Klippel-Trenaunay syndrome is a rare congenital malformation
characterized by a large angiomatous nevus; hypertrophy of soft tissue or
overgrowth of bone, or both; and venous varicosities. The cases of 108
patients who had a diagnosis of this syndrome between 1956 and 1990 were
reviewed. One hundred and twenty-six anomalies were found in twenty-nine
patients, each of whom had one to thirteen malformations of the fingers or
toes, or both. The ratio of female patients to male patients was
approximately two to one. Twenty-six patients had macrodactyly affecting
one to six digits; nine, syndactyly involving two or more digits; five,
metatarsus primus varus; two, clinodacytly; two, polydactyly; one,
camptodactyly; and one, a congenital trigger finger. Thirty-three of the
126 anomalies were in extremities that had no nevi, varicose veins, or
generalized hypertrophy.