Congenital lipofibromatosis associated with macrodactyly of the foot

Congenital lipofibromatosis of the foot, a type of paracrine growth disorder, is a distinct clinical entity that must be differentiated from other causes of macrodactyly, with specific diagnostic and therapeutic considerations. Eleven skeletally immature patients with congenital lipofibromatosis and macrodactyly of the foot were studied. Diagnostic histopathologic criteria were documented. The most specific pathologic finding of congenital lipofibromatosis is an overabundance of fibrofatty tissue on the plantar aspect of the foot and involved toes. Surgical defatting or debulking procedures, ray resection, and phalangeal epiphysiodesis produced significant cosmetic improvement. Syndactylization and phalangeal resection were not as beneficial.