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遗传性多发性骨软骨瘤中短指畸形和手部外生骨疣的发病率。

Incidence of brachydactyly and hand exostosis in hereditary multiple exostosis.

作者信息

Cates H E, Burgess R C

机构信息

Shriners Hospitals for Crippled Children, Lexington Unit, Ky.

出版信息

J Hand Surg Am. 1991 Jan;16(1):127-32. doi: 10.1016/s0363-5023(10)80027-9.

Abstract

Forty-two radiographs and charts of twenty-two patients with a diagnosis of hereditary multiple exostosis and hand involvement were examined to determine the incidence of hand exostoses and association with brachydactyly. An average of 11.6 exostoses were found per hand. The proximal phalanges and metacarpals are affected in the majority of patients and the thumb and distal phalanges are rarely involved. Most exostoses were located in the juxtaepiphyseal region (61.8%) and typically involved less than 50% of the bone diameter. Brachydactyly can be seen in patients with hereditary multiple exostosis when no exostoses is present; however, the presence of an exostosis results in even more shortening. The location and size of the exostosis had no relationship to increased bone shortening. Operative treatment was required in four of twenty-two patients for debulking and impingement.

摘要

对42张X光片和22例诊断为遗传性多发性骨软骨瘤且手部受累患者的病历进行了检查,以确定手部骨软骨瘤的发生率及其与短指畸形的关联。每只手平均发现11.6个骨软骨瘤。大多数患者的近端指骨和掌骨受累,拇指和远端指骨很少受累。大多数骨软骨瘤位于骨骺附近区域(61.8%),通常累及不到50%的骨直径。遗传性多发性骨软骨瘤患者在没有骨软骨瘤时也可见短指畸形;然而,存在骨软骨瘤会导致更严重的缩短。骨软骨瘤的位置和大小与骨缩短增加无关。22例患者中有4例需要手术治疗以进行减容和解除压迫。

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