The Journal of Bone and Joint Surgery, Vol 73, Issue 10 1537-1546, Copyright © 1991 by Journal of Bone and Joint Surgery, Inc
Anomalies of the fingers and toes associated with Klippel-Trenaunay syndrome
BJ McGrory, PC Amadio, JH Dobyns, GB Stickler and KK Unni
Department of Orthopedics, Mayo Clinic, Rochester, Minnesota 55905.
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.
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