Bilateral hand malformations with absence of carpal bones with fusion of proximal metacarpals.

A 14-year-old boy presented with a deformity of his left hand since birth. His parents were nonconsanguineous and his early history was uneventful. The mother was not exposed to any teratogenic drugs during pregnancy. His other siblings are normal. The parents noticed the hand deformity at birth but he had not come to medical attention until he complained of restricted movement of the left hand at the wrist. On examination he did not have any pain on movement of the wrist nor tenderness over the wrist joint. Palmar flexion was restricted completely and active dorsiflexion was restricted in the last 30°. Further passive dorsiflexion was exaggerated and the middle finger could touch the dorsal aspect of the forearm. The hand was deviated to the ulnar side. The patient was of normal stature and had no dysmorphic features. The index finger of the right hand was noted to be small. His feet were normal