McGrory B J, Amadio P C, Dobyns J H, Stickler G B, Unni K K
Department of Orthopedics, Mayo Clinic, Rochester, Minnesota 55905.
J Bone Joint Surg Am. 1991 Dec;73(10):1537-46.
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.
克-特综合征是一种罕见的先天性畸形，其特征为巨大的血管瘤性痣、软组织肥大或骨骼过度生长，或两者皆有，以及静脉曲张。回顾了1956年至1990年间诊断为此综合征的108例患者的病例。在29例患者中发现了126处异常，其中每位患者有1至13处手指或脚趾畸形，或两者皆有。女性患者与男性患者的比例约为二比一。26例患者有巨指（趾）症，累及1至6个手指（趾）；9例有并指（趾）畸形，累及两个或更多手指（趾）；5例有第一跖骨内翻；2例有手指弯曲；2例有多指（趾）畸形；1例有先天性屈曲挛缩指；1例有先天性扳机指。126处异常中有33处位于无痣、无静脉曲张或无全身肥大的肢体。
