Dysharmonic maturation of the hand in the congenital malformation syndromes

Abstract

In many congenital malformation syndromes the pattern of hand-wrist development does not fit the sequence pictured in the Greulich-Pyle atlas. Not infrequently, there is a difference in maturation level of carpal and phalangeal centers in excess of that found in clinically normal children. Usually, the carpal centers are less developed than the phalangeal centers, whereas overall skeletal maturation is retarded (as in trisomy 18) or advanced (as in cerebral gigantism). In still other conditions, specific carpal centers are disproportionately delayed. By way of example, the capitate is differentially delayed in epiphyseal dysplasia, the lunate in homocystinuria, and the scaphoid in Fanconi's anemia and other radial hypoplasia syndromes. Side to side (i.e., bilateral) asymmetries may also occur in the developing hand, as in paralysis, in conditions involving increased local vascularity (as in hemangioma and rheumatoid arthritis) or in conditions associated with decreased vascularity. In the presence of excessive dysharmonic development or major bilateral asymmetry, with or without agenesis of one or more hand bones, assigning meaningful bone ages in congenital malformation syndromes becomes difficult. On the other hand, the degree and pattern of dysharmonic maturation may be helpful in diagnosis.