; adult; aged; cervical vertebra; chi square distribution; female; human; intensive care; male; metabolism; middle aged; mortality; procedures; risk factor; spinal cord injury; spine fusion; time factor; tracheostomy; very elderly; young adult

Predictive values of magnetic resonance imaging features for tracheostomy in traumatic cervical spinal cord injury - Jeong T.S.; Lee S.G.; Kim W.K.; Ahn Y.; Son S. (European Spine Journal, 2018)
Background: Traumatic cervical spinal cord injury (SCI) may causerespiratory complication that may lead to the need for mechanicalventilation. Magnetic resonance (MR) imaging plays a crucial role indetecting and evaluating spinal trauma.Purpose of the study: To evaluate the MR imaging features that havea statistically significant association with the need for a tracheostomyin patients with cervical spinal cord injury during the acute stage ofinjury.Materials and methods: This study retrospectively reviewed theclinical data of 130 patients with cervical SCI. We analyzed thefactors believed to increase the risk of requiring a tracheostomy,including the severity of SCI, the level of injury as determined byradiological assessment, three quantitative MR imaging parameters,and eleven qualitative MR imaging parameters.Results: Significant differences between the non-tracheostomy andtracheostomy groups were determined by the following five factors onmultivariate analysis: complete SCI (p = 0.007), the radiologicallevel of C5 and above (p = 0.038), maximum canal compromise(MCC) (p = 0.010), lesion length (p = 0.022), and osteophyte formation (p = 0.015). For the MCC, the cut-off value was 46%, and therisk of requiring a tracheostomy was three times higher at an intervalbetween 50-60% and ten times higher between 60-70%. For lesionlength, the cut-off value was 20 mm, and the risk of requiring atracheostomy was two times higher at an interval between 20-30 mmand fourteen times higher between 40-50 mm.Conclusion: The ASIA grade A, a radiological injury level of C5 andabove, an MCC C 50%, a lesion length C 20 mm, and osteophyteformation at the level of injury were considered to be predictivevalues for requiring tracheostomy intervention in patients with cervical SCI (Table 1).
Keywords: adult; American Spinal Injury Association impairment scale; cervical spinal cord injury; conference abstract; controlled study; female; human; major clinical study; male; nuclear magnetic resonance imaging; osteophyte; predictive value; quantitative analysis; retrospective study; risk assessment; tracheostomy

Perioperative management of anterior cervical spine fixation in a patient with hereditary angioedema: A case report - Matsuo M.; Shakunaga K.; Sakamoto N.; Yamazaki M. (Japanese Journal of Anesthesiology, 2018)
Hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by recurrent episodes of cutaneous or submucosal angioedema The condition usually accompanies a deficiency in the Cl esterase inhibitor (Cl-INH) that leads to the overproduction of bradykinin, causing an abrupt increase in vascular permeability. In the present case, a 44-year-old woman with a history of HAE was scheduled to undergo cervical anterior fusion surgery. We planned treatment with human Cl-inactivator concentrate (Berinert P) prior to surgery and examined her pharynx and larynx by using video laryngoscopy (McGRATH®MAC) before intubation but without abnormal findings. The operation was performed under total intravenous anesthesia, and no significant issue was observed during surgery. The patient was kept intubated postoperatively because of potential laryngeal edema attack and postoperative hemorrhage. On postoperative day 1, Berinert P and dexamethasone were additionally administrated. Then, we extubated the trachea safely after significant upper airway edema had been excluded by using flexible laryngoscopy. Careful observation and sufficient preparation for reintubation or urgent tracheotomy are needed for patients with HAE after general anesthesia We demonstrated that HAE could be managed without life-threatening airway compromise by employing adequate pharmacological interventions and sensible determination of the timing of extubatioa.
Keywords: berinert p; complement component C1s inhibitor; dexamethasone; adult; angioneurotic edema; anterior spine fusion; article; case report; cervical anterior fusion; clinical article; extubation; female; human; intravenous anesthesia; intubation; laryngoscopy; larynx edema; perioperative period; postoperative hemorrhage; postoperative period; videolaryngoscopy; berinert p

German National Guideline for Treating Chronic Respiratory Failure with Invasive and Non-Invasive Ventilation - Revised Edition 2017: Part 2 - Windisch W.; Geiseler J.; Simon K.; Walterspacher S.; Dreher M.; Windisch W.; Dreher M.; Geiseler J.; Siemon K.; Brambring J.; Dellweg D.; Grolle B.; et al. (Respiration, 2018)
Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010, the German Respiratory Society (DGP) has leadingly published the guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure." However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the guidelines. For this reason, the updated guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease-specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning. In the current guidelines, different societies as well as professional and expert associations have been involved when compared to the 2010 guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.
Keywords: multichannel recorder; amyotrophic lateral sclerosis; artificial ventilation; bronchiectasis; central hypoventilation syndrome; chronic obstructive lung disease; chronic respiratory failure; comorbidity; coughing; cystic fibrosis; decision making; diaphragm stimulation; documentation; Epworth sleepiness scale; forced vital capacity; Germany; health care cost; health care organization; health care system; human; hypercapnia; hypoventilation; lung fibrosis; muscle weakness; nerve stimulation; nursing care; obesity hypoventilation syndrome; oxygen therapy; palliative therapy; paraplegia; patient care; phrenic nerve stimulation; polysomnography; positive end expiratory pressure; practice guideline; priority journal; quality of life; respiratory acidosis; review; sleep disordered breathing; spinal cord transsection; spirometry; terminal care; tracheotomy

Is the face an air bag for the brain and torso? - The potential protective effects of severe midface fractures - Woriax H.E.; Hamill M.E.; Gilbert C.M.; Reed C.M.; Faulks E.R.; Love K.M.; Lollar D.I.; Nussbaum M.S.; Collier B.R. (American Surgeon, 2018)
We investigated the patterns of injury associated with major midface trauma. Our hypothesis is that midface injuries are associated with a decrease in certain traumatic brain injuries as well as major torso injuries. The registry of our Level I trauma center was queried for all adult patients treated over 25 years from 1989 to 2013. Patients with midface fractures were identified based on the ICD-9 code. Associated injuries were defined based both on individual ICD-9 codes as well as the Barell Injury Matrix. Injury etiology was defined based on e-codes. Univariate analysis was performed using chi-squared test, Fisher's exact test, and Wilcoxon test. A total of 29,152 patients were identified. Excluding pediatric patients, those with exclusively penetrating trauma, and patients with incomplete data, 20,971 patients were included for subsequent analysis. Midface fractures were identified in 752 patients. Patients with Le Fort fractures were more likely to be male, have a higher Injury Severity Score, a lower arrival Glasgow Coma Scale, and more likely to require intensive care unit admission and mechanical ventilation, with a longer hospital length of stay. Patients with midface fractures had significantly fewer subdural hematomas, subarachnoid hemorrhages, spine fractures, and were less likely to have associated abdominal and pelvic injuries. Patients with midface fractures were more likely to require facial reconstruction procedures and craniotomy. Patients presenting with midface fractures after blunt trauma have a distinctly different pattern of injuries. One potential mechanism for this is a deceleration effect, where midface impact and resulting fractures dissipate some of the energy.
Keywords: airbag; protective equipment; abdominal injury; adult; article; artificial ventilation; assault; Barell Injury Matrix; blood transfusion; blunt trauma; craniectomy; craniotomy; disease assessment; disease association; disease registry; face fracture; face injury; face surgery; falling; female; Glasgow coma scale; hospital admission; human; ICD-9; injury scale; injury severity; intensive care unit; laparotomy; length of stay; liver resection; major clinical study; male; maxilla fracture; middle aged; open reduction (procedure); osteosynthesis; pelvis injury; sex difference; spine fracture; spine fusion; splenectomy; subarachnoid hemorrhage; subdural hematoma; thoracotomy; thorax injury; tracheostomy; traffic accident; traumatic brain injury; ventriculostomy

Surgical decompression for recurrent cord compression in cancer: a case series and review of the literature - Rajah G.; Rapp A.; Discolo E.; Eltahawy H. (Neurological Research, 2018)
Spine metastases affect a significant number of cancer patients each year, with the spine being the third most common location for cancer spread. As patients live longer with improved treatments, the opportunity for recurrence at previously treated sites increases. Here, we describe seven patients with recurrent, compressive, metastatic spine tumors at previously surgically treated sites that required additional surgical intervention with manipulation of at least one rod. Five of the patients had recurrence including adjacent levels while two had recurrence solely at the previously decompressed level. The patients remained ambulatory for an average of 31.2 months after the initial surgery. We also discuss the role of adjuvant treatment in these patients and review the literature.
Keywords: stomach tube; adult; aged; article; breast cancer; clinical article; colon adenocarcinoma; decompression surgery; deep vein thrombosis; follow up; gastrostomy; human; laminectomy; middle aged; mobilization; pancytopenia; pleura effusion; renal cell carcinoma; sarcoma; scar; sepsis; spinal cord compression; spine metastasis; tracheostomy; tumor recurrence; tumor volume

Percutaneous Dilatational Tracheostomy in Ankylosing Spondylitis (Bechterew Disease) Is Feasible and Not Associated With Higher Complication Rates - Hamsen U.; Kamp O.; Kaczmarek C.; Aach M.; Waydhas C.; Schildhauer T.A.; Jansen O. (Journal of Intensive Care Medicine, 2018)
Background: Ankylosing spondylitis (AS) is a common disease with an incidence of approximately 0.5% in Europe, causing severe limitations of axial spine mobility and cervical kyphosis. Deformities of the cervical spine and the temporomandibular joints could increase the risk of complications while performing an intubation or tracheostomy. The percutaneous dilatational tracheostomy (PDT) is a standard procedure in intensive care medicine. However, the combination of cervical kyphosis and osteoporosis makes patient positioning challenging. Therefore, one could conclude that patients with AS are not candidates for PDT, but neither studies nor case reports yet reported about feasibility of this procedure in AS. Methods: Retrospective analysis at a level 1 trauma center of patient records from 2002 to 2016, assessing all patients with AS and PDT. Results: A total of 31 patients with AS have been subjected to PDT. All PDTs were performed using the modified Ciaglia single-step dilatational technique. Neither cardiopulmonary nor surgical complications occurred during the procedure. One patient received a change of the existing airway prior to the procedure; a small nasal tube was changed for a laryngeal mask. Conclusion: Although head positioning may be challenging, PDT should be taken into consideration for patients with AS. In the hands of an experienced doctor, it is safe and feasible.
Keywords: laryngeal mask; tracheostomy kit; aged; ankylosing spondylitis; article; ciaglia single step dilatational technique; echography; female; human; intensive care unit; intubation; kyphosis; major clinical study; male; osteoporosis; postoperative complication; priority journal; retrospective study; spinal cord injury; spine fracture; surgical technique; tracheostomy; Ciaglia Blue Rhino

Complications following lengthening of spinal growing implants: Is postoperative admission necessary? - Shaw K.A.; Fletcher N.D.; Devito D.P.; Murphy J.S. (Journal of Neurosurgery: Pediatrics, 2018)
OBJECTIVE: The aim of this study was to evaluate the effect of postoperative admission status on 30-day perioperative complications in patients with growing spinal instrumentation undergoing surgical lengthening. METHODS: This retrospective case-control study of records from the 2014-2015 National Surgical Quality Improvement Program-Pediatric database was performed to identify surgical lengthening procedures of spinal implants in patients with growing instrumentation by Current Procedural Terminology code. The 30-day postoperative complications were classified according to the Clavien-Dindo system. Patients were subdivided according to their postsurgical admission status. Admission status, American Society of Anesthesiologists (ASA) Physical Status classification, tracheostomy, neuromuscular diagnosis, ventilator dependence, and nutritional support were considered as possible risk factors in univariate and multivariate logistic regression analyses. RESULTS: A total of 796 patients were identified (mean age 9.09 ± 3.44 years; 54% of patients were female), of whom 73% underwent lengthening on an inpatient basis. Patients with a tracheostomy or ventilator dependence were more likely to be admitted postoperatively. The overall rate of major complications was 3.5% and did not differ based on admission status (2.8% inpatient vs 3.8% outpatient, p = 0.517). On univariate analysis, ventilator dependence (9.5% vs 2.7%, p = 0.002), need for nutritional support (7.1% vs 2.5%, p = 0.006), and ASA class > II (4.8% vs 1.3%, p = 0.04) placed patients at a higher risk for any postoperative complications. Multivariate analysis identified only ventilator dependence as an independent risk factor for any perioperative complication. CONCLUSIONS: Postoperative admission status did not affect the rate of 30-day perioperative complications, readmission, or rate of unplanned operations following lengthening of growing spinal instrumentation. Outpatient lengthening appears to be safe; however, consideration for postoperative admission should be given for those who are ventilator dependent.
Keywords: spine implant; artificial ventilation; case control study; child; cohort analysis; conference paper; female; hospital readmission; human; implant failure; major clinical study; male; nerve injury; nutritional support; peroperative complication; pneumonia; postoperative infection; postoperative period; priority journal; prosthesis complication; reoperation; retrospective study; school child; sepsis; superficial dehiscence; superficial infection; tracheostomy; urinary tract infection; venous thromboembolism

Bilateral vocal cord palsy after a posterior cervical laminoplasty - Iwai C.; Fushimi K.; Nozawa S.; Shirai Y.; Ogawa H.; Yasura K.; Shimizu K.; Akiyama H. (European Spine Journal, 2018)
Purpose: To report a patient with bilateral vocal cord palsy following cervical laminoplasty, who survived following a tracheotomy and intensive respiratory care. Methods: Acute respiratory distress is a fatal complication of cervical spinal surgery. The incidence of bilateral vocal cord palsy after posterior cervical decompression surgery is extremely rare. The authors report a 71-year-old woman who suffered from cervical myelopathy due to ossification of the posterior longitudinal ligament. Open-door laminoplasty from C2 to C6 and laminectomy of C1 were performed. Following surgery, extubation was successfully conducted. Acute-onset dysphagia and stridor had occurred 2 h following extubation. A postoperative fiber optic laryngoscope revealed bilateral vocal cord palsy. After a tracheotomy and intensive respiratory care, she had completely recovered 2 months after surgery. Discussion: One potential cause of this pathology was an intraoperative hyper-flexed neck position, which likely induced mechanical impingement of the larynx, resulting in swelling and edema of the vocal cords and recurrent laryngeal nerve paresis. Direct trauma of the vocal cords during intubation and extubation could have also induced vocal cord paralysis. Conclusions: We reported a case of bilateral vocal cord palsy associated with posterior cervical laminoplasty. Airway complications following posterior spinal surgery are rare, but they do occur; therefore, spine surgeons should be aware of them and take necessary precautions against intraoperative neck position, intubation technique, even positioning of the intratracheal tube.
Keywords: cannula; endotracheal tube cuff; fiberoptic laryngoscope; head holder; nasogastric tube; rigid laryngoscope; videolaryngoscope; fentanyl; hydrocortisone; propofol; remifentanil; rocuronium; aged; anesthesia induction; article; artificial ventilation; aspiration pneumonia; bilateral vocal cord palsy; bladder dysfunction; breathing; case report; cervical myelopathy; cervical spine radiography; clinical article; consciousness; continuous infusion; dexterity test; dysphagia; elective surgery; endotracheal intubation; extubation; female; first cervical vertebra; french open door laminoplasty; human; hyperreflexia; hypertension; intensive care; intestine function disorder; laminectomy; laminoplasty; larynx edema; lower limb; medical history; nuclear magnetic resonance imaging; operation duration; operative blood loss; otolaryngologist; oxygen saturation; paralysis; paresthesia; posterior cervical laminoplasty; posterior longitudinal ligament; postoperative analgesia; postoperative complication; postoperative pain; postoperative period; priority journal; prone position; respiratory care; sixth cervical vertebra; spastic gait; speech therapy; stridor; swallowing; tendon reflex; tracheostomy; tracheotomy; upper limb; vertebral canal stenosis; vocal cord; vocal cord paralysis; voluntary movement

Risk factors for dysphagia after a spinal cord injury: a systematic review and meta-analysis - Iruthayarajah J.; McIntyre A.; Mirkowski M.; Welch-West P.; Loh E.; Teasell R. (Spinal Cord, 2018)
Study design: Systematic review and meta-analysis. Objective: To determine the risk factors predictive of dysphagia after a spinal cord injury (SCI). Setting: None. Methods: A comprehensive literature search was performed in five scientific databases for English articles that identified risk factors for dysphagia after a SCI in adult (≥19 years) individuals. Data extracted included: author name, year and country of publication, participant demographics, sample size, study design, method of dysphagia diagnosis, and risk factor percentages. Methodological quality of studies was assessed using the Newcastle–Ottawa Scale. For identified risk factors, risk percentages were transformed into risk ratios (RR) with 95% confidence intervals. Quantitative synthesis was performed for risk factors reported in two or more studies using restricted maximum-likelihood estimator random effects models. Results: Eleven studies met inclusion criteria of which ten studies were of moderate quality (n = 10). Significant risk factors included: age, injury severity, level of injury, presence of tracheostomy, coughing, voice quality, bronchoscopy need, pneumonia, mechanical ventilation, nasogastric tubes, comorbid injury, and a cervical surgery. Results of the quantitative synthesis indicated that the presence of a tracheostomy posed a threefold greater risk of the development of dysphagia (RR: 3.67); while, cervical surgery posed a 1.3 times greater risk of the development of dysphagia (RR: 1.30). Conclusions: Knowledge of these risk factors can be a resource for clinicians in the early diagnosis and appropriate medical management of dysphagia post SCI.
Keywords: adult; age; article; artificial ventilation; bronchoscopy; coughing; diagnosis; dysphagia; early diagnosis; female; human; injury severity; maximum likelihood method; meta analysis; nasogastric tube; Newcastle-Ottawa scale; pneumonia; publication; quantitative analysis; risk assessment; risk factor; sample size; spinal cord injury; surgery; systematic review; tracheostomy; voice

The rule is: There are no rules - Tshiombo G. (International Journal of Cardiology, 2018)
Keywords: stent graft; aortic aneurysm; aortic dissection; blood vessel rupture; cerebrovascular accident; clinical effectiveness; dialysis; disease severity; editorial; endovascular aneurysm repair; human; mortality; open surgery; outcome assessment; priority journal; reoperation; respiratory failure; sensitivity and specificity; spinal cord injury; thrombosis; tracheostomy; treatment outcome; treatment response; vocal cord paralysis

Indications and complications of rigid bronchoscopy - Batra H.; Yarmus L. (Expert Review of Respiratory Medicine, 2018)
Introduction: Rigid bronchoscopy is an invaluable tool for the management of airway disorders and an essential skill for an interventional pulmonologist. Since its introduction in the late 19th century, it has remained an important technique for the management of central airway obstruction, foreign body aspiration and massive hemoptysis. Areas covered: This article will review the history, indications, contraindication, technique and complications of rigid bronchoscopy. We will also briefly discuss the methods of anesthesia and ventilation and finally our perspective on the future of rigid bronchoscopy. Expert commentary: Although the rise of flexible bronchoscopy in the 1960s led to a decline in the use of rigid bronchoscopy, the last two decades have witnessed resurgence in its popularity. We anticipate that it will remain an important tool used by interventional pulmonologists for decades to come. We suggest that interventional pulmonologists must have training and develop expertise in this technique.
Keywords: biopsy forceps; flexible bronchoscope; grasping forceps; rigid bronchoscope; rigid telescope; self expandable metallic stent; ablation therapy; airway obstruction; artificial ventilation; arytenoid cartilage; bronchoplasty; bronchoscopy; bronchus tumor; cartilage injury; cervical spine; cytoreductive surgery; foreign body; general anesthesia; heart arrhythmia; heart muscle ischemia; hemoptysis; history of medicine; human; hypoxemia; jet ventilation; laceration; laryngoscopy; larynx edema; light related phenomena; mouth injury; perforation; pharynx; preoperative evaluation; pulmonologist; respiratory tract injury; respiratory tract intubation; review; spinal cord injury; spine disease; tracheostomy; treatment contraindication; treatment indication; videorecording; vocal cord

Heart-Shaped Bilateral Medullary Pyramidal Infarction as a Pathognomonic Finding of Anterior Spinal Artery Occlusion - Searcy S.; Akinduro O.O.; Spector A.; Yoon J.W.; Brown B.L.; Freeman W.D. (Neurocritical Care, 2018)
Background: Unilateral anterior spinal artery (ASA) occlusion resulting in bilateral medullary pyramidal (BMP) infarction is a rare and devastating stroke subtype. We present two cases highlighting the diagnostic and clinical challenges of BMP infarction. Methods: Case reports and literature review. Results: A 57-year-old man rapidly had severe vomiting and diarrhea 2 h after a meal. Examination revealed bulbar weakness and areflexic tetraplegia. Respiratory failure developed, requiring intubation and mechanical ventilation. Brain magnetic resonance imaging (MRI) showed a heart-shaped region of diffusion abnormality, characteristic of BMP infarction. Cerebral angiography showed an occluded left vertebral artery with unilateral left-sided origin of ASA. The patient required tracheostomy and percutaneous gastrostomy tube and was discharged to rehabilitation, with little improvement of his tetraplegia at 3-month follow-up. A 43-year-old woman presented to the emergency department with acute onset of lower-extremity paresthesia and history of upper respiratory infection 2 weeks prior. Initial examination findings included bulbar weakness, dysphagia, hyporeflexia, and generalized weakness. After admission, she had severe respiratory distress and required intubation. Lumbar puncture was evaluated for Guillain–Barré syndrome, but cerebrospinal fluid protein concentration was normal. Changes on diffusion-weighted MRI of the brain showed the characteristic heart-shaped BMP infarction, indicating occlusion of a unilateral ASA. She required tracheostomy and percutaneous gastrostomy tube placement, with no paralysis resolution. Conclusion: Acute BMP infarction may present with flaccid tetraplegia mimicking neuromuscular disorders. When the infarction is recognized early, intravenous thrombolysis can be considered to reduce morbidity of this rare stroke subtype.
Keywords: percutaneous endoscopic gastrostomy tube; powered wheelchair; botulinum antiserum; immunoglobulin; abnormal respiratory sound; adult; anterior spinal artery occlusion; antibiotic therapy; anxiety; article; artificial ventilation; Babinski reflex; bilateral medullary pyramidal infarction; botulism; brain angiography; brain infarction; breathing disorder; bronchoscopy; bulbar paralysis; case report; cerebrospinal fluid examination; clinical article; clinical outcome; cognition; coughing; depression; diabetes mellitus; diarrhea; diffusion weighted imaging; drinking behavior; drug response; dysphagia; dysphonia; dyspnea; eye movement; face asymmetry; female; follow up; Guillain Barre syndrome; hospital discharge; human; hypertension; intubation; laboratory test; lumbar puncture; lung lavage; magnetic resonance angiography; male; medical history; middle aged; migraine; morbid obesity; muscle strength; neurologic examination; nuclear magnetic resonance imaging; nursing care; occlusive cerebrovascular disease; paresthesia; physical examination; priority journal; pseudobulbar palsy; pupil reflex; quadriplegia; rehabilitation; respiratory failure; smoking; tracheostomy; treatment duration; upper respiratory tract infection; vomiting; walking difficulty

Nusinersen in infants who initiate treatment in a presymptomatic stage of spinal muscular atrophy (SMA): Interim results from the Phase 2 NURTURE study - De Vivo D.C.; Bertini E.; Hwu W.; Foster R.; Bhan I.; Gheuens S.; Farwell W.; Reyna S.P.; Vajsar J. (Canadian Journal of Neurological Sciences, 2018)
Background: NURTURE (NCT02386553) is an ongoing openlabel single-arm efficacy/safety study of intrathecal nusinersen in infants who initiate treatment in a presymptomatic stage of spinal muscular atrophy (SMA). Methods: Enrolled infants were age ≤6 weeks at first dose, clinically presymptomatic, had genetically diagnosed SMA, and 2 or 3 copies of SMN2. Primary endpoint is time to death or respiratory intervention (≥6 hours/day continuously for ≥7 days or tracheostomy). Results: As of July 5, 2017, 25 infants (2 copies SMN2, n=15;3 copies, n=10) were enrolled. All infants were alive. Two infants (both with 2 copies SMN2) required respiratory intervention (but not tracheostomy or permanent ventilation) during an acute, reversible viral infection and thus met the primary endpoint. At last visit, 22/24 (92%) infants had achieved WHO motor milestones sitting without support and 8/16 (50%;2 SMN2, n=3/11;3 SMN2, n=5/5) on study >13 months achieved walking alone. AEs were reported in 24/25 (96%) infants; most 20/25 (80%) had AEs that were mild/moderate in severity; 9 had serious AEs. Four infants had an AE possibly related to study drug, which resolved despite continued treatment. No new safety concerns were identified. Conclusions: Nusinersen continued to benefit infants who initiated treatment in a presymptomatic stage of SMA.
Keywords: endogenous compound; nusinersen; survival motor neuron protein 2; artificial ventilation; case report; clinical article; conference abstract; death; diagnosis; drug therapy; female; human; infant; male; phase 2 clinical trial; spinal muscular atrophy type 2; tracheostomy; virus infection; walking

Nusinersen and early physiotherapy in patients with spinal muscular atrophy type 1: Case series - Bazancir Z.; Aslan M.; Bözgör B.; Güngör S.; Talu B. (Acta Myologica, 2018)
Objectives. To evaluate effects of combined of nusinersen and early physiotherapy on functional performance, respiratory and nutritional status in four cases with SMA type 1. Method. The children was three females and one male, aged from 10 month to 5 years 9 month. One patient used nasal CPAP, 3 patients benefited from mechanical ventilation with tracheotomy and one patient had gastrostomy at baseline. All patients underwent a physiotherapeutic evaluation at the time of the first consultation (pre-treatment) as well as on the follow up-visit (8 week intervals for 24 weeks). CHOP INTEND is used to evaluate functional performance. The primary endpoint of cases was change in CHOP INTEND score from baseline. As secondary endpoint, we evaluated changes in respiratory and nutritional status as well as parents' impression regarding improvements or worsening in motor and respiratory function. To evaluate parents' impression, we used a Likert scale with five categories. Intrathecal Nusinersen was applied 4 doses. Physiotherapy program were done 2 days in a week for 24 week supervised by physiotherapist and home exercises were given on other days. Results. After 4 doses nusinersen and early physiotherapy, mean improvement of CHOP INTEND score was 10.7 ± 2.21 points, MV pressure was reduced three patient, a patient was observed to need nasal CPAP during sleep only. Nutritional status and parents' impression were improved. Parents of 3 children reported a marked improvement and parents of 1 children a slight improvement in motor function, regarding respiratory function an improvement, parents of 3 children a marked improvement and parent of 1 children did not observe a change. Conclusions. Depending the improvements after nusinersen and early physiotherapy, we believe that the prospective follow up of this data will bring information about benefit ratio of nusinersen and early physiotherapy in this population.
Keywords: nusinersen; case report; case study; child; clinical article; clinical evaluation; conference abstract; consultation; drug therapy; exercise; female; follow up; gastrostomy; human; Likert scale; male; motor performance; nutritional status; physiotherapist; physiotherapy; positive end expiratory pressure; preschool child; prospective study; respiratory function; sleep; tracheostomy; Werdnig Hoffmann disease

The natural course of dysphagia in ALS is different between patients with bulbar and spinal onset - Torrieri M.C.; Manera U.; D'ovidio F.; Calvo A.; Moglia C.; Cammarosano S.; Ilardi A.; Canosa A.; Bersano E.; Mazzini L.; Chiò A. (European Journal of Neurology, 2018)
Background and aims: We aimed at evaluating the relationship between dysphagia and site of onset in amyotrophic lateral sclerosis (ALS) in terms of progression rate. Methods: We enrolled 871 incident ALS patients (580 with spinal onset, 291 with bulbar onset), resident in Piemonte and Valle d'Aosta, Italy, from 2007 to 2013. Based on ALSFRS-R item 3, dysphagia was classified as “severe” (0-1), “moderate” (2-3), “absent” (4). The progression of dysphagia was considered as time-dependent variable and was reassessed at each visit. Progression rate of dysphagia was calculated separately among patients with bulbar and spinal onset (N=642). Time intervals ended with the date when dysphagia became severe. The starting date was the date of onset for bulbar patients (N=279) and the date of first bulbar symptoms (considering ALSFRS-R items 1 and 3<4) for spinal patients (N=363). 217 patients did not develop dysphagia during the follow-up. Results: ALS patients showed moderate dysphagia after a median of 24.4 months (IQR=13.5-44.7) from the onset. Dysphagia became severe after additional 17.7 months (IQR=9.5-29.8); death/tracheostomy occurred after additional 6.9 months (IQR=2.6-14.7). The progression rate showed a median time interval of 17.4 months (IQR=8.9-29.5) between the first bulbar symptom and severe dysphagia. These time intervals were shorter in patients with spinal onset (median 11.9 months; IQR=5.5-24.1) than in cases with bulbar onset (22.9 months; IQR=16.0-31.8) (Wilcoxon test p=0.000). Conclusion: The progression rate of dysphagia significantly differs between ALS patients with bulbar and spinal onset.
Keywords: adult; amyotrophic lateral sclerosis; Aosta Valley; conference abstract; controlled study; death; dependent variable; disease course; dysphagia; female; follow up; human; major clinical study; male; rank sum test; resident; tracheostomy

Cluster of acute flaccid myelitis associated with enterovirus d68 (EV-D68) in five children in South East Scotland, september-october 2016 - Chinchankar N.; Kirolos A.; Mark K.; McDougall C.; Shetty J.; Templeton K.; Pilley E.; Stevenson J.; Eunson P.; Waugh C. (Pediatric Critical Care Medicine, 2018)
Aims & Objectives: We aim to describe the clinical presentation, epidemiology and long term followup of five cases presenting with acute flaccid myelitis (AFM) due to enterovirus D68 (EV-D68) Methods After the index case was diagnosed a multidisciplinary Incident management team was convened to identify possible cases of AFM. Suspected cases were investigated with nasopharyngeal secretion (NPS) testing and MRI. Results Five children, positive for EV-D68 on NPS presented with asymmetrical limb weakness, decreased truncal tone and reduced reflexes. MRI of the brain and spinal cord demonstrated abnormal high T2 signal in the dorsal pons, medulla and spinal cord grey matter, with predominance in the cervical spinal cord. Nerve conduction studies carried out on two of the most severely affected cases showed evidence of acute motor neuropathy. (Figure prsented). Conclusions This was the largest paediatric cluster of AFM associated with EV-D68 in Europe and the first to have affected Scotland. Patients were aged between 2-6 years old. Two children had complications of autonomic involvement with hypertension and evidence of end organ damage. All cases suffered from limb pain which responded to Gabapentin. Three patients had bulbar involvement, 2 of whom required intubation, ventilation and intensive care management. These 2 children then went on to require tracheostomies and long term home ventilation. All the 5 children have required and continue to require ongoing community rehabilitation due to persistent neurological deficits.
Keywords: gabapentin; artificial ventilation; case report; cervical spinal cord; child; clinical article; complication; conference abstract; diagnosis; Enterovirus D68; female; follow up; gray matter; human; hypertension; intubation; limb pain; limb weakness; male; medulla oblongata; motor neuropathy; myelitis; nerve conduction; nonhuman; nuclear magnetic resonance imaging; organ injury; pons; preschool child; reflex; rehabilitation; Scotland; tracheostomy

Pial arteriovenous fistula of the spine in a child with hemiplegia - Hatayama K.; Goto S.; Nishida A.; Inoue M. (Clinical Case Reports, 2018)
Pial arteriovenous fistula (AVF) is an extremely rare disease in children. When a child presents with sudden onset of hemiparesis and headache, it is very important to perform spinal magnetic resonance imaging (MRI) scanning for early diagnosis and treatment.
Keywords: guiding catheter; immunoglobulin; mannitol; poliomyelitis vaccine; steroid; arteriovenous fistula; article; artificial ventilation; case report; child; clinical article; computer assisted tomography; consciousness; digital subtraction angiography; dyspnea; edema; follow up; headache; hemiplegia; human; intensive care unit; lumbar puncture; male; motor performance; neurologic examination; nuclear magnetic resonance imaging; posterior inferior cerebellar artery; preschool child; priority journal; quadriplegia; spinal hematoma; tracheostomy

Cervical spine osteomyelitis in head and neck cancer patients: Looking twice for posterior hypopharyngeal dehiscence - Clifton W.E.; Gupta V.; Prause C.; Vibhute P.G. (Radiology of Infectious Diseases, 2018)
Cervical spinal osteomyelitis due to breakdown of the posterior wall of the hypopharynx is a rare complication following surgery and adjuvant radiation or chemotherapy of head and neck cancer. Very few such cases have been reported in literature. We report 2 cases of cervical spine osteomyelitis in head and neck cancer patients due to posterior hypopharyngeal dehiscence. In the first patient, a 74 year old woman with total laryngectomy performed 12 years ago; the osteomyelitis followed an iatrogenic tear from balloon dilatation of a chronic radiation induced neopharyngeal stricture. There was a slit-like defect on MRI in the posterior wall at the junction of the neopharynx and esophagus immediately anterior to C5-6 osteomyelitis. The second, a 53 year old male patient developed this complication from a spontaneous breakdown of the posterior hypopharynx 4 months after completing chemoradiation for tonsillar cancer. There was failure to recognize the sinus tract on the immediate post-treatment neck CT which predated the onset of osteomyelitis. Both patients were treated with broad spectrum antibiotic therapy and discontinuation of oral feeding. Awareness and diligent detection of hypopharyngeal dehiscence is important in cases of cervical spinal osteomyelitis occurring in head and neck cancer patients.
Keywords: amoxicillin plus clavulanic acid; antibiotic agent; ceftriaxone; doxycycline; fluconazole; adult; aged; article; balloon dilatation; cancer radiotherapy; case report; cervical spine osteomyelitis; clinical article; esophagus; esophagus stenosis; feeding; female; gastrostomy; granulation tissue; head and neck cancer; human; human tissue; hypopharynx; laryngectomy; male; metastasis; middle aged; nuclear magnetic resonance imaging; osteomyelitis; tonsillectomy; tracheostomy; wound dehiscence; x-ray computed tomography; Zenker diverticulum

Amyotrophic Lateral Sclerosis and the Respiratory System - Braun A.T.; Caballero-Eraso C.; Lechtzin N. (Clinics in Chest Medicine, 2018)
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that always affects the respiratory muscles. It is characterized by degeneration of motor neurons in the brain and spinal cord. Respiratory complications are the most common causes of death in ALS and typically occur within 3 to 5 years of diagnosis. Because ALS affects both upper and lower motor neurons, it causes hyperreflexia, spasticity, muscle fasciculations, muscle atrophy, and weakness. It ultimately progresses to functional quadriplegia. ALS most commonly begins in the limbs, but in about one-third of cases it begins in the bulbar muscles responsible for speech and swallowing.
Keywords: pacemaker; abdominal disease; amyotrophic lateral sclerosis; artificial ventilation; breathing muscle; bronchus secretion; bulbar innervated muscle dysfunction; chest wall oscillation; coughing; daytime somnolence; diaphragmatic pacemaker; dyspnea; expiratory muscle weakness; fasciculation; headache; human; hyperreflexia; inspiratory muscle weakness; lung disease; lung function test; maximal expiratory pressure; maximal inspiratory pressure; motoneuron; muscle atrophy; muscle weakness; noninvasive ventilation; orthopnea; overall survival; oximetry; palliative therapy; pneumonia; polysomnography; positive end expiratory pressure; priority journal; quality of life; review; risk reduction; sleep disorder; spasticity; speech disorder; tachypnea; terminal care; tracheostomy; vaccination; ventilator weaning

Progress in the Treatment of Patients with Neuromuscular and Nonmuscular Chest Wall Diseases - McCool F.D.; Benditt J.O. (Clinics in Chest Medicine, 2018)
Keywords: cause of death; disability; Duchenne muscular dystrophy; editorial; human; kyphoscoliosis; morbidity; mortality; neuromuscular disease; noninvasive ventilation; obesity hypoventilation syndrome; pneumonia; priority journal; respiratory failure; sleep disordered breathing; spinal cord injury; survival rate; thorax disease; tracheostomy

Cervical spine injury from unrecognized craniocervical instability in severe pierre robin sequence associated with skeletal dysplasia - Zhu X.; Evans K.N.; El-Gharbawy A.; Lee J.Y.; Brooker J.E.; Jabbour N.; Tyler-Kabara E.C.; Madan-Khertarpal S.; Losee J.E.; Goldstein J.A. (Cleft Palate-Craniofacial Journal, 2018)
Pierre Robin Sequence (PRS) can be associated with skeletal dysplasias, presenting with craniocervical instability and devastating spinal injury if unrecognized. The authors present the case of an infant with PRS and a type II collagenopathy who underwent multiple airway-securing procedures requiring spinal manipulation before craniocervical instability was identified. This resulted in severe cervical cord compression due to odontoid fracture and occipitoatlantoaxial instability. This case highlights the importance of early cervical spine imaging and cautious manipulation in infants with PRS and suspected skeletal dysplasia.
Keywords: cervical collar; halo brace; nasal cannula; stomach tube; collagen 2a1; collagen type 2; unclassified drug; airway obstruction; alkalemia; apnea hypopnea index; article; atlantooccipital joint; brain ventricle dilatation; bronchoscopy; case report; cervical spine; cervical spine injury; chondropathy; cleft palate; clinical article; clonus; computer assisted tomography; disease association; disease severity; distraction osteogenesis; electrolyte disturbance; endotracheal anesthesia; endotracheal intubation; extubation; failure to thrive; feeding difficulty; fetus echography; food aversion; gastroesophageal reflux; gastrostomy; gene mutation; general anesthesia; glossoptosis; human; hyperreflexia; hypocapnia; infant; laminectomy; laryngomalacia; laryngoscopy; larynx surgery; male; micrognathia; neurologic examination; nuclear magnetic resonance imaging; occupational therapy; odontoid process fracture; odontoidectomy; oxygen desaturation; palatoplasty; physiotherapy; Pierre Robin syndrome; plastic surgery; polysomnography; priority journal; quadriplegia; respiratory distress; short stature; sleep disordered breathing; spasticity; speech therapy; spinal cord compression; spinal cord decompression; spinal cord injury; spine instability; spine surgery; spondyloepiphyseal dysplasia; spondyloepiphyseal dysplasia congenita; stomach fundoplication; subdural hematoma; subglottic stenosis; supraglottoplasty; synchondrosis; tongue collapse; tongue disease; tracheostomy; ventilator weaning; vertebral canal stenosis

Outcomes for patients with Guillain Barre Syndrome transferred to a new weaning and long-term ventilation service in Liverpool, UK - Parker R.; Ford V.; Ward K.; Ashcroft H.; Duffy N.; Chakrabarti B.; Angus R. (Journal of the Intensive Care Society, 2018)
Introduction: In October 2010 the long-term ventilation service in Liverpool began providing support for non-spinal cord injury patients ventilated via a tracheostomy. As part of the Ventilation Centre (VIC) building work it became possible to look after stable tracheostomy ventilated patients away from ICU. A service has been established to assess, transfer and offer weaning for slow to wean patients from the North West and North Wales. Methods: Prospective data collection has been done by two of the authors, VF and RP for the first 5 years. Outcomes have been assessed regarding underlying reason for failure to wean, weaning success, and follow up to one year. Failure to wean has been classified as neuromuscular disease, COPD, obesity, kyphoscoliosis and chest wall deformity, post-surgery and other. This enables comparison with published UK data. Of the first 95 transfers for weaning it was noted that 10 had Guillain Barre Syndrome and this was the main reason for weaning failure. This is a group which may be traditionally felt to have a poor prognosis, and in whom the evidence is limited. Results: The median age for GBS patients was 58 years, and 50% were male. All were transferred from a General ICU, the median length of stay in the referring ICU prior to transfer was 69 days (range 16-265 days). The median VIC length of stay before discharge was 65.5 days (range 18-121 days), including discharge planning. They were profoundly weak on admission, mean MRC sum score 22/60. Despite this all were weaned and decannulated, seven discharged with long-term nocturnal NIV, three with no support. All those with NIV were issued cough assist devices initially for home use. Two went directly home, and eight to rehabilitation. Nine were alive one year after VIC discharge and six were living in their own home. No patients had PEG feeding on discharge, all were orally fed. Whilst four patients were treated for pneumonia on the VIC, no patients required re-escalation of care to the General ICU. Conclusions: Patients with GBS as the reason for weaning failure had spent longer on the referring ICU, and then spent longer on the VIC compared with allcomers (median 69 vs 48 days and 65.5 vs 42 days respectively). Despite signifcant peripheral muscle weakness a weaning approach based around nocturnal NIV, chest physiotherapy and physical rehabilitation can produce good outcomes in selected patients.
Keywords: adult; artificial ventilation; breathing exercise; case report; chronic obstructive lung disease; clinical article; conference abstract; feeding; female; follow up; Guillain Barre syndrome; hospital discharge; human; intensive care unit; kyphoscoliosis; length of stay; male; middle aged; muscle weakness; non continuous ventilator; obesity; pneumonia; prognosis; prospective study; rehabilitation; surgery; thorax deformity; treatment failure; weaning

Systematic review of respiratory approach to the quadriplegic patient in the acute stage. The role of the respiratory physiotherapist - Montigiani G.; Papi D.; Bucciardini L.; Sommariva M. (Italian Journal of Medicine, 2018)
Introduction: Pulmonary complications in spinal cord injury are the most common cause of death in the acute phase. During the acute stage is usually managed with two different approaches: the invasive approach, by having the patient undergoing tracheotomy and invasive ventilation or the non-invasive approach, which involves prematurely extubating the patient, supporting him with non-invasive ventilation (NIV) and manual or mechanical cough assistance. Purpose: The aim of the study was to investigate among the recently published articles, which would be the choice of respiratory care to the patient with a complete cervical spinal cord injury in the acute phase. Methods: A systematic review was performed selecting articles where the number of tracheotomy and non-tracheotomy in patients with a complete cervical spinal injury. Results: 19 articles were selected. Six articles show a preference to the non-invasive approach with a percentage of non-tracheotomy patients less than 50%. 13 articles have a percentage of tracheotomies greater than 50%. No study offers a randomized clinical trial in which the two interventions are compared. Discussion: It is preferable to carry out an early tracheotomy, considering it to be a faster and safer approach. Non-invasive ventilation prevents the infections and to short and long-term complications related to the presence of a tracheal cannula. Conclusions: NIV is therefore a valid alternative to invasive ventilation in the spinal cord injured patient but it requires a well-trained team where the respiratory physiotherapist plays a fundamental role.
Keywords: adult; cannula; cervical spinal cord injury; complication; conference abstract; female; human; infection; male; noninvasive ventilation; physiotherapist; randomized controlled trial (topic); respiratory care; systematic review; trachea; tracheostomy

Surgical treatment for old subaxial cervical dislocation with bilateral locked facets in a 3-year-old girl - Li C.; Li L.; Duan J.; Zhang L.; Liu Z. (Medicine (United States), 2018)
Rationale: This study aimed to describe the case of a 3-year-old girl with old bilateral facet dislocation on cervical vertebrae 6 and 7, who had spinal cord transection, received surgical treatment, and achieved a relative satisfactory therapeutic effect. Patient concerns: A 3-year-old girl was urgently transferred to the hospital after a car accident. Diagnoses: she was diagnosed with splenic rupture, intracranial hemorrhage, cervical dislocation, spinal transection, and Monteggia fracture of the left upper limb. Interventions: The girl underwent emergency splenectomy and was transferred to the intensive care unit of the hospital 15 days later. One-stage anterior-posterior approach surgery (anterior discectomy, posterior laminectomy, and pedicle screw fixation) was performed when the patient stabilized after 45-day symptomatic treatment. The operation was uneventful. Outcomes: The reduction of lower cervical dislocation was satisfactory, with sufficient spinal cord decompression. The internal fixation position was good, and the spinal sequence was well restored. The girl was discharged 2 weeks later after the operation and followed up for 2 years. The major nerve function of both upper limbs was recovered, with no obvious retardation of the growth of immature spine. Lessons: A satisfactory therapeutic effect was achieved for a pediatric old subaxial cervical dislocation with bilateral locked facets using anterior discectomy, posterior laminectomy, and pedicle screw fixation. The posterior pedicle screw fixation provided a good three-dimensional stability of the spine, with reduced risk and complications caused by anterior internal fixation. The growth of immature spine was not obviously affected during the 2-year follow-up.
Keywords: pedicle screw; article; assisted ventilation; bilateral locked facet; brain hemorrhage; case report; cervical spine dislocation; child; clinical article; coma; computer assisted tomography; digital radiography; discectomy; emergency surgery; female; follow up; Glasgow coma scale; hospital discharge; human; laminectomy; monteggia fracture; nerve function; nuclear magnetic resonance imaging; old subaxial cervical dislocation; palliative therapy; patient satisfaction; pediatric intensive care unit; physical examination; preschool child; priority journal; rib fracture; spinal cord decompression; spinal cord transsection; spine injury; spleen rupture; splenectomy; tracheotomy; traffic accident

Adult-onset neurocutaneous melanosis with Dandy-Walker malformation - Chang T.-W.; Hsieh P.-C.; Liu Z.-H.; Tu P.-H. (Formosan Journal of Surgery, 2018)
Neurocutaneous melanosis (NCM) is characterized by diffuse or focal proliferation of melanin-producing cells over the skin or leptomeninges. Even without malignant transformation, the prognosis is poor after symptomatic progression of NCM, resulting from either mass effect in central nervous system or hydrocephalus. We reported a 26-year-old female patient with rapid deterioration after the onset of NCM. Despite no solid tumor formation, elevated cerebrospinal fluid protein content, which complicated shunting procedure, associated with hydrocephalus played the key role in clinical deterioration. Adult onset of NCM with Dandy-Walker malformation was never reported in our literature review. Diagnosis and treatment for such disease entity are discussed.
Keywords: cerebrospinal fluid drainage system; mechanical ventilator; glycerol; melanin; protein; adult; adult onset neurocutaneous melanosis; article; behavior disorder; bizarre behavior; brain angiography; brain cyst; brain fourth ventricle; brain hemorrhage; brain tissue; brain tomography; brain ventricle peritoneum shunt; case report; cerebellum hypoplasia; cerebrospinal fluid analysis; clinical article; computer assisted tomography; consciousness; contrast enhancement; craniotomy; Dandy Walker syndrome; dermis; dizziness; drowsiness; emergent cystoperitoneal shunt; facial nerve paralysis; female; headache; hospital admission; hospital discharge; human; hydrocephalus; incoherent speech; limb; lumbar puncture; medical history; melanoma; melanosis; nausea and vomiting; neuroimaging; neurologic examination; nevus; nuclear magnetic resonance imaging; perivascular space; phakomatosis; physical examination; pigmented nevus; posterior fossa; postoperative period; priority journal; protein cerebrospinal fluid level; radiodiagnosis; respiratory function; scar; septic shock; shunt failure; skin biopsy; speech disorder; spinal cord compression; spine radiography; spine tumor; syringomyelia; tonic clonic seizure; tracheostomy; trunk; unsteady gait

Persistent Extreme Hyperextension of the Fetal Neck: Clinical and Neuroimaging Findings - Shrot S.; Johnson C.T.; Golden W.C.; Baschat A.A.; Bullard J.E.; Tekes A.; Poretti A.; Dunn E.; Huisman T.A.G.M. (Journal of Neuroimaging, 2018)
BACKGROUND AND PURPOSE: Persistent hyperextension of the fetal craniocervical junction or neck is one of several fetal positions commonly observed on prenatal imaging. Underlying fetal structural etiologies such as fetal neck masses and iniencephaly can be detected as causes of hyperextension. Caesarean delivery is considered in cases of vaginal delivery or obstructed labor for fear of cervical spinal cord injury. In this case series, we describe the prenatal magnetic resonance imaging (MRI) findings and their potential role in obstetric management and discuss postnatal outcomes in fetuses demonstrating prenatal imaging findings of persistent extreme hyperextension of the neck. METHODS: A retrospective cohort of fetuses with extreme fetal neck hyperextension on prenatal ultrasound and subsequent MRI is described. RESULTS: Persistent extreme neck hyperextension was demonstrated in four pregnancies. One patient showed resolution of head positioning prior to labor. In the other 3 patients, neck hyperextension persisted until labor. In these three pregnancies, caesarean section with early intubation was performed due to concern for airway compromise and cervical cord injury. An underlying structural neck abnormality with airway compromise was noted only in 1 patient (large venolymphatic malformation). No airway compromise, cervical spine, or spinal cord anomaly was identified in the remaining 3 patients. Noncervical fetal anomalies were detected only in 1 patient (arthogryposis multiplex and clubfoot deformities). In patients with no structural neck abnormality, early extubation was performed shortly following labor. CONCLUSIONS: Detailed, high-resolution fetal MRI may serve as a valuable secondary imaging modality for clinical decision making regarding management of pregnancy, in utero therapy, mode of delivery, and postnatal care.
Keywords: nuclear magnetic resonance scanner; article; breech presentation; case report; cervical spinal cord injury; cesarean section; clinical article; clubfoot; cohort analysis; disease severity; female; fetus; fetus echography; fetus malformation; human; hydramnios; intrapartum care; intrauterine growth retardation; intubation; lymphatic malformation; muscle hypotonia; neuroimaging; nuclear magnetic resonance imaging; retrospective study; sleep disordered breathing; tracheostomy; vaginal delivery; whiplash injury

Clinical outcomes and rates of aortic growth and reoperation after 1-stage repair of extensive chronic thoracic aortic dissection - Kouchoukos N.T.; Kulik A.; Castner C.F. (Journal of Thoracic and Cardiovascular Surgery, 2018)
Objective: The study objective was to analyze clinical outcomes, distal segmental aortic growth, and aortic reoperation rates after 1-stage open repair of extensive chronic thoracic aortic dissection via bilateral anterior thoracotomy. Methods: Eighty patients underwent extensive 1-stage repair of chronic aortic dissection that included the ascending aorta, the entire aortic arch, and the varying lengths of the descending thoracic aorta. One half or more of the descending thoracic aorta was replaced in 62 (78%) of the 80 patients. Hospital mortality was 2.5% (2 patients). Stroke occurred in 1 patient (1.2%), spinal cord ischemic injury occurred in 1 patient (1.2%), and renal failure requiring long-term dialysis occurred in 2 patients (2.5%). Sixty-five of the 78 hospital survivors (83%) had serial imaging studies suitable for calculation of growth rates of the remaining dissected thoracic and abdominal aorta. Forty-seven patients were followed for more than 5 years, and 21 patients were followed for more than 10 years. Results: The mean annual growth rate for the distal contiguous aorta was 1.7 mm/y. Forty aortas increased in diameter, 16 aortas remained unchanged, and 9 aortas decreased in diameter. Five patients required reoperation on the contiguous thoracic or abdominal aorta 8, 27, 34, 51, and 174 months postoperatively for progressive enlargement. Actuarial freedom from reoperation on the contiguous aorta at 5 and 10 years was 95.4% and 93%, respectively. Actuarial freedom from any aortic reoperation at 5 and 10 years was 89.2% and 84.4%, respectively. Actuarial survival for the entire cohort at 5 and 10 years was 76.4% and 52.6%, respectively, and survival free of any aortic operation was 68.6% and 43.9%, respectively. No patient whose cause of death was known died of aortic rupture. Conclusions: Our extended experience with the 1-stage open procedure confirms its safety and durability for treatment of chronic aortic dissection with enlargement confined to the thoracic aorta. The procedure is associated with low operative risk and a low incidence of reoperation on the contiguous aorta. It represents a suitable alternative to the 2-stage, frozen elephant trunk, and hybrid procedures that are also used to treat this condition.
Keywords: abdominal aorta; adult; aortic arch; aortic dissection; aortic growth; aortic regurgitation; article; ascending aorta; brain perfusion; cardiovascular parameters; cerebrovascular accident; chronic disease; clinical outcome; computer assisted tomography; coronary artery bypass graft; disease severity; Ehlers Danlos syndrome; false aneurysm; female; follow up; heart failure; hospital mortality; human; iliac artery aneurysm; infrarenal abdominal aneurysm; kidney failure; Loeys Dietz syndrome; long term care; magnetic resonance angiography; major clinical study; male; nerve injury; neurologic disease; open surgery; paraplegia; postoperative complication; priority journal; recurrent laryngeal nerve; reoperation; surgical technique; thoracic aorta; thoracic aortic dissection; tracheostomy; wound infection

Surgery for extensive, chronic aortic dissection: What about the elephant (trunks) in the room? - Girardi L.N. (Journal of Thoracic and Cardiovascular Surgery, 2018)
Keywords: aortic dissection; blood transfusion; cerebrovascular accident; deep hypothermic circulatory arrest; editorial; frozen elephant trunk technique; hemostasis; human; length of stay; morbidity; priority journal; spinal cord injury; sternotomy; surgical technique; thoracic aortic surgery; thoracotomy; tracheostomy

Variety is the spice of life: One-stage or two-stage repair of extensive chronic thoracic aortic dissection - Preventza O.; Orozco-Sevilla V.; Pollock G.; Coselli J.S. (Journal of Thoracic and Cardiovascular Surgery, 2018)
Keywords: mechanical ventilator; aortic dissection; aortic reconstruction; aortic rupture; cerebrovascular accident; chronic obstructive lung disease; connective tissue disease; editorial; hospital mortality; human; incision; intermethod comparison; lung function; one stage repair; priority journal; reoperation; spinal cord injury; sternotomy; surgical technique; thoracic aortic surgery; thoracoabdominal aorta aneurysm; thoracotomy; tracheostomy; treatment outcome; two stage repair

Predictors of failure of decannulation of tracheostomy: A retrospective and observational data analysis from a tertiary care teaching hospital - Gupta A.; Garg A.; Dutt C. (Neurorehabilitation and Neural Repair, 2018)
Objectives: Airway protection is imperative in neurologically unstable patients and one of the most common surgical procedure performed in the intensive care units to provide mechanical ventilation and toileting of pulmonary secretions is Tracheostomy.1 “Decannulation” is defined as the process of removing the tracheostomy tube that facilitates the path of weaning from mechanical ventilation and results in spontaneous breathing. This vital transition is a simple and rapid process in most of the cases. However, this apparently simple step needs a coordinated effort of brain, swallowing, coughing and vocal cord muscles. There have been reported complications including death2 due to lack of evaluation and closure of stoma post tracheostomy tube removal. Despite the importance of such a bedside procedure, it is still unknown in most of the centers of the world when a tracheostomy tube can be safely removed. In this study, we aim to analyze the factors which affect failure of decannulation in neurology and neurosurgery patients, thus helping us create a scoring system for a more objectified evaluation and preventing any complication(s) related to the procedure. Methods: We retrospectively reviewed all the inpatients with neurological and neurosurgical conditions who failed in the process of decannulation during the period of August 2016 to July 2017. We extracted data from our electronic health records including patient characteristics, level of consciousness of patients, indications of tracheostomy, associated co-morbidities and success or failure of swallowing assessment pre-decannulation. Results: The 26 patients (n) who failed decannulation procedure in the given period were majority from Neurology conditions (n=21). There were 22 males (84%) and 4 females (16%), with a mean age of 62 Years and a mean weight of 71 kg who failed decannulation. The most common indication of tracheostomy in these patients was cerebro-vascular accident (n=16, 61%), brain malignancy (n=3, 11%), traumatic brain injury (n=3, 11%), motor neuron disease (n=2, 7%) and spinal cord injury (n=2, 7%). Amongst the patients with stroke, there were 9 patients with infarct (n=34%) and 2 with hemorrhage (7%).There was a significant 2 relation between the level of consciousness and the result of removal of tracheostomy tube as low GCS patients (<8/15) were 12 in number (46%). It was also observed that 23 patients (88.4%) failed in the swallowing assessment which was performed pre-decannulation procedure and most of these required some swallowing intervention to pass the test (n=20). There was a higher chance of decannulation failure in stroke patients (56%) with Right MCA infarct which can be explained by the control of swallow and speech centers by this cerebral territory. Difficult intubation (n=13, 50%) was not associated with decannulation failure. Presence of sepsis (34%) witnessed a higher rate of decannulation failure. Amongst other associated comorbidities were Hypertension (n=23, 88%), Diabetes (n=11, 42%), Seizure disorder (n=4, 15%) Smoking (n=10, 38%) and Bronchial Asthma (n=5, 19%) which accounted for higher chances of decannulation failure. Conclusions: Despite the importance and relevance of the procedure of decannulation, there are subjective and variable algorithms3, multiple aberrations in the complex interplay of systems, dearth of literature and ambiguity in screening and evaluation of patients which limit our understanding in this unexplored area of neurosciences and intensive care. To better understand the practice of decannulation, we propose developing of a scoring system which can assess the patient objectively and prevent aspiration and failure of this process.
Keywords: adult; aspiration; asthma; bleeding; brain cancer; cancer patient; cerebral artery disease; clinical article; clinical evaluation; comorbidity; complication; conference abstract; consciousness; controlled study; data analysis; death; diabetes mellitus; electronic health record; epilepsy; female; human; hypertension; intensive care; intubation; male; middle aged; motor neuron disease; neurology; neurosurgery; prevention; retrospective study; scoring system; sepsis; smoking; speech; spinal cord injury; stoma; stroke patient; swallowing; teaching hospital; tertiary health care; tracheostomy tube; traumatic brain injury; treatment failure

The natural history of dysphagia in ALS patients with bulbar and spinal onset - Manera U.; D'Ovidio F.; Calvo A.; Moglia C.; Cammarosano S.; Ilardi A.; Canosa A.; Bersano E.; Mazzini L.; Chiò A. (Neurology, 2018)
Objective: To assess the progression rate of dysphagia (ALSFRS-R scale) in a population-based series of ALS patients. Background: Few studies investigated the interaction between dysphagia and site of onset, and none evaluated it in terms of progression rate. Design/Methods: We enrolled 871 ALS patients (580 with spinal onset and 291 with bulbar onset), resident in Piemonte and Valle d'Aosta regions, Italy, diagnosed from January 1, 2007 to December 31, 2013. Dysphagia severity was classified in three categories, based on ALSFRS-R item 3: “normal” (item score 4), “moderate” (scores 3 and 2), and “severe” (scores 1 and 0). The progression of dysphagia in ALS patients was treated as time-dependent variable, and was reassessed at each visit (Number of visits=6,845). Progression rate of dysphagia was calculated separately among patients with bulbar and spinal onset (N=642); for both categories, intervals ended with the date when dysphagia became severe (ALSFRS-R item 3 < 2); the starting date was represented by the date of symptom onset for bulbar patients (N=279) and by the date of first bulbar symptoms (considering ALSFRS-R item 1 and item 3 < 4) for spinal patients (N=363); 217 patients did not develop dysphagia during the follow-up. Results: ALS patients had moderate dysphagia after a median of 24.4 months (IQR=13.5-44.7) from date of first symptoms, which progressed to severe dysphagia after additional 17.7 months (IQR=9.5-29.8), and death or tracheostomy after additional 6.9 months (IQR=2.6-14.7). The progression rate of the disease showed a median time interval of 17.4 months (IQR=8.9-29.5) between the first bulbar symptom and severe dysphagia. These time intervals were significantly shorter in patients with spinal onset (median 11.9 months; IQR=5.5-24.1) than in patients with bulbar onset (22.9 months; IQR=16.0- 31.8) (Wilcoxon test p=0.000). Conclusions: Progression rate of dysphagia from first symptoms to severe status differs significantly between ALS patients with bulbar and spinal onset.
Keywords: adult; Aosta Valley; conference abstract; controlled study; death; dependent variable; diagnosis; disease course; dysphagia; female; follow up; history; human; major clinical study; male; rank sum test; resident; tracheostomy

Spinal muscular atrophy type I and the dual role of viruses: An interview with professor basil T. Darras, Professor of neurology (pediatrics) at harvard medical school - Mammas I.N.; Spandidos D.A. (Experimental and Therapeutic Medicine, 2018)
According to Professor Basil T. Darras, Professor of Neurology (Pediatrics) at Harvard Medical School and Director of the Spinal Muscular Atrophy (SMA) Program at Boston Children's Hospital in Boston (MA, USA), the diagnosis of SMA type I is clinical and is based on detailed general physical and neurological examinations. SMA type I remains the most common genetic disease resulting in death in infancy and is really devastating for the child, the parents, as well as the medical professionals with the privilege of caring for patients with SMA and their parents. The proposed management options include: i) no respiratory support; ii) non-invasive ventilation; and iii) tracheotomy with mechanical ventilation. Deciding, which option is the best, is indeed a very personal decision. The optimal clinical care should be extremely mindful of parents' wishes and management goals with regard to the quality of life. Since the end of 2016 in the USA, and recently in Europe, there exists the possibility of accessing a novel treatment drug for SMA, namely Nusinersen. This antisense oligonucleotide is administered intrathecally and increases the production of the fully functional SMN protein, thus improving motor function, the quality of life and survival. Among the ongoing clinical trials, oral treatment with RG7916, a small molecule SMN2 splicing modifier, appears to be really promising. Gene therapy using viral vectors is expected to offer an ‘one and done’ therapy and possibly a cure, if administered early in life, before any symptoms appear. It is really interesting that viruses, which at the moment are the cause of death of children with SMA, if genetically modified, may be used for their treatment.
Keywords: nusinersen; risdiplam; survival motor neuron protein; virus vector; article; artificial ventilation; clinical trial (topic); gene therapy; genetic screening; human; motor performance; pediatrics; quality of life; survival; tracheotomy; Werdnig Hoffmann disease; rg 7916

Understanding demographic variables of neurological patients admitted to an Indian post hospital inpatient rehabilitation center: Exploring the essential needs - Janagama V.; Sagi P.; Kandiraju S.; Addanki S. (Neurorehabilitation and Neural Repair, 2018)
Objectives: To evaluate demographic data of neurological patients admitted to an Indian post hospital inpatient rehabilitation unit. Methods: We have analyzed data of 174 patients admitted to our inpatient rehabilitation center in Hyderabad, India. The data was collected between the period of July 2015 to March 2017 (20 Months). Results: The mean age of patients was 51.96 (SD= 17.11). Out of the 174 patients 74.1% were males and 25.9% were females. 25.9% of patients were retired while 43.7% were employed and 9.2% were students. 93.1% were directly admitted from hospital where as 6.9% came from home. 54.6% had stroke, 27.6% had Traumatic Brain Injury, 8.6% had Spinal cord injury, 4.6% had Polyneuropathies, 4.5% were others. Out of the 174 patients, 70.1% of them had associated medical comorbid conditions like hypertension (44.3%), type 2 diabetes Mellitus (40.8%), coronary artery disease (2.3%), hyperlipidemia (1.1%), atrial fibrillation (1.1%), overweight (1.7%), alcohol (10.3%), Tobacco (8%) and others (12.1%). On admission, more than half (54%) of patients needed artificial feeding, more than onefourth (29.9%) had tracheostomy and a significant number of patients (77%) had urinary catheter. Conclusions: With increasing incidence of neurological patients in India, there is a dire need to understand demographic variables and develop cost effective resources and protocols to improve rehabilitation outcomes. Majority of the effected individuals were still working (43.7%) or were students (9.2%) which can cause significant burden of number of disability adjusted life years. Hence it is very crucial for stake holders to develop multidisciplinary rehabilitation facilities for early, effective and empowered recovery of neurological patients.
Keywords: alcohol; adult; artificial feeding; atrial fibrillation; cerebrovascular accident; conference abstract; controlled study; coronary artery disease; disability-adjusted life year; female; hospital patient; human; hyperlipidemia; hypertension; incidence; India; major clinical study; male; middle aged; non insulin dependent diabetes mellitus; obesity; polyneuropathy; rehabilitation; rehabilitation center; remission; spinal cord injury; student; tobacco; tracheostomy; traumatic brain injury; urinary catheter

Use of pediatric Tracheal Stoma Retainer® in a 24-year-old spinal muscular atrophy patient - Ito H.; Sobue K. (Pediatrics International, 2018)
Keywords: airway creation device; One-way Valve; Tracheal Stoma Retainer; tracheostomy tube; adult; artificial ventilation; case report; clinical article; gastrostomy; hospital discharge; human; influenza B; male; motor retardation; note; phonation; phonation device; priority journal; respiratory distress; respiratory failure; spinal muscular atrophy type 2; tracheostomy; ventilator weaning; verbal communication; young adult; One-way Valve; Tracheal Stoma Retainer

Angiography Before Posterior Mediastinal Tumor Resection: Selection Criteria and Patient Outcomes - Madariaga M.L.; Borges L.F.; Rabinov J.D.; Chang D.C.; Lanuti M.; Mathisen D.J.; Gaissert H.A. (Annals of Thoracic Surgery, 2018)
Background: Resection of posterior mediastinal tumors may be complicated by bleeding or neurologic injury. Preoperative spinal angiography of mediastinal tumors has been underreported or not commonly practiced. This study evaluated the selection criteria and outcomes of patients with posterior mediastinal tumors who underwent preoperative angiography and embolization. Methods: This was a single-institution retrospective study of patients with posterior mediastinal tumors from 2002 to 2016. Multilevel spinal angiography was performed, with or without selective arterial embolization of vascular supply, in patients selected by thoracic or neurologic surgeons. Results: Ten of 87 patients (11%) with posterior mediastinal tumors underwent preoperative angiography. A mean of 11 arteries (range, 2 to 25) were studied. Embolization in 7 of 10 patients successfully occluded 1 to 3 arteries. There was no significant difference in age, sex, body mass index, American Society of Anesthesiologists Physical Status Classification, operative time, operative blood loss, complications, or death between patients with or without angiography. Patients who underwent angiography had larger tumors (1,490 vs 97 cm3, p < 0.0001), involvement of the neuroforamen (44% vs 10%, p < 0.05), and a longer hospital stay (9 vs 4.5 days, p < 0.05). Angiography was complicated in 1 patient by vocal cord ulceration after intubation of a tumor-compressed trachea. Shared blood supply between the tumor and the spinal cord precluded embolization and tumor resection in 1 patient. Use of angiography increased over time. Conclusions: Selective preoperative angiography for evaluation of posterior mediastinal tumors identifies arterial variations, threatened spinal arteries, and targets for embolization. The specific role of angiography and embolization requires further investigation to standardize indications and protocols for the number of arteries examined.
Keywords: adult; age; Anesthesiologists Physical Status Classification; arterial embolization; article; body mass; clinical assessment tool; clinical evaluation; clinical outcome; controlled study; female; follow up; gender; Horner syndrome; hospitalization; human; intubation; length of stay; major clinical study; male; mediastinum tumor; middle aged; mortality; neuroforamen; operation duration; operative blood loss; pneumonia; posterior mediastinal tumor; postoperative complication; preoperative evaluation; priority journal; prolonged air leak; retrospective study; spinal angiography; spinal cord; thorax surgery; trachea compression; tracheostomy; tumor volume; urinary tract infection; vascularization; vertebra; vocal cord disorder; vocal cord ulceration; wound infection

Bridging the acute to chronic care gap - Iantomasi P.; Cagliostro M. (Canadian Journal of Respiratory Therapy, 2018)
In our current health care climate, movement of patients from an acute care setting to a chronic care facility or rehabilitation centre is becoming increasingly challenging due to the limited number of beds and a high demand for these services. The waiting time for transfer can be lengthy, and therefore there are many patients ready to be transitioned, but who remain in an acute care hospital. This is especially true for those patients with specialized needs, such as patients with a tracheostomy or those with a spinal cord injury. Since these patients require particular care and have access to only a limited number of beds, in only a few specific centres, they are often waiting extended periods of time to access a placement. Having these patients remain in acute care hospitals while awaiting transition to another facility (chronic care or rehab), means that acute care providers are faced with unique challenges in terms of their management and clinical progress. At St. Michaels Hospital (SMH), an urban city teaching trauma hospital found in the heart of downtown Toronto, this situation is a frequent reality. As such, a respiratory therapist-led quality improvement project was developed in an effort to identify and implement strategies to minimize the gaps for these patients (tracheostomy and spinal cord injury) transitioning from acute to chronic care. Through the presentation of two case studies, the following will be discussed: the acute to chronic care gaps that were identified, the process changes that were implemented, the outcomes to date, the challenges faced with these initiatives, and areas for future improvement.
Keywords: adult; climate; conference abstract; emergency care; heart; human; rehabilitation; respiratory therapist; spinal cord injury; teaching; total quality management; tracheostomy

Evolution of Simplified Frozen Elephant Trunk Repair for Acute DeBakey Type I Dissection: Midterm Outcomes - Roselli E.E.; Idrees J.J.; Bakaeen F.G.; Tong M.Z.; Soltesz E.G.; Mick S.; Johnston D.R.; Eagleton M.J.; Menon V.; Svensson L.G. (Annals of Thoracic Surgery, 2018)
Background: A modified technique for frozen elephant trunk (FET) repair of acute DeBakey type I dissection has evolved. Procedural modifications are described and midterm outcomes evaluated. Methods: From 2009 to 2016, 72 patients with DeBakey type I dissection underwent emergency simplified FET. Mean age was 59 ± 15 years. Presentation included malperfusion (n = 22, 31%), rupture (n = 12, 16%), and aortic insufficiency (n = 42, 58%). Concomitant procedures included valve replacement (n = 9), root replacement (n = 11; valve sparing n = 6), cusp repair (n = 11), and valve resuspension (n = 21). The first 39 were treated by modifying an early generation stent graft. The next 16 received newer modified stent grafts, and the latest 17 underwent branched single anastomosis technique with left subclavian stent grafting. Results: Operative mortality was 4.2% (n = 3 of 72). Two presented comatose without recovering, the other died from coagulopathy complications. Morbidity included stroke (n = 3, 4.2%), spinal injury (n = 3, 4.2%; 1 permanent), tracheostomy (n = 7, 9.7%), and renal failure (n = 2, 2.8%). Median follow-up was 28 ± 25 months. Survival was 92% at 6 months, 92% at 1 year, 89% at 3 years, and 80% at 5 years. Among 69 survivors, follow-up imaging was available in 63 (91%). Of these, 58 (92%) patients thrombosed the treated false lumen, with shrinkage in 37(54%) patients from 42 ± 8 mm to 37 ± 7 mm. Ten patients underwent 14 late reinterventions for growth and incomplete thrombosis (7 endo extension, 4 left subclavian embolization, 1 bypass, 2 false lumen embolization). Freedom from reintervention was 93% at 6 months, 87% at 1 year, 77% at 3 years, and 72% at 5 years. Conclusions: Simplified FET for treating acute DeBakey type I dissection has evolved and remained safe. It promotes aortic remodeling, and simplifies management of chronic aortic complications.
Keywords: aortic aneurysm endovascular graft; artery prosthesis; polypropylene suture; subclavian artery stent; acute debakey type i dissection; adult; aortic dissection; aortic regurgitation; aortic root surgery; aortic rupture; aortic surgery; aortic valve repair; aortic valve replacement; aortic valve resuspension; arterial embolization; bleeding; blood clotting disorder; brain disease; brain hypoxia; brain injury; carotid artery injury; cerebrovascular accident; chest bleeding; clinical outcome; comatose patient; conference paper; convalescence; coronary artery bypass graft; emergency surgery; endovascular aneurysm repair; female; follow up; hematoma; hemorrhagic shock; human; kidney failure; major clinical study; male; middle aged; morbidity; paralysis; paraplegia; priority journal; reoperation; retreatment; simplified frozen elephant trunk repair; spinal cord injury; surgical mortality; survival analysis; survival rate; survival time; survivor; thorax disease; tracheostomy; vertebral artery stenosis; visual impairment; C-TAG; Viabahn

An unusual cause of recurrent laryngospasm: A case report - Cattermole G.N.; Ndebwanimana V.; Polzin-Rosenberg N. (African Journal of Emergency Medicine, 2018)
Introduction: Laryngospasm is a partial or complete closure of the vocal cords, causing stridor and then complete airway obstruction. We present an unusual case of recurrent laryngospasm following cervical spine trauma. Case report: A 41-year-old pedestrian was hit by a car sustaining several spine fractures including a comminuted fracture of C1. These were initially unrecognised, and his cervical spine was not immobilised. During this time the patient experienced three episodes of laryngospasm requiring intubation. On day 11 his fractures were identified, and a Philadelphia collar was placed. He made a full recovery without any neurological sequelae. Discussion: Laryngospasm is a recognised complication of anaesthesia and intubation. This case illustrates that this life-threatening complication can also follow cervical fractures, and reinforces the need for prompt and careful review of imaging to identify such fractures in trauma patients, especially those with stridor.
Keywords: cervical collar; bronchodilating agent; adult; article; bronchospasm; case report; cervical spine fracture; clavicle fracture; clinical article; clinical handover; comminuted fracture; computer assisted tomography; conservative treatment; convalescence; diagnostic error; echography; endotracheal intubation; extended focused assessment with sonography in trauma; extubation; Glasgow coma scale; hematothorax; hospital admission; hospital discharge; human; larynx spasm; lung function; male; manubrium; multiple fracture; oxygen desaturation; oxygen saturation; pain assessment; patient transport; pelvis radiography; rib fracture; shoulder fracture; spine fracture; spinous process; stridor; tachycardia; tachypnea; thoracic spine; thorax radiography; tracheostomy; transverse process; vertebral canal

The impact of a specialized spinal cord injury center as compared with non-specialized centers on the acute respiratory management of patients with complete tetraplegia: An observational study - Richard-Denis A.; Feldman D.; Thompson C.; Albert M.; Mac-Thiong J.-M. (Spinal Cord, 2018)
Study Design: Retrospective cohort study. Objectives: To compare the proportion of tracheostomy placement and duration of mechanical ventilation (MV) in patients with a complete cervical spinal cord injury (SCI) that were managed early or lately in a specialized acute SCI-center. The second objective was to determine the impact of the timing of admission to the SCI-center on the MV support duration. Setting: A single Level-1 trauma center specialized in SCI care in Quebec (Canada). Methods: A cohort of 81 individuals with complete tetraplegia over a 6-years period was included. Group 1 (N = 57- early group-) was admitted before surgical management in one specialized acute SCI-center, whereas Group 2 (N = 24 -late group-) was surgically managed in a non-specialized center and transferred to the SCI-center for post-operative management only. The proportion of tracheostomy placement and MV duration were compared. Multivariate regression analysis was used to assess the impact of the timing of admission to the SCI-center on the MV duration during the SCI-center stay. Results: Patients in Group 2 had a higher proportion of tracheostomy (70.8 vs. 35.1%, p = 0.004) and a higher mean duration of MV support (68.0 ± 64.2 days vs. 21.8 ± 29.7 days, p = 0.006) despite similar age, trauma severity (ISS), neurological level of injury and proportion of pneumonia. Later transfer to the specialized acute SCI-center was the main predictive factor of longer MV duration, with a strong impact factor (s = 946.7, p < 0.001). Conclusions: Early admission to a specialized acute SCI-center for surgical and peri-operative management after a complete tetraplegia is associated with lower occurrence of tracheostomy and shorter mechanical ventilation duration support. Sponsorship: MENTOR Program of the Canadian Institute of Health Research and US Department of Defense Spinal Cord Injury Research Program.
Keywords: adult; article; artificial ventilation; cohort analysis; complete cervical spinal cord injury; complete tetraplegia; disease severity; emergency health service; female; health center; hospital admission; human; major clinical study; male; nonspecialized center; pneumonia; priority journal; quadriplegia; Quebec; retrospective study; specialized spinal cord injury center; spinal cord injury; spine surgery; tracheostomy

Fusion, Failure, Fatality: Long-term Outcomes After Surgical Versus Nonoperative Management of Type II Odontoid Fracture in Octogenarians - Perry A.; Graffeo C.S.; Carlstrom L.P.; Chang W.; Mallory G.W.; Puffer R.C.; Clarke M.J. (World Neurosurgery, 2018)
Background Type II odontoid fracture is a highly morbid injury among octogenarians, with 41% 1-year mortality. Our objective was to assess long-term fusion, complication, and survival rates. Methods Retrospective review of prospective trauma registry and blinded review of follow-up radiographs. Results Follow-up cohort included 94 nonoperative and 17 operative patients (median, 52 and 79 months). The operative group had significantly higher rates of repeated surgery for primary treatment failure or complication (1% vs. 18%; P = 0.01) and dysphagia, aspiration events, or tracheostomy (29% vs. 78%, P = 0.002; 6% vs. 30%, P = 0.04; 1% vs. 18%, P = 0.01). Three-year all-cause mortalities were 71% and 76%, respectively (P = 0.78). No delayed myelopathy was observed. One-year postinjury radiographs were available for 13 and 6 patients in the nonoperative and operative groups (P = 0.9); bony union was observed in 3 and 5 patients (23% vs. 83%; P = 0.04). Retrolisthesis greater than 2 mm was observed in 2 and 1 patients (15% vs. 17%; P = 1.0). Two patients in the operative group underwent repeated surgery for primary treatment failure. Dysphagia was diagnosed in 3 and 5 operative patients (23% vs. 83%; P = 0.04), whereas aspiration events occurred in 0 and 3 patients (0% vs. 50%; P = 0.02). Three-year mortalities in this cohort were 38% and 67% (P = 0.35). Conclusions Radiographic union is significantly associated with operative management, but the corresponding clinical benefit is unclear. Complications were significantly more common after surgery. Long-term survival in octogenarians following type II odontoid fracture is poor, independent of management. Frequent complications without a proven survival benefit suggest that most patients are better managed conservatively.
Keywords: aged; all cause mortality; arthrodesis; article; aspiration; cervical spine radiography; clinical outcome; cohort analysis; comparative effectiveness; conservative treatment; controlled study; dysphagia; female; follow up; fracture healing; groups by age; human; long term survival; major clinical study; male; odontoid process fracture; postoperative complication; register; reoperation; retrolisthesis; retrospective study; spine fusion; spondylolisthesis; survival rate; tracheostomy; treatment failure; very elderly

Novel approach using transoral robotic surgery for resection of cervical spine chordoma - Mahmoud A.F.; McCann A.; Malhotra N.R.; O'Malley B.W. (Journal of Neurological Surgery, Part B: Skull Base, 2018)
Background: Chordomas are rare infiltrative neoplasms arising from remnants of the notochord and are predominantly located in the sacrum or clivus, whereas only 6% are located in the cervical spine. Surgical resection is the mainstay of treatment; however, tumor location and access often prove challenging, especially when attempting gross total resection. Due to limited visualization, a traditional approach often requires mandibulotomy, palatotomy, or glossotomy which introduce significant morbidity to the patient. Here, we present the first case in which transoral robotic surgery (TORS) was utilized for the resection of a midline C2 chordoma. Case Report: The patient is a 27-year-old man with incidentally discovered chordoma located in the midline C2 spine. The mass was noted to be small and did not involve critical surrounding structures on imaging; therefore, our goal was to obtain cure through en bloc resection with negative margins. Anterior approach preceded by posterior laminectomy was performed via TORS technique with assistance from image guidance technology to obtain exposure through the mouth. After gross total resection, reconstruction was performed with anterior and posterior spinal fusion with hardware and iliac spine bone graft. Temporary tracheostomy was performed and feeding tube placed. Closure achieved with local tissue myofascial advancement. Conclusion: Tumors of the axial spine are associated with perceived higher operative difficulty due to the complex anatomy of the upper cervical spine and restricted access. Amount of tumor resection is one of the most important prognostic factors affecting patient outcomes. TORS has been utilized extensively for access to the oropharynx due to superior visualization and maneuverability, primarily for resecting malignancy but also for benign disease. En bloc resection of a chordoma is limited by invasion of surrounding critical structures and by issues with access. By employing TORS approach, we overcame limitations due to access without introducing morbidity from traditional approaches. In this case, we avoided the need for mandibulotomy, palatotomy, and glossotomy. We advocate for the use of TORS for approach to the midline cervical spine and encourage providers to consider utilizing this technique.
Keywords: adult; anatomy; bone graft; cancer prognosis; cancer surgery; case report; cervical spine; chordoma; clinical article; computer; conference abstract; feeding tube; human; iliac bone; laminectomy; male; morbidity; mouth; oropharynx; robot assisted surgery; spine fusion; tracheostomy

Peripheral nerve pathology at fixed stage in spinal muscular atrophy with respiratory distress type 1 - Ikeda A.; Yamashita S.; Tsuyusaki Y.; Tanaka M.; Tanaka Y.; Hashiguchi A.; Takashima H.; Goto T. (Brain and Development, 2018)
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is characterized by severe respiratory failure due to diaphragmatic paralysis and distal muscular weakness in early infancy. After an initial decline in respiratory state and motor function until 1–2 years of age, residual capabilities reach a plateau. We report the peripheral neuropathological findings of a patient with SMARD1 at 1 year and 1 month of age, when his muscle strength and respiratory symptoms had deteriorated and then stabilized for several months. Peripheral nerve biopsy revealed severely progressed axonal degeneration. This finding suggests the rapid progression of peripheral axonal neuropathy in SMARD1 that leads to its characteristic clinical course of respiratory failure and paralysis in the early infantile period.
Keywords: collagen; myelin; acute respiratory failure; areflexia; article; artificial ventilation; brain size; case report; clinical article; diaphragm paralysis; disease course; electromyography; facial expression; foot malformation; gene; gene mutation; genetic screening; heterozygote; human; hyperhidrosis; IGHMBP2 gene; male; muscle action potential; muscle cell; muscle hypotonia; muscle strength; myelinated nerve; nerve biopsy; nerve conduction; nerve fiber degeneration; nuclear magnetic resonance imaging; peripheral neuropathy; pes cavus; positive end expiratory pressure; respiratory distress; respiratory failure; respiratory tract disease; respiratory tract infection; Schwann cell; sensory nerve conduction velocity; spinal muscular atrophy; spinal muscular atrophy with respiratory distress type 1; subcutaneous fat; sural nerve; tachycardia; tracheostomy; white matter

Paradoxical Distraction with Upright Position After Halo Fixation in 2 Patients with Atlanto-Occipital Dislocation - Clifton W.; Feindt A.; Skarupa D.; McLauchlin L.; Tavanaiepour D.; Rahmathulla G. (World Neurosurgery, 2018)
Background Atlanto-occipital dislocation (AOD) is the most uncommon form of traumatic cervical spine injury. The majority of patients die before reaching higher-level care, and only a small percentage of patients with AOD survive the initial injury after receiving tertiary care. As such, there is a paucity of evidence-based management guidelines for treating this condition. Halo vest fixation has been a proposed method for interim stability while these patients undergo medical optimization for surgical intervention. There have been several reports of worsening AOD after halo placement. Reverse Trendelenburg position after halo fixation has been previously described to aid in the reduction of AOD, as well as concomitant atlantoaxial dislocation by gravitational downward force. Case Description In this series we present 2 cases of obese patients (body mass index >30) with AOD treated by halo fixation that had increased distraction after head of bed elevation. Conclusion Our theorized mechanism for this phenomenon is due to the downward pull of subaxial forces secondary to a large body habitus.
Keywords: spine fixation device; adult; article; atlanto occipital dislocation; atlantoaxial dislocation; avulsion injury; body mass; case report; cervical spine dislocation; clinical article; disease association; female; gastrostomy; Glasgow coma scale; halo fixation; human; hypovolemic shock; intubation; occipital cervical fusion; quadriplegia; spine fusion; spine stabilization; standing; tracheostomy; x-ray computed tomography; young adult

Total aortic arch replacement using frozen elephant trunk technique with J Graft Open Stent Graft for distal aortic arch aneurysm - Koizumi S.; Nagasawa A.; Koyama T. (General Thoracic and Cardiovascular Surgery, 2018)
Objectives: The J Graft Open Stent Graft, the first commercialized stent graft in Japan, has been available since February 2014, and we have used this new device as a frozen elephant trunk technique for distal aortic arch aneurysm. Here, we present our surgical method and result with this new device. Methods: Between October 2014 and October 2016, 30 patients (26 males, mean age 74.3 ± 9.7 years) underwent total aortic arch replacement using the frozen elephant trunk technique with the J Graft Open Stent Graft for distal aortic arch aneurysm. The follow-up period was 10 months. Results: In-hospital mortality and 30-day mortality were 0%. Stroke occurred in three cases (10%), paraplegia in one case (3.3%), respiratory complication in two cases (6.7%), and renal failure in two cases (6.7%). During the follow-up period, there was no aorta- related death, and two patients needed an aortic operation (re-hemiarch replacement for pseudoaneurysm of proximal anastomotic site; axilloaxillary bypass for occlusion of the left subclavian artery), and two patients needed catheter embolization for type II endoleak for reopening of the left subclavian artery. Conclusion: Our short-term surgical results were acceptable. This new device is useful for distal aortic arch aneurysms.
Keywords: j graft open stent graft; stent graft; adult; aged; aortic aneurysm; aortic arch surgery; artery bypass; artery occlusion; article; artificial embolization; brain infarction; cerebrovascular accident; clinical article; delirium; distal aortic arch aneurysm; embolism; endoleak; false aneurysm; female; frozen elephant trunk technique; hemiplegia; hospital mortality; human; kidney failure; left subclavian artery; lung complication; male; operative blood loss; paraplegia; postoperative complication; prosthetic valve endocarditis; reoperation; respiratory tract disease; seizure; spinal cord injury; surgical technique; thoracotomy; tracheotomy; very elderly

Pre-emptive awake airway management under dexmedetomidine sedation in a parturient with spinal muscular atrophy type-2 - Godlewski C.A.; Castellanos P.F. (International Journal of Obstetric Anesthesia, 2018)
Historically, pregnancy in females with spinal muscular atrophy was contraindicated due to the great risk to the parturient, but with improved management and increased survival more patients are becoming pregnant. We describe the management of a pregnant patient with spinal muscular atrophy type-2, who had severe restrictive lung disease, extensive spinal fusion that precluded neuraxial anesthesia, and chronic respiratory failure on nocturnal Bilevel Positive Airway Pressure. Airway management was further complicated by limited mouth opening and cervical spine ankylosis.
Keywords: anesthesia mask; endotracheal tube; nasal cannula; nebulizer; operating room; operating table; oxygen nasal cannula; pediatric face mask; wheelchair; bicarbonate; dexmedetomidine; epinephrine; fentanyl; glucose; lidocaine; midazolam; nitrous oxide; phenylephrine; sevoflurane; adult; African American; anion gap; Apgar score; article; bicarbonate blood level; bradycardia; case report; cesarean section; clinical article; conscious sedation; emergency surgery; end tidal carbon dioxide tension; endotracheal intubation; extubation; female; fetus heart rate; fetus lung; gestational age; hip dislocation; human; hypoglycemia; ketonuria; neonatal intensive care unit; newborn hypoxia; oxygen saturation; pediatric anesthesia; positive end expiratory pressure; posterior spine fusion; prematurity; rehydration; respiration control; respiratory distress syndrome; resuscitation; shock; spinal muscular atrophy type 2; surgical intensive care unit; thoracolumbar spine; tracheostomy; trismus; urinary tract infection

Refractory orthostatic hypotension in a patient with a spinal cord injury: Treatment with droxidopa - Canosa-Hermida E.; Mondelo-García C.; Ferreiro-Velasco M.E.; Salvador-de la Barrera S.; Montoto-Marqués A.; Rodríguez-Sotillo A.; Vizoso-Hermida J.R. (Journal of Spinal Cord Medicine, 2018)
Context: Orthostatic hypotension (OH) is a common complication in patients with a spinal cord injury, mainly affecting complete injuries above neurological level T6. It is generally more severe during the acute phase but can remain symptomatic for several years. Findings: A 65-year-old male with a grade ASIA A post-traumatic cervical spinal cord injury, at neurological level C4, presenting with symptomatic refractory OH. Increased blood pressure (BP) levels and an overall clinical improvement was observed after administering an increasing dose of droxidopa. Treatment was started at a dose of 100 mg twice daily (bid), one to be taken upon rising in the morning and another one in the afternoon, at least three hours before bedtime. According to the patient's symptomatic response, each individual dose was increased by 100 mg at 48-hour intervals. Both increased mean BP levels and a subjective symptomatic improvement were evidenced at a dose of 300 mg bid. Clinical relevance: Treatment with droxidopa increases BP levels and improves symptoms related to refractory OH using all physical and pharmacological measures available. It could therefore constitute an effective alternative treatment for OH in patients with a spinal cord injury.
Keywords: abdominal binder; bandages and dressings; compression stocking; droxidopa; etilefrine; fludrocortisone; aged; American Spinal Injury Association impairment scale; arthrodesis; article; artificial ventilation; case report; cervical spinal cord injury; clinical article; cognition; decompression surgery; dizziness; drug dose increase; drug withdrawal; elevated blood pressure; evening dosage; faintness; fatigue; head injury; human; intervertebral disk hernia; lethargy; male; morning dosage; multiple trauma; orthostatic hypotension; osteosynthesis; quadriplegia; sitting; spine fracture; supine position; tracheostomy; traffic accident

A Meta-Analysis of the Influencing Factors for Tracheostomy after Cervical Spinal Cord Injury - Wang Y.; Guo Z.; Fan D.; Lu H.; Xie D.; Zhang D.; Jiang Y.; Li P.; Teng H. (BioMed Research International, 2018)
Background. Traumatic cervical spinal cord injury (CSCI) is a common disease that has high complication, disability, and mortality rates and a poor prognosis. Tracheostomy is an important supportive therapy for patients with CSCI. However, a consensus on the predictive factors for tracheostomy after CSCI has not been reached. Objective. This meta-analysis study assessed the influencing factors for tracheostomy after CSCI. Methods. We searched for relevant studies on the influencing factors for tracheostomy after CSCI. The extracted data were analyzed using RevMan 5.3 software. We calculated the odds ratio (OR) or mean difference (MD) and 95% confidence intervals (CIs). Results. Sixteen eligible studies containing 9697 patients with CSCI were selected. The pooled OR (MD) and 95% CI of the influencing factors were as follows: age (mean ± SD): -0.98 (-4.00 to 2.03), advanced age: 1.93 (0.80 to 4.63), sex (male): 1.29 (1.12 to 1.49), American Spinal Injury Association Impairment Scale (AIS) A grade: 7.79 (5.28 to 11.50), AIS B grade: 1.15 (1.13 to 2.02), AIS C grade: 0.28 (0.20 to 0.41), AIS D grade: 0.04 (0.02 to 0.09), neurological level of injury (upper CSCI): 2.36 (1.51 to 3.68), injury severity score (ISS): 8.97 (8.11 to 9.82), Glasgow Coma Scale (GCS) score ≤8: 6.03 (2.19 to 16.61), thoracic injury: 1.78 (1.55 to 2.04), brain injury: 0.96 (0.55 to 1.69), respiratory complications: 5.97 (4.03 to 8.86), smoking history: 1.45 (0.99 to 2.13), traffic accident injury: 1.27 (0.92 to 1.74), and fall injury: 0.72 (0.52 to 1.01). Conclusions. The current evidence shows that male sex, AIS A grade, AIS B grade, neurological level of injury (upper CSCI), high ISS, GCS≤8, thoracic injury, and respiratory complications are risk factors for tracheostomy after CSCI, and AIS C grade and AIS D grade are protective factors. This study will allow us to use these factors for tracheostomy decisions and ultimately optimize airway management in patients with CSCI.
Keywords: American Spinal Injury Association impairment scale; article; brain injury; cervical spinal cord injury; Glasgow coma scale; human; injury scale; meta analysis; respiratory complication; respiratory tract disease; risk factor; sex difference; smoking; systematic review; thorax injury; tracheostomy

Fibrocartilaginous emboli: An uncommon cause of spinal cord infarction - Chay W. (Journal of Spinal Cord Medicine, 2018)
Objective: To describe a case of spinal cord infarction in an otherwise healthy individual that was suspected to be from fibrocartilaginous embolism. Background: Fibrocartilaginous embolism (FCE) is a rare cause of spinal cord infarction in humans. In FCE, the spinal cord infarction is postulated to result from migration of fibrocartilaginous nucleus pulposus material through the nearby vasculature into one of the spinal cord vessels. This was first described in 1961 and since, several cases have been reported to cause infarction of the spinal cord, lung, brain, vertebrae, and ribs. Participants/methods: A 20 year-old left-hand dominant male was playing volleyball with friends when he reportedly experienced sharp pain on the right side of the neck after turning his head. He subsequently developed right arm weakness/numbness. He was taken to local ED by his friends and upon arrival, he could no longer move his arms or legs. In the ED, he started having difficulty breathing and was intubated. Initial imaging studies including CTA brain and neck were negative. Subsequent MRI of brain reportedly demonstrated multiple acute to subacute posterior circulation lacunar infarctions involving bilateral cerebellar hemispheres, bilateral medial thalami and parasagittals, and right parietal cortex. MRI cervical spine reportedly demonstrated cervical spinal cord hyperintensity, concerning for inflammatory vs ischemic myelopathy. Acute hospital course was significant for prolonged respiratory failure necessitating tracheostomy/PEG placement, and intermittent fevers. He was eventually medically stabilized and transferred to Shepherd Center for comprehensive rehabilitation. On admission to Shepherd Center, neurological testing was consistent with C2 AIS A SCI with sensory ZPP to L5 on the right and S1 on the left. At discharge, repeat ASIA exam was consistent with C1 AIS C SCI. Significance for SCI practice: There are a multitude of etiologies of non-traumatic spinal cord injuries. More commonly, infections, inflammatory conditions, demyelinating diseases, compressive/mass lesions, congenital diseases, and vascular disorders. More rare causes of spinal cord injury/infarction (such as FCE) should be considered if one of the more common etiologies is not identified. Conclusion: In an otherwise healthy individual who experiences a non-traumatic spinal cord injury, one should consider FCE as a potential cause.
Keywords: adult; arm weakness; brain infarction; case report; cell migration; cerebellum; cervical spinal cord; cervical spine; clinical article; conference abstract; demyelinating disease; dyspnea; embolism; fever; friend; hand; head; human; hypesthesia; infection; inflammation; lacunar stroke; leg; lung infarction; male; nuclear magnetic resonance imaging; nucleus pulposus; pain; parietal cortex; rehabilitation; respiratory failure; rib; spinal cord infarction; spinal cord injury; spinal cord ischemia; thalamus; tracheostomy; vascularization; vertebra; volleyball; young adult

Risk factors for laryngeal penetration-aspiration in patients with acute traumatic cervical spinal cord injury - Ihalainen T.; Rinta-Kiikka I.; Luoto T.M.; Thesleff T.; Helminen M.; Korpijaakko-Huuhka A.-M.; Ronkainen A. (Spine Journal, 2018)
Background Context Laryngeal penetration-aspiration, the entry of material into the airways, is considered the most severe subtype of dysphagia and is common among patients with acute cervical spinal cord injury (SCI). Purpose The aim of this study was to investigate risk factors for penetration-aspiration in patients with acute traumatic cervical spinal cord injury (TCSCI). Study Design This is a prospective cohort study. Patient Sample Thirty-seven patients with TCSCI were included in the study. Outcome Measures The highest Rosenbek penetration-aspiration scale (PAS; range 1–8) score of each patient was the primary outcome measure. The risk factors consisted of patient characteristics, demographics, and clinical signs observed during a clinical swallowing trial. Materials and Methods A clinical swallowing trial and videofluoroscopic swallowing study (VFSS) was performed on all patients within 28 days post injury. For group comparisons, the patients were divided into two groups: (1) penetrator-aspirators (PAS score ≥3) and (2) non-penetrator-aspirators (PAS score ≤2). Results Of the 37 patients, 83.8% were male. The mean age at the time of the injury was 61.2 years. Most patients had an incomplete TCSCI (78.4%) caused by a fall (75.7%). In the VFSS, 51.4% of the patients were penetrator-aspirators, and 71.4% had silent aspiration. The risk factors for predicting penetration-aspiration were (1) necessity of bronchoscopies, (2) lower level of anterior cervical operation, (3) coughing, throat clearing, choking related to swallowing, and (4) changes in voice quality related to swallowing. Binary logistic regression identified coughing, throat clearing, choking, and changes in voice quality related to swallowing as independent risk factors for penetration-aspiration. Conclusions The necessity of bronchoscopies, postinjury lower cervical spine anterior surgery, coughing, throat clearing, choking, and changes in voice quality related to swallowing was a markedrisk factor for aspiration and penetration following a cervical SCI. These factors and signs should be used to suspect injury-related pharyngeal dysfunction and to initiate preventive measures to avoid complications. The clinical swallowing evaluation is a relevant adjunct in the management of these patients and can improve the detection of penetration and aspiration.
Keywords: iohexol; adult; article; aspiration; assessment of humans; bronchoscopy; cervical spinal cord injury; cervical vertebra; clinical article; cohort analysis; controlled study; coughing; female; fluoroscopy; human; male; outcome assessment; pharynx; priority journal; prospective study; swallowing; tracheostomy; videofluoroscopic swallowing study; voice; omnipaque

Incidence, risk factors, and outcomes of acute respiratory distress syndrome among critically injured children - Killien E.; Mills B.; Watson R.S.; Vavilala M.S.; Rivara F.P. (American Journal of Respiratory and Critical Care Medicine, 2018)
Rationale: While acute respiratory distress syndrome (ARDS) is a recognized complication of severe traumatic injury, little is known about trauma-related ARDS in children. It is not clear which children are at risk for ARDS after trauma nor how ARDS affects outcomes in this population. This investigation aimed to measure the incidence, risk factors, and outcomes associated with development of ARDS among critically injured children included in the largest trauma registry worldwide. Methods: We conducted a retrospective cohort study of patients <18 years included in the National Trauma Data Bank from 2007-2014. Patients with traumatic injury (excluding burns and drowning) admitted to an intensive care unit at a Level 1 or 2 adult or pediatric trauma center were included. We identified patients for whom ARDS was recorded as a complication of their hospitalization. We determined associations between patient demographics, injury characteristics, and illness severity with development of ARDS in bivariate analyses and used a backwards selection model-building process with significance level p<0.05 to develop our final multivariable generalized linear model controlling for year and transfer status. Results: ARDS occurred in 2.01% (n=2449) of 121,891 critically injured children. Risk factors independently associated with development of ARDS are presented in the table. Patient characteristics associated with higher odds of ARDS included African American race (versus White) and comorbid bleeding disorders, congenital anomalies, stroke, and hypertension. Injury characteristics included motor vehicle crashes and firearm injuries, higher injury severity score, and presence of chest, abdominal, or spinal injuries. Intubation, tachypnea if unintubated, lower oxygen saturation, and lower Glasgow Coma Scale at hospital presentation were associated with higher odds of ARDS. Compared to children without ARDS, those with ARDS experienced longer duration of ventilation (median 5 days [IQR 2-10] versus 2 days [1-6], p<0.001), more frequent tracheostomy placement (15.5% versus 2.1%, chi2 p<0.001), and longer hospitalization (13 days [5-23] versus 4 days [2-7], p<0.001). Case fatality for ARDS was 19.9% versus 4.3% among children without ARDS (chi2 p<0.001), and 43.9% of patients with ARDS had ongoing care needs after discharge versus 15.6% of those without ARDS (chi2 p<0.001). Conclusions: We provide the first description of the incidence, risk factors, and outcomes of ARDS among pediatric trauma patients. Early recognition of ARDS is essential to implementing treatment strategies associated with lower mortality. Improved understanding of the risk factors associated with ARDS following pediatric trauma may help providers anticipate its development and improve outcomes for severely injured children. (Table Presented) .
Keywords: abdominal injury; adult; adult respiratory distress syndrome; African American; artificial ventilation; bivariate analysis; bleeding; burn; case fatality rate; cerebrovascular accident; child; childhood injury; cohort analysis; complication; conference abstract; congenital malformation; drowning; emergency health service; female; Glasgow coma scale; gunshot injury; hospitalization; human; hypertension; incidence; intensive care unit; intubation; major clinical study; male; motor vehicle; oxygen saturation; race; retrospective study; risk factor; spine injury; tachypnea; thorax injury; tracheostomy

The Burden of Pediatric Neurocritical Care in the United States - Williams C.N.; Piantino J.; McEvoy C.; Fino N.; Eriksson C.O. (Pediatric Neurology, 2018)
Background: Disorders requiring pediatric neurocritical care (PNCC) affect thousands of children annually. We aimed to quantify the burden of PNCC through generation of national estimates of disease incidence, utilization of critical care interventions (CCI), and hospital outcomes. Methods: We performed a retrospective cohort analysis of the Kids Inpatient Database over three years to evaluate pediatric traumatic brain injury, neuro-infection or inflammatory diseases, status epilepticus, stroke, hypoxic ischemic injury after cardiac arrest, and spinal cord injury. We evaluated use of CCI, death, length of stay, hospital charges, and poor functional outcome defined as receipt of tracheostomy or gastrostomy or discharge to a medical care facility. Results: At least one CCI was recorded in 67,058 (23%) children with a primary neurological diagnosis, and considered a PNCC admission. Over half of PNCC admissions had at least one chronic condition, and 23% were treated in children's hospitals. Mechanical ventilation was the most common CCI, but utilization of CCIs varied significantly by diagnosis. Among PNCC admissions, 8110 (12%) children died during hospitalization and 14,067 (21%) children had poor functional outcomes. PNCC admissions cumulatively accounted for over 1.5 million hospital days and over $4 billion in hospital costs in the study years. Most PNCC admissions, across all diagnoses, had prolonged hospitalizations (more than one week) with an average cost of $39.9 thousand per admission. Conclusions: This large, nationally representative study shows PNCC diseases are a significant public health burden with substantial risk to children's health. More research is needed to improve outcomes in these vulnerable children.
Keywords: article; artificial ventilation; cerebrovascular accident; child; child health; cohort analysis; death; diagnosis; epileptic state; female; gastrostomy; heart arrest; hospital charge; hospital cost; hospital patient; hospitalization; human; infection; inflammatory disease; length of stay; male; medical care; morbidity; neurology; public health; retrospective study; spinal cord injury; tracheostomy; traumatic brain injury; United States

A study of predictors for hyponatraemia in patients with cervical spinal cord injury - Song P.W.; Dong F.L.; Feng C.C.; Shen Y.N.; Wang Y.; Zhang R.J.; Ge P.; Shen C.L. (Spinal Cord, 2018)
Objectives: The objectives of the study were to investigate the predictors for hyponatraemia in patients with cervical spinal cord injuries (CSCIs) and to define the relationship between magnetic resonance imaging (MRI) scans and hyponatraemia. Setting: The study was carried out at The First Affiliated Hospital of Anhui Medical University. Methods: A total of 292 patients with CSCIs were retrospectively reviewed to determine the predictors of hyponatraemia. Fourteen variables were extracted from the medical records: Age, sex, blood pressure (BP), tracheostomy, serum potassium, serum chloride, serum bicarbonate, serum albumin, intravenous fluid intake and urine volume for 24 h, haematocrit, haemoglobin, neurological assessment and four MRI signal patterns. Univariate and multivariate analyses were used to determine the effect of each variable on hyponatraemia. Results: Eighty-two of the 270 patients (30%) developed hyponatraemia. Univariate analyses indicated that the following variables were significant predictors of hyponatraemia: Tracheostomy; the initial American Spinal Injury Association (ASIA) Impairment Scale (AIS) A assessment; and haemorrhage changes on T2-weighted MRI scans, and low BP. Multivariate regression analyses revealed two variables were significant predictors of hyponatraemia: Haemorrhage changes on T2-weighted MRI scans and low BP. Conclusions: Haemorrhage changes on MRI scans were closely associated with the onset of hyponatremia and could provide objective data for forecasting hyponatraemia in CSCI patients. Low BP was also a reasonable predictor of hyponatremia.
Keywords: bicarbonate; chloride; hemoglobin; potassium; serum albumin; adolescent; adult; aged; American Spinal Injury Association impairment scale; article; bicarbonate blood level; bleeding; cervical spinal cord injury; chloride blood level; female; fluid intake; hematocrit; human; hyponatremia; hypotension; major clinical study; male; neurologic examination; nuclear magnetic resonance imaging; potassium blood level; priority journal; retrospective study; risk factor; tracheostomy; urine volume

Cooling in quad fever: successful use of targeted temperature management for fever in acute traumatic cervical spinal cord injury - Addy M.J.; Kennedy C.D.; Nicholson Roberts T.C.; Boyle K. (Journal of Neurosurgical Anesthesiology, 2018)
Introduction: Half of patients with acute traumatic spinal cord injury (SCI) suffer fever.1 Autonomic dysfunction leading to aberrant thermoregulation is thought to underlie this susceptibility. The term neurogenic fever is used where no identifiable etiology other than the SCI itself exists; it is a diagnosis of exclusion. Its incidence may be 4% to 5% in traumatic SCI.1 It can be progressive and fatal, a case series of 5 patients with cervical SCI and neurogenic fever had 100% mortality within 8 days of injury.2 We present a case of suspected neurogenic fever in which the patient survived with prolonged use of targeted temperature management (TTM). Case History: A previously well 61-year-old man sustained a complete C4 SCI after falling head first over the handle bars of his road bike. He was admitted to neuro-ICU in neurogenic shock. Noninvasive ventilation was commenced on admission but within 24 hours of injury he required tracheal intubation and ventilation. On day 1 his core temperature rose to 39°C, passive cooling measures were commenced. By day 3 it reached 41.8°C, intravascular TTM to 37.5°C was commenced. TTM continued for 12 days (Fig. 1), with 3 failed attempts at cessation and a change to a surface TTM method. He underwent a tracheostomy on day 40 and was repatriated to a local ICU day 54. Five months postinjury he is decanulated and soon to be discharged home. Discussion: Although there is growing use of TTM in neurocritical care, this is predominantly for intracranial insults. Savage et al1 recent systematic review highlights the lack of evidence available to clinicians managing neurogenic fever. Indeed, challenges in this case included early recognition of life-threatening hyperthermia, distinguishing the cause of the fever and its clinical management. We found no literature to aid decision making on the threshold, duration, or method of cooling. There are now invasive and noninvasive cooling methods; we have shown that both can be efficacious in this condition. (Figure Presented).
Keywords: adult; brain; case report; case study; cervical spinal cord injury; clinical article; conference abstract; cooling; core temperature; decision making; endotracheal intubation; fever; head; human; hyperthermia; incidence; male; middle aged; mortality; neurological intensive care unit; noninvasive ventilation; tracheostomy

Increased risk of stroke in penetrating versus blunt injuries to cervical spine - Sullivan P.Z.; Blue R.; Leonard J.; Kung D.; Chen I.; Schuster J. (Journal of Neurotrauma, 2018)
Identification and treatment of vertebral artery injury in cervical spine trauma patients is necessary to prevent and optimally treat strokes of the brainstem, thalamus, posterior circulation and cerebellum. The true rate of stroke following penetrating vertebral artery injury is poorly described in the trauma and neurosurgical literature. In this series, 620 patients with traumatic cervical spine injuries were identified, and eighty-three vertebral artery injuries were found. In total, seven patients (1%) developed strokes consistent with the distribution of the vertebral artery and its branches following blunt or penetrating trauma to the cervical spine. Twenty patients were identified with penetrating injuries to the vertebral artery and five patients in this cohort demonstrated strokes that were consistent with vertebral artery occlusion (25%). Three patients with penetrating injury required embolization procedures. In contrast, among sixty-three patients with blunt cervical spine trauma and vertebral artery injury, only two patients were identified with vertebral artery distribution strokes (3%). None of the patients with blunt injury and stroke required endovascular procedures. Recovery after vertebral artery injury and stroke was variable and tracheostomy and PEG tube placement were required in all patients with penetrating trauma. Data on aspirin use for this population was variable, as co-morbid injuries lead to variable administration. There is a significantly higher risk of stroke from a penetrating versus blunt vertebral artery injury in this series (25% vs 3%, p = 0.002). Our results suggest that penetrating vertebral injuries should incite a higher level of suspicion for stroke in this difficult to assess population. Further, patients with penetrating vertebral artery injury should undergo repeat CTA at 7-10 days after injury to rule out development of pseudo-aneurysms or fistulas, as these may lead to life-threatening complications.
Keywords: acetylsalicylic acid; adult; artificial embolization; blunt trauma; cerebrovascular accident; cervical spine injury; cohort analysis; complication; conference abstract; endovascular surgery; false aneurysm; female; fistula; human; major clinical study; male; percutaneous endoscopic gastrostomy tube; remission; tracheostomy; trinucleotide repeat; vertebral artery stenosis

Use of nonsteroidal anti-inflammatory drugs to prevent heterotopic ossification after spinal cord injury: a retrospective chart review - Zakrasek E.C.; Yurkiewicz S.M.; Dirlikov B.; Pence B.T.; Crew J.D. (Spinal Cord, 2018)
Study Design: Retrospective chart review. Objectives: The objective of this study is to evaluate the efficacy of nonsteroidal anti-inflammatory drug (NSAID) prophylaxis for heterotopic ossification (HO) in the acute phase after spinal cord injury (SCI). Setting: Acute rehabilitation hospital in California, USA. Methods: This retrospective chart review (October 2013–March 2017) included individuals with motor complete SCI followed by the SCI service within 60 days of injury. Group demographics and HO diagnosis were compared in those who received and those who did not receive NSAID prophylaxis. A backward stepwise multiple regression was employed to assess the predictive association between injury characteristics and HO prophylaxis on HO diagnosis. Results: A total of 108 AIS A and B cases were included, and 27 received ≥ 15 days of therapy (overall range 6–44 days). Logistic regression analysis revealed those who received ≥ 15 days of NSAID prophylaxis had an odds ratio of 0.1 of being diagnosed with HO compared with those who did not (95% CI, 0.02 to 0.52). Significant predictors of HO diagnosis were tracheostomy (OR 2.8, 95% CI, 1.05 to 7.5), urinary tract infection (OR 4.3, 95% CI, 1.5 to 12.2), and pressure injury (OR 3.3, 95% CI, 1.1 to 9.5). Adverse effects of NSAID use were minimal. Conclusions: NSAID prophylaxis appears to help prevent HO development during the acute phase after SCI. Prospective study with prolonged follow up is necessary to confirm the long-term efficacy of HO prevention and to further evaluate safety following spinal fusion. Sponsorship: None.
Keywords: adult; adverse drug reaction; article; California; comparative effectiveness; controlled study; diagnosis; drug safety; drug therapy; female; follow up; heterotopic ossification; human; male; medical record review; multiple regression; prevention; prophylaxis; prospective study; rehabilitation; retrospective study; side effect; spinal cord injury; spine fusion; tracheostomy; urinary tract infection; nonsteroid antiinflammatory agent

Risk factors associated with mortality after traumatic cervical spinal cord injury - Higashi T.; Eguchi H.; Wakayama Y.; Sumi M.; Saito T. (OTA International, 2018)
Objectives: To investigate the mortality rate following cervical spinal cord injury (SCI) injury and analyze the associated risk factors. Design: Retrospective cohort study. Setting: One Level 1 trauma center. Patients/participants: A cohort of 76 patients with traumatic cervical SCI was reviewed between January 2010 and May 2015, of which 54 patients were selected for the present retrospective study. Intervention: Operative or conservative treatment. Main outcome measurements: The following patient parameters were analyzed; age, sex, American Spinal Injury Association (ASIA) impairment scale, neurological impairment level, injury mechanism, radiological findings, treatment, tracheostomy rate, and mortality. Results: The mean age of the patient cohort was 65 ± 17 years, with 11 females (20%) and 43 males (80%). A total of 16 (30%), 4 (7%), 22 (41%), and 12 patients (22%) were scored A, B, C, and D, respectively, on the ASIA impairment scale. Most of the injuries were at the C4 (30%) and C5 (33%) levels. Falls from standing (35%) and heights (39%) were the most common injury mechanisms. SCI in 40 patients (74%) occurred without major fracture or dislocation. Surgery was performed on 26 patients. The overall mortality was 19%. Patients in the deceased group were significantly older at the time of injury, compared with those who survived. Paralysis had been more severe in the deceased group. A significantly high number of patients in the deceased group received a tracheostomy. When analyzed using a multivariate logistic regression model, an ASIA impairment scale of A was a significant risk factor for mortality. Conclusions: The risk factors associated with mortality were age, tracheostomy, and an ASIA impairment scale of A, the latter had the highest risk.
Keywords: aged; American Spinal Injury Association impairment scale; article; cervical spinal cord injury; cohort analysis; conservative treatment; controlled study; dislocation; falling; female; fracture; human; Japan; major clinical study; male; mortality; paralysis; priority journal; retrospective study; risk factor; standing; tracheostomy

Longer-term assessment of nusinersen safety/efficacy in infantile-onset spinal muscular atrophy: Interim analysis of shine - Finkel R.S.; Castro D.; Farrar M.A.; Tulinius M.; Krosschell K.J.; Saito K.; Zhang Y.; Bhan I.; Farwell W.; Reyna S.P. (Journal of Neuromuscular Diseases, 2018)
Background: Nusinersen is an antisense oligonucleotide approved for the treatment of SMA. It has demonstrated a favorable benefit:risk profile and shown significant and clinically meaningful efficacy on motor function across a broad spectrum of SMA populations, and event-free survival (time to death or permanent ventilation) in infantile-onset SMA. The objective of the current analysis was to report interim results from the SHINE study (NCT02594124) for patients with infantile-onset SMA (most likely to develop Type I) who transitioned from ENDEAR. Methods: SHINE is an open-label extension study for infants/children who participated in the ENDEAR, CHERISH, CS12, or CS3A nusinersen trials. Nusinersen doses were administered according to the regimen and participant's cohort from the previous trial. The primary endpoint is safety/tolerability; secondary endpoints include achievement of Hammersmith Infant Neurological Examination - Section 2 (HINE-2) motor milestones and event-free survival defined as time to death or permanent ventilation (tracheostomy or ≥16 hours ventilation/day continuously for >21 days in the absence of acute reversible event). Results: The cutoff date was June 30, 2017; 89 patients transitioned from ENDEAR, 65/81 previously randomized to nusinersen and 24/41 to sham-control. Within SHINE only, 83 patients had an adverse event (AE). There were no treatment-related serious AEs. The most frequent AEs were pyrexia and upper respiratory tract infection. Mean (95% CI) change in HINE- 2 total score from nusinersen initiation to last observed visit was 1.1 (0.20-1.90) for patients who received sham-control in ENDEAR and nusinersen in SHINE (n=20/24) and 5.8 (4.58-7.04) for those who received nusinersen in ENDEAR and SHINE (n=74/81; pooled ENDEAR/SHINE data). Median (95% CI) event-free survival time among patients treated with sham-control in ENDEAR was 22.6 (13.6-31.3) weeks versus 73.0 (36.3-NA) weeks among those who received nusinersen in ENDEAR and SHINE. Conclusion: Improvements in motor function and event-free survival continued among patients who initiated nusinersen in ENDEAR and motor function improved among those who initiated nusinersen in SHINE. Further analysis of SHINE data will provide additional information on the longterm safety/tolerability and efficacy of repeated nusinersen doses across multiple SMA populations.
Keywords: nusinersen; achievement; adverse event; artificial ventilation; child; cohort analysis; conference abstract; controlled study; death; drug combination; drug safety; drug therapy; event free survival; female; fever; human; infant; major clinical study; male; motor performance; neurologic examination; pharmacokinetics; spinal muscular atrophy; tracheostomy; upper respiratory tract infection

Post Adjuvant neck irradiation related cervical spine arthritis mimics bony metastasis in MRI and PET/CT scan- A Case Report - Su C.-W.; Yen T.-Y. (Neuroradiology, 2018)
PURPOSE: To report a tongue cancer patient who had C-spine inflammatory arthritis mimicking bone metastasis after postoperative adjuvant irradiation. MATERIAL ANDMETHODS: A 54-year-old man underwent a composite resection for right tongue cancer with right radical neck dissection, mandibulotomy and tracheostomy, followed by adjuvant radiotherapy as total dose of 60Gy divided into 30 fractions in 2015. Five months later, he presented neck pain and rigidity for two weeks. The cervical spine two views X-ray film revealed multiple spurs formation and disc space narrowing at C4-5, C5-6, and C6-7 level. Pain killer, steroids and muscle relaxant were given but in vain. The MRI scan one week later disclosed abnormal signal with enhancement at T2 phase and decreased signal at T1 dark-fluid phase over lateral side of C1 and soft tissue surrounding occipital condyle (Figure1, 2). For further information, we prescribed PET/CT as well. PET/CT revealed increased uptake in the left superior articular facet of C1 vertebra with osteolytic change as SUVmax: 7.4 (Figure 3). After discussion between radiation oncologist, ENT oncologist, and neurosurgeon, we prescribed oral antibiotics as fusidic acid as initial treatment for suspicious arthritis rather than bony metastasis due to patient's clinical pattern. After taking fusidic acid for several weeks, patient felt better, and took this oral antibiotic continually. RESULTS: After prescription fusidic acid for three months, this patient's neck pain and rigidity relieved markedly. Followed MRI scans after finishing oral antibiotics one month and one year later both showed dramatically improved as only mild enhancement at left lateral mass between C1 spine and left occipital condyle. CONCLUSIONS: Neck irradiation is a risk factor of C-spine inflammatory arthritis and it may mimic bony metastasis as osteolytic lesion. This benign diagnosis should be kept in mind when cancer patient received irradiation before. (Figure Presented).
Keywords: adjuvant; fusidic acid; muscle relaxant agent; steroid; adjuvant radiotherapy; adult; arthritis; bone atrophy; cancer adjuvant therapy; cancer patient; cancer surgery; case report; cervical spine; clinical article; conference abstract; diagnosis; drug therapy; first cervical vertebra; human; irradiation; liquid; male; metastasis; middle aged; neck dissection; neck pain; neurosurgeon; nuclear magnetic resonance imaging; osteophyte; positron emission tomography-computed tomography; prescription; radiation oncologist; rigidity; risk factor; soft tissue; tongue cancer; tracheostomy; X ray film

Two arresting cases in the ICU - Denny J.; Bauza G.; Denny J. (Critical Care Medicine, 2018)
Learning Objectives: “Out of the blue” asystole is always startling in the ICU. We describe two different patients who presented with multiple episodes of asystole in a one month period in the Surgical ICU. Differential diagnosis is discussed and features common to both cases are explored. This will enhance the ability of attendees to deal with similar such presentations. Methods: Case #1 was a 73-year-old restrained female passenger in a motor vehicle accident who sustained multiple injuries: C2 body fracture (fx.), T3, 7, 8, 11 compression fx., L3 fx. s/p fusion, Left 1-6 rib fxs. Hospital course included a tracheostomy and PEG, serratia pneumonia, and prolonged ventilator dependence. Neurologically she weakly moved all extremities. After 2 weeks in the ICU, she had an episode of asystole which resolved after 5 chest compressions. A cardiology consult recommended observation. Her asystole recurred 3 days later, and again resolved with 3 compressions. At that time, cardiology recommended a scopolamine patch to reduce vagal tone. No further episodes occurred. Case #2 was a 64-year-old male who fell 6 feet and sustained a C5 fracture with spinal cord injury (T4 sensory level), and underwent an ORIF of the C5 fracture. There was also a T1 fracture, subsequently treated with a TLSO brace. His hospital course was complicated by neurogenic shock requiring norepinephrine infusion, polymicrobial pneumonia and ventilator dependence. After 1 week in the ICU, he also suffered asystole, which resolved after 5 compressions. Cardiology evaluation was done. Asystole recurred and cardiology started a scopolamine patch with good effect. Results: Sinus arrest is usually followed by a junctional or ventricular escape beat. In these 2 ICU cases, skilled nurses were already in the patients' rooms and promptly began compressions, with subsequent NSR. Common causes of asystole include high vagal tone, a terminal rhythm after a code, and medication causes, among others. Notably, hypoxia usually provokes first a bradycardia, then possible asystole. There was no desaturation in these cases. Cardioaccelerator fibers occur from T1-4, and counteract vagal tone. As both patients also had thoracic spine injuries, this may have weakened this normal opposition to vagal tone. In these 2 patients, cardiology did not elect a pacemaker because of ongoing infection, and life expectancy of arguably less than one year. Successful treatment was achieved with the vagolytic effects of scopolamine patch.
Keywords: noradrenalin; scopolamine; adult; aged; artificial ventilation; brace; bradycardia; cardiology; case report; differential diagnosis; female; fiber; foot; fracture; human; hypoxia; infection; infusion; life expectancy; male; middle aged; multiple trauma; nonhuman; nurse; pacemaker; pneumonia; resuscitation; rib; Serratia; sinus arrest; spinal cord injury; surgical intensive care unit; tracheostomy; traffic accident; vagus tone; ventilator

Tracheal-spinal fistula, a late complication of tracheostomy - Jun D.; Jun J.; Martires J.; Barot N. (American Journal of Respiratory and Critical Care Medicine, 2018)
Introduction: Tracheostomy is commonly done to assisting patients who require long-term ventilator support. Despite demonstrated safety in most cases, tracheostomies can be associated with numerous short and long-term complications. Here, we describe a rare case of tracheal-spinal fistula as a late complication in a patient requiring long term ventilator support. Presentation: A 27 year-old man with Duchenne muscular dystrophy and chronic respiratory failure status who underwent tracheostomy in 2012 presents with several days of fevers, dyspnea, and increasing secretions. Patient was last seen in ear-nose-throat clinic a year prior for tracheostomy follow-up with subsequent routine care performed by the patient's mother. On admission, he was febrile to 39 degrees Celsius with stable hemodynamics and ventilator requirements. On examination, he was noted to have thick purulent secretions from his tracheostomy tube and diminished left lower base breath sounds. He also complained of tingling sensation in his left fourth and fifth digits with normal sensation otherwise with baseline motor weakness in his bilateral upper extremities. Laboratory examination showed leukocytosis with left shift and normal gas exchange and lactate. Chest film confirmed presence of a left lower lung field infiltrate consistent with pneumonia. Patient was started on broad-spectrum; however, despite several days of antibiotics the patient's fever continued and a computer tomography scan was obtained to better characterize his chest film findings. Here, we discovered that in addition to a dense left lower lobe infiltrate, the patient had severely dilated tracheostomy balloon with a possible fistula to the cervical spine. A direct laryngoscopy was performed at the bedside, confirming the presence of a small 2 millimeter fistulous tract along the right mid/central area of dilated posterior tract wall. Patient was treated for diskiitis and osteomyelitis with long-term ceftzidime with gradual neurologic improvement. Discussion: Previously described long-term complications of tracheostomy include recurrent pneumonias, tracheal stenosis, tracheomalacia, and tracheoesophageal fistulas (TEF). The latter is thought to be due to an iatrogenic complication during placement or related excessive cuff pressures causing injury to the surrounding structure. This case illustrates another rare complication of tracheostomy, likely due to similar mechanism of injury proposed in TEFs. Clinicians should be wary of new neurologic symptoms especially in the upper extremities with this condition.
Keywords: antibiotic agent; lactic acid; abnormal respiratory sound; adult; artificial ventilation; balloon; bodily secretions; case report; cervical spine; chronic respiratory failure; clinical article; complication; computer assisted tomography; conference abstract; digit (body part); Duchenne muscular dystrophy; dyspnea; ear; female; fever; follow up; gas exchange; hemodynamics; human; iatrogenic disease; laboratory test; laryngoscopy; leukocytosis; male; neurologic disease; nose; osteomyelitis; pneumatic cuff; pneumonia; sensation; suppuration; thinking; thorax; throat; trachea stenosis; tracheoesophageal fistula; tracheomalacia; tracheostomy tube; upper limb; ventilator; weakness

A case of delayed diagnosis: Late onset tracheal stenosis after multiple short term intubations - Gao X.; Bachan M.; Khan Z.; Siegel R.E. (American Journal of Respiratory and Critical Care Medicine, 2018)
Introduction: Tracheal stenosis is a common complication in patients undergoing endotracheal intubation and can cause life threatening complications. However its presentations may be atypical and lead to delay in diagnosis. We present a case of late onset tracheal stenosis which was initially misdiagnosed as anxiety. Case Description: A 44-year-old man was admitted to Medical Intensive Care Unit (MICU) for 2 weeks' history of intermittent dyspnea. His history was significant for motor vehicle accident (MVA) which was complicated with thoracic spine fracture, paraplegia and multiple rib fracture with pneumothorax. He was intubated 5 times over a month while he was comatose. The intubations last from 2 to 6 days. He was successfully extubated one month after MVA and had no respiratory issues until his admission to MICU. He complained of generalized chest tightness and shortness of breath. A trial of bronchodilator failed to relieve symptoms. On examination patient was very anxious but speaking in full sentences, oxygen saturation was well maintained above 98% on room air and lungs were clear without any wheezing. His chest x ray was grossly normal. A bedside flexible fiberoptic laryngoscopy was performed for suspicion of possible airway stenosis, but it was reported as normal. Therefore his symptoms were considered more due to anxiety also since he responded to Lorazepam. However, during the next few days his symptoms worsened and a biphasic respiratory stridor developed. A CT of chest and neck showed possible tracheal stenosis. An urgent rigid bronchoscopy was performed and showed excessive granulation tissue in trachea leading to profound narrowing. He underwent emergent tracheostomy with tracheal tube placement. Symptoms were significantly relieved afterwards, and oral steroid was started to suppress granulation tissue formation. Discussion: In spite of improvement in the design of tracheal tubes, almost all patient undergoing translarygneal intubation develop airway stenosis, and severe stenosis (>50%) still occurs in approximate 19% patients. However, symptoms usually are not prominent especially at rest until the narrowing is more than 70% and can be very atypical, such as in our patient. It is crucial to maintain a high suspicion for tracheal stenosis in any patients with a history of intubation and exertional dyspnea, particularly not improving with bronchodilators, as immediate evaluation is needed.
Keywords: bronchodilating agent; lorazepam; steroid; adult; airway; ambient air; anxiety; bronchoscopy; case report; chest tightness; clinical article; coma; conference abstract; delayed diagnosis; diagnostic error; drug therapy; dyspnea; endotracheal tube; granulation tissue; human; intubation; laryngoscopy; male; medical intensive care unit; neck; oxygen saturation; paraplegia; pneumothorax; rest; rib fracture; speech; stridor; thoracic spine; thorax radiography; trachea stenosis; tracheostomy; traffic accident; wheezing

Preoperative Variables Associated With Respiratory Complications After Pediatric Neuromuscular Spine Deformity Surgery - Luhmann S.J.; Furdock R. (Spine Deformity, 2018)
Objective: The objective of this study is to identify preoperative laboratory values and patient factors that are associated with postoperative respiratory complications in pediatric neuromuscular scoliosis (NMS) populations undergoing posterior spinal fusion (PSF) with instrumentation. Summary of Background Data: PSF in NMS patients are high-risk surgeries. Respiratory complications are the most common postoperative event, with rates up to 28.2% following surgery. Methods: A single-surgeon, two-hospital pediatric spine surgery database was reviewed to identify all patients who underwent PSF for NMS. Diagnoses included cerebral palsy (n=83), myelomeningocele (n=13), spinal muscular atrophy (n=4), and other (n=11). This study defined respiratory complications as postoperative pneumonia, pleural effusion, pneumothorax, need for reintubation, respiratory status requiring a return to the pediatric intensive care unit (PICU), or prolonged (>4-day) need for mechanical ventilation. Preoperative laboratory values for transferrin, prealbumin, hemoglobin/hematocrit, total protein, albumin, and total lymphocyte count were collected. Results: There were 50 males and 61 females with a mean age of 14 years 2.5 months (8–20 years). Seventeen patients (15.3%) experienced postoperative respiratory complications. On univariate analysis, any history of pneumonia, the presence of gastrostomy tube, and low transferrin levels were associated with postoperative respiratory complications, and a strong trend (p=.06) was observed for tracheostomy. On multivariate analysis, the presence of gastrostomy tube and history of pneumonia remained as clinically significant predictors of postoperative respiratory complications. Conclusion: Pediatric NMS patients undergoing PSF that have history of pneumonia or gastrostomy tube present at time of surgery are at increased risk for postoperative respiratory complications. The univariate associations of tracheostomy presence and low transferrin levels with postoperative respiratory complications deserve further examination. Level of Evidence: Level II.
Keywords: adolescent; article; artificial ventilation; cerebral palsy; child; complication; controlled study; diagnosis; female; gene expression; hematocrit; human; lymphocyte count; major clinical study; male; meningomyelocele; pediatric intensive care unit; pleura effusion; pneumonia; pneumothorax; preoperative evaluation; protein expression; risk assessment; scoliosis; spinal muscular atrophy; spine fusion; spine malformation; stomach tube; surgeon; surgery; tracheostomy; univariate analysis; albumin; endogenous compound; hemoglobin; transthyretin; protein; transferrin

Spinal Muscular Atrophy With Respiratory Distress Type 1—A Child With Atypical Presentation - Chiu A.T.G.; Chan S.H.S.; Wu S.P.; Ting S.H.; Chung B.H.Y.; Chan A.O.K.; Wong V.C.N. (Child Neurology Open, 2018)
The authors report a child with spinal muscular atrophy with respiratory distress type 1 (SMARD1). She presented atypically with hypothyroidism and heart failure due to septal defects that required early heart surgery and microcephaly in association with cerebral atrophy and thin corpus collosum. The subsequent asymmetrical onset of diaphragmatic paralysis, persistent hypotonia, and generalized muscle weakness led to the suspicion of spinal muscular atrophy with respiratory distress type 1. Sanger sequencing confirmed a compound heterozygous mutation in the Immunoglobulin Mu Binding Protein 2 (IGHMBP2) gene, with a known mutation c.2362C > T (p.Arg788*) and a novel frameshift mutation c.2048delG (p.Gly683A1afs*50). Serial nerve conduction study and electromyography confirmed progressive sensorimotor polyneuropathy and neuronopathy. In summary, this case report describes a child with spinal muscular atrophy with respiratory distress type 1 also with congenital cardiac disease and endocrine dysfunction, expanding the phenotypic spectrum of this condition. A high index of suspicion is needed in diagnosing this rare condition to guide the management and genetic counseling.
Keywords: antihypertensive agent; catecholamine; homovanillic acid; metadrenalin; noradrenalin; normetadrenalin; thyrotropin; vanilmandelic acid; article; artificial ventilation; bladder capacity; body height; body weight; brain atrophy; case report; cause of death; child; clinical article; constipation; corpus callosum; developmental delay; disease classification; disease course; disease severity; echocardiography; electromyography; female; gene; gene mutation; genetic analysis; head circumference; heart atrium septum defect; heart ventricle septum defect; human; hyperhidrosis; hypertension; hyporeflexia; IGHMBP2 gene; intrauterine growth retardation; low birth weight; medical history; microcephaly; muscle action potential; muscle biopsy; muscle hypotonia; myelination; nerve biopsy; nerve conduction velocity; nerve fiber degeneration; neuroimaging; nose feeding; nuclear magnetic resonance imaging; pathogenesis; postoperative period; priority journal; respiratory distress; spinal muscular atrophy; sural nerve; thyroid disease; thyrotropin blood level; tracheostomy; treatment refusal; urinalysis; urinary tract infection; urosepsis; vesicoureteral reflux

Blastomycosis-related acute respiratory distress syndrome successfully managed with extracorporeal membrane oxygenation - Pearson S.D.; Bhavani S.; Katsis J.; Banach B.; Antic T.; Patel B.K.; Naureckas E.T. (American Journal of Respiratory and Critical Care Medicine, 2018)
Introduction: Blastomyces dermatitidis is an endemic mycosis found primarily in the midwestern and south-central United States. Blastomycosis can cause acute or chronic pneumonia, and can rarely cause overwhelming pulmonary infection and related acute respiratory distress syndrome (ARDS). Case Description: A 21-year-old female with morbid obesity and no other past medical history was transferred to the University of Chicago medical intensive care unit for management of acute hypoxemic respiratory failure and spinal cord compression. She had initially presented one month prior with back pain and dyspnea. Chest radiography showed a right upper lobe opacity for which she was treated on two separate occasions for community acquired pneumonia. She then presented a third time for worsening dyspnea in addition to back pain. Her chest imaging demonstrated a diffuse military pattern with coincident large right sided thoracic mass at the level of T2-T4. Subsequent MRI showed the thoracic mass was associated with severe cord compression. The patient developed hypoxemia requiring high flow nasal cannula (HFNC) and was transferred to the University of Chicago for further care. Upon transfer, empiric broad spectrum antibiotics, antifungals, and RIPE therapy were initiated and the patient required intubation for worsening hypoxemia. She underwent a bronchoscopy with broncho-alveolar lavage revealing normal airways with thick tan mucous secretions, from which fungal cultures grew Blastomyces dermatitidis. She underwent percutaneous drainage of the thoracic mass with removal of 200 cc of frank pus which also demonstrated Blastomyces on culture. The patient's course was complicated by acute renal failure, severe acute respiratory distress syndrome with PaO2:FiO2 ratio < 100, and plateau pressures exceeding 40 cmH2O despite lung protective ventilation and paralysis. To allow adequate oxygenation while awaiting lung recovery, venovenous extracorporeal membrane oxygenation (ECMO) was initiated on day 2 of intubation. She was extubated to HFNC 8 days later, ECMO support was gradually weaned, and she underwent decannulation on ECMO day 39. She subsequently had full recovery of renal function, significant neurologic recovery, and was ultimately discharged home on oral antifungal therapy on hospital day 76. Discussion: ARDS is an uncommon complication of pulmonary blastomycosis, with mortality rates exceeding 50% in published case series. A recent retrospective case series reported successful application of ECMO in four patients with blastomycosis-related ARDS. Although the role of ECMO in blastomycosis-related ARDS is far from certain, transfer to an institution with experience in ECMO should be considered. (Figure presented) .
Keywords: antibiotic agent; antifungal agent; acute kidney failure; adult; adult respiratory distress syndrome; airway; antifungal therapy; army; backache; blastomycosis; bronchoscopy; case report; case study; clinical article; community acquired pneumonia; complication; conference abstract; drug therapy; dyspnea; extracorporeal oxygenation; female; human; hypoxemia; Illinois; intubation; kidney function; lung lavage; medical history; medical intensive care unit; morbid obesity; mortality rate; mucus secretion; nasal cannula; nervous system; nuclear magnetic resonance imaging; paralysis; percutaneous drainage; pus; remission; respiratory airflow; respiratory failure; retrospective study; spinal cord compression; thorax radiography; young adult

Judicious utilisation of surgical and advanced radiotherapy resources to manage triple trouble: A case of synchronous dual malignancies with severe cervical spine deformity - Ahmad I.; Lohan R.; Chufal K.S.; Raina S. (BMJ Case Reports, 2018)
Keywords: computed tomography scanner; Eclipse; linear accelerator; oropharynx airway; radiotherapy planning system; Somatom Sensation Open; adult; biopsy; blood analysis; cancer grading; cancer growth; cancer radiotherapy; cancer staging; cancer surgery; case report; cell differentiation; chemoradiotherapy; clinical article; clinical feature; computer assisted tomography; disease severity; dysphagia; glossectomy; head and neck squamous cell carcinoma; histopathology; human; human tissue; male; medical examination; middle aged; mucosa inflammation; neck dissection; note; nuclear magnetic resonance imaging; patient care; patient positioning; postoperative period; priority journal; pyriform sinus; radiation dose; respiration control; spine malformation; thorax radiography; torticollis; tracheostomy; treatment duration; treatment outcome; tumor invasion; ulcer healing; xerostomia; Eclipse; Somatom Sensation Open; TrueBeam

Homozygosity for a nonsense variant in AIMP2 is associated with a progressive neurodevelopmental disorder with microcephaly, seizures, and spastic quadriparesis - Shukla A.; Das Bhowmik A.; Hebbar M.; Rajagopal K.V.; Girisha K.M.; Gupta N.; Dalal A. (Journal of Human Genetics, 2018)
We ascertained two unrelated consanguineous families with two affected children each having microcephaly, refractory seizures, intellectual disability, and spastic quadriparesis. Magnetic resonance imaging showed atrophy of cerebrum, cerebellum and spinal cord, prominent cisterna magna, symmetric T2 hypo-intensities in the bilateral basal ganglia and thinning of corpus callosum. Whole-exome sequencing of three affected individuals revealed c.105C>A [p.(Tyr35Ter)] variant in AIMP2. The variant lies in a common homozygous region of 940 kb on chromosome 7 and is likely to have been inherited from a common ancestor. The phenotype noted in our subjects' shares marked similarity with that of hypomyelinating leukodystrophy-3 caused by mutations in closely related gene AIMP1. We hereby report the first human disease associated with deleterious mutations in AIMP2.
Keywords: amino acid transfer RNA ligase; anticonvulsive agent; acute gastroenteritis; anteverted nostril; article; artificial ventilation; blood sampling; brain atrophy; case report; cerebellum atrophy; child; clinical article; contracture; corpus callosum; dehydration; developmental delay; electroencephalography; failure to thrive; female; genetic association; genetic variability; hirsutism; homozygosity; homozygote; human; intractable epilepsy; male; mental disease; microcephaly; myoclonus seizure; neuroimaging; neurologic disease; nuclear magnetic resonance imaging; pneumonia; prognathia; quadriplegia; school child; seizure; spasticity; spike wave; spinal cord atrophy; spontaneous abortion; tonic clonic seizure; tracheostomy; whole exome sequencing

Medical complications and mortality in octogenarians undergoing elective spinal fusion surgeries - Rajpal S.; Lee Nelson E.; Villavicencio A.T.; Telang J.; Kantha R.; Beasley K.; Burneikiene S. (Acta Neurochirurgica, 2018)
Background: The consequences of suffering postoperative complications in elderly undergoing spinal surgeries may be different compared to younger patients. The primary objective of this study was to identify the types and frequency of medical complications and mortality rates in patients 80 years of age or older undergoing elective spinal fusion surgeries for degenerative spinal disease. Methods: A prospective observational study with a retrospective chart review was performed, which included all consecutive patients ≥80 years old undergoing elective spinal fusion surgeries from May 2012 to August 2015. We identified a total of 95 patients, of which 39 cervical and 56 lumbar surgeries were performed. There were 41 female and 54 male patients with the mean age of 82.8 years (range, 80–91). The perioperative complications were allocated into the following categories: infection, pulmonary, cardiac, gastrointestinal, hematologic, urologic, neurovascular, thromboembolic, and other. Baseline and postoperative clinical outcome scores were compared to evaluate efficacy. Results: The mean follow-up time was 14.8 months (range, 5 days to 37 months) with an overall mortality rate of 8.4%. The 30-day, 90-day, and 1-year mortality rates were 2.1, 2.1, and 4.2%, respectively. There were 53.9 and 71.4% patients with complications in the cervical and lumbar patient groups, respectively. The presence of general comorbidities and the number of intervertebral levels predicted the occurrence of perioperative complications. Also, longer OR times were associated with a higher number of complications per patient and the occurrence of a UTI. Dysphagia was a significant predictor in developing pneumonia and atelectasis. Conclusions: The incidence of perioperative medical complications and mortality rates in octogenarians undergoing elective spinal surgeries are quite high. The benefits of having surgery must be weighed against the risks of not only surgical but also adverse medical events. An informed decision-making process should include discussion of potential postoperative morbidity specific to this patient population in order to guide patient’s acceptance of higher risks and expectations postoperatively. It is also important to identify potential complications and adapt preventive measures in order to help minimize them in this patient population.
Keywords: acute kidney failure; acute respiratory failure; aged; anemia; article; aspiration pneumonia; atelectasis; atrial fibrillation; bacteremia; blood transfusion; bronchitis; cerebrovascular accident; cervical spine; clinical outcome; comorbidity; compression fracture; congestive heart failure; deep vein thrombosis; dysphagia; elective surgery; esophagus perforation; failed back surgery syndrome; female; follow up; gout; heart muscle ischemia; hematoma; hospital readmission; human; hyponatremia; hypotension; inappropriate vasopressin secretion; intervertebral disk hernia; kyphoplasty; length of stay; liquorrhea; lumbar spinal stenosis; lumbar spine; lung edema; lung embolism; major clinical study; male; Mallory Weiss syndrome; medical record review; mortality rate; nursing home; observational study; Oswestry Disability Index; overall survival; peroperative complication; posterior lumbar interbody fusion; postoperative complication; priority journal; prospective study; pseudarthrosis; pseudomembranous colitis; rectum prolapse; rehabilitation care; reoperation; retroperitoneal abscess; retrospective study; sepsis; seroma; Short Form 36; spinal cord decompression; spinal cord disease; spine fusion; spine malformation; spondylolisthesis; surgical drainage; surgical infection; surgical mortality; tachycardia; thrombophlebitis; tracheostomy; transient ischemic attack; urinary tract infection; urine retention; very elderly

Compound heterozygous RYR1 mutations in a preterm with arthrogryposis multiplex congenita and prenatal CNS bleeding - Brackmann F.; Türk M.; Gratzki N.; Rompel O.; Jungbluth H.; Schröder R.; Trollmann R. (Neuromuscular Disorders, 2018)
RYR1 mutations, the most common cause of non-dystrophic neuromuscular disorders, are associated with the malignant hyperthermia susceptibility (MHS) trait as well as congenital myopathies with widely variable clinical and histopathological manifestations. Recently, bleeding anomalies have been reported in association with certain RYR1 mutations. Here we report a preterm infant born at 32 weeks gestation with arthrogryposis multiplex congenita due to compound heterozygous, previously MHS-associated RYR1 mutations, with additional signs of prenatal hemorrhage. The patient presented at birth with multiple joint contractures, scoliosis, severe thoracic rigidity and respiratory failure. He continued to depend on mechanical ventilation and tube feeding. Muscle histopathology showed a marked myopathic pattern with eccentric cores. Interestingly, the patient had additional unusual prenatal intraventricular hemorrhage, resulting in post-hemorrhagic hydrocephalus as well as epidural hemorrhage affecting the spinal cord. This report adds to the phenotypic variability associated with RYR1 mutations, and highlights possible bleeding complications in affected individuals.
Keywords: creatine kinase; ryanodine receptor 1; arterial pH; arthrogryposis; article; artificial ventilation; birth weight; brain hemorrhage; brain radiography; case report; cesarean section; clinical article; clinical feature; computer assisted tomography; creatinine blood level; disease severity; enteric feeding; gene mutation; genetic analysis; genetic association; genetic counseling; genetic variability; gestation period; high frequency oscillation; histopathology; human; human tissue; hydramnios; hydrocephalus; infant; joint contracture; male; muscle biopsy; muscle hypertonia; nuclear magnetic resonance imaging; percutaneous endoscopic gastrostomy; prematurity; priority journal; ptosis (eyelid); respiratory failure; risk factor; RYR1 gene; scoliosis; sweating; tendon reflex; tracheotomy; whole exome sequencing

Distal Spinal Muscular Atrophy: An Overlooked Etiology of Weaning Failure in Children with Respiratory Insufficiency - Habibi Zoham M.; Eghbalkhah A.; Kamrani K.; Khosroshahi N.; Yousefimanesh H.; Eskandarizadeh Z. (Journal of Pediatric Intensive Care, 2018)
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive neuromuscular disorder that involves the anterior horn motor neurons. It is a disease with a poor prognosis presenting with progressive distal motor weakness and respiratory insufficiency from diaphragmatic paralysis followed by distal muscle weakness before 6 months of age. With the intent to spread the awareness of this rare and life-threatening disease, we report a 2.5-month-old female infant with a subsequent diagnosis of SMARD1, who was admitted in our pediatric intensive care unit with chief complaint of progressive respiratory distress and poor feeding.
Keywords: antibiotic agent; DNA; abnormal respiratory sound; antibiotic therapy; article; breech presentation; case report; cesarean section; clinical article; crackle; diaphragm paralysis; DNA sequence; exon; feeding disorder; female; fluoroscopy; gene; gene mutation; genetic analysis; genetic variation; hospital infection; human; human tissue; IGHMBP2 gene; infant; pediatric intensive care unit; priority journal; respiratory acidosis; respiratory distress; septicemia; skin biopsy; spinal muscular atrophy; spinal muscular atrophy with respiratory distress type 1; tracheostomy; urine retention; ventilator weaning

Screw migration and oesophageal perforation after surgery for osteosarcoma of the cervical spine - Denaro L.; Longo U.G.; Di Martino A.C.; Maffulli N.; Denaro V. (BMC Musculoskeletal Disorders, 2017)
Background: Even though internal fixation has expanded the indications for cervical spine surgery, it carries the risks of fracture or migration, with associated potential life threatening complications. Removal of metal work from the cervical spine is required in case of failure of internal fixation, but it can become challenging, especially when a great amount of scar tissue is present because of previous surgery and radiotherapy. Case presentation: We report a 16 year old competitive basketball athlete who underwent a combined anterior and posterior approach for resection of an osteosarcoma of the sixth cervical vertebra. Fourteen years after the index procedure, the patient eliminated spontaneously one screw through the intestinal tract via an oesophageal perforation and developed a severe dysphagia. Three revision surgeries were performed to remove the anterior plate because of the great amount of post-surgery and post-irradiation fibrosis. Conclusions: Screw migration and oesophageal perforation after cervical spine surgery are uncommon potentially life-threatening occurrences. Revision surgery may be challenging and it requires special skills.
Keywords: bone plate; bone screw; orthopedic cast; orthopedic fixation device; suction drain; antiinflammatory agent; mesna; adolescent; article; athlete; basketball player; bone graft; bone radiography; bone tumor; cancer surgery; case report; cervical spine; clinical article; colonoscopy; computer assisted tomography; device migration; device removal; disease severity; dysphagia; emergency ward; endoscopist; esophagus injury; esophagus perforation; fibrosis; fluoroscopy; histopathology; human; human tissue; iliac crest; index finger; male; medical history; muscle contracture; myelography; Neck Disability Index; neck pain; neurologic examination; orthopedic surgery; osteosarcoma; paresthesia; physiotherapy; postoperative complication; radiotherapy; rest; spinal cord compression; tendinitis; total parenteral nutrition; tracheostomy; treatment duration; tumor biopsy; body weight loss; uromitexan

Trends in Patient Care for Traumatic Spinal Injuries in the United States: A National Inpatient Sample Study of the Correlations with Patient Outcomes from 2001 to 2012 - Holland C.M.; Mazur M.D.; Bisson E.F.; Schmidt M.H.; Dailey A.T. (Spine, 2017)
Study Design. A retrospective database review. Objective. The aim of this study was to examine whether patient characteristics, distribution of care, and patient outcomes for spinal cord injury (SCI) in the United States have changed between 2001 and 2012. Summary of Background Data. Although patient outcomes after cranial injury are better at high-volume centers with specialized, multidisciplinary teams, similar assessments have not been done for spinal injuries. Methods. We retrospectively reviewed the National and Nationwide Inpatient Samples for the years 2001, 2002, 2011, and 2012 to identify patients with spinal fracture with or without SCI. The demographic characteristics of the patient cohort, clinical course, hospital characteristics, interhospital transfer, and disposition were statistically analyzed relative to patient mortality, total hospital costs, and length of stay. How these data changed over this 11-year period was also evaluated. Results. A total of 159,875 cases were identified, with 141,737 fractures without SCI and 18,138 SCIs with or without fracture. There was a statistically significant decrease in the percentage of patients transferred with spine injury from 4.2% to 3.4% (P<0.001) from the early years to the later years and in patient transfers for SCIs (8.1% vs. 6.5%, P<0.001). Interestingly, the overall mortality rate (3.5% vs. 3.6%) remained unchanged (P=0.679), but mortality from SCI increased (6.6-7.4%, P=0.021). Conclusion. From 2002 to 2012, the rate of interhospital transfer of spinal injury patients declined, while the mortality rate for patients with SCI increased. Interestingly, there was an increase in transfers after spinal surgery at the index hospital. The decentralization of spine care may be responsible for the increase in mortality.
Keywords: stomach tube; adult; controlled study; correlational study; demography; disease course; female; hospital cost; human; ICD-9-CM; length of stay; major clinical study; male; middle aged; mortality rate; patient care; priority journal; retrospective study; review; spinal cord injury; spine fracture; tracheostomy; treatment outcome; United States

A service evaluation of surgical tracheostomies in the emergency theatre at University Hospital, Coventry: Time for a dedicated tracheostomy team? - Virdi M.; Chohan P.; Elledge R.; Townsend R.; Walton G. (British Journal of Oral and Maxillofacial Surgery, 2017)
Introduction/aims: The routine practice of surgical tracheostomy has been challenged by bedside percutaneous dilatational tracheostomy (PDT). Whilst PDT is costeffective and time-efficient, there is still a place for the open technique in patients with unfavourable anatomy, unstable cervical spine injuries and/or coagulopathies. Complication rates of the open technique are often lower, but booking such cases into busy emergency theatres may prove difficult. Material/methods: We conducted a prospective service evaluation of all surgical tracheostomies referred to ENT and Maxillofacial surgeons from the Intensive Care Unit over a 6-month period. Results/statistics: There were 39 patients referred who had been intubated for a mean (SD) of 7.3 (4.0) days at the point of referral. Following referral, 21/39 were delayed beyond one day, with a mean time to surgical tracheostomy provision of 2.2 (0.9) days. The commonest reasons for delay were surgeon decision (13/21) and theatre availability (7/21). Surgeons often cited an unwillingness to perform surgical tracheostomies outside of elective lists and clashes with planned clinical commitments. Conclusions/clinical relevance: We feel that there is an argument for a dedicated theatre slot to provide surgical tracheostomies in our unit. The average cost of an ICU bed increased from £1,551 to £1,647 in the period 1999-2006. Ever increasing costs coupled with a predicted rise in demand for Critical Care bed-days of4%per annum highlight the need to facilitate early discharges from ICU. Identifying candidates for surgical tracheostomy early and ensuring theatre and surgeon availability may help curb these costs and provide a more efficient service.
Keywords: clinical article; dental surgeon; emergency; female; human; intensive care unit; male; patient referral; prospective study; statistics; surgery; tracheostomy; university hospital

Acute flaccid paralysis by Enterovirus D68 infection: First Italian description on adult patient and role of electrophysiology - Ceccanti M.; Rubino A.; Romanzi F.; Onesti E.; Tartaglia G.; Inghilleri M. (Clinical Neurophysiology, 2017)
A Peruvian woman was admitted to the Emergency Department of “Policlinico Umberto I” in Rome for drop head associated to an acute flaccid paralysis of the upper limbs, beginning from the proximal muscles and rapidly extending to the distal. No sensory involvement was objectified. Her clinical history was significant for Non- Hodgkin Lymphoma and hematopoietic stem cell transplantation, complicated with a chronic graft-versus-host disease. Medullar spinal cord MRI showed a long tract of T2 hyperintensity in the anterior and central region of the cervical cord, with an elective involvement of grey matter. Lumbar puncture showed pleocytosis. An early NCS and EMG exam demonstrated reduction in cMAP amplitude from Ulnar and Median nerves bilaterally with absence of F-waves, with normal sensory conduction. Phrenic nerve assessment revealed bilateral reduction in amplitude. Severe reduction in spatial recruitment, doublets and triplets were also detected in the needle examination of upper limbs. Normal SEP were registered. Entherovirus D68, a polio-like virus firstly isolated in US in 2014, was isolated in patient's cerebrospinal fluid, plasma and throat swab. Intravenous immunoglobulins, together with steroids and Acyclovir, were dispensed as replacement therapy and passive immunization, with no effect. Patient underwent to tracheostomy and died after two weeks from hospitalization.
Keywords: aciclovir; endogenous compound; immunoglobulin; steroid; adult; cerebrospinal fluid; cervical spinal cord; chronic graft versus host disease; electrophysiology; emergency ward; Enterovirus D68; female; flaccid paralysis; gray matter; head; hematopoietic stem cell transplantation; Hodgkin disease; hospitalization; human; human tissue; lumbar puncture; median nerve; medulla oblongata; muscle; nuclear magnetic resonance imaging; passive immunization; Peruvian; phrenic nerve; plasma; pleocytosis; poliomyelitis; substitution therapy; throat culture; tracheostomy; ulna; upper limb

Weaning outcomes of mechanically ventilated spinal cord injured persons with acute tetraplegia admitted to a regional UK centre over a 10 year period - Chakrabarti B.; Forrest A.; Bevan M.; Ward A.; Chaudhary K.; Soni B.; Selmi F.; Parker R.; Plant P.K.; Manuel A.; Duffy N.; Lari S.; et al. (Thorax, 2017)
Introduction The occurrence of Spinal Cord Injury (SCI) is often complicated by the need for assisted ventilation. This study charts the weaning outcomes of mechanically ventilated SCI subjects admitted over a 10 year period to a regional Spinal Injuries unit. Methodology Acute SCI subjects with Tetraplegia admitted from April 2007-2017 to the Northwest Regional Spinal Injuries Centre (NWRSIC) were identified. Only those presenting with all 3 criteria: A) admission injury level C1-C6 b) admission ASIA score A-C and c) need for mechanical ventilation on arrival to the NWRSIC were included in the final analysis. Results The cohort consisted of 84 subjects (mean age 57 (SD 18) years; 76% male; 81 surviving to discharge). On admission, the level of injury was C1-3 in 28% (C1-3 ASIA A 20%; C1-3 ASIA B 2%; C1-3 ASIA C 6%) and C4-6 in 72% (C4-6 ASIA A 38%; C4-6 ASIA B 17%; C4-6 ASIA C 17%). On admission, 86% (72/84) were tracheostomy ventilated 24 hours/day, 12% (10/84) tracheostomy ventilated at night only and 2% (2/84) using NIV. By discharge, 13% (11/ 81) were tracheostomy ventilated 24 hours/day (including 2 Phrenic nerve paced), 13% (11/81) tracheostomy ventilated at night only, 7% (6/81) prescribed nocturnal NIV with 65% (53/81) breathing independently. Thus, when taking the entire cohort, 63% (53/81) achieved complete Ventilatory liberation, 12% (10/81) weaned to nocturnal tracheostomy ventilation only and 6% (5/81) were weaned to NIV whilst no further weaning was possible in 16% (13/81). The ability to breathe independently by discharge was found to correlate with level of injury on admission (CC 0.39; p<0.001), level of injury on discharge (CC 0.47; p<0.001) and non-significant trend with improvement in neurological function during admission (CC 0.21; p=0.06) but not age or gender. Conclusion Our data demonstrates that in a cohort of consecutive SCI patients requiring mechanical ventilation on admission to a regional Spinal injuries unit, weaning from mechanical ventilation was possible in 84% of subjects with 63% being liberated completely from Ventilatory support by discharge. The use of NIV in the SCI cohort appears to be an emergent strategy during the weaning process.
Keywords: adult; American Spinal Injury Association impairment scale; cohort analysis; female; gender; human; major clinical study; male; middle aged; nervous system function; night; phrenic nerve; quadriplegia; spinal cord injury; tracheostomy; ventilator weaning

Spinal cord stimulation for pain treatment failed to modify disease progression in a patient with amyotrophic lateral sclerosis - Di Lazzaro V.; Mazzone P.; Insola A.; Florio L.; Capone F.; Ranieri F. (Clinical Neurology and Neurosurgery, 2017)
Keywords: implantable pulse generator; implanted spinal cord stimulator; riluzole; aged; amyotrophic lateral sclerosis; analgesia; article; atrophy; Babinski reflex; case report; clinical article; cortical excitability; denervation; disease course; dysarthria; enteric feeding; fasciculation; human; limb weakness; male; neurophysiology; nuclear magnetic resonance imaging; paresthesia; spastic paraplegia; spinal cord stimulation; tracheostomy; Octrode

Spinal intramedullary tuberculoma following pulmonary tuberculosis: A case report and literature review - Wang G.-C.; Wu S.-W. (Medicine (United States), 2017)
Rationale: Spinal intramedullary tuberculoma (IMTB) is a rare disease that accounts for 1 to 2/100,000 patients with tuberculosis. We presented a case with pulmonary tuberculosis and concurrent IMTB at C3 to C5 level and reviewed the recent case series and discussed the diagnosis, treatment, and outcome. Patient concerns: A 33-year-old male had concurrent pulmonary TB and IMTB at the C3 to C5 level. He had quadriplegia (muscle power 0 at 4 limbs) and sensory loss below C5 level. He also had incontinence, anal tone loss, and paradoxical respiratory pattern. Diagnosis: Spinal magnetic resonance imaging (MRI) showed a 25 11mm intramedullary lesion at C3/C4 level. Under the impression of IMTB, he underwent surgery. Intervention: We performed C3 to C5 laminectomy and en bloc removal of the tumor. The patient kept receiving anti-TB medications after the surgery. Outcome: His 4 limbs muscle power had improved but could not be liberated from the endotracheal tube, so tracheostomy was performed. Muscle power gradually increased to 3 points in his upper limbs and to 2 points in his lower limbs. Sensation in his 4 limbs gradually improved as well. Lessons: IMTB is a rare disease that should be treated with a combination of medication and surgery. For patients with prominent spinal cord compression and neurological symptoms, early operation to remove the tumor is necessary.
Keywords: betamethasone; tuberculostatic agent; adult; alcohol liver cirrhosis; cancer surgery; case report; clinical article; computer assisted tomography; drug withdrawal; feces incontinence; human; laminectomy; limb weakness; lung tuberculosis; male; nuclear magnetic resonance imaging; paresthesia; priority journal; quadriplegia; review; spinal intramedullary tuberculoma; tuberculoma; X ray

Another cause of CNS hypomyelination-FIG4 mutations - Hussain S.; Berry I.; Soulioti A.; Uka S.; Livingston J.H.; Vadlamani G. (Developmental Medicine and Child Neurology, 2017)
Introduction: FIG4 mutations are associated with CMT4J, Amyotrophic lateral sclerosis-11(ALS11), bilateral polymicro-gyria and Yunis-Varon Syndrome. We describe a novel phe-notype manifesting with peripheral neuropathy and CNS hypomyelination in 2 cases. Case series: Patient A was born at term to consanguineous Asian parents. He had early developmental delay, depressed reflexes, poor facial movements and four limb hypotonia. Neurophysiology confirmed a demyelinating polyneuropathy. At 11yrs of age he is wheelchair, tracheostomy and gastros-tomy dependent. He has profound weakness with contrac-tures, communication via eye gaze and gestures. Serial MRI at 30-months, 7 years and 8-years of age, showed hypomyelina-tion with hyperintense T2-signal in the internal capsules, deep white matter and cerebellar hypoplasia. Genetic testing by whole exome sequencing confirmed a mutation in FIG4. Patient B was born after IVF to unrelated caucasian parents. He had global delay with four limb hypotonia and difficult to elicit reflexes, suggesting a peripheral neuropathy. He had a squint and myopic vision. At around 34 months of age, he walks with his hands held and babbles. Serial MRI at 10-months and 27-months showed hypomyelination throughout the subcortical white matter with increased T2 signal in the internal capsule and cerebellar hypoplasia. Genetic testing confirmed a FIG4 mutation. Discussion: FIG4 encodes Sac-3 protein, a phosphatase involved in vesicle trafficking. In the mouse model of Null-FIG4, hypomyelination of the brain and spinal cord occurs in addition to peripheral neuropathy seen in humans. Transgenic mice with overexpression of human pathogenic FIG4-141T variant have protection from the hypomyelination. To our knowledge, these cases are the first evidence of hypomyelina-tion seen in the human phenotype of FIG4. Functional studies on fibroblasts are in progress to confirm this further.
Keywords: endogenous compound; phosphatase; animal experiment; animal model; Asian; capsula interna; case study; Caucasian; cerebellum hypoplasia; demyelinating neuropathy; developmental delay; face; fibroblast; gaze; gene overexpression; genetic screening; gesture; human; limb; male; mouse; mouse model; muscle hypotonia; mutation; myopia; neurophysiology; nonhuman; nuclear magnetic resonance imaging; peripheral neuropathy; phenotype; reflex; strabismus; tracheostomy; transgenic mouse; vision; weakness; wheelchair; white matter; whole exome sequencing

Transpedicular direct osteosynthesis of hangman's fracture from a mini-open exposure as a less invasive procedure: A technical note - Kovari V.Z.; Josvai A.; Csokay A. (Trauma Case Reports, 2017)
This surgical technical case report presents initial clinical experience and preliminary results with a less invasive surgical solution for selected hangman's fracture. A well-known stabilization technique (i.e. direct transpedicular osteosynthesis) was applied through a minimally invasive small incision transmuscular posterior approach guided by a standard C-arm fluoroscopy. This mini-open approach to C2 vertebra allows similar dissection, visualization of the bony landmarks, visual control of the transpedicular screw path drilling, tapping and screw insertion to the standard posterior cervical spine approach. At the same time it has the benefits of less invasive procedures.
Keywords: bone screw; pedicle screw; retractor; surgical wire; adult; article; bleeding; case report; clinical article; componet fracture; computer assisted tomography; disease classification; female; fluoroscopy; fracture; grip strength; hangman fracture; human; Kirschner wire; Levine Edward classification; medical history; neurologic disease; nuclear magnetic resonance imaging; open fracture; operation duration; osteosynthesis; priority journal; second cervical vertebra; semispinalis capitis muscle; spinal cord compression; spine fracture; spine stabilization; splenius capitis; spondylolisthesis; surgical technique; tracheostomy; traffic accident; transpedicular direct osteosynthesis; trapezius muscle; treatment outcome; zygapophyseal joint

Isolated C5 vertebrae dislocation with trauma: An extremely rare case of isolated C5 dislocation - Özdoğan S.; Kaya M.; Demirel N.; Düzkalır A.H.; Yaltırık C.K. (American Journal of Case Reports, 2017)
Objective: Rare disease Background: Total spondylolisthesis, or dislocation of 1 cervical vertebrae, is only caused by high-energy trauma and is usually fatal. Cervical spine fractures and dislocations often cause 3-column structural damage to the cervical spine, injury to the spinal cord, and precipitating alignment of the cervical vertebrae, as well as cervical instability, which are detrimental, show poor prognosis, and are associated with high rates of mortality rate and disability. Case Report: We report an extremely rare case of isolated C5 dislocation caused by falling out of a tree, with sudden tetraplegia. Conclusions: Total spondylolisthesis or dislocation of 1 cervical vertebrae can be surgically treated with anterior approach because it is possible to completely remove the vertebra body, intervertebral disc, and bone fragments, to directly decompress the spinal cord with stabilization.
Keywords: dissector; urinary catheter; ventilator; adult; artery injury; article; artificial ventilation; bone graft; case report; clinical article; computed tomographic angiography; computer assisted tomography; emergency health service; falling; female; fracture fixation; human; intensive care unit; liquorrhea; neurologic examination; nuclear magnetic resonance imaging; plate fixation; positive end expiratory pressure; postoperative period; quadriplegia; spinal hematoma; spine injury; tracheostomy; urine incontinence; vertebra dislocation; vertebral artery

Transoral robotic-assisted surgery for the approach to anterior cervical spine lesions - Molteni G.; Greco M.G.; Presutti L. (European Archives of Oto-Rhino-Laryngology, 2017)
The Da Vinci robotic surgical system is increasingly being used by head and neck surgeons in transoral approaches for head and neck cancer. Our experience using the Da Vinci system for transoral robotic-assisted surgery (TORS) is presented. The feasibility of TORS for lesions involving the anterior portion of C1–C2 and the cranio-cervical junction has been evaluated from an anatomical viewpoint in a cadaveric laboratory. Two patients treated using the Da Vinci system to reach C1–C2 benign lesions are presented. The anatomical cadaveric study showed that this approach is safe and feasible. The first two cases which we describe confirmed the advantages of the Da Vinci system in the anterior approach to the cervical spine and allowed the limitations of this procedure to be assessed. TORS may be useful to reach anterior lesions of the cervical spine localized at the level of C1 and C2: first, for removal of small benign and well-delineated lesions; and second, for diagnostic purposes with biopsy of large lesions. Further studies and new instruments are needed to confirm the safety and results of this approach in terms of morbidity.
Keywords: barbed suture; bone drill; Da Vinci; forceps; laparoscopic surgical instrument; laparoscopic video camera; nasogastric tube; silastic tube; absorbable barbed suture; aged; antibiotic therapy; article; atlantooccipital joint; cadaver; case report; cervical spine; cervical spine radiography; clinical article; computer assisted tomography; diarrhea; feasibility study; female; fibrocartilage; histopathology; human; human tissue; limb weakness; neuroimaging; nuclear magnetic resonance imaging; operation duration; osteodystrophy; paresthesia; postoperative infection; priority journal; robot assisted surgery; spinal cord decompression; spine disease; surgical approach; surgical technique; tracheotomy; transoral robotic assisted surgery; tube removal; vomiting; Da Vinci; V-loc

Spinal muscular atrophy carriers with two SMN1 copies - Ar Rochmah M.; Awano H.; Awaya T.; Harahap N.I.F.; Morisada N.; Bouike Y.; Saito T.; Kubo Y.; Saito K.; Lai P.S.; Morioka I.; Iijima K.; et al. (Brain and Development, 2017)
Background Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder. Over 95% of SMA patients have homozygous deletions of the SMA-causative gene, SMN1. Thus, SMA carriers are usually diagnosed based on SMN1 copy number, with one copy indicating SMA carrier status. However, two SMN1 copies do not always exclude carrier status. In this study, we identified SMA carriers with two SMN1 copies. Subjects and methods From 33 families, 65 parents of genetically confirmed SMA patients were tested to determine SMA carrier status. Molecular genetic analyses, including multiplex ligation-dependent probe amplification (MLPA) assay, were performed using blood samples from family members. Results Of the 65 parents, three parents from three families had two SMN1 copies. Accordingly, the frequency of carriers with two SMN1 copies was 4.6%. Two of these families were further studied. Patient 1 was homozygous for SMN1 deletion. Patient 1's mother had two SMN1 copies on one chromosome, with deletion of SMN1 on the other chromosome ([2 + 0] genotype). Patient 1 inherited SMN1-deleted chromosomes from both parents. Patient 2 was compound heterozygous for two SMN1 mutations: whole-gene deletion and intragenic missense mutation, c.826T > C (p.Tyr276His). Patient 2's father had two SMN1 copies with the same intragenic mutation in one copy ([1 + 1d] genotype, d intragenic mutation). Patient 2 inherited the chromosome with an SMN1 mutation from the father and SMN1-deleted chromosome from the mother. Conclusion SMA carriers with two SMN1 copies may be rare, but its possibility should be taken into consideration in carrier testing and counseling for SMA families or population-based carrier screening.
Keywords: genomic DNA; survival motor neuron protein 1; agar gel electrophoresis; allele; Apgar score; article; assisted ventilation; birth weight; bradycardia; chromosome deletion; disease course; female; gene amplification; gene conversion; gene deletion; gene mutation; genetic analysis; heterozygosity; human; infant; major clinical study; missense mutation; multiplex ligation dependent probe amplification; muscle hypotonia; nucleotide sequence; pneumonia; point mutation; polymerase chain reaction; real time polymerase chain reaction; respiratory failure; restriction fragment length polymorphism; spinal muscular atrophy; tracheotomy

One-year outcomes following treatment with nusinersen: Interim results from the NURTURE study of presymptomatic infants with genetically diagnosed spinal muscular atrophy (SMA) - De Vivo D.; Bertini E.; Hwu W.-L.; Foster R.; Gheuens S.; Farwell W.; Reyna S. (Annals of Neurology, 2017)
Objective: NURTURE is an ongoing phase 2, open-label, single-arm study, evaluating intrathecal nusinersen (12-mg equivalent dose) in infants with presymptomatic SMA. Methods: Enrolled infants were presymptomatic at screening, age ≤6 weeks at first dose, had genetic diagnosis of SMA, and 2 or 3 SMN2 copies. The primary endpoint is time to death or respiratory intervention (tracheostomy/ventilation for ≥6 hours/day for ≥7 days). Secondary end-points include: proportion of infants developing protocol- (Table Presented) defined SMA symptoms (defined by age-adjusted weight, g-tube placement or failure to achieve appropriate WHO motor milestones), motor milestone attainment assessed by Hammersmith Infant Neurological Examination (HINE) Section 2 and change in Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) scale. Outcomes for infants completing Day365 visit are reported. Results: As of 31 Oct 2016, 20 infants have enrolled; 9 have completed Day365. After 1 year, no infants died or required respiratory intervention. All infants (9/9) achieved expected HINE motor milestones for healthy infants based on age in the categories ability to kick and head control, 7/9 achieved rolling, 6/9 sitting, 5/9 crawling, 5/9 walking, and 3/9 standing (Table). Three infants achieved all HINE motor milestones expected for age. Median total CHOP INTEND score was 62.0 (range:44-64) points at Day365. Four infants (all 2 SMN2 copies) exhibited protocol-defined SMA symptoms at Day365, 1 lost weight between Day184-365; however, all 4 were sitting without support. Conclusions: These results suggest that early intervention with nusinersen, before symptom onset, provides clinical benefits in infants with genetically diagnosed SMA.
Keywords: endogenous compound; nusinersen; survival motor neuron protein 2; child; clinical article; conference abstract; controlled study; death; diagnosis; drug therapy; early intervention; female; head; human; infant; male; mental capacity; neurologic examination; neurology; Pennsylvania; spinal muscular atrophy type 2; tracheostomy; walking

Analysis of a series of patients with acute flaccid myelitis (AFM) Presenting to a Tertiary Care Referral Center - Kuwabara N.; Shah S.; Igarashi M.; Jack R.; Karmarkar S.; Caron E.; Bhattarai P.; Shah N. (Annals of Neurology, 2017)
Objective: Acute Flaccid Myelitis (AFM) affects the spinal cord leading to serious flaccid neurologic sequelae. While Enterovirus D68 (EVD68) infections are commonly implicated, its overall management options are poorly described. We present the analysis of a series of children with AFM presenting to a mid-south tertiary care referral center. Methods: Retrospective chart review of six cases of AFM admitted from January 2016 to December 2016. Results: In our series only boys were affected; 50% were less than 6 years, with a mean age of 7.4 years. The peak incidence corresponds to CDC reports in September 2016. Clinical and/or radiographic abnormalities in the brainstem were present in 67% patients with 50% patients requiring mechanical ventilatory support. 67% patients could not ambulate at admission. A gastrostomy tube and tracheostomy was required in 33% patients. MRI abnormalities were predominantly noted in the cervical spinal cord in 83% patients. Peripheral nerve enhancement was determined in 33% patients. CSF pleocytosis was seen in 66%. Only two patients tested positive for EVD68 by nasopharyngeal PCR. All patients had residual neurological deficits at discharge despite immunomodulatory treatments including steroid, intravenous gamma globulin, plasma exchange. Four patients required further inpatient rehabilitation. Conclusions: This AFM case series demonstrates variable clinical presentations with brainstem and peripheral nerve involvement with residual neurological deficits. The role of immunomodulatory therapy in AFM requires further investigation. We recommend early recognition and prompt neuro-rehabilitation for patients with AFM to improve their long-term outcomes.
Keywords: human immunoglobulin; steroid; artificial ventilation; brain stem; case report; case study; cervical spinal cord; child; clinical article; conference abstract; congenital malformation; drug therapy; Enterovirus D68; hospital patient; human; incidence; infection; male; medical record review; myelitis; nasopharynx; neuroimaging; neuroimmunology; neurorehabilitation; nonhuman; nuclear magnetic resonance imaging; patient referral; peripheral nerve; plasma exchange; pleocytosis; polymerase chain reaction; rehabilitation; retrospective study; school child; stomach tube; tertiary health care; tracheostomy

The Experience of Families with Children with Spinal Muscular Atrophy Type i Across Health Care Systems - Murrell D.V.; Lotze T.E.; Farber H.J.; Crawford C.A.; Wiemann C.M. (Journal of Child Neurology, 2017)
Spinal muscular atrophy type I is a genetic disease characterized by degeneration of spinal cord motor neurons resulting in weakness, technology dependence and early demise. While the newly approved treatment nusinersen may alter the morbidity/mortality of this disease there continues to be complex treatment challenges to consider. The aim of this qualitative study was to understand from the parent's perspective, experiences of the family and child in the emergency center, hospital, and clinical care settings to identify gaps in care. Nineteen families interviewed had 22 children with spinal muscular atrophy I (11 deceased, 11 living). Three overarching themes emerged from parent interviews describing a range of experiences surrounding diagnosis, informed medical decision making and acute care practice. Identified quality improvements include development of a diagnostic screening tool, a medical decision tool, and emergency center informational template individualized to the child and providing an overview of spinal muscular atrophy I.
Keywords: nusinersen; adolescent; adult; article; bulbar paralysis; child; child experience; child parent relation; controlled study; environment; experience; family counseling; family experience; female; health care personnel; health care system; human; infant; long term care; male; medical decision making; palliative therapy; priority journal; quality of life; respiratory distress; tracheostomy; Werdnig Hoffmann disease

Risk factors for severe dysphagia in acute cervical spinal cord injury - Hayashi T.; Fujiwara Y.; Sakai H.; Maeda T.; Ueta T.; Shiba K. (Spinal Cord, 2017)
Study design:A retrospective, consecutive case series.Objectives:The relationship between dysphagia and acute cervical spinal cord injury (CSCI) has been recently reported; however, the cause and mechanism of dysphagia are still not well understood. No definitive factors have yet been established according to multivariate analysis. The objective is to elucidate the incidence and risk factors of dysphagia in patients with acute CSCI.Setting:Spinal Injuries Center, Fukuoka, Japan.Methods:A total of 298 patients with acute CSCI, who were evaluated for neurological impairment within 3 days after injury, were reviewed. CSCI patients with tube dependence due to obvious aspiration after injury were defined as having dysphagia. The factors postulated to increase the risk for dysphagia, including the patient's age, sex, American Spinal Injury Association (ASIA) impairment scale at 3 days after injury, level of injury, tracheostomy and operative treatment, were analyzed using a multiple logistic regression model.Results:Of 298 patients, 21 were suffering from severe dysphagia after acute CSCI (7.0%). Of these 21 patients, 12 (57%) had CSCI at the C3-C4 level. Multivariable logistic regression analysis revealed that old age (>72 years), severe ASIA impairment scale (A or B) and presence of tracheostomy were significant risk factors of dysphagia. Level of injury ≥C3-C4 was not a significant risk factor after adjustment for several potential confounders.Conclusion:The incidence of severe dysphagia associated with aspiration was 7%. Old age, severe paralysis and presence of tracheostomy may be the risk factors for dysphagia. The risk for dysphagia should be evaluated to prevent aspiration pneumonia.
Keywords: adult; aged; American Spinal Injury Association impairment scale; article; case study; cervical spinal cord injury; dysphagia; female; human; Japan; major clinical study; male; priority journal; retrospective study; risk factor; tracheostomy

Variation in definition of prolonged mechanical ventilation - Rose L.; McGinlay M.; Amin R.; Burns K.E.; Connolly B.; Hart N.; Jouvet P.; Katz S.; Leasa D.; Mawdsley C.; McAuley D.F.; Schultz M.J.; et al. (Respiratory Care, 2017)
Consistency of definitional criteria for terminology applied to describe subject cohorts receiving mechanical ventilation within ICU and post-acute care settings is important for understanding prevalence, risk stratification, effectiveness of interventions, and projections for resource allocation. Our objective was to quantify the application and definition of terms for prolonged mechanical ventilation. We conducted a scoping review of studies (all designs except single-case study) reporting a study population (adult and pediatric) using the term prolonged mechanical ventilation or a synonym. We screened 5,331 references, reviewed 539 full-text references, and excluded 120. Of the 419 studies (representing 38 countries) meeting inclusion criteria, 297 (71%) reported data on a heterogeneous subject cohort, and 66 (16%) included surgical subjects only (46 of those 66, 70% cardiac surgery). Other studies described COPD (16, 4%), trauma (22, 5%), neuromuscular (17, 4%), and sepsis (1, 0.2%) cohorts. A total of 741 terms were used to refer to the 419 study cohorts. The most common terms were: prolonged mechanical ventilation (253, 60%), admission to specialized unit (107, 26%), and long-term mechanical ventilation (79, 19%). Some authors (282, 67%) defined their cohorts based on duration of mechanical ventilation, with 154 studies (55%) using this as the sole criterion. We identified 37 different durations of ventilation ranging from 5 h to 1 y, with > 21 d being the most common (28 of 282, 7%). For studies describing a surgical cohort, minimum ventilation duration required for inclusion was ≥ 24 h for 20 of 66 studies (30%). More than half of all studies (237, 57%) did not provide a reason/rationale for definitional criteria used, with only 28 studies (7%) referring to a consensus definition. We conclude that substantial variation exists in the terminology and definitional criteria for cohorts of subjects receiving prolonged mechanical ventilation. Standardization of terminology and definitional criteria is required for study data to be maximally informative.
Keywords: article; artificial ventilation; brain injury; chronic obstructive lung disease; clinical outcome; hemodynamics; hospital admission; hospital mortality; human; intubation; length of stay; neuromuscular disease; quality of life; sepsis; spinal cord injury; tracheostomy; ventilator weaning

Airway Management Protocol after Anterior Cervical Spine Surgery - Kim M.; Choi I.; Park J.H.; Jeon S.R.; Rhim S.C.; Roh S.W. (Spine, 2017)
Study Design. Retrospective comparative cohort analysis. Objective. To evaluate the effect of postoperative airway management protocol (ASAN Extubation Protocol, AEP) on incidence of airway complications for patients undergoing anterior cervical spine surgery (ACSS). Background. Postoperative airway compromise remains crucial for patients undergoing ACSS. Despite the potential severity of these complications, the data in the published literature addressing this issue is sparse. Methods. A retrospective cohort study was performed regarding airway complications (postoperative airway edema requiring unplanned reintubation or tracheostomy) between groups of patients undergoing ACSS before and after applying our standardized protocol (AEP) for postoperative extubation. The AEP was developed based on 5 clinical risk factors reported having relation to airway complication. Postoperative patients with any oneor more risk factors were kept intubated for at least overnight and extubation was conducted according to the amount of prevertebral soft tissue swelling. Results. A total of 538 ACSS patients were identified from 2008 to 2016. The nonprotocol group (before protocol application, 275 patients) and the Protocol group (after protocol, 263 patients) were compared; airway complication rates were significantly different between two groups (nonprotocol: 3.64% (10/275) vs. protocol: 0.76% (2/263), P=0.024). The possible factors that may increase airway complication include operative indications (P=0.002), trauma (P=0.000), medical comorbidity risk (P=0.011), combined anterior and posterior surgery (P=0.002), and operation time longer than 5hours (P=0.045). In multivariate analysis, medical comorbidity risk, trauma, and airway protocol adoption were significant factors. AEP reduced the airway complication rate by odds ratio 0.125 (P=0.013). Conclusion. Postoperative airway complication is not very common after ACSS. AEP contributed to reduce the incidence of airway complications. The potentially life-threatening event of loss of airway patency, even though it is a rare complication, should be cautiously analyzed with identification of risk factors before the surgery.
Keywords: adult; age; anterior spine fusion; article; body mass; clinical protocol; cohort analysis; comorbidity; controlled study; female; human; injury; major clinical study; male; medical history; middle aged; operation duration; postoperative care; postoperative complication; postoperative edema; priority journal; respiration control; respiratory failure; retrospective study; risk assessment; sex difference; smoking; standardization; surgical technique; therapy effect; tracheostomy; treatment indication; treatment outcome

Classification and regression tree model for predicting tracheostomy in patients with traumatic cervical spinal cord injury - Lee D.-S.; Park C.-M.; Carriere K.C.; Ahn J. (European Spine Journal, 2017)
Purpose: In patients with cervical spinal cord injury (CSCI), respiratory compromise and the need for tracheostomy are common. The purpose of this study was to identify common risk factors for tracheostomy following traumatic CSCI and develop a decision tree for tracheostomy in traumatic CSCI patients without pulmonary function test. Methods: Data of 105 trauma patients with CSCI admitted in our institution from April, 2008 to February, 2014 were retrospectively analyzed. Patients who underwent tracheostomy were compared to those who did not. Stepwise logistic regression analysis and classification and regression tree model were used to predict the risk factors for tracheostomy. Results: Tracheostomy was performed in 20% of patients with traumatic CSCI on median hospital day 4. Patients who underwent tracheostomy tended to be more severely injured (higher Injury Severity Score, lower Glasgow Coma Score, and lower systolic blood pressure on admission) which required more frequent intubation in the emergency room (ER) with a higher rate of complete CSCI compared to those who did not. Upon multiple logistic analysis, Age ≥ 55 years (OR: 6.86, p = 0.037), Car accident (OR: 5.8, p = 0.049), injury above C5 (OR: 28.95, p = 0.009), ISS ≥ 16 (OR: 12.6, p = 0.004), intubation in the ER (OR: 23.87, p = 0.001), and complete CSCI (OR: 62.14, p < 0.001) were significant predictors for the need of tracheostomy after CSCI. These factors can predict whether a new patient needs future tracheostomy with 91.4% accuracy. Conclusions: Age ≥ 55 years, injury above C5, ISS ≥ 16, Car accident, intubation in the ER, and complete CSCI were independently associated with tracheostomy after CSCI. CART analysis may provide an intuitive decision tree for tracheostomy.
Keywords: abdominal injury; adult; article; cervical spinal cord injury; comparative study; controlled study; decision tree; disease classification; emergency ward; female; Glasgow coma scale; hospital admission; hospital discharge; hospitalization; human; injury scale; injury severity; intubation; logistic regression analysis; lung complication; major clinical study; male; motor dysfunction; pneumonia; postoperative complication; prediction; priority journal; retrospective study; surgical patient; surgical risk; systolic blood pressure; thorax injury; tracheostomy; traumatic brain injury

Dysphagia in acute cervical spinal cord injury-development of a screening tool through a Delphi process of expert consensus - McRae J.; Smith C.; Beeke S.; Emmanuel A. (Intensive Care Medicine Experimental, 2017)
INTRODUCTION. Dysphagia in cervical spinal cord injury (CSCI) is associated with respiratory complications, increased length of stay and mortality risks [1]. The cause is multi-factorial with subtle clinical presentation [2]. Early dysphagia identification helps to prevent complications (including silent aspiration) however a lack of national guidance and effective screening methods has led to varied clinical practices in non-specialised units resulting in poor outcomes [3]. OBJECTIVES. To use a Delphi technique, gathering expert consensus on risk factors for dysphagia, to develop a screening tool and clinical recommendations for critical care healthcare professionals. METHODS. Following a literature review, 85 statements were generated across seven domains: co-morbid factors, definition, screening, assessment, identification, management, to form the first round of a Delphi. A multi-disciplinary international expert panel of 27 clinicians ranked the statements using a 5 point Likert scale. Statements not achieving consensus of >70% of the panel were modified and recirculated for a subsequent round until levels of agreement were static. Participants received individual feedback at the end of each round detailing their response compared to the group's response. RESULTS. Following two rounds of the Delphi, 62 statements achieved consensus (round 1, 59%; round 2, 48%) with statements for dysphagia screening and assessment achieving the least consensus. Based on the results, a screening tool was developed to identify dysphagia risks with 3 domains: injury risk (brain injury, cervical surgery, spinal injury level and severity); clinical risk (intubation, tracheostomy, ventilation, and nutrition) and urgency (chest infection, pyrexia, oral care and suction). A set of clinical recommendations provided additional guidance on optimal respiratory and nutritional management. CONCLUSIONS. This Delphi study achieved multi-professional consensus on factors contributing to dysphagia risk in CSCI allowing development of a screening tool. This will aid referral to Speech and Language Therapy for prompt and consistent diagnostic assessment and intervention in non-specialised units. A future multi-site pilot study of the tool is necessary to validate its utility and impact on patient outcomes.
Keywords: adult; artificial ventilation; aspiration; brain injury; cervical spinal cord injury; chest infection; clinical practice; complication; controlled study; Delphi study; dysphagia; female; fever; human; intubation; Likert scale; mouth hygiene; multicenter study; nutrition; patient referral; pilot study; prevention; risk factor; speech and language rehabilitation; suction; surgery; tracheostomy

Risk Analyses of Pressure Ulcer in Tetraplegic Spinal Cord-Injured Persons: A French Long-Term Survey - Le Fort M.; Espagnacq M.; Perrouin-Verbe B.; Ravaud J.-F. (Archives of Physical Medicine and Rehabilitation, 2017)
Objective To identify the long-term clinical, individual, and social risk factors for the development of pressure ulcers (PUs) in traumatic spinal cord–injured persons with tetraplegia (TSCIt). Design Cohort survey with self-applied questionnaires in 1995 and 2006. Setting Thirty-five French-speaking European physical medicine and rehabilitation centers participating in the Tetrafigap surveys. Participants Tetraplegic adults (N=1641) were surveyed after an initial posttraumatic period of at least 2 years. Eleven years later, a follow-up was done for 1327 TSCIt, among whom 221 had died and 547 could be surveyed again. Interventions Not applicable. Main Outcome Measures The proportion of PUs documented at the various defined time points, relative to the medical and social situations of the TSCIt, by using univariate analyses followed by logistic regression. Results Of the participants, 73.4% presented with a PU during at least 1 period after their injury. Four factors had an effect on the occurrence of PUs in the long-term. Protective features for this population were incomplete motor impairment (odds ratio, 0.5) and the ability to walk (odds ratio, 0.2), whereas a strong predictive factor was the development of a PU during the initial posttrauma phase (odds ratio, 2.7). Finally, a significant situational factor was the lack of a social network (odds ratio, 3.1). Conclusions We believe that the highlighting of a motor incomplete feature of SCI (protective against the development of a PU) and of a medical risk factor, an early PU (which served as a definitive marker of the trajectory of TSCIt), together with a social situational factor, indicates the crucial role of initial management and long-term follow-up.
Keywords: adult; article; brain injury; cohort analysis; decubitus; employment status; female; follow up; French (language); home accident; human; longitudinal study; major clinical study; male; marriage; motor dysfunction; quadriplegia; risk assessment; risk factor; sensory dysfunction; social network; social participation; spinal cord injury; sport injury; tracheotomy; traffic accident; treatment duration

Bilateral vocal cord paralysis caused by hanging - Lerrick A.J. (Otolaryngology - Head and Neck Surgery (United States), 2017)
Objectives: Death by hanging is typically accomplished by severing the spinal cord or by strangulation. A patient's failed suicide attempt by hanging enabled hospital care to be rendered. Bilateral vocal cord paralysis was identified, possibly the first reported case of this entity. Methods: A 56-year-old man was found unconscious following an attempted suicide by hanging. Cardiopulmonary resuscitation restored spontaneous circulation. The preliminary diagnosis was asphyxiation. A 9-cm-long 4-mm-wide excoriation extended from the left anterior neck to the right posterior triangle, overlying the inferior thyroid cartilage. It was postulated that his neck had been flexed rightward and hyperextended on the left. Imaging revealed normal cervical spine alignment and cervical soft-tissue edema, R > L. An angiogram was unremarkable. Early extubation failed because of stridor and dyspnea but was successful at 1 week. The patient had inspiratory > expiratory stridor. His voice was hypophonic and raspy. Laryngoscopy identified right vocal cord paralysis (0/4) and left vocal cord paresis (<1/4). Both cords were medialized. A tracheotomy secured the patient's airway. Three months postinjury, vocal cord function was fully recovered, permitting decannulation. Results: Clinical findings suggested compression of the right and stretching of the left vagus nerves. It is entirely possible that the right recurrent laryngeal nerve was compressed as it entered the larynx at the cricothyroid articulation and that the left recurrent laryngeal nerve was stretched at the same anatomic level. Conclusions: The vagus nerves may have been more resilient when injured than the more deeply situated recurrent laryngeal nerves regardless of the mechanism.
Keywords: adult; case report; cervical spine; diagnosis; dyspnea; edema; ego development; extubation; female; human; human tissue; laryngoscopy; male; middle aged; recurrent laryngeal nerve; remission; resuscitation; soft tissue; stridor; suffocation; suicide attempt; thyroid cartilage; tracheostomy; vagus nerve; vocal cord paralysis; voice

PM and R consults for combined TBI/sci patients in the shock trauma ICU impact ventilation days, morbidity, and disposition: A retrospective study - Linville C.R.; Morris M.; Chacko J.J.; Verduzco-Gutierrez M.; Stampas A. (PM and R, 2017)
Objective: Determine differences in characteristics, complications, length of stay and disposition for combined traumatic brain injury and spinal cord injury (SCI) patients admitted to the shock trauma intensive care unit (STICU) who received and did not receive a physical medicine and rehabilitation (PMR) consult. Design: A retrospective clinical study was conducted of acute trauma patients admitted to the STICU at a Level 1 trauma center with acute care stay days of at least 14 days. Patients admitted from January 1, 2013, through December 31, 2013, who were 16 years of age or older with a spinal cord injury diagnosis were included in this study. General characteristics, complications and disposition were then recorded and analyzed for these patients. Setting: Level I Trauma Center. Participants: 36 patients met inclusion criteria. Interventions: Not applicable. Main Outcome Measures: Demographics, characteristics, complications, length of stay, disposition. Results: 36 patients had a combined TBI/SCI. There were no significant differences in patient characteristics if PMR was or was not consulted. Complications for combined TBI/SCI patients with a PMR consult, compared to those without a PMR consult e dysphagia requiring PEG/PEJ placement: 15 vs. 0 (p=.01). There were no other significant differences in other complications including pneumonia, UTI, PE, DVT, or tracheostomy placement. There were no significant differences in length of stay, however, patients with a PMR consult had more ventilation free days compared to those without a PMR consult: 13.5 vs. 8.5 (p=.04). Disposition for combined TBI/SCI patients with a PMR consult, compared to those without a PMR consult-Inpatient rehabilitation: 14 vs. 0 (p=.01); LTAC: 5 vs. 4 (p=.08); SNF: 3 vs. 0 (p=1); home: 3 vs. 2 (p=.1). Conclusions: This retrospective study shows combined TBI/SCI patients with a PMR consult were more likely to require gastrostomy tube placement, had more ventilation free days, and were more likely to discharge to inpatient rehabilitation.
Keywords: adult; artificial ventilation; clinical article; complication; conference abstract; controlled study; diagnosis; dysphagia; emergency care; emergency health service; female; hospital patient; human; intensive care unit; length of stay; male; morbidity; outcome assessment; pneumonia; rehabilitation; rehabilitation medicine; retrospective study; spinal cord injury; stomach tube; tracheostomy

Percutaneous tracheostomy: A comprehensive review - Rashid A.O.; Islam S. (Journal of Thoracic Disease, 2017)
Tracheostomy is a common procedure. It can be done surgically or percutaneously by dilating the stoma using Seldinger technique. Percutaneous tracheostomy (PT) is now routinely performed by surgeons and non-surgeons such as intensivists and anesthesiologists in the intensive care units (ICU) all over the world. Although obesity, emergent tracheostomy, coagulopathy, inability to extend the neck and high ventilator demand (HVD) were initially thought to be a relative contraindication, recent data suggest safety of PT in these patient population. Ultrasound can be helpful in limited cases to identify the neck structure especially in patients with a difficult anatomy. Bronchoscopy during PT can shorten the duration and avoid complications. PT has favorable complication rate, lower infection rate, shorter procedural duration and is cost-effective. Experience with the technique and careful planning is needed to minimize any avoidable potential complication.
Keywords: tracheostomy kit; Portex ULTRAperc; adult respiratory distress syndrome; artificial ventilation; blood clotting disorder; bronchoscopy; cannulation; complication; endotracheal tube; human; hypotension; intermethod comparison; medical education; nonsurgical invasive therapy; obesity; open surgery; percutaneous tracheostomy; positive end expiratory pressure; review; safety; spinal cord injury; suture; tracheal dilator; tracheostomy; tracheostomy tube; treatment contraindication; treatment indication; ultrasound; Ciaglia Blue Rhino; Portex ULTRAperc

Tandem spinal stenosis a systematic review - Overley S.C.; Kim J.S.; Gogel B.A.; Merrill R.K.; Hecht A.C. (JBJS Reviews, 2017)
Background: Tandem spinal stenosis refers to spinal canal diameter narrowing in at least 2 distinct regions of the spine, most commonly the lumbar and cervical regions. This entity can be an asymptomatic radiographic finding, or it can present with severe myelopathy and lower-extremity symptoms. Tandem spinal stenosis may impact surgeon decision-making when planning either cervical or lumbar spine surgery, and there is currently no consensus in the literature regarding the treatment algorithm for operative intervention. Methods: A MEDLINE literature search was performed using PubMed, the Cochrane Database of Systematic Reviews, and Embase from January 1980 to February 2015 using Medical Subject Heading queries for the terms “tandem spinal stenosis,” “cervical stenosis AND lumbar stenosis,” and “concomitant spinal stenosis.” We included studies involving adult patients, tandem spinal stenosis of the cervical and lumbar regions, and a minimum of 5 patients. Articles that did not discuss spinal disorders or only explored disorders at a single spinal region were excluded. Results: The initial database review resulted in 234 articles. After abstracts were reviewed, only 17 articles that met inclusion criteria were identified: 2 cadaveric studies, 5 clinical studies of patients with radiographic tandem spinal stenosis, and 10 clinical studies of patients with symptomatic tandem spinal stenosis. Conclusions: Tandem spinal stenosis is a common condition present in up to 60% of patients with spinal stenosis. This disorder, however, is often overlooked, which can lead to serious complications. Identification of tandem spinal stenosis is paramount as a first step in management and, although there is still no preferred intervention, both staged and simultaneous procedures have been shown to be effective. Surgeons may utilize a single, staged, or combined approach to decompression, always addressing cervical myelopathy as a priority.
Keywords: bibliographic database; bone radiography; cauda equina; cervical myelopathy; clinical outcome; decompression; hematoma; human; morphology; ossification; patient positioning; quality control; reoperation; review; septicemia; spondylolisthesis; spondylosis; systematic review; tracheostomy; vertebral canal stenosis; wound infection

Reconstruction of late esophagus perforation after anterior cervical spine fusion with an adipofascial anterolateral thigh free flap: A case report - Coelho R.; Ekberg T.; Svensson M.; Mani M.; Rodriguez-Lorenzo A. (Microsurgery, 2017)
Reconstruction of late esophageal perforation usually requires flap surgery to achieve wound healing. However, restoring the continuity between the digestive tract and retropharyngeal space to allow for normal swallowing remains a technical challenge. In this report, we describe the use of a thin and pliable free adipofascial anterolateral thigh (ALT) flap in a 47-year-old tetraplegic man with a history of C5–C6 fracture presented with a large posterior esophagus wall perforation allowing an easier flap insetting for a successful wound closure. The postoperative course was uneventful and mucosalization of the flap was confirmed by esophagoscopy 4 weeks postsurgery. The patient tolerated normal diet and maintained normal swallowing during a follow-up of 3 years postoperatively. The adipofascial ALT flap may provide easier insetting due to the thin and pliable layer of adipofascial tissue for reconstructing large defects of the posterior wall of the esophagus by filling the retroesophageal space.
Keywords: barium; adipofascial flap; adult; anastomosis; anterior spine fusion; anterolateral thigh flap; article; case report; cervical spine; clinical article; computer; debridement; esophagoscopy; esophagus perforation; esophagus reconstruction; feeding apparatus; free tissue graft; human; male; medical history; middle aged; nutrition; percutaneous endoscopic gastrostomy; priority journal; quadriplegia; reconstructive surgery; spine fracture; swallowing; tracheostomy; wound healing

Reoperative repair of descending thoracic and thoracoabdominal aneurysms - Lau C.; Gaudino M.; Gambardella I.; Mills E.; Munjal M.; Elsayed M.; Girardi L. (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2017)
OBJECTIVES: To evaluate the results of reoperation on descending thoracic and thoracoabdominal aneurysms. METHODS: Sixty-nine consecutive patients undergoing reoperative aneurysm repair (20 descending thoracic and 49 thoracoabdominal) were compared to 602 contemporary primary repairs. Propensity matching was used to reduce observable differences in preoperative characteristics. RESULTS: The reoperation group was younger (60.2 vs 65.3 years, P = 0.005) and less were extent I or II (28.6% vs 76%, P < 0.001). In the reoperation group, 82.6% were repaired with clamp-and-sew, 14.5% circulatory arrest and 2.9% partial bypass versus the primary surgery group 62.1%, 8.1% and 29.7%, respectively (P < 0.001). In the reoperation versus primary surgery group, respectively, spinal drainage was used in 73.9% vs 83.7% (P = 0.05), intercostal reimplantation in 11.6% vs 44.2% (P < 0.001), and cold renal perfusion in 36.2% vs 19.8% (P = 0.001). Operative mortality was comparable (8.7% vs 5.3% primary, P = 0.25) but the reoperative extent I subgroup had higher mortality (20% vs 3.1%; P = 0.04). Incidence of major complications was comparable (stroke 0 vs 0.9%, tracheostomy 5.8% vs 8%, renal failure 7.2% vs 5%, spinal cord injury 4.3% vs 2.7%; P > 0.05 for all variables), with the exception of myocardial infarction (2.9% vs 0.5%, P = 0.028). Five-year survival was 57.6% in reoperations and 58% in the primary surgery group (P = 0.878). No differences in the in-hospital and follow-up outcomes were found in the propensity matched comparison. CONCLUSIONS: Reoperative repair of descending thoracic and thoracoabdominal aneurysms can be safely performed with reasonable in-hospital and follow-up outcomes compared to primary aneurysm repair.
Keywords: abdominal aortic aneurysm; aged; cause of death; dissecting aneurysm; female; follow up; hospital mortality; human; incidence; male; middle aged; mortality; New York; postoperative complication; procedures; reoperation; retrospective study; survival rate; thoracic aorta aneurysm; trends; vascular surgery

Lysosomal Storage Diseases: Past, Present, and Future - Richtsfeld M.; Belani K.G. (Anesthesia and Analgesia, 2017)
Keywords: laryngeal mask; videolaryngoscope; lysosome enzyme; bone marrow transplantation; comorbidity; elective surgery; endotracheal intubation; enzyme replacement; human; lysosome storage disease; priority journal; review; spinal cord compression; tracheostomy

Successful Reinnervation of the Diaphragm After Intercostal to Phrenic Nerve Neurotization in Patients With High Spinal Cord Injury - Nandra K.S.; Harari M.; Price T.P.; Greaney P.J.; Weinstein M.S. (Annals of plastic surgery, 2017)
OBJECTIVE: Our objective in this study was to extend diaphragmatic pacing therapy to include paraplegic patients with high cervical spinal cord injuries between C3 and C5. INTRODUCTION: Diaphragmatic pacing has been used in patients experiencing ventilator-dependent respiratory failure due to spinal cord injury as a means to reduce or eliminate the need for mechanical ventilation. However, this technique relies on intact phrenic nerve function. Recently, phrenic nerve reconstruction with intercostal nerve grafting has expanded the indications for diaphragmatic pacing. Our study aimed to evaluate early outcomes and efficacy of intercostal nerve transfer in diaphragmatic pacing. METHODS: Four ventilator-dependent patients with high cervical spinal cord injuries were selected for this study. Each patient demonstrated absence of phrenic nerve function via external neck stimulation and laparoscopic diaphragm mapping. Each patient underwent intercostal to phrenic nerve grafting with implantation of a phrenic nerve pacer. The patients were followed, and ventilator dependence was reassessed at 1 year postoperatively. RESULTS: Our primary outcome was measured by the amount of time our patients tolerated off the ventilator per day. We found that all 4 patients have tolerated paced breathing independent of mechanical ventilation, with 1 patient achieving 24 hours of tracheostomy collar. CONCLUSIONS: From this study, intercostal to phrenic nerve transfer seems to be a promising approach in reducing or eliminating ventilator support in patients with C3 to C5 high spinal cord injury.
Keywords: adult; artificial ventilation; cervical vertebra; complication; diaphragm; follow up; human; innervation; intercostal nerve; male; nerve transplantation; paraplegia; phrenic nerve; procedures; respiratory failure; spinal cord injury; transplantation; treatment outcome

Open repair of chronic thoracic and thoracoabdominal aortic dissection using deep hypothermia and circulatory arrest - Corvera J.; Copeland H.; Blitzer D.; Hicks A.; Manghelli J.; Hess P.; Fehrenbacher J. (Journal of Thoracic and Cardiovascular Surgery, 2017)
Background Chronic dissection of the thoracic and thoracoabdominal aorta as sequela of a prior type A or B dissection is a challenging problem that requires close radiographic surveillance and prompt operative intervention in the presence of symptoms or aneurysm formation. Open repair of chronic thoracic and thoracoabdominal aortic dissection using deep hypothermia has been our preferred method to treat this complex pathology. The advantages of this technique include organ and spinal cord protection, the flexibility to extend the repair proximally into the arch, and the ability to limit ischemia to all vascular beds. Methods Open repair of arch by left thoracotomy and descending thoracic and thoracoabdominal aortic pathology using deep hypothermia was performed in 664 patients from 1995 to 2015. A subset of this cohort had chronic thoracoabdominal aortic dissection (n = 196). All nonemergency cases received coronary angiography and echocardiography preoperatively. Significant coronary artery disease or severe aortic insufficiency was addressed before repair of the chronic dissection. In recent years, lumbar drains were placed preoperatively in the most extensive repairs (extents II and III). Important intercostal arteries from T8 to L1 were revascularized with smaller-diameter looped grafts. Multibranched grafts for the visceral segment have been preferred in recent years. Results Mean age of patients was 58 ± 14 years. Men comprised 74% of the cohort. Aortopathy was confirmed in 18% of the cohort. Prior thoracic aortic repair occurred in 57% of patients, and prior abdominal aortic repair occurred in 14% of patients. Prior type A aortic dissection occurred in 44% of patients, and prior type B occurred in 56% of patients. Operative mortality was 3.6%, permanent spinal cord ischemia occurred in 2.6% of patients, permanent hemodialysis occurred in 0% of patients, and permanent stroke occurred in 1% of patients. Reexploration for bleeding was 5.1%, and respiratory failure requiring tracheostomy occurred in 2.6%. Postoperative length of stay was 11.9 ± 9.7 days. Reintervention for pseudoaneurysm or growth of a distal aneurysm was 6.9%. The 1-, 5-, and 10-year survivals were 93%, 79%, and 57%, respectively. Conclusions Open repair of chronic thoracic and thoracoabdominal aortic dissection using deep hypothermia and circulatory arrest has low morbidity and mortality. The need for reintervention is low, and long-term survival is excellent. We believe that open repair continues to be the gold standard in patients who are suitable candidates for surgery.
Keywords: acute kidney failure; adult; aortic dissection; aortic reconstruction; aortic regurgitation; article; cardiopulmonary bypass; cerebrovascular accident; chronic disease; computer assisted tomography; coronary angiography; coronary artery disease; descending aortic surgery; echocardiography; false aneurysm; female; follow up; heart arrest; hemodialysis; hospitalization; human; hypothermia; length of stay; long term survival; major clinical study; male; middle aged; paraplegia; pneumonia; postoperative hemorrhage; priority journal; reoperation; respiratory failure; spinal cord ischemia; surgical mortality; survival; thoracic aortic dissection; thoracic aortic surgery; thoracoabdominal aorta aneurysm; thoracoabdominal aortic dissection; thoracotomy; tracheostomy

Adaptation of speech valve in child with spinal cord injury - Da Silva Freitas J.; Da Cunha Pereira G.; Dos Santos A.C.K.; Kurtz L.; De Oliveira A.C.; Garcez L.W. (International Archives of Otorhinolaryngology, 2017)
Introduction: Spinal cord trauma is an external caused injury in the spinal cord and leads to motor and sensory alterations. Tetraplegia comes up from lesions between the first and seventh cervical vertebrae. The speech valve allows the air to be exhaled by the upper airways, this mechanism allows the oral communication of the tracheostomized patient, facilitates the management of secretions through the training of the laryngeal function, reducing the need for aspirations. Objective: To report the process of speech valve adaptation in a quadriplegic child due to spinal cord trauma. Resumed report: Child with one year and eight months, quadriplegic due to spindle trauma, fracture in C2-C5 by stretching, with tracheostomy, using invasive mechanical ventilation.In process of adaptation with passy muir valve, model 007 for mechanical ventilation. That was realized seven training sessions with deflated cuff and peep equal to 0, showed lack of oxygenation and signs of air entrapment. After modified patient sitting position, using rigid vest and parameters of ventilator changed to peep of 3, greater expiratory time and lower respiratory rate, it allows the tolerance of the valve for a longer time, no longer showing signs of air entrapment. Conclusion: During the period using the valve, can be observed vocalizations of monosyllables and disyllabic, better handling of cough and the swallowing of secretions and saliva. The adaptation of this device aims to contribute to an improvement in breathing, phonation and deglutition mechanisms, directly impacting in the life quality of the patient.
Keywords: artificial ventilation; aspiration; bodily secretions; breathing rate; case report; cervical vertebra; child; clinical article; conference abstract; coughing; female; fracture; human; male; oxygenation; phonation; pneumatic cuff; quadriplegia; quality of life; salivation; spinal cord injury; swallowing; tracheostomy; upper respiratory tract; ventilator; vocalization; voice prosthesis

Oral ephedrine is useful to wean patients off long term parenteral vasopressors after cervical spinal cord injury - Khan M.F.; Siddiqui K.M.; Asghar M.A.; Ullah H. (Anaesthesia, Pain and Intensive Care, 2017)
Traumatic spinal cord injury (SCI) in young adults not only increases the risk of mortality but more commonly it complicates with life-long disability. Cervical SCI patients are particularly susceptible and sensitive to phases of cardiovascular instability and respiratory failure directly consequential from their injuries. Furthermore, long term vasopressor requirement is not uncommon though weaning from parenteral vasopressor is a challenge. We document a case of the use of oral ephedrine, which we used to wean our patient from parenteral vasopressors. Oral ephedrine can be an appropriate option to get rid of long term use of infusion of vasopressor.
Keywords: endotracheal tube; dopamine; ephedrine; hypertensive factor; noradrenalin; adult; article; artificial ventilation; autonomic dysfunction; blood pressure; case report; cervical spinal cord injury; clinical article; comminuted fracture; crystalloid; disease severity; fluid resuscitation; gunshot injury; heart rate; hematopneumothorax; human; hypotension; intensive care; intensive care unit; male; mean arterial pressure; sinus arrhythmia; sweating; tracheostomy

Locked-in syndrome: Traumatic anatomy of a rare condition - Maynard W.; Sewell M.; Laherty R. (Clinical Anatomy, 2017)
A 45-year-old normally fit and well female was involved in a highspeed road traffic accident. At the scene she was hypotensive, tachycardic and had a Glasgow Coma Score of 3/15. She was not moving and had sluggish dilated pupils. She was taken to the emergency department, resuscitated and underwent imaging. Ct-scanning of the head, neck, chest, abdomen and pelvis was performed which showed an active abdominal bleed from the superior mesenteric artery close to its origin. Additional injuries included bony disruption at the craniocervical junction and a subarachnoid haemorrhage. The patient went to theatre for a trauma laparotomy to ligate the bleeding superior mesenteric artery. Following haemorrhage control, the patient continued to demonstrate labile cardiorespiratory function (blood pressure varied between 50-180 mm Hg systolic). It was felt this was due to possible brainstem compression from the occipito-cervical dislocation which had originally been shown on CT. The patient underwent occipito-cervical fusion and decompression and the patient's haemodynamic status normalised. The patient required a tracheostomy for long-term mechanical ventilation; remained quadriplegic and lost the ability to speak or swallow. MRI of the cervical spine showed significant brainstem and proximal spinal cord oedema. Eye movements to command and voice were preserved and a diagnosis of locked-in syndrome was made. There was no neurological improvement at 6 months. Locked in syndrome following traumatic occipito-cervical dislocation is rare. Clinicians need to be aware of this potential diagnosis as a cause of refractory haemodynamic instability in the trauma setting and to inform decision making regarding future care.
Keywords: antihypertensive agent; accident; adult; anatomy; blood pressure; brain stem; cervical spine dislocation; compression; controlled study; decision making; decompression; diagnosis; edema; emergency ward; eye movement; Glasgow coma scale; head; human; laparotomy; locked in syndrome; middle aged; mydriasis; nuclear magnetic resonance imaging; pelvis; spinal cord; subarachnoid hemorrhage; superior mesenteric artery; tachycardia; thorax; tracheostomy; traffic; voice; x-ray computed tomography

Traumatic vertebral and carotid artery dissections related to motor vehicle accidents-injury characteristics and outcomes from a national inpatient sample - Khatri R.; Afzal M.R.; Qureshi M.A.; Kassar D.; Rodriguez G.J.; Cruz-Flores S.; Maud A. (Cerebrovascular Diseases, 2017)
Background: Carotid and vertebral artery dissections are relatively common causes of ischemic stroke. Comparative analysis between these two entities related to motor vehicle trauma at the national level is not well studied. Objective: To compare patterns of injury and in-hospital outcomes in motor vehicle related traumatic carotid versus vertebral artery dissections at national level. Methods: A retrospective study was conducted using a national database (years 20011 to 2014). Patients with a principal diagnosis of carotid dissection (ICD9 = 443.21) or vertebral dissections (ICD9 = 443.24) were identified. Baseline characteristics, comorbidities, in-hospital complications, in-hospital procedures, length of stay, hospital charges, disability and mortality were compared between the two groups. Results: A total of 2969 patients had either carotid (n= 1300,44%>) or vertebral (n= 1669, 56 %>) artery dissection during the study period. Patients with vertebral artery dissections were older (mean ±SD: 46.4 ±19.4 versus 38.2 ±16.8, p = 0.01) and had more vascular risk factors (hypertension, diabetes mellitus, atrial fibrillation, dyslipidemia and nicotine dependence). There was no statistically significant difference in the rate of ischemic stroke (22% carotid vs 16.6 % vertebral, p 0.1052). Vertebral artery dissection patients were more commonly associated with (83%) fractures of cervical vertebrae either without spinal cord injury (63.5%>) or with spinal cord injury (16.5%>). Carotid dissection patients had more traumatic subarachnoid hemorrhage (17.8% vs 11%), p 0.0206), brain injury (68.8%o vs 40.6%o p <.0001) and other organs involved including liver, spleen, intrathoracic organs and bone fractures (pelvic fracture, long bone fractures, multiple rib fractures, skull base fracture and facial bones fracture). They also had higher rates of pneumonia (11.5%o vs 5.6%o, p 0.0093) and underwent more procedures (mechanical ventilation, gastrostomy, blood transfusion and tracheostomy). The length of stay was higher for carotid artery dissection (15.5 days vs 10.2 days p <.0001) patients with a higher moderate to severe disability (61.8%o vs 46.1%o), and the difference remained the same after adjusting for age, gender and the presence of other injuries (OR 1.679, CI 1.136-2.483, p 0.0096). There was no difference in mortality (12.0%o carotid artery dissection vs 7.8%o vertebral artery dissection, p 0.1100). Conclusion: Carotid artery dissection related to motor vehicle accidents are associated with other multiple organ injuries and bone fractures suggesting higher impact trauma, and they lead to higher moderate to severe disability compared to vertebral artery dissections. There is an association of vertebral artery dissection and the presence of vertebrae fractures.
Keywords: adult; artificial ventilation; atrial fibrillation; blood transfusion; brain injury; brain ischemia; cardiovascular risk; carotid artery injury; cervical vertebra; comorbidity; complication; controlled study; diabetes mellitus; diagnosis; disability; dyslipidemia; facial bone; female; gastrostomy; gender; hospital charge; hospital patient; human; hypertension; ICD-9; length of stay; liver; major clinical study; male; mortality; multiple fracture; organ injury; pelvis fracture; pneumonia; retrospective study; rib fracture; skull base fracture; spinal cord injury; spleen; subarachnoid hemorrhage; tobacco dependence; tracheostomy; traffic accident

Quality of life after resection of a chordoma of the mobile spine - Schwab J.H.; Janssen S.J.; Paulino Pereira N.R.; Chen Y.L.E.; Wain J.C.; DeLaney T.F.; Hornicek F.J. (Bone and Joint Journal, 2017)
Aims The aim of the study was to compare measures of the quality of life (QOL) after resection of a chordoma of the mobile spine with the national averages in the United States and to assess which factors influenced the QOL, symptoms of anxiety and depression, and coping with pain post-operatively in these patients. Patients and Methods A total of 48 consecutive patients who underwent resection of a primary or recurrent chordoma of the mobile spine between 2000 and 2015 were included. A total of 34 patients completed a survey at least 12 months post-operatively. The primary outcome was the EuroQol-5 Dimensions (EQ-5D-3L) questionnaire. Secondary outcomes were the Patient- Reported Outcome Measurement Information System (PROMIS) anxiety, depression and pain interference questionnaires. Data which were recorded included the indication for surgery, the region of the tumour, the number of levels resected, the status of the surgical margins, re-operations, complications, neurological deficit, length of stay in hospital and rate of re-admission. Results The median EQ-5D-3L score was 0.71 (interquartile range (IQR) 0.44 to 0.79) which is worse than the national average in the United States of 0.85 (p < 0.001). Anxiety (median: 55 (IQR 49 to 61), p = 0.031) and pain (median: 61 (IQR 56 to 68), p < 0.001) were also worse than the national average in the United States (50), while depression was not (median: 52 (IQR 38 to 57), p = 0.513). Patients who underwent a primary resection had better QOL and less anxiety, depression and pain compared with those who underwent resection for recurrent or residual disease. The one- and five-year probabilities were 0.96 and 0.74 for survival, 0.07 and 0.25 for tumour recurrence, and 0.02 and 0.16 for developing distant metastasis. A total of 25 local complications occurred in 20 patients (42%), and there were 50 systemic and other complications in 25 patients (52%) within 90 days. Conclusion These patient reported outcomes and oncological and surgical outcomes can be used when counselling patients and to aid decision-making when planning surgery.
Keywords: pedicle screw; abdominal pain; acute kidney failure; adult; aged; anxiety disorder; article; assessment of humans; bone allograft; cancer patient; cancer radiotherapy; cancer surgery; chordoma; clinical article; clinical assessment; compartment syndrome; controlled study; coping behavior; decubitus; deep vein thrombosis; depression; distant metastasis; dural tear; dysphagia; dyspnea; EuroQol 5 Dimensions; female; femoral shaft; femoral shaft allograft; fever; fibula; fibula graft; fibular allograft; health survey; heart infarction; hospital readmission; hospitalization; human; humeral shaft; humeral shaft allograft; hygroma; intubation; length of stay; lung embolism; male; medical information system; meninx disorder; middle aged; minimal residual disease; mobile spine chordoma; neurogenic bladder; neurologic disease; nutritional deficiency; outcome assessment; overall survival; paraplegia; Patient Reported Outcome Measurement Information System; patient-reported outcome; pedicle screw; pedicle screw malposition; peripheral neuropathy; pleura effusion; pneumonia; postoperative complication; postoperative delirium; postoperative ileus; postoperative nausea and vomiting; postoperative pain; postoperative period; priority journal; prosthesis complication; prosthesis loosening; pyelonephritis; pyrexia idiopathica; quality of life; respiratory failure; risk factor; serotonin syndrome; spinal cord decompression; spine stabilization; spine tumor; surgical infection; surgical margin; surgical patient; symptom; tracheostomy; tumor recurrence; United States; ureter stone; urinary tract infection; urosepsis; vocal cord paralysis

Experience of Sun′s procedure for chronic type B dissection with aortic arch involvement - Qi R.; Zhu J.; Chen L.; Li C.; Qiao Z.; Cheng L.; Ge Y.; Hu H.; Xia Y.; Xing X.; Zheng T.; Liu Y.; et al. (National Medical Journal of China, 2017)
Objective: To study the surgical treatment of chronic type B dissection with aortic arch involvement using Sun′s procedure. Methods: Between February 2009 and December 2015, 29 patients [20 males, 9 females, with a mean age of (41±12) years, range 24-64 years] with type B dissection with aortic arch involvement underwent Sun′s procedure. Sixteen patient had a history of hypertension. Marfan syndrome was observed in 9 cases, coronary artery disease in 3 cases, mitral regurgitation in 3 patients, cerebrovascular disease in one patient. Twenty-two patients suffered proximal aortic arch disease, 4 cases experienced history of aortic root procedure and 2 subjects had history of pregnancy. Four patients had aortic arch malformation. Results: One case suffered from massive cerebral infarction after surgery and died in another hospital. Concomitant procedures included mitral valve replacement in 3 cases, coronary artery bypass grafting in 3 patients, reconstruction of the right aberrant subclavian artery in one patient. Ventilator support exceeding 24 hours obseved in 2 patients. One of them recieved continuous renal replacement therapy and recovered before discharge. Spinal cord injury was obseved in one case, brain infarction in one patient and pericardial drainage in one case. Two patients required tracheotomy. During 12-94 (43±23) months′ follow-up, thoracoabdominal aortic replacment was performed in 4 patients, thoracic endovascular aortic repair (TEVAR) in 2 subjects and repair of perivalvular leakage in one patient. Conclusions: Sun′s procedure obtained satisfactory results in patients with chronic type B dissection with aortic arch involvement. Concomitant repair of proximal aortic arch lesions and distal type B dissection can be adopted using Sun′s procedure.
Keywords: adult; aortic arch; aortic arch anomaly; aortic disease; aortic dissection; aortic root surgery; artery reconstruction; article; artificial ventilation; brain infarction; cerebrovascular disease; clinical article; coronary artery bypass graft; coronary artery disease; endovascular aneurysm repair; female; follow up; hospital discharge; human; hypertension; male; Marfan syndrome; medical history; mitral valve regurgitation; mitral valve replacement; personal experience; renal replacement therapy; subclavian artery; surgical technique; thoracic aortic surgery; tracheotomy

Successful tracheostomy decannulation after complete or sensory incomplete cervical spinal cord injury - Kim D.H.; Kang S.W.; Choi W.A.; Oh H.J. (Spinal Cord, 2017)
Study Design: Retrospective study. Objectives: To report the successful tracheostomy decannulation/extubation in cervical spinal cord injury (C-SCI) patients. Setting: Tertiary university hospital, pulmonary rehabilitation center. Methods: Complete or sensory incomplete C-SCI patients who had received invasive acute phase respiratory management, and succeeded in decannulation/extubation became candidates of this study. Retrospective review was conducted on the transitions of the respiratory status. Results: Sixty-two patients (M: 55, F: 7) were identified. Traumatic etiologies accounted for 93.5%, and mean onset age was 47.6±15.8 years. Sixty patients (96.8%) had undergone tracheostomy and the other two received endotracheal intubation during acute phase. All patients succeeded in decannulation/extubation after employing mechanically assisted coughing and noninvasive mechanical ventilation (NIV). Mean time since tracheostomy to decannulation was 7.0±14.5 months. Of the 60 tracheostomized patients, 12 succeeded in decannulation without applying long-term NIV, 31 switched to continuous NIV after decannulation. Fifteen patients totally weaned off from ventilators after NIV. Two patients who once succeeded in decannulation were re-tracheostomized. For the 31 patients with continuous NIV, mean hours of daily need for ventilatory support had reduced from 15.3±8.0 to 5.7±5.7 h at final follow-ups. Conclusion: Complete or sensory incomplete C-SCI patients, even with high neurological level can be successfully decannulated through NIV and aggressive use of mechanically assisted coughing. Undesirable tracheostomy can be avoided by employing the noninvasive respiratory management.
Keywords: adult; airway obstruction; American Spinal Injury Association impairment scale; article; artificial ventilation; cannulation; cervical spinal cord injury; comorbidity; coughing; decannulation; decubitus; depression; endotracheal intubation; extubation; female; heterotopic ossification; human; hypercapnia; intensive care unit; lung function; major clinical study; male; non invasive procedure; onset age; pleura effusion; pneumonia; positive end expiratory pressure; priority journal; pulmonary rehabilitation; respiratory care; retrospective study; sleep disordered breathing; tracheostomy; tracheostomy tube; urinary tract infection; ventilator weaning

Implementation of a respiratory rehabilitation protocol: weaning from the ventilator and tracheostomy in difficult-to-wean patients with spinal cord injury - Gundogdu I.; Ozturk E.A.; Umay E.; Karaahmet O.Z.; Unlu E.; Cakci A. (Disability and rehabilitation, 2017)
PURPOSE: Following repeated weaning failures in acute care services, spinal cord injury (SCI) patients who require prolonged mechanical ventilation and tracheostomy are discharged to their homes or skilled nursing facilities, with a portable mechanical ventilator (MV) and/or tracheostomy tube (TT) with excess risk of complications, high cost and low quality of life. We hypothesized that many difficult-to-wean patients with cervical SCI can be successfully managed in a rehabilitation clinic. The aim of our study was to develop a respiratory rehabilitation, MV weaning and TT decannulation protocol and to evaluate the effectiveness of this protocol in tetraplegic patients. METHODS: A multidisciplinary and multifaceted protocol, including respiratory assessment and management themes, was developed and performed based on the findings from other studies in the literature. Tetraplegic patients with the diagnosis of difficult-to-wean, who were admitted to the rehabilitation clinic after having been discharged from the intensive care unit to their home with home-type MV and/or TT, were included in this prospective observational study. RESULTS: The respiratory rehabilitation protocol was applied to 35 tetraplegic patients (10 home-type MV and tracheostomy-dependent, and 25 tracheostomized patients) with C1-C7 ASIA impairment scale grade A, B, and C injuries. Seven out of 10 patients successfully weaned from MV and 30 of 35 patients were decannulated. Four patients were referred for diaphragm pace stimulation and tracheal stenosis surgery. The mean durations of MV weaning and decannulation were 37 and 31 days, respectively. CONCLUSIONS: A multifaceted, multidisciplinary respiratory management program can change the process of care used for difficult-to-wean patients with SCI. Implications for rehabilitation Findings from this study indicate the significance of a multidimensional evaluation of any reversible factors for prolonged MV- and/or TT-dependent SCI patients. Thus, rehabilitation specialists should take this into consideration and should provide the appropriate amount of time to these patients. The proposed protocol of respiratory rehabilitation for MV- and/or TT-dependent SCI patients shows promising results in terms of changing the care used for these patients. Successful implementation of a respiratory rehabilitation and weaning protocol is dependent on careful planning and detailed communication between the rehabilitation specialist and intensivist during the respiratory rehabilitation process. Because many of the so-called difficult- or impossible-to-wean patients were successfully weaned from MV and TT in the PMR clinic, the need for such an outlet for countries without specialized centers is supported.
Keywords: adolescent; adult; aged; clinical protocol; female; home care; hospital discharge; human; male; middle aged; pathophysiology; prospective study; quality of life; spinal cord injury; standards; tertiary care center; tracheostomy; ventilator weaning; young adult

Pulmonary outcomes following specialized respiratory management for acute cervical spinal cord injury: A retrospective analysis - Zakrasek E.C.; Nielson J.L.; Kosarchuk J.J.; Crew J.D.; Ferguson A.R.; Mckenna S.L. (Spinal Cord, 2017)
Study Design: Retrospective analysis. Objectives: To identify multivariate interactions of respiratory function that are sensitive to spinal cord injury level and pharmacological treatment to promote strategies that increase successful liberation from mechanical ventilation. Setting: United States regional spinal cord injury (SCI) treatment center. Methods: Retrospective chart review of patients consecutively admitted to Santa Clara Valley Medical Center between May 2013 and December 2014 for ventilator weaning with C1-C5 American Spinal Injury Association Impairment Scale (AIS) A or B SCI, <3 months from injury and who had a tracheostomy in place. A nonlinear, categorical principal component analysis (NL-PCA) was performed to test the multivariate interaction of respiratory outcomes from patients (N=36) being weaned off ventilator support after acute SCI with (N=15) or without (N=21) theophylline treatment. Results: In total, 36 patients met inclusion criteria (2 C1, 5 C2, 11 C3, 14 C4 and 4 C5). The NL-PCA returned three independent components that accounted for 95% of the variance in the data set. Multivariate general linear models hypothesis tests revealed a significant syndromic interaction between theophylline treatment and SCI level (Wilks' Lambda, P=0.028, F (12,64)=2.116, ' • 2 =0.256, 1-β=0.838), with post hoc testing demonstrating a significant interaction on PC1, explained by a positive correlation between improved forced vital capacity and time it took to reach 16 h of ventilator-free breathing. Thirty-three patients (92%) achieved 16 h of ventilator-free breathing (VFB) and 30 patients (83%) achieved 24 h of VFB. Conclusions: We suspect that some portion of the high success rate of ventilator weaning may be attributable to theophylline use in higher cervical SCI, in addition to our aggressive regimen of high volume ventilation, medication optimization and pulmonary toilet (positive pressure treatments and mechanical insufflation-exsufflation).
Keywords: theophylline; adolescent; adult; aged; American Spinal Injury Association impairment scale; article; artificial ventilation; cervical spinal cord injury; clinical article; female; forced vital capacity; human; male; mechanical ventilator; medical record review; middle aged; positive end expiratory pressure; priority journal; respiratory failure; respiratory function; tracheostomy; ventilator weaning

Cervicomedullary decompression through expanded endoscopic endonasal approach: Our clinical experience - Alkherayf F.; Agbi C.; Kilty S.; Lamothe A. (Canadian Journal of Neurological Sciences, 2017)
Background: patients with ventral cervical-medullary compression require anterior decompression of the cervicomedullary junction. Odontoid resection can be accomplished through expanded endoscopic approach especially in cases of irreducible basilar invagination in which the pathology is situated well above the palatine line. Methods: We are presenting our experience at the Ottawa Hospital (TOH) over the last seven years in patients who underwent expanded endoscopic endonasal decompression of their cervicomedullary junction. 16 patients underwent such procedure, those patients with preoperative cervical instability underwent posterior fusion for stabilization at the same surgical setting. Follow up ranged from 9 months to 5 years. Results: All patients had severe symptoms of myelopathy and some lower cranial nerves dysfunction. All patients were extubated after recovery from anesthesia and allowed oral intake next day. patients demonstrated improvement in their symptoms and none of them required tracheostomy. 12.5% experienced transient velopharyngeal insufficiency. one patient had CSF leak which was successfully treated with lumbar drain and one patient developed infection from the posterior cervical fusion and required debridement. All patients were eventually discharged home. Postoperative imaging demonstrated excellent decompression of the anterior cervicomedullary junction pathology. Conclusions: The expanded endoscopic endonasal approach for odontoidectomy should be considered as a minimally invasive approach for anterior decompression in selected cases.
Keywords: anesthesia; child; clinical article; cranial nerve; debridement; decompression; drain; female; follow up; human; infection; liquorrhea; lumbar region; palatopharyngeal incompetence; pathology; preschool child; remission; spinal cord disease; surgery; tracheostomy

Update on traumatic acute spinal cord injury. Part 2 - Mourelo Fariña M.; Salvador de la Barrera S.; Montoto Marqués A.; Ferreiro Velasco M.E.; Galeiras Vázquez R. (Medicina Intensiva, 2017)
The aim of treatment in acute traumatic spinal cord injury is to preserve residual neurologic function, avoid secondary injury, and restore spinal alignment and stability. In this second part of the review, we describe the management of spinal cord injury focusing on issues related to short-term respiratory management, where the preservation of diaphragmatic function is a priority, with prediction of the duration of mechanical ventilation and the need for tracheostomy. Surgical assessment of spinal injuries based on updated criteria is discussed, taking into account that although the type of intervention depends on the surgical team, nowadays treatment should afford early spinal decompression and stabilization. Within a comprehensive strategy in spinal cord injury, it is essential to identify and properly treat patient anxiety and pain associated to spinal cord injury, as well as to prevent and ensure the early diagnosis of complications secondary to spinal cord injury (thromboembolic disease, gastrointestinal and urinary disorders, pressure ulcers).
Keywords: anxiety; article; artificial ventilation; decubitus; early diagnosis; gastrointestinal disease; human; patient care; spinal cord decompression; spinal cord injury; spinal pain; spine stabilization; thromboembolism; tracheostomy; traumatic acute spinal cord injury; treatment duration; urinary tract disease

Determinants of cough effectiveness in patients with respiratory muscle weakness - Laghi F.; Maddipati V.; Schnell T.; Langbein W.E.; Tobin M.J. (Respiratory Physiology and Neurobiology, 2017)
Experiments were undertaken to mechanistically define expiratory-muscle contribution to effectiveness of cough while controlling glottic movement. We hypothesized that electrical abdominal-muscle stimulation in patients with respiratory-muscle weakness produces effective coughs only when glottic closure accompanies coughs. In ten spinal-cord-injury patients, esophago-gastric pressure and airflow were recorded during solicited-coughs, coughs augmented by abdominal-muscle stimulation, and passive open-glottis exhalations. During solicited-coughs, patients closed the glottis initially; five were flow-limited, five non-flow-limited. Stimulations during solicited-coughs or open-glottis exhalations elicited similar driving pressures (changes in gastric pressure; p < 0.001). Despite high driving pressures, stimulations induced flow-limitation only when patients transiently closed the glottis – not during open-glottis exhalations. That is, transient glottic closure enabled transmission of abdominal (driving) pressure to the thorax during cough, while impeding dissipation of intrathoracic pressure. In conclusion, transient glottic closure is necessary to render cough effective in patients with respiratory-muscle weakness, indicating that failure to close the glottis contributes to ineffective cough in weak tracheostomized patients and patients with bulbar disorders.
Keywords: abdominal pressure; abdominal wall musculature; adult; aged; airflow; article; breathing muscle; clinical article; coughing; esophagus pressure; exhalation; glottis; human; middle aged; muscle contraction; muscle excitation; muscle weakness; neuromuscular electrical stimulation; priority journal; spinal cord injury; stomach pressure; thorax pressure; tracheostomy

Efficacy and safety of nusinersen in infants with presymptomatic spinal muscular atrophy (SMA): Interim results from the NURTURE study - Bertini E.; Hwu W.-L.; Reyna S.P.; Farwell W.; Gheuens S.; Sun P.; Zhong Z.J.; De Vivo D.C. (European Journal of Paediatric Neurology, 2017)
Objective: SMA is an autosomal recessive neuromuscular disorder caused by mutations in the survival motor neuron 1 (SMN1) gene, which result in SMN protein deficiency. Nusinersen is an antisense oligonucleotide that modifies SMN2 pre-mRNA splicing, thereby promoting increased functional SMN protein levels. NURTURE (NCT02386553) is an ongoing phase 2, open-label, single-arm study, evaluating intrathecal nusinersen (12-mg equivalent dose) in infants with presymptomatic SMA. Methods: Infants enrolled in the NURTURE study must be presymptomatic at the time of screening, ≤<=6 weeks old at first dose, have genetic documentation of 5q SMA homozygous gene deletion or mutation, and have 2 or 3 SMN2 gene copies. The primary endpoint is the time to death or respiratory intervention (tracheostomy or ventilation for ≥>=6 hours/day for ≥>=7 days). Secondary endpoints include survival, growth parameters, motor milestone attainment assessed by section 2 of the Hammersmith Infant Neurological Examination (HINE) and WHO criteria, and change from baseline in Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) motor function scale. Sibling data was also collected. Study enrollment started in May 2015. Results: As of 8 June 2016, at the time of the first interim analysis, 22 infants had been screened; 17 were enrolled and dosed. All infants remained enrolled for up to ∼13 months, and none met the primary endpoint of respiratory intervention or death. Improvements in mean HINE motor milestones scores versus baseline were observed. The majority of infants gained weight over time, consistent with normal development. Additional analyses on data collected as of November 1, 2016 are ongoing. Data from this most recent interim analysis will be reported, including results for patient subgroups based on demographic and clinical characteristics. Conclusion: Interim analysis results demonstrate the efficacy and safety of nusinersen in presymptomatic infants with SMA.
Keywords: endogenous compound; nusinersen; survival motor neuron protein 2; child; clinical trial; death; development; documentation; gene deletion; homozygosity; human; infant; mental capacity; motor performance; neurologic examination; Pennsylvania; pharmacokinetics; sibling; spinal muscular atrophy; tracheostomy

Pediatric long-term non-invasive ventilation definition and situation PLTNIV - Pavone M.; Cutrera R. (Pediatric Pulmonology, 2017)
Definition Respiratory support can be distinguished as “invasive” and “noninvasive”. The distinction depends on the interface used for patientventilator connection. For non-invasive ventilation (NIV), gases are conducted into the airways via an external interface. For invasive ventilation (IMV), gases are conducted into the airways through an endotracheal tube or tracheostomy [1,2]. Indications for and Goals of NIV Non-invasive ventilation in children is indicated essentially for: 1) Diseases due to increased respiratory load (intrinsic cardiopulmonary disorders, abnormalities of the upper airways, chest wall deformities); 2) Disorders characterized by weakness of the respiratory muscles (neuromuscular diseases, spinal cord injuries); 3) Abnormal neurological control of ventilation(congenitalor acquiredalveolarhypoventilationsyndrome) [1,2]. Non-invasive ventilation can alleviate chronic respiratory failure through the correction of hypoventilation, the improvement of respiratory muscle function and reducing the workload of the respiratory system [1,2]. Goals of NIV are the relief from symptoms, reduction of the work of breathing, improvement and stabilization of gas exchanges, patient-ventilator synchrony, improvement of duration and quality of sleep, improvement of the quality of life and functional status, and prolongation of survival [3]. Patients and Interface Selection Long-term NIV is applicable to cooperative and stable patients with a certain degree of respiratory autonomy [1,2]. Usually, NIV is applied at night and/or during daytime naps [1-3]. The choice of interface depends on the characteristics of the patient (age, facial characteristics, degree of cooperation, and severity of respiratory impairment). In children, interface acceptance is the first step for a successful NIV program [1,2]. Nasal masks are the most often used interfaces, although there are promising experiences with the use of oro-nasal and full-face masks, nasal pillows and mouthpieces [1,2]. Ventilation Mode Pressure-targeted ventilation is the modality most often used for noninvasive ventilation [1-3]. Continuous positive airway pressure (CPAP) support is based on the delivery to the airways of a constant pressure for the whole respiratory cycle. With CPAP, the work of breathing is entirely up to the patient [1-3]. CPAP acts by elevating the intraluminal pressure of the upper airway at levels higher than those of the critical transmural pressure that determines the collapse of the upper airway. This pressure keeps the airways open, promotes relaxing of the upper airway dilator muscles, and reduces inspiratory muscle activity of the upper airways and diaphragm [1-3]. CPAP prevents alveolar collapse favoring alveolar recruitments and the increase in functional residual capacity. Through this mechanism, CPAP improves oxygenation and downloading the inspiratory muscles reduces the work of breathing. Bi-level positive airway pressure (Bi-levelPAP) provides respiratory support at two different levels. Using bi-level PAP is possible, therefore, to separately adjust a lower expiratory positive airway pressure (EPAP, CPAP) and a higher inspiratory positive airway pressure (IPAP, PIP). The inspiratory pressure enhances the patient's spontaneous inspiratory act [1-3]. The expiratory pressure allows eliminatingmore easily exhaled air and CO2.TheEPAP plays the same role discussed above forCPAP[1-3]. The tidal volumewill be generated as the result of the delta between the inspiratory and expiratory pressures [1-3]. In Pressure Support Ventilation (PSV) mode, the ventilator ensures a maximum value of inspiratory pressure in the airways equal to that set by the operator. This pressure support allows the patient to achieve more effective breaths. The patient determines respiratory rate, inspiratory flow and inspiratory time by determining the onset of inspiration, muscle strength applied during the inspiration and the passage to expiration [1]. The use of the PSV mode allows preserving the patient's spontaneous breathing while ensuring the reduction of excessive work of breathing undergone by the patient. This mode is preferable in patients capable of spontaneous breathing and able to activate the ventilator cycles. In Pressure Control Ventilation (PCV) mode, the operator sets the maximum level of pressure that is delivered by the ventilator during the inspiratory act, the respiratory rate and the inspiratory:expiratory ratio (I:E), in the absence of respiratory effort. Breaths delivered by the ventilator are determined by a pressure, duration of inspiration and expiration default. This mode is preferable in severely ill patients with significant impairment of the muscle pump efficiency or ventilatory drive [1]. Training Program and Discharge Plan for Long-Term Use If NIV can be established gradually, an accurate clinical training session aimed at the introduction of the patient and family to its practice must be planned [1,2]. Training should start by using very low pressures and when the patient tolerates pressures throughout the night, the pressures can be gradually increased [1,2]. The choice of pressures is the process by which the clinician searches for a compromise between defect correction (through the increase in pressures), and the limitation of the side effects (with the use of a pressure as low as possible, although still effective) [1,2]. Pressure requests depend on the individual patient's current clinical condition and must be obtained from the evaluation of its monitoring [1-3]. Before discharge, the patient's respiratory status should be stable on the same ventilator, circuit and interfaces that the child will use at home. A personalized follow-up plan must always be provided [1,2]. The optimal frequency for follow-up evaluations has not yet been readily determined. These evaluations should generally be scheduled more frequently in infants and younger children [1,2]. On such occasions, the history and a complete clinical and instrumental assessment (ventilator, circuits, humidification, interfaces) must be performed [1,2]. Compliance should be systematically evaluated through the internal memory of the instrument to verify the actual time of ventilator use. This check also allows assessing air leakages, pressures delivered and nocturnal SpO2 values [1,2]. Polysomnographic evaluations are recommended before initiating NIV and discharging with the ventilator, and during each in-hospital follow-up admission [1-3]. Pulmonary function tests, blood gas analysis, chest x-ray and lateral projection of the skull, echocardiography should be periodically repeated [1,2]. Situation An increasing number of children with chronic hypercapnic respiratory failure are currently treated with NIV [1,2]. Non-invasive ventilation allows preserving functions such as swallowing, feeding, speaking, coughing, heating/humidification of the inspired air [1]. The introduction of NIV has reduced the number of emergency room visits per year, tracheostomies, intubations and the length of stay in the pediatric intensive care units. Non-invasive ventilation has allowed early weaning from IMV and extubations. Non-invasive ventilation has also enabled preventing vocal cord or trachea damages, and reduce the risk of lower respiratory tract infections [1]. Convincing data have been reported from national surveys on long-term experiences with NIV performed especially in Western countries [4-7]. In the last years, new data have come out from developing and Eastern countries [8-10]. Neuromuscular disease such as Duchenne Muscular Dystrophy (DMD) and Spinal Muscular Atrophy (SMA), and diseases of the central nervous system such as the congenital central alveolar hypoventilation syndrome represent two main indications for NIV [4-7]. Among respiratory diseases, airway malacia and obstructive sleep apnea have been the most frequently treated with CPAP/NIV [4-7]. Children with severe physical and cognitive disabilities are also increasingly offered long-term ventilation to prolong life [4,6]. The survival is longer in patients treated with NIV than in those undergoing IMV [4-7]. Usually, the median age at the beginning of IVM ventilation is significantly lower than in those treated with NIV [4]. Non-invasive ventilation has been successfully started even in children under 1 year of age [6]. Data are available on the possible weaning from long-term NIV, as well as on deaths during NIV (for example in children in whom a palliative approach was taken) [4-10]. Children with neuromuscular and neurological disease are least likely to wean off from NIV. Children most likely to discontinue long-term NIV are those with chronic lung disease of prematurity, airway malacia, and upper-airways abnormalities [4-10]. Non-invasive ventilation failures and consequently tracheostomy and IVM have been reported for example in children with Cerebral Palsy [4-10].A significant number of patients with NIV have transitioned to adult care [7]. Compliance with NIV is a major issue. Data downloaded from built-in software showed a wide range on mean nightly use [2]. Parental assessment of PAP use may overestimate actual home ventilator use. In this latter study, patients with greater improvement in apnea-hypopnea index were more likely to be adherent. Clinical parameters and nighttime and daytime symptoms improved after PAP therapy regardless of age or adherence. Treatment adherence was not correlated with age, type of underlying disease, interfaces used, nocturnal gas exchanges, and duration of PAP treatment. Children who attempted to use CPAP at least 6 nights a week were treated with CPAP for a longer time on the nights of use. Usage in the first week of treatment predicted longer term use over 2 to 3 months. A predictor of PAP use was maternal education. Adherence was demonstrated lower in African American children.
Keywords: carbon dioxide; adult; African American; apnea hypopnea index; apnea monitoring; blood gas analysis; breathing muscle; breathing pattern; breathing rate; cerebral palsy; child; chronic respiratory failure; cognitive defect; collapse; congenital central hypoventilation syndrome; coughing; daytime somnolence; death; diaphragm; Duchenne muscular dystrophy; echocardiography; education; emergency ward; endotracheal tube; expired air; extubation; face mask; family study; feeding; female; follow up; functional residual capacity; gas exchange; heating; human; humidifier; hypobarism; infant; intestine pressure; intubation; length of stay; lower respiratory tract infection; lung dysplasia; lung function test; memory; muscle function; muscle strength; neuromuscular disease; night; noninvasive ventilation; pediatric intensive care unit; physical disability; pressure support ventilation; quality of life; respiration load; side effect; skull; sleep disordered breathing; sleep quality; software; speech; spinal cord injury; spinal muscular atrophy; survival; swallowing; thorax deformity; thorax radiography; tissue oxygenation; tracheobronchomalacia; tracheostomy; upper respiratory tract; ventilator; vocal cord; weakness; weaning; work of breathing; workload

Assessment of Sleep in Patients Receiving Invasive Mechanical Ventilation in a Specialized Weaning Unit - Huttmann S.E.; Wilms K.; Hamm C.; Magnet F.S.; Windisch W.; Storre J.H. (Lung, 2017)
Introduction: A restful sleep is essential for regenerative processes and remains crucial for patients recovering from stressful periods in the intensive care unit. The current study aimed to assess sleep quality in critically ill patients receiving invasive mechanical ventilation within a specialized weaning unit in hospital. Methods: Tracheotomized subjects undergoing prolonged weaning from mechanical ventilation were included in the study. Polysomnography and gas exchange monitoring was performed during nocturnal ventilation. Subjective evaluation of sleep quality and health-related quality of life were also assessed. Results: Nineteen subjects completed the study protocol. Sleep architecture was highly heterogeneous across individual subjects. Mean total sleep time (TST) was 273 ± 114 min, sleep efficacy 70 ± 23%, slow-wave sleep 25.7 ± 18.4%/TST, rapid eye movement sleep 9.6 ± 7.5%/TST, and arousal index 18.7 ± 12.4/h. No significant difference in sleep quality was found between subjects with successful (N = 7) or unsuccessful (N = 12) weaning. Bicarbonate levels were negatively correlated both with sleep efficacy and sleep quality, that latter of which was subjectively assessed by the subjects using a visual analogue scale. Conclusion: Subjects who were undergoing prolonged weaning from mechanical ventilation and admitted to a specialized weaning unit, showed reduced sleep quality with preservation of high amounts of slow-wave sleep.
Keywords: DRKS00004736; acute respiratory failure; aged; article; artificial ventilation; chronic obstructive lung disease; clinical article; critical illness; critically ill patient; female; follow up; gas exchange; human; kidney failure; kyphoscoliosis; latent period; lung congestion; male; neuromuscular disease; polysomnography; pressure transducer; priority journal; quality of life; REM sleep; sleep disordered breathing; sleep quality; sleep stage; sleep time; slow wave sleep; spinal cord injury; tracheostomy tube; tracheotomy; ventilator weaning; visual analog scale

Neurological form of hemophagocytic lymphohistiocytis presenting as ADEM - Benallegue N.; Miot C.; Beloncle F.; Pellier I.; Gueden S.; Van Bogaert P. (European Journal of Paediatric Neurology, 2017)
Objective: Hemophagocytic Lymphohistiocytis (HLH), previously called macrophage activation syndrome, is a rare and very aggressive disease related to uncontrolled massive immune activation. The typical presentation is a multiorgan failure with frequent central central nervous system (CNS) involvement. Some cases are described with only CNS involvement without systemic disease presentation. Aggressive immunosuppressive treatment is required considering high rate of mortality and morbidity. We report here a pure neurological form of HLH presenting as acute disseminated encephalomyelitis (ADEM) to stress on the difficulties diagnosing HLH, which may delay early appropriate treatment. Results: This 14 year-old girl presented with acute tetraparesis and perineal hypoesthesia, drowsiness, and meningism following a Ebstein-Barr Virus primary infection. Rapid respiratory failure required mechanical ventilation, then tracheotomy. MRI showed T2- weighted lesions of cerebrum with matter, basal ganglia, brainstem and spinal cord. CSF analysis showed 174 cells permm3, mostly lymphocytes (97%), and 0.56 g/L of proteins. ADEM was first suspected but her clinical condition did not improve after corticotherapy, and plasma exchanges. In view of unfavorable evolution, high cell count in the CSF and some atypical MRI features for ADEM, especially asymmetric lesion in large area, HLH was considered. Counts of activated T lymphocytes in the CSF (CD4+ 15% ; CD8+ 50% (N<15%))and blood were markedly elevated. The patient was treated by cyclosporine combined with intrathecal methotrexate injections, intravenous rituximab and alemtuzumab. Tetraparesis improved seven days after the last administration of alemtuzumab, autonomous walking was possible three months after initial presentation, cognitive impairment improved. The patient was free of relapse fifteen months after initial symptoms. Conclusion: Isolated CNS HLH should be considered when facing with ADEM not responding to usual treatment. Some particular MRI features might be helpful for early diagnosis. This is crucial considering that early effective immunosuppressive therapy is essential to prevent poor outcomes.
Keywords: alemtuzumab; CD4 antigen; cyclosporine; endogenous compound; methotrexate; rituximab; acute disseminated encephalomyelitis; adolescent; adverse drug reaction; artificial ventilation; basal ganglion; brain injury; brain stem; case report; cell count; clinical trial; cognitive defect; corticosteroid therapy; diagnosis; drowsiness; drug combination; drug therapy; early diagnosis; female; girl; human; human cell; hypesthesia; immunosuppressive treatment; injection; meningism; morbidity; mortality; nonhuman; nuclear magnetic resonance imaging; plasmapheresis; primary infection; quadriplegia; relapse; respiratory failure; side effect; spinal cord; stress; T lymphocyte; tracheostomy; virus; walking

Giant Cell Tumor of Cervical Spine Presenting as Acute Asphyxia - Kumar R.; Meis J.M.; Amini B.; McEnery K.W.; Madewell J.E.; Rhines L.D.; Benjamin R.S. (Spine, 2017)
Study Design. Case report and literature review. Objective. To describe treatment of a unique case of acute airway obstruction by a large C7 giant cell tumor (GCT) with preoperative denosumab followed by surgical resection, and review the literature on this rare entity. Summary of Background Data. Standard treatment for GCTs includes surgical resection or curettage and packing. Large lesions in the spine may require preoperative therapy with denosumab, a human monoclonal antibody to RANKL, to facilitate surgery. It is highly unusual for GCT arising in cervical spine to present with acute asphyxia (requiring tracheostomy). Methods. We report a patient with large C7 GCT that caused tracheal compression with almost complete airway obstruction requiring emergency intubation. Results. The tumor responded to subcutaneously administered denosumab with marked decrease in size and relief of symptoms. Increased tumor mineralization in response to therapy facilitated subsequent successful surgical tumor resection. The patient remains symptom-free 2 years after surgery without tumor recurrence. Conclusion. Denosumab can shrink the size of large GCTs, providing symptom relief before surgery and facilitate tumor resection.
Keywords: denosumab; adult; airway obstruction; article; asphyxia; cancer chemotherapy; cancer patient; cancer surgery; case report; computer assisted tomography; curettage; dyspnea; female; follow up; giant cell tumor; giant cell tumor of cervical spine; human; laminectomy; nuclear magnetic resonance imaging; outcome assessment; preoperative care; priority journal; radiology; respiratory tract intubation; seventh cervical vertebra; spine tumor; trachea compression; tumor recurrence; tumor volume; wheezing; young adult

Physician driven variation in the care of children with spinal muscular atrophy type 1 - Oskoui M.; Ng P.; Liben S.; Zielinski D. (Pediatric Pulmonology, 2017)
Background: Increasing numbers of families are requesting active supportive management for their child with spinal muscular atrophy type 1 (SMA1), leading to longer survival and greater prevalence of affected children. Strong opinions exist among physicians for and against the provision of care measures prolonging life. Objective: To describe current practice in the care of SMA1 in Canada, and explore the factors underlying inter-physician variability. Methods: A cross-sectional survey of Canadian hospital-based pediatric neurologists and pediatric respirologists was performed in 2015. Odds ratios and 95% confidence intervals were calculated to compare proportions between groups. Results: There was a 54% completion rate (99 physicians). Over half of participants believed that a disease modifying therapy was likely within 10 years. Quebec respirologists were 50 times less likely to offer long-term non-invasive ventilation (NIV) than respirologists in other provinces (OR 50.6, 95% CI 2.4–1075.3), and 20 times less likely to discuss tracheostomy with families (OR 20.4, 95% CI 2.0–211.8). High raters of perceived happiness of affected children were more likely to find NIV an acceptable measure for acute (OR 6.7, 95% CI 1.7–26.0) and chronic (OR 13.7, 95% CI 4.0–46.4) respiratory failure and prophylactic use (OR 5.8, 95% CI 2.2–15.6). Conclusion: Physician knowledge, opinions, subjective perception of child happiness, and regional factors, all influence physicians’ practices and the shared decision-making process. Parents may not be informed or offered all the services available to their child. Knowledge translation initiatives are needed to enhance SMA1 care. Pediatr Pulmonol. 2017;52:662–668. © 2016 Wiley Periodicals, Inc.
Keywords: antiinflammatory agent; disease modifying treatment; unclassified drug; aeration; article; breathing exercise; Canada; child; child care; chronic respiratory failure; clinical practice; controlled study; cross-sectional study; endotracheal intubation; female; gas exchange; gas mask; happiness; health care access; hospitalization; human; hypoventilation; inter physician variability; life expectancy; lung clearance; lung volume; major clinical study; male; mechanical insufflation exsufflation device; medical decision making; medical education; medical specialist; neurologist; noninvasive ventilation; palliative therapy; pediatrics; perception; physician; positive end expiratory pressure; prophylaxis; quality of life; Quebec; respirologist; social support; spinal muscular atrophy; Werdnig Hoffmann disease; suction; tracheostomy; treatment duration

Lateral mass lesion of C1 - Cabrita F.; Carvalho M.; Sincari M. (Sinapse, 2017)
The surgical removal of C1 lateral mass lesions is challenging due to the close relationship with vertebral artery and plexus and upper spinal cord. We report a case of a 49-year-old female presenting with severe occipitocervical pain, aggravating during the night period and with neck mobilization. The patient with a previous diagnosis of breast cancer (9 years before) submitted to left mastectomy. MRI and CT Scans showed an osteolytic lesion on the left lateral mass of C1 with signs of C0-C1-C2 instability. Vascular studies showed left vertebral artery compression on the transverse foramen without occlusion. Osteoarticular scintigraphy reveled no other skeletal lesions. The lesion was removed and and substituted by with a Harms cage and an occipito-cervical fusion was performed by single midline dorsal cervical approach for the upper spine. The patient recovered immediately after surgery with no neurological deficits recorded. In the early post-operative period the patient developed acute respiratory distress due to traqueal oedema with need for re-intubation and ventilatory support for three days and kept tracheostomy for one month with full recovery, posteriorly. Posterior midline approach is suitable for these lesions specially in cases when posterior fixation and fusion are warranted.
Keywords: adult; assisted ventilation; bone atrophy; bone lesion; breast cancer; cancer size; case report; compression; diagnosis; dorsal region; edema; female; human; intubation; mastectomy; metastasis; middle aged; neck; neurologic disease; night; nuclear magnetic resonance imaging; occlusion; pain; postoperative period; respiratory distress; scintigraphy; spine; surgery; tracheostomy; vertebral artery; x-ray computed tomography

Airway adverse events following posterior occipito-cervical spinal fusion - Sheshadri V.; Moga R.; Manninen P.; Goldstein C.L.; Rampersaud Y.R.; Massicotte E.M.; Fehlings M.G.; Venkatraghavan L. (Journal of Clinical Neuroscience, 2017)
Management of the airway may be challenging in patients undergoing occipito-cervical spine fusions (OCF). Changes in the occipito-cervical angle (dOC2A) of fusion after surgery may result in acute airway obstruction, dyspnea and/or dysphagia. Objectives of the study were to review the airway management of patients during posterior OCF, determine the incidence, nature and risk factors for postoperative airway adverse events (AEs), and to determine the relationship between airway AEs and the change in dOC2A. In this retrospective cohort of 59 patients, following extubation in the operating room (OR), there were no complications in 43 (73%) patients (Group 1). Sixteen (27%) patients (Group 2) had airway complications; 4 requiring reintubation and 12 having delayed extubation. The number of vertebral levels fused (>6), presence of difficult intubation and duration of surgery (>5 h) were significantly associated with AEs. There was no significant difference in the dOC2A between the groups (−1.070 ± 5.527 versus −4.375 ± 10.788, p = 0.127). Airway management in patients undergoing OCF poses a challenge for the anesthesiology and surgical teams. The incidence of AEs was 27%. The decision to extubate immediately after surgery needs to be individualized. Factors such as difficult intubation, number of vertebral levels fused and duration of surgery have to be considered. A significant correlation between dOC2A and postoperative AEs could not be established. Risk factors for postoperative AEs are multifactorial and prospective evaluation of these factors is indicated.
Keywords: adult; aged; ankylosing spondylitis; article; cohort analysis; controlled study; extubation; female; fiberoptic bronchoscopy; human; intubation; Klippel Feil syndrome; laryngeal mask; macroglossia; major clinical study; male; middle aged; mucopolysaccharidosis; operation duration; oxygen desaturation; posterior occipitocervical spine fusion; posterior spine fusion; reintubation; repeat procedure; respiratory distress; respiratory tract disease; retrospective study; rheumatoid arthritis; spine fracture; spine metastasis; stridor; tracheostomy; upper respiratory tract obstruction; young adult

Real-time ultrasound imaging biofeedback training is diaphragmatic function in nontraumatic cervical spinal cord injury: a single-subject experimental study - Kim J.-S.; Oh D.-W. (European Journal of Physiotherapy, 2017)
This study aimed to describe, and report the results from a detailed procedure of a diaphragmatic breathing exercise using real-time ultrasound imaging biofeedback (RUIB) training. The subject was a 16-year-old man with an incomplete cervical spinal cord injury (CSCI) caused by spinal intramedullary arteriovenous malformations at the level of his C4–C5 spinal cord. Although he could walk independently, decreased respiratory function was one of the major factors reducing his ability to perform daily activities, such as walking. Outcome measures included a 10-meter walk test (10MWT) results, balance ability in eyes open and closed, diaphragm movement distance on sniffing, and forced vital capacity. All parameters except the 10MWT results appeared to significantly improve during the intervention phase. Data points during both the intervention and follow-up phases showed improved trends in comparison to those in the baseline phase, indicating that intervention might be beneficial in improving respiratory function and performance and that these gains can be maintained after the intervention. Our findings suggest that the use of RUIB during breathing exercise may be valuable in managing respiratory and physical function of patients with cervical cord injuries.
Keywords: adolescent; arteriovenous malformation; article; Barthel index; biofeedback; body equilibrium; breathing exercise; case report; cervical spinal cord; cervical spinal cord injury; daily life activity; diaphragm; diaphragm movement; experimental study; follow up; forced vital capacity; human; laminectomy; lung capacity; male; muscle training; nuclear magnetic resonance imaging; outcome assessment; real time echography; respiratory function; sniffing; spinal intramedullary arteriovenous malformation; stretching exercise; tracheostomy; walk test; walking

Paediatric spinal cord infarction—a review of the literature and two case reports - Sheikh A.; Warren D.; Childs A.-M.; Russell J.; Liddington M.; Guruswamy V.; Chumas P. (Child's Nervous System, 2017)
Ischemic spinal cord infarction is rare in the paediatric population, and when it does occur, it is usually associated with traumatic injury. Other potential causes include congenital cardiovascular malformations, cerebellar herniation, thromboembolic disease and infection. Magnetic resonance imaging (MRI) findings can be subtle in the early evaluation of such patients. The outcome is variable and depends on the level and extent of the spinal cord infarct and subsequent rehabilitation. Here, we present two cases of ischemic spinal cord infarction in children.
Keywords: antiinfective agent; blood clotting factor 5 Leiden; cardiolipin antibody; fibrinogen; glycoprotein; hemoglobin; immunoglobulin G; lupus anticoagulant; phospholipid antibody; steroid; anti-infective therapy; arm movement; artery catheterization; article; case report; cervical spinal cord; chest infection; child; childhood disease; erythrocyte transfusion; female; flaccid paralysis; follow up; gene mutation; hemoglobin blood level; human; international normalized ratio; male; mean arterial pressure; myelitis; neuroimaging; neurorehabilitation; nuclear magnetic resonance imaging; pediatric physiotherapy; pediatric rehabilitation; pediatric surgery; positive end expiratory pressure; preschool child; priority journal; prothrombin time; spinal cord infarction; steroid therapy; supine position; tracheostomy; walking

Healthcare resource use in patients with diagnosis of spinal muscular atrophy (SMA) in Optum™ U.S. Claims Database - Teynor M.; Hou Q.; Zhou J.; Hall E.; Wells W.; Avendano J. (Neurology, 2017)
Objective: Describe HRU among patients with diagnostic codes for SMA in a US commercial claims database. Background: SMA is a devastating rare, autosomal recessive neuromuscular disease clinically characterized by severe and progressive muscular atrophy and weakness. HRU in SMA has been shown to be high; however, no published studies have assessed HRU in U.S. commercial claims data. Design/Methods: We conducted a retrospective analysis of Optum's Clinformatics™ DataMart for MultiPlan, a large US insurance claims database covering approximately 96.7 million lives from 49 plans across seven regions. Patients included in this analysis were born between January 2004 and September 2015 with >2 diagnostic codes for SMA (ICD-9: 335.0 or 335.1×) ≥31 days apart. Descriptive statistics were used. Results: 3,146 patients had ≥2 diagnostic codes for SMA ≥31 days apart, of which 342 met inclusion criteria (51% male; average age 2.1 years). Median follow-up time was 19.1 months (average 36.9 months). Top diagnosis claims included lack of coordination (63%), acute respiratory infections (62%), respiratory abnormality (56%), cough (54%), and feeding difficulties/mismanagement (50%). Only 3.8% of patients had a claim for genetic testing. 66.4% had ≥1 inpatient stay (average 3.5 per patient) with a mean length of stay (LOS) of 10 days (median 4 days), and a maximum LOS of 389 days. Top inpatient claims included pneumonia (23%), pulmonary collapse (18%), respiratory failure (16%), gastrostomy status (15%), and esophageal reflux (15%). 64% had ≥1 ER visit. Patients averaged 225 outpatient visits. Claims for g-tubes and tracheostomy occurred in 25% and 7% of patients, respectively. Suction machines (47%), cough assist (38%), and BiPAP/CPAP (36%) were the most prevalent durable medical equipment claims. Conclusions: Patients with diagnostic codes for SMA demonstrated high HRU, consistent with the clinical course of SMA. Further analysis is warranted to understand the economic burden of HRU in SMA to inform research efforts for therapeutic interventions.
Keywords: atelectasis; child; congenital malformation; coordination disorder; coughing; data base; diagnosis; disease course; feeding difficulty; female; follow up; gastroesophageal reflux; gastrostomy; genetic screening; hospital patient; human; ICD-9; insurance; length of stay; machine; major clinical study; male; non continuous ventilator; outpatient; pneumonia; positive end expiratory pressure; preschool child; respiratory failure; respiratory tract infection; retrospective study; spinal muscular atrophy; statistics; suction; tracheostomy

Interim efficacy and safety results from the Phase 2 NURTURE study evaluating nusinersen in presymptomatic infants with spinal muscular atrophy - De Vivo D.C.; Hwu W.-L.; Reyna S.P.; Farwell W.; Gheuens S.; Sun P.; Zhong Z.J.; Su J.; Schneider E.; Bertini E. (Neurology, 2017)
Objective: NURTURE (NCT02386553) is an ongoing phase 2, open-label, single-arm study, evaluating intrathecal nusinersen (12-mg equivalent dose) in infants with presymptomatic spinal muscular atrophy (SMA). Background: SMA is a neuromuscular disorder caused by a mutation in the survival motor neuron 1 (SMN1) gene resulting in SMN protein deficiency. Nusinersen is an investigational antisense oligonucleotide drug that modifies SMN2 pre-mRNA splicing, promoting increased functional SMN protein production. Design/Methods: NURTURE is enrolling presymptomatic infants with genetically diagnosed SMA and evaluating their development from near birth to 2.5 years. The primary endpoint is time to death or respiratory intervention (tracheostomy/any ventilation support for ≥6 hours/day continuously for ≥7 days). Secondary endpoints include the proportion of infants developing clinical symptoms of SMA and achievement of motor milestones. Changes in compound muscle action potential (CMAP) and adverse events (AEs) also were assessed. Study enrollment started May 2015; interim results are reported. Results: As of 8 Jun 2016, 22 infants were screened; 17 were enrolled and dosed; 5 failed screening. Most (12/17) had 2 SMN2 copies, and most were aged ≤1 month at enrollment. All 17 infants remain enrolled; none have met the primary endpoints of respiratory intervention or death. Improvements in mean Hammersmith Infant Neurological Examination (HINE) motor milestones scores vs Baseline have been observed. Infants have generally gained weight over time consistent with normal development, and mean CMAP scores have mostly improved vs Baseline. There were no severe AEs reported, 5 infants experienced a serious AE. Three infants experienced AEs considered possibly related to study drug. No new safety concerns were identified. Conclusions: The NURTURE interim analysis shows that all the infants treated with nusinersen are alive and generally achieving new motor skills and appropriate age-related developmental gains. The efficacy and safety profile of nusinersen supports continued clinical development.
Keywords: endogenous compound; nusinersen; protein c jun; survival motor neuron protein; survival motor neuron protein 1; achievement; adverse drug reaction; assisted ventilation; clinical article; clinical trial; controlled clinical trial; controlled study; death; development; diagnosis; drug therapy; female; gene expression; gene mutation; genetic predisposition; human; infant; male; motor performance; muscle action potential; neurologic examination; pharmacokinetics; phase 2 clinical trial; protein deficiency; RNA splicing; safety; screening; side effect; spinal muscular atrophy; symptom; tracheostomy

TBCD may be a causal gene in progressive neurodegenerative encephalopathy with atypical infantile spinal muscular atrophy - Ikeda T.; Nakahara A.; Nagano R.; Utoyama M.; Obara M.; Moritake H.; Uechi T.; Mitsui J.; Ishiura H.; Yoshimura J.; Doi K.; Kenmochi N.; et al. (Journal of Human Genetics, 2017)
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disorder caused by survival motor neuron gene mutations. Variant forms of SMA accompanied by additional clinical presentations have been classified as atypical SMA and are thought to be caused by variants in as yet unidentified causative genes. Here, we presented the clinical findings of two siblings with an SMA variant followed by progressive cerebral atrophy, and the results of whole-exome sequencing analyses of the family quartet that was performed to identify potential causative variants. We identified two candidate homozygous missense variants, R942Q in the tubulin-folding cofactor D (TBCD) gene and H250Q in the bromo-adjacent homology domain and coiled-coil containing 1 (BAHCC1) gene, located on chromosome 17q25.3 with an interval of 1.4 Mbp. The in silico analysis of both variants suggested that TBCD rather than BAHCC1 was likely the pathogenic gene (TBCD sensitivity, 0.68; specificity, 0.97; BAHCC1 sensitivity, 1.00; specificity, 0.00). Thus, our results show that TBCD is a likely novel candidate gene for atypical SMA with progressive cerebral atrophy. TBCD is predicted to have important functions on tubulin integrity in motor neurons as well as in the central nervous system.
Keywords: anticonvulsive agent; bromo adjacent homology domain and coiled coil containing 1 protein; tubulin; tubulin folding cofactor D; unclassified drug; article; brain atrophy; case report; chromosome 17q; computer assisted tomography; computer model; controlled study; developmental disorder; dysphagia; dystocia; failure to thrive; fasciculation; female; focal epilepsy; genetic variability; genotype; gestational age; hepatosplenomegaly; high arched palate; homozygote; human; hypoxemia; immobility; infant; involuntary movement; mental deficiency; missense mutation; motoneuron; muscle hypotonia; muscle weakness; nerve degeneration; pes equinus; psychomotor retardation; respiratory distress; sensitivity and specificity; sibling; single nucleotide polymorphism; tracheostomy; vaginal delivery; Werdnig Hoffmann disease; whole exome sequencing

The impact of temperature in aortic arch surgery patients receiving antegrade cerebral perfusion for >30 minutes: How relevant is it really? - Preventza O.; Coselli J.S.; Akvan S.; Kashyap S.A.; Garcia A.; Simpson K.H.; Price M.D.; Mayor J.; de la Cruz K.I.; Cornwell L.D.; Omer S.; Bakaeen F.G.; et al. (Journal of Thoracic and Cardiovascular Surgery, 2017)
Objective We examined the early outcomes and the long-term survival associated with different degrees of hypothermia in patients who received antegrade cerebral perfusion (ACP) for >30 minutes. Methods During a 10-year period, 544 consecutive patients underwent proximal and total aortic arch surgery and received ACP for >30 minutes and 1 of 3 levels of hypothermia: deep (14.1°C-20°C; n = 116 [21.3%]), low-moderate (20.1°C-23.9°C; n = 262 [48.2%]), and high-moderate (24°C-28°C; n = 166 [30.5%]). A variable called “predicted temperature” was used in propensity-score analysis. Multivariate analysis was done to evaluate the effect of actual temperature on outcomes. Results The operative mortality rate was 12.5% (n = 68) overall and was 15.5%, 11.8%, and 11.5% in the deep, low-moderate, and high-moderate hypothermia patients, respectively (P = .54). The persistent stroke rate was 6.6% overall and 12.2%, 4.6%, and 6.0% in these 3 groups, respectively (P = .024 on univariate analysis). On multivariate analysis, actual temperature was not associated with mortality, but lower temperatures predicted persistent stroke and reoperation for bleeding. In the propensity-matched subgroups, the patients with predicted deep hypothermia had (nonsignificantly) greater rates of persistent stroke (12.2% vs 4.9%; relative risk, 1.08; 95% CI, 0.87-1.15) and reoperation for bleeding (14.6% vs 2.4%; relative risk, 1.14; 95% CI, 0.87-1.15) than the patients with predicted moderate hypothermia. On long-term follow-up (mean duration, 5.12 years), 4- and 8-year survival rates were 62.3% and 55.7% in the deep hypothermia group and 75.4% and 74.2% in the moderate hypothermia group (P = .0015). Conclusions In proximal and arch operations involving ACP for >30 minutes, greater actual temperatures were associated with less stroke and reoperation for bleeding. There were no significant differences among the predicted hypothermia levels, although a trend toward a higher rate of adverse events was noticed in the deep hypothermia group. Long-term survival was better in the moderate hypothermia group.
Keywords: adult; adverse outcome; aged; antegrade cerebral perfusion; aortic dissection; aortic valve replacement; aortic arch surgery; aortic root surgery; brain perfusion; cerebrovascular accident; conference paper; controlled study; coronary artery bypass graft; disease duration; endocarditis; extracorporeal oxygenation; female; follow up; human; hypothermia; kidney failure; long term survival; major clinical study; male; mortality rate; multiple organ failure; neurologic disease; operative blood loss; paraplegia; pericardiotomy; postoperative inflammation; priority journal; reoperation; risk factor; spinal cord injury; surgical mortality; survival rate; tracheostomy

Complications of spinal cord injuries: Two case reports and a review of the literature - Karabacak P.; Gülsoy K.Y.; Osmanlioglu H.O.; Yavuz L. (Journal of Clinical and Analytical Medicine, 2017)
Spinal cord injuries are a health problem that affects many systems and leads to physical restrictions associated with the level at which the spinal cord is affected. It may be necessary to maintain early and late-stage treatment under intensive care conditions according to the degree of dysfunction that develops as a result of the level affected in the spinal cord. With the appropriate early treatment,survival increases in these patients. However,care must be taken with respect to complications that may develop while the patient is undergoing treatment and intervention must be made for these complications to provide patient comfort. In this paper,we present two cases of patients with cervical fractures who developed cardiovascular and respiratory problems while in the intensive care unit.
Keywords: antibiotic agent; atropine; dopamine; nitrate; quetiapine; adult; artificial heart pacemaker; artificial ventilation; bacterial endocarditis; bradycardia; breathing exercise; cardiovascular disease; case report; cervical spine fracture; computer assisted tomography; death; deterioration; drug substitution; drug withdrawal; falling; fever; heart arrest; human; hypertension; hypotension; intensive care; male; percutaneous endoscopic gastrostomy; pneumonia; pulmonary aspiration; quadriplegia; respiratory tract disease; review; sedation; sepsis; septic shock; spinal cord injury; spine stabilization; tracheostomy; unconsciousness; urinary tract infection

Anesthetic considerations for patients with acute cervical spinal cord injury - Bao F.-P.; Zhang H.-G.; Zhu S.-M. (Neural Regeneration Research, 2017)
Anesthesiologists work to prevent or minimize secondary injury of the nervous system and improve the outcome of medical procedures. To this end, anesthesiologists must have a thorough understanding of pathophysiology and optimize their skills and equipment to make an anesthesia plan. Anesthesiologists should conduct careful physical examinations of patients and consider neuroprotection at preoperative interviews, consider cervical spinal cord movement and compression during airway management, and suggest awake fiberoptic bronchoscope intubation for stable patients and direct laryngoscopy with manual in-line immobilization in emergency situations. During induction, anesthesiologists should avoid hypotension and depolarizing muscle relaxants. Mean artery pressure should be maintained within 85-90 mmHg (1 mmHg = 0.133 kPa; vasoactive drug selection and fluid management). Normal arterial carbon dioxide pressure and normal blood glucose levels should be maintained. Intraoperative neurophysiological monitoring is a useful option. Anesthesiologists should be attentive to postoperative respiratory insufficiency (carefully considering postoperative extubation), thrombus, and infection. In conclusion, anesthesiologists should carefully plan the treatment of patients with acute cervical spinal cord injuries to protect the nervous system and improve patient outcome.
Keywords: atropine; barbituric acid derivative; benzodiazepine; ethinylestradiol plus norgestimate; isoprenaline; methylprednisolone; propofol; salbutamol; suxamethonium; anesthesia; article; asthma; bradycardia; cervical spinal cord injury; computer assisted tomography; electromyography; Frankel grade; glucose blood level; heart arrest; heart arrhythmia; heart supraventricular arrhythmia; human; hyperglycemia; hypotension; hypothermia; hypovolemia; intensive care unit; laryngoscopy; mean arterial pressure; nuclear magnetic resonance imaging; operative blood loss; sinus rhythm; tracheotomy; treatment outcome; urinary tract infection

Prognostic Differences: Epstein-Barr Virus–Associated Primary Leiomyosarcoma of the Spine Versus Spinal Leiomyosarcoma Metastases - Agarwal N.; Hansberry D.R.; Kavi A.; Agarwal P.; Baisre A.; Goldstein I.M. (World Neurosurgery, 2017)
Objective The authors studied 6 cases of osseous leiomyosarcoma of the spine. Two of these cases were of immunocompromised human immunodeficiency virus (HIV)-positive patients with Epstein-Barr virus (EBV)-associated primary vertebral leiomyosarcomas. The remaining 4 cases were of patients with leiomyosarcoma metastases to the spine. Methods Each patient underwent surgical resection of their vertebral mass; however, the patients with the EBV-associated tumors had the best postoperative prognosis. Results The HIV-positive patients have had no further local recurrence, while the other 4 patients had rapid local recurrences requiring multiple surgical interventions. Furthermore, the patients living with HIV have lived longer with fewer leiomyosarcoma-related health complications. Conclusions These findings suggest that EBV-associated vertebral leiomyosarcoma is of a less aggressive variety than metastatic leiomyosarcoma of the spine.
Keywords: dexamethasone; docetaxel; gemcitabine; ifosfamide; mesna; abdominal hysterectomy; adult; bone metastasis; cancer combination chemotherapy; cancer palliative therapy; cancer patient; cancer prognosis; cancer radiotherapy; cancer surgery; clinical article; computer assisted tomography; confusion; controlled study; decompression surgery; disease association; drug safety; drug withdrawal; dysphagia; Epstein Barr virus; Epstein Barr virus infection; female; gastrointestinal toxicity; histopathology; human; Human immunodeficiency virus; Human immunodeficiency virus infected patient; Human immunodeficiency virus infection; hydronephrosis; in situ hybridization; laminectomy; leiomyosarcoma; liver metastasis; lung metastasis; male; mediastinum mass; metastasis resection; middle aged; multimodality cancer therapy; multiple cycle treatment; neurotoxicity; neutropenia; nuclear magnetic resonance imaging; occupational therapy; physiotherapy; pneumonia; positron emission tomography; positron emission tomography-computed tomography; postoperative period; retroperitoneal tumor; review; salpingooophorectomy; spinal cord biopsy; spinal cord compression; spinal cord metastasis; spinal cord tumor; spinal leiomyosarcoma; spine; spine surgery; spine tumor; thorax radiography; tracheostomy; tumor localization; tumor recurrence; tumor volume; urinary tract infection; vertebra body; decadron; gemzar

Resection of the odontoid process through expanded endoscopic endonasal approach: Our clinical experience and surgical techniques - Alkherayf F.; Agbi C.; Kilty S.; MacDonald K.; Lamothe A. (Journal of Neurological Surgery Part B: Skull Base, 2017)
Introduction: Patients with ventral cervical-medullary compression e.g., rheumatoid arthritis patients require anterior decompression of the cervico-medullary junction. Resection of the odontoid process can be accomplished through expanded endoscopic endonasal approach especially in cases of irreducible basilar invagination in which the pathology is situated well above the palatine line. Methods: We are presenting our experience at the Ottawa Hospital (TOH) and University of Ottawa over the last seven years in patients who underwent expanded endoscopic endonasal decompression of their cervicomedullary junction. Over the last seven years, 16 patients underwent such procedure, those patients with preoperative cervical instability underwent posterior fusion of the upper cervical spine to the occiput for stabilization at the same surgical setting. Our follow up ranged from 9 months to 5 years. Results: All patients presented with severe symptoms of myelopathy and some lower cranial nerves dysfunction. All patients demonstrated improvement in their symptoms. All patients were extubated after recovery from anesthesia and allowed oral food intake the next day. None of our patients required tracheostomy. 12.5% experienced transient velopharyngeal insufficiency. one patient had CSF leak which was successfully treated with lumbar drain. One of our patients developed infection from the posterior cervical fusion and required debridement and antibiotics treatment. All of our patients were eventually discharged home. Postoperative imaging demonstrated excellent decompression of the anterior cervicomedullary junction pathology. Conclusions: The expanded endoscopic endonasal approach for odontoidectomy should be considered as a minimally invasive approach for anterior decompression in selected cases.
Keywords: antibiotic agent; adult; anesthesia; case report; cervical spine; clinical article; conference abstract; cranial nerve; debridement; decompression; drain; drug therapy; female; follow up; food intake; human; infection; liquorrhea; lumbar region; odontoid process; palatopharyngeal incompetence; pathology; remission; spinal cord disease; surgery; tracheostomy

Comorbidities and complications of spinal fusion for scoliosis - Berry J.G.; Glotzbecker M.; Rodean J.; Leahy I.; Hall M.; Ferrari L. (Pediatrics, 2017)
BACKGROUND AND OBJECTIVES: General pediatricians and hospitalists are increasingly summoned to optimize the comorbid conditions of children with medical complexity (CMC) undergoing major surgery. We assessed the relationship between specific chronic conditions of CMC and hospital resource use with spinal fusion for scoliosis, an operation with high cost and morbidity. METHODS: Retrospective analysis of 7252 children age ≥5 years with an underlying complex chronic condition undergoing spinal fusion between January 1, 2010 through December 31, 2014 in 41 children's hospitals. Hospital length of stay (LOS), cost, and 30-day readmission rate were compared across comorbid conditions by using linear and logistic regression accounting for demographic characteristics and clustering of patients by hospital. RESULTS: Fifty-nine percent of children had ≥4 comorbid conditions. As the number of chronic conditions increased from 1-3 to ≥10, median LOS increased 60% (5 [interquartile range (IQR), 4-7] to 8 [IQR, 5-13] days); median hospital cost increased 53% ($52 319 [IQR, $37 937-71 513] to $80 429 [IQR, $58 602-$111 965]); and readmission rates increased 293% (5.4% to 15.8%) (P < .001 for all). In multivariable analysis, conditions strongly associated with LOS and cost were chronic respiratory insufficiency (LOS: +2.1 days; cost: +$12 070; and bladder dysfunction (LOS: +0.8 days; cost: +$4014) (P < .001 for all). Readmission likelihood was highest with bladder dysfunction (odds ratio, 1.5; 95% confidence interval, 1.1-2.0) and epilepsy (odds ratio, 1.2; 95% confidence interval, 1.0-1.5). CONCLUSIONS: Chronic respiratory insufficiency, bladder dysfunction, and epilepsy had significant associations with hospital resource use for CMC undergoing spinal fusion. Pediatricians, patients, and families may find it useful to consider these conditions when striving to benefit the children's perioperative health and outcomes.
Keywords: adult; article; asthma; bladder dysfunction; brain malformation; cerebral palsy; child; childhood disease; Children with medical complexity; chromosome disorder; chronic disease; chronic respiratory failure; cohort analysis; comorbidity; controlled study; decubitus; dysphagia; epilepsy; female; gastroesophageal reflux; genetic disorder; hospital cost; hospital discharge; hospital readmission; human; hydrocephalus; hypertension; length of stay; major clinical study; male; medicaid; medical information system; muscle disease; muscular dystrophy; outcome assessment; respiratory arrest; retrospective study; scoliosis; spinal dysraphism; spine fusion; tracheostomy

Dysphagia in patients with cervical spinal cord injury: Retrospective study - Cascioli R.; Castellani G.B.; Bosi S.; Fiorani M.; Fontana D.; Locatelli R. (Dysphagia, 2017)
Introduction: It is clinically recognized that patients with a cervical lesion of the spinal cord have frequently swallowing problems . Many studies have shown that a combination of multiple factors, such as surgical approach, mechanical ventilation and presence of tracheotomy, have to be considered as risk factors of developing swallowing disorders, although every single factor cannot be statistically associated with dysphagia. Materials and Methods: A total of 19 patients with cervical spinal cord injury (SCI) from Montecatone Rehabilitation Institute were included in our study. Respiratory issues and variously mixed factors (prolonged mechanical ventilation, tracheotomy, surgical fixation and neck collar) were detected in all patients. Theirs swallowing abilities was assessed clinically, by a SLP (Logemann 1989) and endoscopically (FEES). Results: Dysphagia was identified in 18 patients. BSE has always recorded cough ineffectiveness, pharynx sensibility and propulsive wave impairment, but an altered vocal quality and food trail at the suctioning have been noticed just occasionally. The pharyngeal residue rate was determined precisely just by FEES, likewise anatomically pharyngeal alterations due to surgical fixation. Thereby a specific therapy (inclusive of neuromuscular treatment and the most suitable dietary modifications) has been organized in order to prevent pulmonary complications. Currently, 18 patients have sufficient oral caloric intake, although some of them need textural dietary restrictions. Conclusions: In patients with cervical SCI, complex clinical conditions and long hospitalized, an integrate assessment both by means of BSE and FEES could be useful in order to estimate as best the post deglutition aspiration risk and thereby organized the most suitable behavioural/dietary measures. Further analysis is needed to establish specific risk factors such as critical illness condition.
Keywords: artificial ventilation; aspiration; caloric intake; cervical spinal cord injury; clinical article; coughing; critical illness; diet restriction; dysphagia; human; lung complication; neck; pharynx; prevention; rehabilitation; retrospective study; risk factor; sensibility; surgery; tracheostomy

Early percutaneous dilational tracheostomy does not lead to an increased risk of surgical site infection following anterior spinal surgery - Kaczmarek C.; Aach M.; Hoffmann M.F.; Yilmaz E.; Waydhas C.; Schildhauer T.A.; Hamsen U. (Journal of Trauma and Acute Care Surgery, 2017)
BACKGROUND: Most patients with cervical spinal cord injuries require tracheostomy. The optimal timing is still a matter of debate. Previous studies showed that patients receiving early tracheostomy had fewer ventilator days and decreased rates of pneumonia and were mobilized earlier. Because of the proximity of the anterior approach to the tracheostoma, there is concern about an increased risk of surgical site infection (SSI) related to tracheostomy. METHODS: This was a retrospective analysis at a Level I trauma center of patient records from 2008 to 2014, identifying all patients with spinal cord injury who received anterior cervical spinal surgery and had early percutaneous dilational tracheostomy (PDT). Follow-up for SSI was performed throughout hospital stay (mean, 110 days; median, 96 days, with lower quartile 89 days and upper quartile 119 days) and at 6 weeks and 3 months (clinical examination and computed tomography scans). RESULTS: Fifty-one patients underwent anterior spinal surgery with PDT performed within a median of 5 days (range, 1-18 days). Seventy-eight percent (n = 40) of patients had anterior spinal surgery, whereas 22% (n = 11) had a combined anterior-posterior repair. All percutaneous dilational tracheostomies were performed using the Ciaglia single-step dilation technique. Despite an SSI of one patient's cannulation site, no SSI of the anterior approach was observed. CONCLUSION: Performing a PDT in a timely fashion after anterior spinal surgery does not increase the risk of SSI.
Keywords: adult; anterior spinal surgery; clinical examination; computer assisted tomography; conference paper; emergency health service; female; follow up; hospitalization; human; infection risk; intervertebral disk degeneration; major clinical study; male; medical record; middle aged; neurological surgical equipment; orthopedic surgical equipment; priority journal; retrospective study; spinal cord injury; spine surgery; surgical infection; surgical intensive care unit; tracheostomy; Ciaglia Blue Rhino

Erratum: Early percutaneous dilational tracheostomy does not lead to an increased risk of surgical site infection following anterior spinal surgery (Journal of Trauma and Acute Care Surgery (2017) 82:2 (383-386) DOI: 10.1097/TA.0000000000001320) (Journal of Trauma and Acute Care Surgery, 2018)
In the 82(2) issue of the journal, published in February 2017, the Authorship section of this article should read as follows: CK designed the study collected the data and drafted themanuscript. UH designed the study, collected the data and revised the manuscript. CWhelped with the study design, revised the study, and helped with the literature review. EY helped with the data collection and revised the manuscript.MFH helped with the study design and revised themanuscript.MArevised the manuscript. TAS coordinated the study and helped with the study design. All authors read and approved the final manuscript. This correction has been noted in the online version of the article, which is available at www.jtrauma.com.
Keywords: erratum; error

Cough augmentation techniques for extubation or 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, 2017)
Background: There are various reasons why weaning and extubation failure occur, but ineffective cough and secretion retention can play a significant role. Cough augmentation techniques, such as lung volume recruitment or manually- and mechanically-assisted cough, are used to prevent and manage respiratory complications associated with chronic conditions, particularly neuromuscular disease, and may improve short- and long-term outcomes for people with acute respiratory failure. However, the role of cough augmentation to facilitate extubation and prevent post-extubation respiratory failure is unclear. Objectives: Our primary objective was to determine extubation success using cough augmentation techniques compared to no cough augmentation for critically-ill adults and children with acute respiratory failure admitted to a high-intensity care setting capable of managing mechanically-ventilated people (such as an intensive care unit, specialized weaning centre, respiratory intermediate care unit, or high-dependency unit). Secondary objectives were to determine the effect of cough augmentation techniques on reintubation, weaning success, mechanical ventilation and weaning duration, length of stay (high-intensity care setting and hospital), pneumonia, tracheostomy placement and tracheostomy decannulation, and mortality (high-intensity care setting, hospital, and after hospital discharge). We evaluated harms associated with use of cough augmentation techniques when applied via an artificial airway (or non-invasive mask once extubated/decannulated), including haemodynamic compromise, arrhythmias, pneumothorax, haemoptysis, and mucus plugging requiring airway change and the type of person (such as those with neuromuscular disorders or weakness and spinal cord injury) for whom these techniques may be efficacious. Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL; Issue 4, 2016), MEDLINE (OvidSP) (1946 to April 2016), Embase (OvidSP) (1980 to April 2016), CINAHL (EBSCOhost) (1982 to April 2016), and ISI Web of Science and Conference Proceedings. We searched the PROSPERO and Joanna Briggs Institute databases, websites of relevant professional societies, and conference abstracts from five professional society annual congresses (2011 to 2015). We did not impose language or other restrictions. We performed a citation search using PubMed and examined reference lists of relevant studies and reviews. We contacted corresponding authors for details of additional published or unpublished work. We searched for unpublished studies and ongoing trials on the International Clinical Trials Registry Platform (apps.who.int/trialsearch) (April 2016). Selection criteria: We included randomized and quasi-randomized controlled trials that evaluated cough augmentation compared to a control group without this intervention. We included non-randomized studies for assessment of harms. We included studies of adults and of children aged four weeks or older, receiving invasive mechanical ventilation in a high-intensity care setting. Data collection and analysis: Two review authors independently screened titles and abstracts identified by our search methods. Two review authors independently evaluated full-text versions, independently extracted data and assessed risks of bias. Main results: We screened 2686 citations and included two trials enrolling 95 participants and one cohort study enrolling 17 participants. We assessed one randomized controlled trial as being at unclear risk of bias, and the other at high risk of bias; we assessed the non-randomized study as being at high risk of bias. We were unable to pool data due to the small number of studies meeting our inclusion criteria and therefore present narrative results rather than meta-analyses. One trial of 75 participants reported that extubation success (defined as no need for reintubation within 48 hours) was higher in the mechanical insufflation-exsufflation (MI-E) group (82.9% versus 52.5%, P < 0.05) (risk ratio (RR) 1.58, 95% confidence interval (CI) 1.13 to 2.20, very low-quality evidence). No study reported weaning success or reintubation as distinct from extubation success. One trial reported a statistically significant reduction in mechanical ventilation duration favouring MI-E (mean difference -6.1 days, 95% CI -8.4 to -3.8, very low-quality evidence). One trial reported mortality, with no participant dying in either study group. Adverse events (reported by two trials) included one participant receiving the MI-E protocol experiencing haemodynamic compromise. Nine (22.5%) of the control group compared to two (6%) MI-E participants experienced secretion encumbrance with severe hypoxaemia requiring reintubation (RR 0.25, 95% CI 0.06 to 1.10). In the lung volume recruitment trial, one participant experienced an elevated blood pressure for more than 30 minutes. No participant experienced new-onset arrhythmias, heart rate increased by more than 25%, or a pneumothorax. For outcomes assessed using GRADE, we based our downgrading decisions on unclear risk of bias, inability to assess consistency or publication bias, and uncertainty about the estimate of effect due to the limited number of studies contributing outcome data. Authors' conclusions: The overall quality of evidence on the efficacy of cough augmentation techniques for critically-ill people is very low. Cough augmentation techniques when used in mechanically-ventilated critically-ill people appear to result in few adverse events.
Keywords: acute respiratory failure; artificial ventilation; breathing exercise; controlled clinical trial (topic); coughing; critically ill patient; depressed blood pressure; elevated blood pressure; extubation; heart arrhythmia; hemoptysis; human; length of stay; lung volume; mortality rate; noninvasive ventilation; pneumothorax; priority journal; randomized controlled trial (topic); review; tracheostomy; ventilator weaning

QUAD fever: Beware of non-infectious fever in high spinal cord injuries - Goyal J.; Jha R.; Bhatia P.; Mani R.K. (BMJ Case Reports, 2017)
A case of cervical spinal cord injury and quadriparesis with prolonged fever is being described. Initially, the patient received treatment for well-documented catheter-related bloodstream infection. High spiking fever returned and persisted with no obvious evidence of infection. The usual non-infectious causes too were carefully excluded. QUAD fever or fever due to spinal cord injury itself was considered. The pathogenetic basis of QUAD fever is unclear but could be attributed to autonomic dysfunction and temperature dysregulation. Awareness of this little known condition could help in avoiding unnecessary antimicrobial therapy and in more accurate prognostication. Unlike several previous reported cases that ended fatally, the present case ran a relatively benign course. The spectrum of presentations may therefore be broader than hitherto appreciated.
Keywords: C reactive protein; cefoperazone; D dimer; enoxaparin; hypertensive factor; meropenem; procalcitonin; sulbactam; abdominal radiography; adult; antibiotic sensitivity; antibiotic therapy; article; blood culture; blood pressure; breathing rate; case report; cervical spinal cord injury; clinical outcome; computer assisted tomography; deep vein thrombosis; differential diagnosis; drug indication; drug substitution; drug withdrawal; echography; emergency ward; erythrocyte sedimentation rate; extended spectrum beta lactamase producing Escherichia coli; falling; fever; follow up; Glasgow coma scale; heart rate; hospital acquired pneumonia; hospital discharge; human; hyperthyroidism; laboratory test; leukocyte count; limb weakness; male; middle aged; musculoskeletal system examination; neuroimaging; neurologic examination; nuclear magnetic resonance imaging; oxygen saturation; paralysis; priority journal; prophylaxis; protein blood level; QUAD fever; quadriplegia; spine radiography; thorax radiography; thyroid function test; tracheostomy; treatment duration; treatment response; urinalysis; venous thromboembolism; vital sign

The question of whether or not to perform therapeutic hypothermia: A case of neonatal spinal cord injury - Yokoi K.; Kobayashi S.; Muramatsu K.; Suzuki S.; Gotou H. (Journal of Neonatal-Perinatal Medicine, 2017)
Neonatal spinal cord injury is an extremely rare perinatal complication that often occurs concurrently with hypoxic ischemic encephalopathy (HIE), further complicating diagnosis of spinal cord injury. Although therapeutic hypothermia for moderate to severe HIE is widely recommended in Japan, it is difficult to determine whether it satisfies the neurological findings-related entry criteria in some patients. We describe a female infant with neonatal spinal cord injury after forceps delivery, who underwent therapeutic hypothermia upon diagnosis of HIE. The Apgar scores were 5 at 1 min, 6 at 5 min, and not recorded at 10 min. Blood gas analysis of her umbilical artery was not performed. Since respiratory failure, hypotonia and the absence of primitive reflexes were found at 2 hours after birth, she was initially diagnosed with moderate HIE and underwent a therapeutic hypothermia. Magnetic resonance imaging after therapeutic hypothermia revealed the spinal cord was narrowed from the lower medulla oblongata to the upper cervical cord. Thus she was diagnosed with an upper spinal cord injury at that time. Some patients with neonatal spinal cord injuries satisfy the criteria for therapeutic hypothermia. When neonates with asphyxia present with prolonged respiratory failure and hypotonia, spinal cord injury should be considered in the differential diagnosis. Thus, an early MRI is vital for the diagnosis of spinal cord injury.
Keywords: achilles reflex; Apgar score; article; asphyxia; basal ganglion; blood gas analysis; breathing mechanics; case report; electroencephalography; endotracheal intubation; female; fetus distress; fetus movement; forceps delivery; gastrostomy; human; induced hypothermia; medulla oblongata; muscle hypotonia; neonatal intensive care unit; neuroimaging; newborn; nuclear magnetic resonance imaging; priority journal; respiratory failure; spinal cord injury; subdural hematoma; tendon reflex; tracheostomy; umbilical artery; umbilical cord blood

Airway obstruction and neurogenic shock due to severe cervical spine injury - Matthews S.; Shenvi C.L. (American Journal of Emergency Medicine, 2017)
Keywords: epinephrine; noradrenalin; phenylephrine; vasopressin; aged; artery injury; article; blunt trauma; case report; cervical spine injury; computer assisted tomography; erythrocyte transfusion; Glasgow coma scale; heart arrest; heart ejection fraction; hemorrhagic shock; human; male; neurogenic shock; oxygenation; posttraumatic complication; priority journal; resuscitation; return of spontaneous circulation; shock; subarachnoid hemorrhage; supraglottic airway device; trachea obstruction; tracheostomy; traffic accident; vertebral artery; King airway device

Esophago-cutaneous fistula as a late and rare complication of neglected infection in anterior cervical plating - Kong Y.M.; Chan S.K.; Foo C.H.; Jafaar M.S.A.; Tan B.B.; Teo Y.Y.; Wong C.C. (Malaysian Orthopaedic Journal, 2017)
Introduction: Anterior cervical plating is a common surgery for the treatment of spondylosis, myelopathy, radiculopathy, and cervical spine trauma. Formation of esophago-cutaneous fistula has a rare incident of less than 0.1%1. Common causes of fistula formation are pressure necrosis; loose implants or bone graft; or esophageal injury during operation;2 but in our cases, neglected low grade infection might be the most possible cause. We report 2 cases of esophageo-cutaneous fistula after anterior cervical plating, in traumatic spine injury patients, with chronic history of infection. Case series: Mr A is a 25 years old gentleman with quadriplegia following C6 burst fracture. Anterior C6 cervical corpectomy and fusion was performed. He was subsequently on tracheostomy and PEG tube. 2 years later, he presented with fever, productive cough and shortness of breath for 2months. On examination, he had an anterior neck wound with pus discharge. CT fistulogram revealed esophagocutaneous fistula. He refused further surgery and opted conservative management. Mr B is a 22 years old gentleman with traumatic C5 burst fracture, treated with anterior C5 cervical corpectomy and fusion. 2 years later, he presented with chronic productive cough for 3 months, associated with low grade fever and dysphagia. He also had a discharging sinus from previous operation site. Direct laryngoscopy showed anterior cervical plate erosion into posterior esophageal wall, and communicated with the anterior neck fistula with persistent pus discharge. Anterior cervical plate was removed and posterior esophageal wall was repaired by plastic surgery team. Discussion: Esophagocutaneous fistula is a rare complication, which are usually associated with esophageal injury. As for our case series, the possible cause could be chronic neglected infection, along with resultant salivary leakage into surrounding soft tissue. This ultimately leads to communication of the esophagus with the skin and hence a fistula. Multidisciplinary teams approach is recommended in the management of this complication. Conservative treatment is suggested for early and small esophageal perforations, whereas surgical intervention may be considered for large defects. Elias et al recommended all anterior hardware to be removed, and posterior instrumentation in case of non fusion3. In our cases, both patients had delay presentation of esophagocutaneous fistula, and chose 2 totally different way in managing a similar complication. So far operative management have showed immediate of fistula closure. Whereas patient who chose conservative management still on daily dressing. Conclusion: Esophagocutaneous fistula is a rare complication following anterior cervical plating, where neglected infection could be a possible cause. Nevertheless, it can be managed conservatively or surgically. Surgery always expedite fistula closure.
Keywords: adult; anterior cervical plate; case report; case study; clinical article; complication; computer; conference abstract; conservative treatment; coughing; dysphagia; dyspnea; esophagus injury; esophagus perforation; female; fever; fistulography; fracture; human; laryngoscopy; male; neck; percutaneous endoscopic gastrostomy tube; plastic surgery; pus; quadriplegia; skin fistula; soft tissue; spine injury; surgery; tracheostomy; wound; young adult

Low cost quality initiatives for management of neurosurgical patients in developing nations: Perspective from a tertiary care centre in Pakistan - Rai H.; Waqas M.; Shamim M. (Surgical Neurology International, 2017)
Background: Targeting resources on cost-effective care strategies is pertinent for developing nations, specifically for already burdened specialties such as neurosurgery, where without state support and insurance companies, expenses are borne by the patient themselves. Methods: This was a descriptive review of literary work published by the section of neurosurgery at Aga Khan University Hospital, Pakistan. We searched PubMed and CiNAHL databases to identify articles, which were then critically analyzed and discussed from a perspective of low-cost quality care. Results: We demonstrate the cost effectiveness of three initiatives, namely, blood ordering protocol for elective spine arthrodesis, nonoperative management being superior to surgical stabilization in spine injury patients with complete neurological deficits, and early tracheostomy in patients with isolated severe traumatic brain injury. Conclusion: Initiation and implementation of such cost-effective strategies without compromising quality health standards must be emphasized by neurosurgical centers throughout the developing world for smart allocation and utilization of funds.
Keywords: arthrodesis; article; cost effectiveness analysis; developing country; elective surgery; health care cost; health care quality; human; injury severity; major clinical study; Pakistan; priority journal; spine arthrodesis; spine injury; tertiary care center; tracheostomy; traumatic brain injury; treatment planning

Clinical characteristics and short-term outcome of critical neuro-invasive west nile virus infection - Hawkes M.A.; Carabenciov I.D.; Rabinstein A.A. (Neurocritical Care, 2017)
Introduction Neuro-invasive West Nile Virus infection (NI-WNVI) can cause cerebral and spinal cord involvement, which can result in critical illness. The characteristics and prognosis of patients with NI-WNVI who require ICU admission and their prognosis are not well known. Methods Retrospective review of patients with diagnosis of NI-WNV treated at Mayo Clinic ICUs from 1999 to 2016. Demographic data, presenting symptoms, history of immunosuppression, diagnostic work-up, hospital length of stay, medical complications and modified Rankin Scale (mRs) at discharge were assessed. Available follow-up was also reviewed to assess mortality. Results Sixteen patients (94% male), aged 57±16 years with NI- WNVI were admitted to the ICU. Seven were immunocompromised. Clinical presentations were encephalomyelitis (n=11), encephalitis (n=4), and myelitis (n=1). Three patients developed coma and 14 required mechanical ventilation. Median cells, proteins and glucose in CSF were 70/mm3 (0-698), 101 mg/dl (52-229) and 52 mg/dl (32-95) respectively. WNV serology was diagnostic in 15 patients. CSF antibodies were positive in 9/10 patients. CSF PCR was positive in only 1 of 9 patients tested. Brain and spine MRI were abnormal in 4/12 and 7/8 patients respectively. Main medical complications were pneumonia (n=6), DVT (n= 4), urinary tract infection (n= 2) and gastrointestinal bleeding (n=2). Eight patients had tracheostomy and PEG tube placement. Median hospital stay was 28 days (5-145). The mRS at discharge was 6 (n=1), 5 (n=10), 4(n=3) and 3(n=2). Four additional patients died after a median follow-up of 5.3 months (4.1-86). Conclusions The short-term prognosis of NI-WNVI requiring ICU care is poor and mortality rate is relatively high. More data on the long-term prognosis of these patients are needed to guide the goals of care in the acute setting.
Keywords: antibody; glucose; adult; artificial ventilation; clinical article; coma; complication; demography; diagnosis; encephalitis; encephalomyelitis; female; follow up; gastrointestinal hemorrhage; hospitalization; human; immunosuppressive treatment; length of stay; male; mortality rate; nonhuman; nuclear magnetic resonance imaging; pneumonia; polymerase chain reaction; prognosis; Rankin scale; serology; spine; tracheostomy; urinary tract infection; West Nile virus

Non-invasive positive pressure ventilation during the management of severe spinal muscular atrophy type I - Inoue K.; Kimura N.; Higuchi Y.; Nakamoto M.; Imai T.; Nishio H. (No To Hattatsu, 2017)
Patients with spinal muscular atrophy type (SMA) with the onset before the age of 3 months are considered as severe form of SMA (severe SMA) and have poor prognosis. Here, we report the efficacy of non-invasive positive pressure ventilation(NPPV)in a patient with severe SMA. She was born with generalized hypotonia and feeding difficulties, and had SMN1 gene mutations (the deletion of exons 7 and 8). At 1 month of age, she was intubated because of respiratory failure due to a respiratory tract infection, and extubation proved difficult. Her parents decided that NPPV and a mechanical in-exsufflator (MI-E) should be used for respiratory management rather than a tracheotomy. The NPPV improved her peripheral coldness, cold sweats, chest wall movement, and heart rate and enabled her to sleep well. At 1 year and 2 months, chest computed tomography revealed mild pneumonia and did not show any atelectasis. The NPPV facilitated discharge, and the patient had a good quality of life (QOL) from the point of view of voice production, the ability to move easily, the simplicity of bathing, and the low level of discomfort she experienced. However, she suffered repeated episodes of aspiration pneumonia and airway obstruction (by sputum) after 11 months of age. Thereafter, she required continuous NPPV and high-span inspiratory positive airway pressure (21 cmH2O). At 1 year and 4 months, she died of respiratory failure at home. As her bulbar weakness worsened, respiratory management with NPPV became difficult. However, the long-term use of NPPV together with high-span positive inspiratory pressure plus positive end-expiratory pressure, and a high-pressure MI-E at an early age might improve respiratory management outcomes and patient prognosis. In our case, NPPV was effective at improving ventilation and preventing atelectasis and helped to provide the patient with a good QOL.
Keywords: airway obstruction; article; aspiration pneumonia; bulbar paralysis; case report; cold sweat; computer assisted tomography; extubation; female; gene mutation; heart rate; human; muscle hypotonia; noninvasive ventilation; pneumonia; quality of life; respiratory failure; respiratory tract infection; spinal muscular atrophy

Ventilator weaning in cervical level spinal injuries with diaphragmatic pacing - Morris M.; Wenzel L.R. (Journal of Spinal Cord Medicine, 2017)
Objective: We report the cases of several individuals with tetraplegia after cervical trauma or injury who subsequently underwent diaphragmatic pacemaker implantation to assist with weaning from a ventilator. The individuals included in this report were admitted to inpatient rehabilitation with goals for general rehabilitation and the possibility of ventilator weaning. The individuals we report ultimately underwent diaphragmatic pacemaker implantation following injuries that subsequently required tracheostomy and ventilator placement. The individuals we report were successively able to partially or completely wean from the ventilator with the use of a diaphragmatic pacer. We will review the major indications and contraindications for diaphragmatic pacing and ventilator weaning processes. We will discuss the benefits and barriers noted with diaphragmatic pacing in individuals that are ventilator dependent with cervical level spinal cord injuries, as well as the general process utilized for ventilator weaning with the use of a diaphragmatic pacer. We will explain the roles of various healthcare providers that assist with ventilator weaning including physicians, respiratory therapists and speech language pathologists. Finally, we will highlight the positive outcomes related to ventilator weaning in patients with cervical injuries as it relates to improved quality of life outcomes. Design: Retrospective research design completed in a rehabilitation hospital. Participants/methods: Three individuals with tetraplegia were included in a retrospective research study looking at weaning from the ventilator with use of a diaphragmatic pacer. All research completed was retrospective. Results: All individuals achieved full or partial weaning from the ventilator with successful placement of diaphragmatic pacers. Conclusion: Diaphragmatic pacers can greatly improve quality of life outcomes in individuals with cervical level spinal cord injuries.
Keywords: adult; case report; cervical spine injury; diaphragm; female; health care personnel; hospital patient; human; pacemaker implantation; quadriplegia; quality of life; rehabilitation; respiratory therapist; retrospective study; speech language pathologist; spinal cord injury; tracheostomy; ventilator weaning

Progressive Cervical Spinal Cord Atrophy after a Traffic Accident - Inoue K.; Kumada T.; Fujii T.; Kimura N. (Journal of Pediatrics, 2017)
Keywords: anuria; article; case report; cervical spinal cord; child; disease course; female; human; medulla oblongata; muscle strength; nuclear magnetic resonance imaging; patient transport; physical examination; preschool child; priority journal; quadriplegia; spinal cord; spinal cord atrophy; swelling; tendon reflex; tracheostomy; traffic accident; ventilator

Microorganisms associated with vibrating mesh and jet nebulizers during mechanical ventilation in the acute care setting: A randomized controlled trial - Dubosky M.N.; Lamorena E.; Jones C.; Roberts K.; Kim Y.; Flaws M.L.; Vines D.L. (American Journal of Respiratory and Critical Care Medicine, 2017)
Background: Contaminated nebulizers may spread bacteria to the ventilator circuit and lungs. Differences in design and circuit placement between jet nebulizer (JN) and vibrating mesh nebulizers (VMN) may influence nebulizer contamination and subsequently, the patient. The aim of this research was to compare bacterial growth of the nebulizer, ventilator circuit and sputum between JNs and VMNs. Methods: A randomized, controlled trial, approved by the Institutional Review Board of a Midwest academic medical center. Mechanically ventilated adult subjects with an endotracheal tube and a physician order for aerosol treatment were randomized to either a JN (Misty Max 10™, CareFusion, San Diego, California) or VMN (AeroNeb® Solo, Aerogen, Galway, Ireland). Subjects were excluded if intubated for less than 24 hours, if tracheostomy present on admission, or if diagnosed with diaphragmatic paralysis high spinal cord injury, or brain death. JNs were placed into a spring loaded t-piece 15 cm from the y-piece and operated at 8 L/min, and replaced every 3 days. VMNs were placed on the dry side of the heater water chamber and changed at end of ventilation, up to 28 days. Cultures of aerosol produced by the nebulizers and inspiratory circuit limb were obtained and plated every three days and after mechanical ventilation ended. A dose of 3 mL of normal saline was nebulized with plume directed towards each of 4 agar plates for 1 minute. The 4 agar plates (Remel Products, Lenexa, Kansas) included MacConkey, Chocolate, CNA Blood, and Sheep blood. Ventilator circuit cultures were collected from the rim of the inspiratory limb of the circuit at the y-piece by rubbing a culture swab along the circuit in a circular motion three times. Swabs, along with plate samples were sealed in a biohazard bag labeled with the subject number and sent to the microbiology lab for analysis. Differences in contamination were assessed using Chi-square. Results: See Table 1. 120 Subjects were enrolled with 30 subjects excluded leaving 48 in the JN and 42 in the VMN groups. Conclusions: There was no statistical difference in overall bacteria growth between the aerosol produced by the nebulizers. The VMN group had a significantly higher growth in the circuit, however no difference was found in sputum bacteria growth. Sputum and nebulizer bacteria matched in only one subject. Future studies should concentrate on handling practices as a primary source of nebulizer contamination.
Keywords: sodium chloride; water; adult; aerosol; artificial ventilation; bacterial growth; biosafety; blood; brain death; California; chocolate; contamination; controlled clinical trial; controlled study; diagnosis; diaphragm paralysis; emergency care; endotracheal tube; female; human; institutional review; Ireland; Kansas; limb; major clinical study; male; microbiology; microorganism; motion; nebulizer; nonhuman; physician; plume; randomized controlled trial; sheep; spinal cord injury; spring; sputum; tracheostomy; university hospital; ventilator

Retrograde intubation in a patient with neglected cervical spine injury for sacral advancement flap - Sriramka B.; Pattnaik S.K. (Sri Lankan Journal of Anaesthesiology, 2017)
Neglected cervical spine injury is a challenging scenario for an anaesthetist. Fixed deformities, altered local anatomy, fear of neurological worsening makes intubation difficult and prevents use of manoeuvers in classical intubation. Advances in equipment like fiberoptic bronchoscope or the video laryngoscope aid in such situations. Retrograde intubation (RI) is a useful alternative with high success rate in safe and experienced personnel. The purpose of the article is to highlight the use of this slowly becoming antique technique which becomes quite handy in difficult airway situations.
Keywords: fentanyl; glycopyrronium; lidocaine; midazolam; neostigmine; adult; article; body mass; case report; cervical spine injury; clinical observation; decubitus; falling; hospital readmission; human; intensive care; laryngoscopy; male; neck malformation; occupational accident; paraplegia; patient positioning; postoperative care; premedication; preoperative evaluation; respiratory tract intubation; skin transplantation; thorax injury; thorax radiography; thorax surgery; tissue flap; tracheostomy

Pulmonary rehabilitation using mechanical insufflation-exsufflation therapy for spinal cord injury – Two case studies in the university Malaya medical centre - Tan J.H.; Fauzi A.A.; Hasnan N. (Journal of Health and Translational Medicine, 2017)
There is a high incidence of 36% to 83% of respiratory dysfunction in patients with acute cervical spinal cord injury. Complications arising from respiratory dysfunction remain one of the most common causes of morbidity and mortality in the spinal cord injured population. Specialized pulmonary care and therapy can help individuals with tetraplegia to maintain a stable respiratory status allowing active participation in active rehabilitation. This would allow them to achieve rehabilitation goals of independent function and community reintegration. Mechanical insufflation-exsufflation (MIE) therapy is an option for secretion management in individuals with acute spinal cord injury. In this paper, we present our experience using MIE as an adjunct to management of secretions in the spinal cord injured population at the University of Malaya Medical Centre.
Keywords: bronchodilating agent; mucolytic agent; adult; article; atelectasis; breathing exercise; bronchoscopy; case report; cervical spine fracture; clinical article; coughing; distal radius fracture; falling; fifth cervical vertebra; hospital acquired pneumonia; hospital infection; human; intensive care; Malaysia; male; mechanical insufflation exsufflation therapy; middle aged; osteosynthesis; peak cough flow; pulmonary rehabilitation; quadriplegia; recurrent infection; respiratory tract parameters; spinal cord injury; spinal cord transsection; spinal cord tumor; spine fusion; tracheobronchial toilet; tracheostomy; university hospital

An autopsy case of respiratory failure induced by repetitive cervical spinal cord damage due to abnormal movement of the neck in athetoid cerebral palsy - Takei Y.-I.; Koshihara H.; Oguchi K.; Oyanagi K.; Ohara S. (Internal Medicine, 2017)
We herein report the clinical and autopsy findings of a 48-year-old right-handed man with athetoid cerebral palsy who suffered from cervical myelopathy due to abnormal neck movement, and who died of respiratory failure. Pathologically, the external appearance of the ventral surface of the cervical spinal cord revealed a linear indentation running obliquely at the level between the C4 and C5 segments. In the most severely compressed lesion, the gray matter was predominantly affected and severely atrophic. Microscopically, clusters of oligodendrocytes associated with thinly myelinated axons were also observed in the lateral funiculus. The latter findings are unique, and could be interpreted as regenerative and/or restorative phenomena of the central nervous system following chronic repetitive spinal cord compression.
Keywords: abnormal neck movement; adult; arterial gas; article; autopsy; cerebral palsy; cervical myelopathy; cervical spinal cord injury; cervical spine radiography; consciousness disorder; constipation; dentate nucleus; dyskinesia; fifth cervical vertebra; fourth cervical vertebra; gray matter; human; human tissue; hypercapnia; immunohistochemistry; lung alveolus hypoventilation; lung emphysema; lung fibrosis; male; middle aged; muscle atrophy; muscle weakness; nuclear magnetic resonance imaging; pleura effusion; positive end expiratory pressure; quadriplegia; respiratory failure; scoliosis; spinal cord atrophy; spinal cord compression; substantia nigra; tendon reflex; tracheostomy; urine retention; x-ray computed tomography

Multi-disciplinary outpatient care of ventilator-assisted adults in a university setting - Brown J.P.; Hanley J.; Rochefort K.; Kurili A.; Schotland H.; Sitrin R.G. (American Journal of Respiratory and Critical Care Medicine, 2017)
Rationale: Due to complex qualifying and documentation requirements and the availability of increasingly complex multifunctional devices, input from multiple disciplines has become increasingly important to provide effective care for ventilator dependent individuals. Complex care in a multi-disciplinary clinic environment has been shown to improve quality of care and reduce cost of care for diseases such as cystic fibrosis, organ transplant and ALS. Methods: IRB approved review of current and former patient records. Results: The adult Assisted Ventilation Clinic (AVC) was created at U of M in fiscal year 2010 as a multi-disciplinary outpatient service to accommodate the needs of patients who would benefit from long-term ventilatory support as outpatients. The clinic team is comprised of a pulmonologist, respiratory therapist, nurse practitioner, physiatrist, neurologist, social worker and a dietitian. Underlying diagnoses in FY 2016 include spinal cord injury with quadriplegia, ALS, muscular dystrophies and myopathies, phrenic nerve injury, and multiple sclerosis. Of these diagnoses, spinal cord injury comprises 30% of total patients and ALS is 11% of total patients. Approximately 75% of patients in the clinic have a diagnosis of a primary neuromuscular disease. Of the remaining patients, 7% have a congenital disorder such as cerebral palsy, 3% have a central hypoventilation disorder. 10% have complex sleep apnea and obesity hypoventilation. Only 4% have COPD or other intrinsic lung disease as the primary cause of respiratory failure. 87% of patients are receiving either non-invasive or invasive ventilatory support and tracheostomy is present in 29% of our patients. New patient clinic visits increased from 24 in FY 2010 to 66 in FY 2016, an increase of 275%. Return visits increased from 175 in FY 2010 to 560 in FY 2016, an increase of 320%. By 7/2016, a total of 806 patients were provided service, with a roster of 502 active patients. Conclusions: We conclude that an academic tertiary care center can support a large multi-disciplinary outpatient service specializing in the care of ventilator-dependent adults. Our outpatient clinic population is primarily serving patients with neuromuscular respiratory failure requiring positive pressure ventilator support and we have experienced substantial growth in both new and return visits over the last four fiscal years. Our data could be used to develop comparable services at other institutions to address the challenging needs for this complex clinical population.
Keywords: adult; artificial ventilation; assisted ventilation; cerebral palsy; chronic obstructive lung disease; diagnosis; dietitian; female; human; hypoventilation; major clinical study; male; medical record; multiple trauma; muscular dystrophy; neurologist; nurse practitioner; obesity; outpatient care; outpatient department; phrenic nerve; physiatrist; pulmonologist; quadriplegia; respiratory failure; respiratory therapist; sclerosis; sleep disordered breathing; social worker; spinal cord injury; tertiary care center; tracheostomy; university; ventilator

Acquired tracheomalacia secondary to excessive cuff inflation - Atchley W.; Kakkera K.; Vilensky V.; Meena N. (American Journal of Respiratory and Critical Care Medicine, 2017)
Introduction: Acquired tracheomalacia (TM) in the setting of prolonged tracheostomy dependence is a potential complication that is thought to result from local trauma and resultant tissue injury secondary to elevated cuff pressures and intubation injuries. This case highlights a severe illustration of TM and its complications in a chronically ventilated patient. Case Presentation: A 28 year-old man with medical history significant for spinal cord injury and quadriplegia secondary to a gunshot wound in 2012 with resulting chronic respiratory failure and tracheostomy dependence presented from outside facility for persistent air leak around his indwelling tracheostomy. He also was treated for sepsis in the setting of recurrent ventilator associated pneumonia with broad-spectrum antimicrobials after radiographic findings were consistent with pneumonia. The interventional pulmonary service was consulted for evaluation of the tracheostomy and trachea after a CT scan revealed apparent severe dilatation of the proximal trachea near the site of the tracheostomy cuff. A bronchoscopic evaluation via the nasal pathway and trachea was performed revealing the trachea to be severely dilated in the area around the balloon with signs of complete deterioration of the tracheal rings and formation a blind pouch extending outward from the trachea. The trachea distal to the tracheostomy site was normal and patent (see figure). The patient's home tracheostomy was a Bivona size 8, which the patient inflated with 30cc of saline as per his personal preference. After discovery of the tracheomalacia, he had a Shiley 8 XLT tracheostomy placed with improvement in his air leak. Later this was changed to a Bivona 8.5 120 mm to completely bypass the dilatation with continued improvement. Despite the above measures, the patient continues to be at high risk for tracheal rupture, erosion and aspiration secondary to severely weakened tracheal integrity. Discussion: This case highlights a potential serious complication of prolonged ventilation via tracheostomies and the danger of hyper-inflating the cuff. We recommend that in patients with a requirement for a cuffed tracheostomy, the cuff be inflated with the minimal air/saline required to safely ventilate the patient.
Keywords: sodium chloride; adult; adverse device effect; artificial ventilation; aspiration; balloon; case report; chronic respiratory failure; complication; deterioration; dilatation; gunshot injury; human; male; medical history; patent; pneumatic cuff; quadriplegia; rupture; sepsis; spinal cord injury; tracheomalacia; tracheostomy; ventilator associated pneumonia; x-ray computed tomography

Two cases of ventricular dysrhythmias from aconitine poisoning - Li K.; Vo K.; Repplinger D.; Fouladkou F.; Lynch K.; Smollin C. (Clinical Toxicology, 2017)
Background: Aconitine is a deterpenoid alkaloid found in plants of the Aconitium genus that contain potent cardiovascular and neurological toxins. Most reported cases of aconitine poisoning have been related to the use of traditional Chinese medications. These herbs are used in the treatment of rheumatism, arthritis, and other ailments. Aconitine acts as a voltage-sensitive sodium channel opener that results in paresthesias, muscle weakness, ventricular ectopic rhythms, heart block and ventricular arrhythmias. Case reports: Patient 1: A 56-year-old female presented with chest pain and weakness 1 h after preparing and drinking an herbal tea. On arrival, she was found to have rapidly evolving tachydysrhythmias including bidirectional ventricular tachycardia. She was treated with adenosine, diltiazem, cardioversion, amdiodarone, lidocaine, and ultimately developed hypotension requiring a dopamine infusion and intubation. Given her persistently unstable hemodynamics, she was started on extracorporal membrane oxygenation. She had multiple complications including a retroperitoneal hemorrhage, right leg ischemia requiring an above the knee amputation, spinal cord ischemia with paralysis, and prolonged ventilation requiring tracheostomy. The patient died from her complications approximately one month after initial presentation. Serum concentrations of aconitine on presentation and at 4 h were 3.4 ng/mL and 5.6 ng/mL. Patient 2: A 36-year-old male presented with paresthesias approximately 1 h after preparing and drinking an herbal tea. He had witnessed syncope prior to presentation. Clinical course was complicated by rapidly evolving tachydysrhythmias including polymorphic ventricular tachycardia, superventricular tachycardia, bigeminy, and bidirectional ventricular tachycardia. The patient had progressively worsening altered mental status and hypotension requiring intubation and norepinephrine. Treatments also included calcium, sodium bicarbonate, magnesium, cardioversion, and amiodarone. The patient was extubated on hospital day 2, and subsequently discharged in good condition. Serum concentration of aconitine was 1.8 ng/mL approximately 3.5 h after initial presentation. Case discussion: These two patients presented to separate hospitals in the same city within a one-month period triggering a public health investigation. Samples of the original herbs used in the tea preparations were obtained from each patient's homes and also confirmed positive for aconitine. Both products were ultimately traced back to the same vendor. Conclusions: Aconitine is commonly used in traditional Chinese herbal medicines and is a potential source of significant cardiovascular toxicity.
Keywords: aconitine; adenosine; amiodarone; bicarbonate; calcium; diltiazem; endogenous compound; herbaceous agent; lidocaine; magnesium; noradrenalin; voltage gated sodium channel; aconite; adult; cardiotoxicity; cardioversion; case report; doctor patient relation; drinking; drug therapy; extracorporeal oxygenation; faintness; female; heart block; heart ventricle arrhythmia; hemodynamics; herbal medicine; herbal tea; human; human tissue; hypotension; intoxication; intubation; knee amputation; leg ischemia; male; mental health; middle aged; muscle weakness; nonhuman; paralysis; paresthesia; polymorphic ventricular tachycardia; public health; retroperitoneal hemorrhage; spinal cord ischemia; thorax pain; tracheostomy

The results of early surgical decompression and stabilisation for acute traumatic spinal cord injury in patients with concomitant chest injuries - Alrawi A.; Sewell M.; Maguire R.; Williams R.; Mecci M.; Prasad M.; Aziz F.; Tizzard S. (British Journal of Neurosurgery, 2017)
Objectives: The benefits of early surgical decompression and stabilisation (within 24 hours of injury) for patients with acute traumatic spinal cord injury (SCI) is unclear. The objective of this study was to investigate the effects of early (<24 hrs of injury) versus late (>24 hrs of injury) decompressive and stabilisation surgery for traumatic cervical SCI in patients with concomitant chest injuries. Design: Multicentre retrospective cohort Subjects: Adults with traumatic cervical SCI, GCS >13 and concomitant chest injuries (e.g. haemopneumothoraces, flail chest, multiple rib fractures >3 and/or pulmonary contusions) requiring intensive care unit (ICU) admission. Methods: Thirty-eight consecutive patients who met the inclusion criteria and underwent surgery within 24 hours were compared with 43 patients who underwent decompressive surgery after 24 hours. The primary outcomes were ordinal change in ASIA impairment scale (AIS) at 6 months and ICU stay. Secondary outcomes included complications within 30 days and requirement for a tracheostomy. Results: In the early surgery group, AIS grade improvement was as follows: 21 (55%) no improvement, 13 (34%) had a 1 grade improvement and 4 (11%) had a 2 grade improvement. Mean ICU stay was 17 days (3-71). 42% developed a complication and 50% required a tracheostomy. In the late surgery group, AIS grade improvement was as follows: 28 (65%) no improvement, 13 (30%) had a 1 grade improvement and 2 (4.5%) had a 2 grade improvement. Mean ICU stay was 24 days (4-68). 53% developed a complication and 56% required a tracheostomy. There was one mortality in the late surgery group. Conclusions: For patients with acute traumatic cervical SCI and concomitant chest trauma, early surgical decompression and stabilisation was associated with improved neurological outcome, reduced ICU stay and a lower complication rate.
Keywords: adult; American Spinal Injury Association impairment scale; cervical spinal cord injury; clinical article; clinical trial; controlled clinical trial; controlled study; decompression; flail chest; human; intensive care unit; lung contusion; mortality; multicenter study; surgery; thorax injury; tracheostomy

Spinal complications after tracheal resection with anastomosis for cicatricial stenosis - Parshin V.D.; Isaykin A.I.; Parshin V.V.; Gorshkov K.M.; Nogtev P.V.; Starostin A.V.; Parshin A.V. (Khirurgiia, 2017)
Keywords: adolescent; anastomosis; brain; bronchoscopy; case report; diagnostic imaging; female; human; male; neurologic examination; nuclear magnetic resonance imaging; pathophysiology; patient care; postoperative complication; procedures; spinal cord disease; trachea stenosis; tracheotomy; treatment outcome; young adult

Biotinidase deficiency mimicking neuromyelitis optica beginning at the age of 4: A treatable disease - Girard B.; Bilbault C.; Bonnemains C.; Schmitt E.; Raffo E. (Multiple Sclerosis Journal, 2017)
Background: Metabolic and inflammatory conditions may lead to neurological disorders. Neuromyelitis optica spectrum disorders (NMOSDs) refer to a rare group of demyelinating diseases of the central nervous system which essentially involve the optic nerves and spinal cord. Methods: We report a case of biotinidase deficiency (BD) initially misdiagnosed as NMOSD in a pediatric patient. Results: An 8-year-old girl was initially diagnosed with NMOSD on the basis of optic neuritis (ON) associated with three episodes of longitudinally extensive transverse myelitis (LETM). Intravenous highdose corticosteroids were effective during the first two episodes of LETM. The third acute episode which resulted in tetraplegia, respiratory distress, and blindness was refractory to corticosteroids, plasmapheresis, and rituximab. The unusual clinical course and persistent high levels of plasma and cerebrospinal fluid (CSF) lactate led to additional metabolic investigations being performed. Acylcarnitine profile revealed increased C5-OH acylcarnitine suggestive of BD. Diagnosis was confirmed by direct assessment of plasma enzyme activity (quantified as 5% of the control value). Genetic analysis revealed two mutations, c.643C>T (p.L215F) and c.1612C>T (p.R538C), in the BTD gene (3p25). Dramatic clinical improvement occurred after long-term oral biotin treatment. Conclusion: BD is a treatable condition that may closely mimic the neurological findings of LETM and NMOSD.
Keywords: biotin; biotinidase; corticosteroid; gadolinium; immunoglobulin G; lactic acid; rituximab; article; biotinidase deficiency; blindness; brain radiography; BTD gene; case report; Caucasian; child; chromosome 3p; clinical article; clinical feature; color blindness; correlational study; diagnostic error; disease association; disease course; disease severity; drug efficacy; drug megadose; drug resistance; drug withdrawal; eczema; electrophoresis; endotracheal intubation; enzyme activity; enzyme blood level; eye disease assessment; female; fever; follow up; gene; gene mutation; genetic analysis; hemiparesis; human; immunoglobulin blood level; lactate blood level; long term care; longitudinally extensive transverse myelitis; longitudinally extensive transverse myelitis; lung function; medical history; myelitis; myelooptic neuropathy; neurologic disease assessment; neuroradiology; nuclear magnetic resonance imaging; optic neuritis; paraplegia; physical examination; plasmapheresis; protein cerebrospinal fluid level; quadriplegia; relapse; respiratory distress; tracheotomy; treatment duration; urine retention; visual system examination

Acute transverse myelitis associated with salmonella bacteremia: A case report - Richert M.E.; Hosier H.; Weltz A.S.; Wise E.S.; Joshi M.; Diaz J.J. (American Journal of Case Reports, 2016)
Objective: Rare disease Background: Acute transverse myelitis (ATM) is an uncommon and often overlooked complication of certain bacterial and viral infections that can have a rapid onset and result in severe neurological deficits. Case Report: This case report describes a previously healthy 28-year-old woman who presented to the trauma center after developing acute paralysis and paresthesias of all four extremities within the span of hours. The initial presumptive diagnosis was spinal cord contusion due to a fall versus an unknown mechanism of trauma, but eventual laboratory studies revealed Salmonella bacteremia, indicating a probable diagnosis of parainfectious ATM. Conclusions: This case illustrates the importance of considering the diagnosis of parainfectious ATM in patients presenting with acute paralysis with incomplete or unobtainable medical histories.
Keywords: aquaporin 4; ceftriaxone; glucose; immunoglobulin G; metronidazole; protein; vancomycin; acute transverse myelitis; adult; African American; article; bacteremia; bacterial growth; blood culture; case report; computer assisted tomography; disease association; endotracheal intubation; erythrocyte count; falling; female; hospital admission; human; hyperlactatemia; intensive care unit; laboratory test; leukocyte count; lumbar puncture; lymphocyte count; myelitis; nonhuman; nuclear magnetic resonance imaging; paralysis; paresthesia; plasma exchange; quadriplegia; reference value; respiratory failure; Salmonella enterica; spinal cord injury; spine fracture; tracheostomy

A novel method of fixation of tracheostomy tube in cervical spine surgeries with posterior approach - Gupta D.; Khandelwal A.; Haldar R. (Journal of Neurosciences in Rural Practice, 2016)
Keywords: tracheostomy tube; absence of complications; human; intermethod comparison; letter; patient positioning; priority journal; procedures; spine surgery; surgical approach; suture technique; tracheostomy tube

Central sleep apnea following cervical spine injury - Pannu A.; Patlak J.; Ciampa E.; Oren-Grinberg A. (Critical Care Medicine, 2016)
Learning Objectives: Spinal cord injury (SCI) increases the incidence of sleep-disordered breathing (SDB). While loss of diaphragmatic function from high cervical lesions is easily recognized, patients with apparently intact respiratory function may still suffer from central sleep apnea that can have dangerous sequelae. We describe a case of central sleep apnea which developed in the acute phase following a cervical SCI. Methods: A previously healthy 36 year old man with a BMI of 32 had immediate lower extremity paralysis following a diving injury. CT scan revealed jumped facets at C6-7 bilaterally with anterolisthesis of C6 on C7. He underwent uneventful repair of C6-7 fracture dislocation with posterior instrumentation and C3-T2 spinal fusion and was easily extubated following the procedure. Postoperative exam showed paralysis of the lower extremities with sensory loss below T4. On postoperative day 3, he continued to be alert and conversant while awake but was noted to have recurrent apneic episodes while asleep. Close observation demonstrated no respiratory effort during these episodes and no signs of upper airway obstruction. Oxygen saturation fell during these episodes to as low as 72% despite supplemental oxygen. Each episode resolved completely when the patient was awakened with immediate return of oxygen saturation to >96%. Respiratory mechanics were optimized by repositioning the patient several times to minimize any airway obstruction and medication review revealed no sedating drugs. Non-invasive bilevel positive airway pressure (BiPAP) failed to prevent hypoxia during these episodes in the setting of absent respiratory effort. Given suspicion for central sleep apnea, elective intubation was discussed in detail with the patient who agreed with the same. He was intubated and subsequently underwent elective tracheostomy. Results: Central sleep apnea is a challenging diagnosis to make in the absence of polysomnography testing given the multiple effects of SCI on respiratory function. While risk factors remain unclear, patients with cervical SCI may present with SDB of central origin.
Keywords: oxygen; adult; airway pressure; body mass; breathing mechanics; central sleep apnea syndrome; cervical spinal cord injury; clinical article; computer assisted tomography; diagnosis; diving; fracture dislocation; human; hypoxia; intubation; lower limb; male; oxygen saturation; paraplegia; polysomnography; risk factor; sensory dysfunction; spine fusion; surgery; tracheostomy; upper respiratory tract obstruction; wakefulness

Ventilatory Management of the Noninjured Lung - Bowton D.L.; Scott L.K. (Clinics in Chest Medicine, 2016)
This article reviews aspects of mechanical ventilation in patients without lung injury, patients in the perioperative period, and those with neurologic injury or disease including spinal cord injury. Specific emphasis is placed on ventilator strategies, including timing and indications for tracheostomy. Lung protective ventilation, using low tidal volumes and modest levels of positive end-expiratory pressure, should be the default consideration in all patients requiring mechanical ventilatory support. The exception may be the patient with high cervical spinal cord injuries who requires mechanical ventilatory support. There is no consensus on the timing of tracheostomy in patients with neurologic diseases.
Keywords: artificial ventilation; blood gas; cerebrovascular accident; human; lung injury; lung ventilation; neurologic disease; neuromuscular disease; organ donor; perioperative period; positive end expiratory pressure; priority journal; review; spinal cord injury; tidal volume; tracheostomy; traumatic brain injury; treatment indication

Intraoperative neck positioning for maxillofacial procedures on patients with cervical spine injuries, is it safe to remove the collar? - Schilling C.; Spiers H.; Hayes E.; Jones T.L.; Manisali M. (British Journal of Oral and Maxillofacial Surgery, 2016)
Introduction: Concomitant cervical spine and maxillofacial injury is reported in 2.2-11% of trauma patients. High-risk c-spine injuries not requiring immediate fixation are immobilised in a semi-rigid collar support, interfering with assessment and timely treatment of maxillofacial injuries. This can lead to extensive (delayed) maxillofacial surgery once healing has occurred. We present (for the first time) magnetic resonance images (MRI) demonstrating the alignment of the cervical spine during common maxillofacial procedures in order to assist spinal surgeons in deciding whether it is safe to undertake surgery without collar support.Methods: T2-weighted axial and sagittal MRI of the c-spine was acquired in a healthy volunteer and reviewed by a spinal surgeon. The following intraoperative neck positions were simulated using a head ring +/- shoulder bolster for (i) central midface/mandible fixation (neutral), (ii) surgical tracheostomy (extension), (iii) mandibular condyle fixation (lateral rotation).Results: Neutral position displays minimal effect on normal c-spine alignment (mouth open or closed). Positions (ii) and (iii) show changes in alignment providing invaluable information for the spinal surgeon to decide if the fracture pattern is stable enough to allow positioning in (ii) flexion/extension or (iii) in rotation.Conclusion: Our results have shown that maxillofacial surgery where the neck is held in a neutral position (e.g. ORIF anterior mandible) is highly unlikely to cause further damage in most patients. For other procedures risk is dependent upon the injury pattern. These results are the first step in producing at guideline to aid inter-specialty decision making in the multiply injured patient.
Keywords: cervical spine injury; clinical study; controlled study; decision making; doctor patient relation; face; fracture; human; mandible condyle; maxillofacial injury; maxillofacial surgery; mouth; nuclear magnetic resonance; practice guideline; rigid collar; rotation; shoulder; surgeon; surgery; tracheostomy; volunteer

Complete Tracheal Resection in Closed Cervical Spine Injury - Rodríguez Gómez C.M.; Rubio Garay M.; Baldó Padró X. (Archivos de Bronconeumologia, 2016)
Keywords: endotracheal tube; tracheostomy tube; adolescent; article; case report; cervical spine injury; clinical article; computer assisted tomography; dysphonia; emergency surgery; endotracheal intubation; extubation; hemodynamics; human; male; trachea resection; tracheotomy

Long-Term Mechanical Ventilation - Sahetya S.; Allgood S.; Gay P.C.; Lechtzin N. (Clinics in Chest Medicine, 2016)
Although precise numbers are difficult to obtain, the population of patients receiving long-term ventilation has increased over the last 20 years, and includes patients with chronic lung diseases, neuromuscular diseases, spinal cord injury, and children with complex disorders. This article reviews the equipment and logistics involved with ventilation outside of the hospital. Discussed are common locations for long-term ventilation, airway and secretion management, and many of the potential challenges faced by individuals on long-term ventilation.
Keywords: acute lung injury; adult respiratory distress syndrome; amyotrophic lateral sclerosis; artificial ventilation; chronic obstructive lung disease; cohort analysis; depression; deterioration; disease exacerbation; fibrosing alveolitis; health care facility; health service; hospital discharge; hospitalization; human; hypercapnia; hypoventilation; hypoxemia; intensive care unit; interstitial lung disease; mechanical exsufflator; mechanical insufflator; mechanical ventilator; noninvasive ventilation; patient autonomy; patient care; practice guideline; priority journal; quality of life; reimbursement; resuscitation; review; spinal cord injury; stress; terminal care; thorax deformity; tracheostomy

Vertebral Artery Occlusion and Recanalization After Cervical Facet Dislocation - Zhang Z.; Wang H.; Mu Z. (World Neurosurgery, 2016)
Objective To investigate clinical features of vertebral artery (VA) occlusion and recanalization after cervical facet dislocation. Methods During a 2-year period from January 2014 to December 2015, 29 consecutive patients with cervical facet dislocation were treated in investigator group. VA occlusion and recanalization were identified retrospectively by magnetic resonance imaging (MRI) at the time of injury and follow-up. Results VA occlusion occurred in 6 of the 29 patients (20.7%). All patients were unilateral occlusion and had no vertebrobasilar symptoms. Surgical anterior spinal fusion was performed in 5 patients, and 1 was treated by posterior fusion. Follow-up MRIs revealed VA recanalization in 5 patients. One patient did not undergo MRI because he died of respiratory failure. The time course for VA recanalization was from 6 days after injury to 4 months after operation depending on the length of VA occlusion. Conclusions A fifth of patients with cervical facet dislocation will develop VA occlusion with rare symptomatic vertebrobasilar ischemia. VA recanalization occurs mainly within the first 4 months after injury, regardless of the length of VA occlusion.
Keywords: adult; anterior spine fusion; article; cervical facet dislocation; cervical spine dislocation; clinical article; clinical feature; clinical trial; female; follow up; human; male; middle aged; nuclear magnetic resonance imaging; recanalization; respiratory failure; retrospective study; spinal cord injury; thorax injury; tracheostomy; vertebral arteriography; vertebral artery stenosis; vertebrobasilar insufficiency

Protein-losing enteropathy with intestinal lymphangiectasia in skeletal dysplasia with Lys650Met mutation - Yang C.; Dehner L.P. (American Journal of Medical Genetics, Part A, 2016)
Protein-losing enteropathy is a primary or secondary manifestation of a group of conditions, and etiologies which are broadly divisible into those with mucosal injury on the basis of inflammatory and ulcerative conditions, mucosal injury without erosions or ulcerations, and lymphatic abnormalities. We describe the first case of protein-losing enteropathy in a pediatric patient, with severe skeletal dysplasia consistent with thanatophoric dysplasia type I and DNA analysis that revealed a c.1949A>T (p.Lys650Met) in exon 15 of the FGFR3 gene. She presented with protein-losing enteropathy in her 6th month. Post-mortem examination revealed lymphangiectasia in the small intestine. To our knowledge, this is the first report of intestinal lymphangiectasia as a complication of skeletal dysplasia resulting in severe protein-losing enteropathy. © 2016 Wiley Periodicals, Inc.
Keywords: alpha 1 antitrypsin; fibroblast growth factor receptor 3; immunoglobulin; immunoglobulin G; sildenafil; acanthosis nigricans; achondroplasia; article; assisted ventilation; bone dysplasia; bone radiography; bradycardia; case report; disease course; disease severity; DNA determination; exon; feces level; female; FGFR3 gene; foramen magnum; gene mutation; human; hypercapnia; hypoalbuminemia; immunoglobulin blood level; infant; intestine lymphangiectasia; lymphocytopenia; neonatal respiratory distress syndrome; nuclear magnetic resonance imaging; oxygen desaturation; priority journal; protein losing gastroenteropathy; pulmonary hypertension; skeleton malformation; spinal cord compression; thanatophoric dwarfism; total parenteral nutrition; tracheostomy

Surgical Treatment of Occipitocervical Dislocation with Atlas Assimilation and Klippel-Feil Syndrome Using Occipitalized C1 Lateral Mass and C2 Fixation and Reduction Technique - Yin Y.-H.; Qiao G.-Y.; Yu X.-G. (World Neurosurgery, 2016)
Objective To introduce and assess a surgical treatment of occipitocervical (OC) dislocation with atlas assimilation and Klippel-Feil syndrome (KFS) using occipitalized C1 lateral mass and C2 fixation and reduction technique. Methods From January 2007 to August 2013, 58 symptomatic patients with OC dislocation and KFS of C2-3 congenital fusion and atlas assimilation were surgically treated in our institution via this technique. After opening the C1-2 facet joints via a posterior approach, OC reduction was conducted by intraoperative manipulation and C1 lateral mass and C2 pedicle screw and rod fixation. The instrument position, fusion status, and clinical outcome were analyzed. Results The average follow-up was 36 months (range, 18–52 months). Radiologically, effective reduction was achieved in 56 patients (96.6%) and <50% reduction in 2 (3.4%) who had additional transoral decompression. Neurologic improvement and solid bone fusion were achieved in all patients. The clinical symptoms improved for all patients, with the averaged Japanese Orthopedic Association myelopathy scores increasing from 11.5 to 15.6 (P < 0.01). Conclusions In patients with OC dislocation and KFS of C2-3 fusion and atlas assimilation, posterior manipulative reduction combined with occipitalized C1 lateral mass and C2 fixation provides a reliable and effective treatment.
Keywords: interlaminal fixation device; pedicle screw fixation device; antibiotic agent; anticoagulant agent; fibrinolytic agent; adolescent; adult; antibiotic therapy; anticoagulant therapy; artery injury; article; cervical collar; cervical spine radiography; clinical effectiveness; clinical outcome; computer assisted tomography; coughing; cranial drill; deep vein thrombosis; dyspnea; feasibility study; female; fibrinolytic therapy; first cervical vertebra; follow up; fracture fixation; fracture reduction; human; interlaminal fixation device; intramedullary nailing; Japanese Orthopedic Association Cervical Myelopathy Evaluation; Klippel Feil syndrome; lung infection; major clinical study; male; middle aged; neuroimaging; nuclear magnetic resonance imaging; occipitocervical dislocation; pedicle screw fixation device; pneumonia; postoperative infection; retrospective study; second cervical vertebra; skull injury; spinal cord decompression; spinal cord injury; spinal cord vascular disease; spine fusion; spine manipulation; surgical patient; third cervical vertebra; tracheotomy; ventilator; vertebra dislocation; vertebral artery injury; Vertex

Successful tracheostomy decannulation after motor complete cervical spinal cord injury - Kim D.H.; Kang S.-W.; Choi W.-A.; Suh M.-R. (Chest, 2016)
PURPOSE: Respiratory dysfunction is a major cause of morbidity and mortality in spinal cord injury (SCI), which causes impairment of respiratory muscles, reduced vital capacity, inefficient cough, and impaired mechanics of ventilation. Patients with cervical SCI (CSCI) are at increased risk of tracheostomy although the majority can be managed noninvasively without empiric tracheostomy. We aimed to analyze the successful tracheostomy decannulation or extubation in motor complete CSCI patients. METHODS: Motor complete CSCI patients who had received invasive acute phase respiratory management, and succeeded in either decannulation or extubation at pulmonary rehabilitation center of Gangnam Severance hospital became candidates of this study. Retrospective review was conducted on the transitions of the respiratory status. RESULTS: Sixty-two motor complete CSCI patients (M:55, F:7) were identified. Traumatic causes of SCI accounted for 93.5% (n=58), and mean age at SCI onset was 47.6 ± 15.8 yrs. Sixty patients (96.8%) had undergone tracheostomy and the other 2 received endotracheal intubation during acute phase management. All patients succeeded in decannulation/extubation after employing mechanically assisted coughing (MAC) and noninvasive mechanical ventilation (NIV). Mean time since tracheostomy to decannulation was 7.0 ± 14.5 months. Of the 60 tracheostomized patients, 12 succeeded in decannulation without applying long-term NIV, 31 switched to continuous NIV after decannulation. Fifteen patients totally weaned off from ventilators after NIV. Two patients who once succeeded in decannulation was re-tracheostomized due to unexpected emergencies. For the 31 patients with continuous NIV, mean hours of daily need for ventilatory support had reduced from initial 15.3 ± 8.0 hrs to 5.7 ± 5.7 hrs at final follow ups. CONCLUSIONS: Motor complete CSCI patients even with high neurologic level of injury can benefit from NIV and aggressive use of MAC. Undesirable tracheostomy can be avoided by employing the noninvasive respiratory management.
Keywords: adult; artificial ventilation; cervical spinal cord injury; coughing; endotracheal intubation; extubation; hospital; human; major clinical study; middle aged; nervous system; pulmonary rehabilitation; retrospective study; tracheostomy; ventilator

Bradycardia and cardiac arrest during tracheal suction in a patient with traumatic spinal cord injury - Shimamoto S.; Saito N.; Suzuki H.; Akiduki N.; Namiki M.; Takeda M.; Yaguchi A. (Shock, 2016)
Spinal shock is a common complication in patients with acute spinal cord injury. It involves a transient loss of spinal reflexes for approximately 48 h after the injury. Neurogenic shock is another possible complication, particularly in patients with high complete cervical spinal cord injuries; this usually diminishes within the first 2-6 weeks after the injury, but occurs during the chronic phase. We present the case of a tetraplegic patient who developed cardiac arrest following bradycardia during tracheal suction in the ICU 6 weeks after the injury. A 72-year-old male fell down the stairs and was transferred to the emergency department with tetraplegia. He was admitted to the intensive care unit with a diagnosis of complete cervical spinal cord injury at C4 and C5. He required mechanical ventilation. A tracheostomy was performed at 14 days, and laminectomy and laminoplasty were performed 24 days after the injury for the dislocation fracture of the cervical vertebrae. Mechanical ventilatory support was still required. Forty-two days after the injury, bradycardia during tracheal suction was accompanied by a decline in SpO2, and cardiac arrest occurred. After 4 min of resuscitation and administration of 1 mg adrenaline, the patient recovered without complications. Although cardiac pacemaker and administration of atropine or aminophylline were considered for preventing further occurrences of bradycardia and cardiac arrest, providing high oxygenation before tracheal suction and shortening the duration of tracheal suction prevented those adverse events. This complication did not occur again in the clinical course of this patient. There are some reports on cardiopulmonary complications following spinal cord injury regardless of spinal shock. These complications are associated with more severe spinal cord injuries and those at higher levels. In particular, tracheal stimuli such as suction, intubation, and bronchoscopy seem to induce bradycardia and even asystole in the presence of hypoxia due to the vasovagal reflex. In normal conditions, this reflex is controlled by sympathetic activity due to increased breathing; however, this compensatory sympathetic activity is inhibited by cervical cord lesions and does not function in patients with cervical spinal injury. The patient in this case has not had any complication since the cardiac arrest and does not require additional therapy; however, some patients die because of this complication and some require a cardiac pacemaker or maintenance treatment with atropine. Cardiac dysfunctions are usually life-threatening complications which occur during the non-acute phase in patients with severe cervical spinal cord injury.
Keywords: aminophylline; atropine; epinephrine; adverse drug reaction; aged; artificial ventilation; bradycardia; bronchoscopy; case report; cervical spinal cord injury; cervical vertebra; clinical trial; complication; diagnosis; disease duration; emergency ward; fracture dislocation; heart arrest; human; hypoxia; intensive care unit; intubation; laminectomy; laminoplasty; maintenance therapy; male; oxygenation; prevention; quadriplegia; reflex; resuscitation; side effect; sinus node; stimulus; suction; sympathetic tone; trachea; tracheostomy

The long-term outcome of early spine fusion for scoliosis in children with cerebral palsy - Sitoula P.; Holmes L.; Sees J.; Rogers K.; Dabney K.; Miller F. (Clinical Spine Surgery, 2016)
Study Design: Retrospective review of radiographs and charts (case-only). Objective: The aim of this study was to describe the long-term outcomes of spine fusion for neuromuscular scoliosis in children below 10 years of age with cerebral palsy (CP). Summary of Background Data: Severely involved children with CP may develop early-onset scoliosis. The outcome of spine fusion is not clear and there are no studies focused on spine fusion in this young patient population. Methods: This is a retrospective review of 33 children who underwent spine fusion with unit-rod instrumentation between 1989 and 2006 for CP neuromuscular scoliosis, aged below 10 years at spine fusion, and with follow-up >5 years. Demographic, medical, and radiographic data were retrospectively assessed. Repeated measure analysis of variance and Kaplan-Meier survival estimates were used for data assessment. Results: Thirty-three of 42 patients who underwent spine fusion in this period, 19 boys and 14 girls, met the inclusion criteria. Of 9 patients who were excluded, 3 were lost to follow-up and remaining 6 died within 5 years of surgery. Mean age at surgery was 8.3 years (range, 4.4-9.9 y). Mean follow-up was 9.8 years (range, 5.5-15.8 y). Gross motor function classification system level was V in 31 patients and IV in 2 patients. Thirty-one patients (94%) had seizure disorder, 29 patients (88%) had gastric feeding tubes, and 9 patients (27%) had tracheostomy tubes. Eighty-five percent of the patients had posterior-only surgery. Mean Cobb angles preoperative, immediately postoperative, and at final follow-up were 85, 21, and 24 degrees, respectively. Mean postoperative pelvic obliquity correction was 15±9 degrees (P<0.001). At final follow-up, there was no significant change from the postoperative measurements. Complications included 1 deep wound infection and 10 other problems. Eleven patients (28.2%) died after a mean follow-up of 5.6±3.8 years. Conclusions: In our cohort with early-onset neuromuscular scoliosis, spine fusion was associated with minimal short-term and long-term morbidity, but there was 28% mortality at 10 years of follow-up and 50% predicted mortality at 15 years.
Keywords: cerebral palsy; child; clinical article; Cobb angle; comorbidity; female; human; male; mortality; motor performance; outcome assessment; postoperative complication; retrospective study; review; scoliosis; spine fusion; spine radiography; surgical technique; treatment indication; treatment outcome; wound infection

Air in the brain seven years after cervical spine surgery - Bugov D.; Raoof N.; Pastores S.; Halpern N. (Chest, 2016)
INTRODUCTION: Medical patients presenting with new or recurrent seizures often have a workup that focuses exclusively on infectious and inflammatory etiologies. However, it is vital to consider the patient's surgical history, however remote. CASE PRESENTATION: The patient is a 52-year old man with childhood seizure disorder well controlled on single agent therapy and C2 chondrosarcoma for which he underwent transmandibular resection with reconstruction and posterior occipitocervical fusion in 2007 followed by adjuvant radiation. Seven years later, he was treated for E. coli sepsis and recurrent infections after dental extractions. Soon after, he developed severe dysphagia and was readmitted with aspiration pneumonia and Peptostreptococcus sepsis. He was supported with noninvasive ventilation (NIV) until he developed seizures and required intubation which was difficult due to anatomy (only pharyngeal exudate was noted). Transesophageal echocardiography was unsuccessful due to a pharyngeal mass. Seizure workup included CT head (pneumocephalus attributed to earlier attempts at nasogastric tube placement) and CSF analysis (leukocytosis only). Extubation was deemed high risk given upper airway anatomy and he underwent PEG and tracheostomy. Endoscopy revealed perforation of the cervical hardware through the posterior pharynx. He was transferred to our institution where his posterior pharyngeal defect was repaired with a myocutaneous flap. He remained hospitalized for four weeks with recurrent pneumonia and poor pulmonary toilet. He was ultimately discharged to a subacute rehabilitation facility. DISCUSSION: While pneumocephalus has been described with noninvasive ventilation, this is often in the setting of neurosurgery or trauma. In this case, it was related to a delayed complication of spinal surgery. The patient had poorly explained pneumocephalus and numerous indicators that there may have been a problem in the pharynx (aspiration, difficult intubation and unsuccessful TEE). However a definitive pharyngeal exam was not performed until endoscopy two weeks after presentation. CONCLUSIONS: Patients with previous cervical spine surgery, even remote, may be at risk for pneumocephalus after NIV. This risk is increased if they are chronically ill and at risk for pharyngeal mucosal breakdown.
Keywords: adjuvant; adult; anatomy; aspiration pneumonia; brain; case report; childhood; chondrosarcoma; chronic patient; computer; dysphagia; endoscopy; extubation; exudate; female; head; human; leukocytosis; male; middle aged; myocutaneous flap; nasogastric tube; neurosurgery; noninvasive ventilation; perforation; pharynx; pneumocephalus; pneumonia; radiation; recurrent infection; rehabilitation; seizure; sepsis; spine surgery; tooth extraction; tracheostomy; transesophageal echocardiography; upper respiratory tract

Cough augmentation techniques in the critically ill: A canadian national survey - Rose L.; Adhikari N.K.; Poon J.; Leasa D.; McKim D.A. (Respiratory Care, 2016)
BACKGROUND: Critically ill mechanically ventilated patients experience impaired airway clearance due to ineffective cough and impaired secretion mobilization. Cough augmentation techniques, including mechanical insufflation-exsufflation (MI-E), manually assisted cough, and lung volume recruitment, improve cough efficiency. Our objective was to describe use, indications, contraindications, interfaces, settings, complications, and barriers to use across Canada. METHODS: An e-mail survey was sent to nominated local survey champions in eligible Canadian units (ICUs, weaning centers, and intermediate care units) with 4 telephone/e-mail reminders. RESULTS: The survey response rate was 157 of 238 (66%); 78 of 157 units (50%) used cough augmentation, with 50 (64%) using MI-E, 53 (68%) using manually assisted cough, and 62 (79%) using lung volume recruitment. Secretion clearance was the most common indication (MI-E, 92%; manually assisted cough, 88%; lung volume recruitment, 76%), although the most common units (44%) used it <50% of the time. Use during weaning from invasive (MI-E, 21%; manually assisted cough, 39%; lung volume recruitment, 3%) and noninvasive ventilation (MI-E, 21%; manually assisted cough, 33%; lung volume recruitment, 21%) was infrequent. The most common diagnoses were neuromuscular disease (97%) and spinal cord injury (83%). Pneumothorax was the most frequently identified absolute contraindication for MI-E (93%) and lung volume recruitment (83%); rib fracture was most frequently identified for manually assisted cough (69%). MI-E mean inspiratory pressure was 31 cm H2O, and expiratory pressure was-32 cm H2O. Mucus plugging requiring tracheostomy inner change was the most frequent complication for MI-E (23%), chest pain for manually assisted cough (36%), and hypotension for lung volume recruitment (17%). The most commonly cited barriers were lack of expertise (70%), knowledge (65%), and resources (52%). CONCLUSIONS: We found moderate adoption of cough augmentation techniques, particularly for secretion management. Lack of expertise and knowledge are potentially modifiable barriers addressed with educational interventions.
Keywords: adult; article; augmentation index; chronic obstructive lung disease; clinical article; coughing; cross-sectional study; demography; female; health care survey; human; hypotension; intervention study; knowledge; lung clearance; lung volume; male; middle aged; neuromuscular disease; positive end expiratory pressure; questionnaire; spinal cord injury; thorax pain; young adult

Clinical Implications of Assisted Peak Cough Flow Measured With an External Glottic Control Device for Tracheostomy Decannulation in Patients With Neuromuscular Diseases and Cervical Spinal Cord Injuries: A Pilot Study - Kang S.-W.; Choi W.A.; Won Y.H.; Lee J.W.; Lee H.Y.; Kim D.J. (Archives of Physical Medicine and Rehabilitation, 2016)
Objective To investigate the clinical usefulness and significance of an external control device substituting for glottic function in determining the feasibility of decannulation in tracheostomized patients with neuromuscular diseases and cervical spinal cord injuries whose assisted peak cough flow (APCF) was unmeasurable or <160L/min. Design Before-after trial. Setting Inpatient setting in a university hospital. Participants Tracheostomized patients (N=16; 11 with neuromuscular diseases and 5 with cervical spinal cord injuries) were recruited. Interventions Unassisted peak cough flow (UPCF) and APCF were measured with and without an external glottic control device. Among patients whose APCF without the device was <160L/min, if their APCF with the device was measured as ≥160L/min, they were decannulated. After decannulation, UPCF and APCF were measured again. Main Outcome Measures APCF with and without an external glottic control device as well as APCF after decannulation. Results After successful decannulation, APCFs were greater than or equal to those measured with the device before decannulation. No patients underwent intubation or retracheostomy, and there were no respiratory complications. Conclusions The external glottic control device substituting for innate glottic function is beneficial for determining tracheostomy decannulation. It provides an objective and accurate APCF. It is particularly helpful for patients whose APCF is ≥160L/min while using the device, even if APCF is <160L/min without this device.
Keywords: adult; aged; article; assisted peak cough flow; cervical spinal cord injury; clinical article; external glottic control device; feasibility study; female; glottis; human; male; manual emergency ventilator; measurement accuracy; medical device; middle aged; neuromuscular disease; peak flowmeter; pilot study; respiratory tract parameters; tracheostomy; tracheostomy tube; unassisted peak cough flow; young adult

The timing of tracheotomy in cervical spinal cord injury patients: A retrospective study - Guirgis A.H. (Intensive Care Medicine Experimental, 2016)
Introduction In Intensive Care Unit (ICU) per cutaneous tracheotomy plays a vital role in airway management of patients with Cervical Spinal Cord Injury (CSCI). This retrospective study evaluated the favorable effect of early tracheotomy in patients CSCI. OBJETICVES. To evaluate the timing of tracheotomy in patients with cervical injury and its effect on ICU stay Methods Retrospective data analysis of 72 patients with CSCI who underwent tracheotomy. The primary objective was to evaluate the impact of early tracheotomy(performed within 1 week of CSCI) on better outcome, in terms of days on mechanical ventilation and ICU stay in patients with high (C1, C2) and low (C3 to C7) CSCI. Outcome measures were also compared between the high and low CSCI patients when early tracheotomy was performed in both. In addition the impact of early tracheotomy in terms of survival benefit, ventilator dependence, requirement of inotropic medications, and whether surgical intervention resulted in better outcome in terms of days in mechanical ventilation and ICU stay were also analysed. Results Patients with high CSCI patients with early tracheotomy had significantly less days on mechanical ventilation and inotropic support compared to those with late tracheostomy (9.3+/-7.2 and 13.7 +/-3.2; p = 0.04 and 0.3+/-1.8 and 4.7+/-11.4; p = 0.035 respectively). Low CSCI patients with early tracheotomy also had significantly less days on mechanical ventilation compared to those with late tracheotomy (12.1+/-10.4 and 25.2+/-17.7; p = 0.034). Early tracheotomy resulted in significantly less days of inotropic support in high CSCI patients compared to low CSCI (0.3+/-1.8 and 2.7+/-5.6; p = 0.022). A trend was observed towards lesser ICU stay and days on mechanical ventilation in patients of high CSCI with early tracheotomy. There was no difference in terms of survival benefits, ventilator dependence, and impact of surgery on outcome measures Conclusions Our retrospective analysis supports the hypothesis that early tracheotomy is beneficial in reducing the period on mechanical ventilation and inotropic support in patients with CSCI, irrespective of the anatomical level of injury.
Keywords: artificial ventilation; cervical spinal cord injury; controlled study; data analysis; female; human; inotropism; intensive care unit; major clinical study; retrospective study; surgery; survival; tracheostomy; ventilator

Tethered cord syndrome in a patient with 40-year history of spinal cord injury: A case report - Lazaro A.; Escalon M.X.; Zakhary M. (PM and R, 2016)
Case/Program Description: A 55-year-old man with chronic tetraplegia. The patient initially sustained cervical spinal cord injury following a lacrosse accident in 1977 and was subsequently C5 AIS A. He was placed in a halo with no surgical intervention. Neurologic examination stabilized with C5 function bilaterally. He was independent with power mobility, bed mobility with rails, feeding, shaving and could don and doff a shirt. He reported progressive neck pain and weakness involving biceps and deltoid function bilaterally starting 6 months prior to admission. Cervical spine MRI showed ventral tethering at C5-C6, dorsal tethering at C4, C6-7 and severe myelomalacia. He underwent C5-6 anterior cervical diskectomy and fusion, followed by C2-C7 laminectomies with intradural dethetering and fusion from C3- T1. Post-op course complicated by respiratory failure, with vent dependence requiring tracheostomy. Patient was transferred to spinal cord injury unit for acute inpatient rehabilitation on post-op day 18. Patient is now C4 AIS A. Setting: Tertiary care hospital Results: The patient regained 1/5 muscle strength on C5 bilaterally. He was able to power manual wheelchair with lip control for 150 feet x2. He was weaned from ventilator and tracheostomy tube was downsized. While he did improve during inpatient rehabilitation stay, he did not regain prior level of function. Discussion: This case demonstrates neurologic decline in a patient with a 40-year history of spinal cord injury. It highlights the importance of early detection, management and rehabilitation of tethered cord and other late complications of chronic SCI such as progressive spondylosis or syringomelia. Conclusions: New or changing clinical features such as increasing weakness, pain, or muscle spasm may be a sign of late neurologic complication in chronic SCI that require prompt treatment in order to attempt prevention of potentially permanent neurologic decline.
Keywords: accident; adult; biceps brachii muscle; case report; cervical spinal cord injury; clinical feature; complication; deltoid muscle; discectomy; dorsal region; feeding; foot; hospital patient; human; laminectomy; male; manual wheelchair; middle aged; muscle spasm; muscle strength; neck pain; nervous system; neurologic examination; neurological complication; nuclear magnetic resonance imaging; prevention; quadriplegia; rehabilitation; respiratory failure; spondylosis; surgery; tertiary care center; tethered cord syndrome; tracheostomy tube; ventilator; weakness

Migration of anterior cervical fixation screw causing both esophageal and tracheal erosive injuries: Case report - Thuku G.I.; McKinley W. (PM and R, 2016)
Case/Program Description: A 34-year-old woman with history of IV drug use and prior back surgery presented with fever and acute mental changes. She was diagnosed with spinal epidural abscesses, cervical spinal cord compression and C5 motor incomplete tetraplegia. She underwent abscess drainage and ACF. Her acute hospital course was complicated by empyemas, pnuemothoracies and dysphagia. She required additional acute care procedures including: chest tubes, tracheostomy tube and percutaneous endoscopic feeding tube. After a prolonged stay in acute care, she was transferred to rehabilitation. At 6 weeks after ACF, a chest x-ray incidentally revealed a metallic object (resembling a screw) in the right medial lung base. Subsequent imaging work-up revealed that a screw was missing from the ACF plate. Bronchoscopy revealed a tracheal perforation and screw in the posterior bronchus of the right medial lobe. Modified barium swallow identified an esophageal perforation. The screw and ACF hardware were removed and tracheal injury was repaired. Unsuccessful conservative management was attempted for the esophageal injury, which eventually required surgical closure. Setting: A Tertiary Referral Hospital. Results: Patient did very well and after 6 months follow-up was eating a regular diet without respiratory issues. Discussion: We present a unique case of anterior cervical fusion (ACF) screw extrusion and migration, with resultant simultaneous esophageal and tracheal injuries in a very common procedure, with more than 100,000 performed annually. Conclusions: Esophageal and tracheal perforations can occur simultaneously with ACF screw extrusion and migration. Coexisting respiratory and swallowing issues may pose difficulties with diagnosis of such injuries and recommendations for regular hardware image surveillance are needed.
Keywords: abscess drainage; adult; bronchoscopy; bronchus; case report; cervical spinal cord; chest tube; compression; computer; conservative treatment; diagnosis; diet; dysphagia; eating; emergency care; empyema; epidural abscess; esophagography; esophagus perforation; feeding apparatus; female; fever; follow up; human; imaging; lung; mental disease; quadriplegia; rehabilitation; surgery; tertiary care center; thorax radiography; trachea injury; tracheostomy tube

Lifesaving cyclophosphamide for fulminant multiple sclerosis: A case report - Kumar G.; Sanchez V.; Brex P.; Coles A.; Harikrishnan S. (Multiple Sclerosis, 2016)
Background: Cyclophosphamide is a cytotoxic agent occasionally used to treat inflammatory conditions of the nervous system. We present an exceptional case of its successful use to treat a first presentation of life threatening Multiple Sclerosis (MS). Case: A 31 year old Caucasian female, with background of opiate addiction presented with six month history of unsteadiness, falls and confusion. Initial examination demonstrated pyramidal weakness, hyper-reflexia, cerebellar signs, and cognitive impairment. Six days into admission she suffered respiratory compromise requiring intubation and mechanical ventilation. Magnetic Resonance Imaging demonstrated global cerebral atrophy and multiple widespread, confluent, partially-enhancing T2 and FLAIR white matter hyperintensities throughout both cerebral hemispheres, brainstem, cerebellum and cervical spinal cord. This was felt to most likely represent a demyelinating pathology. Investigations for other inflammatory, infective, toxic, metabolic, neurodegenerative or autoimmune conditions were negative. Cerebrospinal fluid was acellular but significant for the presence of oligoclonal bands. A clinical diagnosis of demyelinating disease, likely MS, was obtained. She was initially treated with high dose steroids (pulsed intravenous methylprednisolone for a total for eight days and then subsequently oral prednisolone) and five cycles of plasma exchange achieving only mild improvement. She remained in high dependency care 27 days into admission, still requiring tracheostomy, enteral feeding and fully dependant on nursing care; equating to Expanded Disability Status Scale (EDSS) of 9.5. Immunosuppression was escalated to six cycles of cyclophosphamide (15mg/kg) in two week intervals. Subsequently there was a dramatic improvement; she began to talk fluently on day 51 and walk independently after day 91. Currently, she remains globally cognitively impaired (Addenbrooke's Cognitive Examination Score 42/100) and has subtle pyramidal weakness in her right arm only; giving EDSS 4.5. To date, she has not exhibited signs of cyclophosphamide toxicity and there has been no progression of radiological findings. Conclusion: This case highlights the remarkable outcome that can be achieved from using potentially toxic immunosuppression in the context of first presentation of highly aggressive MS, even when initiated in a critical care setting.
Keywords: cyclophosphamide; methylprednisolone; oligoclonal band; prednisolone; adult; brain atrophy; brain stem; case report; Caucasian; cerebrospinal fluid; cervical spinal cord; cognitive defect; diagnosis; enteric feeding; Expanded Disability Status Scale; female; hemisphere; human; hyperreflexia; immunosuppressive treatment; intubation; multiple sclerosis; nuclear magnetic resonance imaging; nursing care; opiate addiction; pathology; plasma exchange; toxicity; tracheostomy; unsteadiness; weakness; white matter

Variation in 60-day Readmission for Surgical-site Infections (SSIs) and Reoperation Following Spinal Fusion Operations for Neuromuscular Scoliosis - McLeod L.; Flynn J.; Erickson M.; Miller N.; Keren R.; Dormans J. (Journal of Pediatric Orthopaedics, 2016)
Background: Readmission for surgical-site infection (SSIs) following spinal fusion for NMS impacts costs, patient risk, and family burden; however, it may be preventable. The purpose of this study was to examine variation in hospital performance based on risk-standardized 60-day readmission rates for SSI and reoperation across 39 US Children's Hospitals. Methods: Retrospective cohort study using the Pediatric Health Information Systems (PHIS) database involving children aged 10 to 18 years with ICD9 codes indicating spinal fusion, scoliosis, and neuromuscular disease discharged from 39 US children's hospitals between January 1, 2007 and September 1, 2012. Readmissions within 60 days for SSI were identified based on the presence of ICD9 codes for (1) infectious complication of device or procedure, or (2) sepsis or specific bacterial infection with an accompanying reoperation. Logistic regression models accounting for patient-level risk factors for SSI were used to estimate expected (patient-level risk across all hospitals) and predicted (weighted average of hospital-specific and all-hospital estimates) outcomes. Relative performance was determined using the hospital-specific predicted versus expected (pe) ratios. Results: Average volume across hospitals ranged from 2 to 23 fusions/quarter and was not associated with readmissions. Of the 7560 children in the cohort, 534 (7%) were readmitted for reoperation and 451 (6%) were readmitted for SSI within 60 days of discharge. Reoperations were associated with an SSI in 70% of cases. Across hospitals, SSI and reoperation rates ranged from 1% to 11% and 1% to 12%, respectively. After adjusting for age, sex, insurance, presence of a gastric tube, ventriculoperitoneal shunt, tracheostomy, prior admissions, number of chronic conditions, procedure type (anterior/posterior), and level (>9 or <9 vertebrae), pe ratios indicating hospital performance varied by 2-fold for each outcome. Conclusions: After standardizing outcomes using patient-level factors and relative case mix, several hospitals in this cohort were more successful at preventing readmissions for SSIs and reoperations. Closer examination of the organization and implementation of strategies for SSI prevention at high-performing centers may offer valuable clues for improving care at lower performing institutions. Level of Evidence: Level III.
Keywords: adolescent; adult; anterior spine fusion; article; bacterial infection; brain ventricle peritoneum shunt; child; child hospitalization; chronic disease; cohort analysis; Duchenne muscular dystrophy; female; groups by age; health insurance; hospital discharge; hospital readmission; human; ICD-9; infection prevention; major clinical study; male; medical information system; multicenter study; outcome assessment; posterior spine fusion; priority journal; reoperation; retrospective study; risk factor; scoliosis; sepsis; sex difference; spinal muscular atrophy; stomach tube; surgical infection; tracheostomy