Symphalangism in children - Case report and review of the literature

Symphalangism is an uncommon syndrome characterized by fusion of the interp halangeal joints of the hands and feet. The fusion can involve the proximal or the distal joints; however, involvement of the proximal interphalangeal joints is more common. Symphalangism often is associated with several othe r skeletal and nonskeletal abnormalities. Analysis of the pedigrees of affe cted families reveals this trait to be autosomal dominant. The authors pres ent the case of a 9-year-old boy with bilateral symphalangism of the proxim al interphalangeal joints in the fingers and toes. His father is affected s imilarly. A comparison of these cases with those published in the literatur e indicates that although the radiologic appearance of symphalangism appear s disabling, the fused phalanges seldom cause disability or loss of functio n of the hand. Surgical intervention is not required in most patients. The father and son reported also had capitellar hypoplasia and subluxation of t he radial head associated with limitation of elbow flexion and extension.