Salazard B, Casanova D
Institut de la Main, clinique Monticelli, 88, rue du Commandant-Rolland, 13008 Marseille, France.
Chir Main. 2008 Dec;27 Suppl 1:S115-20. doi: 10.1016/j.main.2008.07.014. Epub 2008 Aug 20.
Apert's syndrome is the most common among acrocephalosyndactylies with complex malformations of the hands. Treatment of the Apert hand is complex and numerous procedures are required. The aim of this study is to propose a strategy for hand management. Sixteen Apert syndrome hands were submitted to early surgery which included opening of the first web, separation of the fingers, realignment of the thumb and correction of the clinodactylies. We performed an average of six operations per child. Treatment of the first web depended on the classification of Upton: for the severe stages, we used a dorsal hand flap. Radical clinodactyly was treated by osteotomy of the delta phalanx and Z-plasty. We treated nine hands with four fingers and seven hands with five fingers. There was always bilateral opposition and symphalangism, excluding the fifth finger. All of the children have a rudimentary but functional pinch grip. Revision of the webs was necessary in 16% of the cases. The Apert hand requires early and specialised treatment that aims to provide a functional hand before two or three years, with the least surgical complications. The functional prognosis is darkened by symphalangism.
阿佩尔综合征是头并指畸形中最常见的一种，伴有手部复杂畸形。阿佩尔手部的治疗很复杂，需要进行许多手术。本研究的目的是提出一种手部处理策略。对16例阿佩尔综合征患者的手部进行了早期手术，包括切开第一蹼、分指、拇指复位和矫正弯指畸形。每个患儿平均接受了6次手术。第一蹼的治疗取决于厄普顿的分类：对于严重阶段，我们采用手背皮瓣。重度弯指畸形采用三角指骨截骨术和Z成形术治疗。我们治疗了9例四指手和7例五指手。除第五指外，均存在双侧对指和关节融合。所有患儿都有基本但功能正常的捏握动作。16%的病例需要进行蹼的修复。阿佩尔手部需要早期和专门的治疗，目标是在两到三年前提供一只功能正常的手，同时手术并发症最少。关节融合会使功能预后变差。
