Macrodactyly.

Abstract

Digital gigantism is an unusual congenital anomaly that is present at birth or recognized in early infancy and is progressive during the period of normal skeletal maturation. It involves cell types that are predominately affected by neurogenic growth control and follow a distribution similar to the sensory supply of a major peripheral nerve, usually the median nerve. There seem to be three variants that overlap somewhat in clinical presentation. They are type I digital gigantism with lipofibromatous hamartoma of a peripheral nerve, type II digital gigantism associated with neurofibromatosis, and type III hyperostotic digital gigantism. Treatment should be individualized and initiated early in life. Epiphysiodeses, osteotomies, and division of branches of the supplying digital nerves may be indicated during childhood. In the adult, arthrodesis of the interphalangeal joints with bony shortening or ray resection may be indicated. Skin flap necrosis is a common complication postoperatively.