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先天性并指(趾)畸形:重新评估

Congenital syndactyly: a reappraisal.

作者信息

Entin M A

出版信息

Can J Surg. 1978 Jul;21(4):360-4.

PMID:209879
Abstract

Digital syndactyly is a common congenital anomaly and is associated with other anomalies affecting the digits, the hand, the arm or even the entire body. In the past it has been considered a single entity. However, recent information provided by experimental embryology, studies of morphogenesis and other sources, suggests that there are at least two distinct entities: primary syndactyly due to interference with the sequence of events that normally culminate in the division of digits into discrete parts and secondary syndactyly due to readhesion of adjacent digits as a result of close contact between raw surfaces. Primary syndactylyl may arise de novo, or it may occur as a manifestation of a mutation phenomenon or as a genetically controlled syndrome. Secondary syndactyly is the result of mechanical adhesion of adjacent parts involved in a general reparative or healing process. The initial insult leading to amputation of a portion of the digits may or may not be genetically controlled; adhesion is fortuitous. Management of syndactyly is determined by the complexity of the malformation. For simple cutaneous syndactyly the surgeon should make a zigzag incision and provide a rectangular, proximally based flap for the floor of the web, usually before the child is 2 years of age. A full- or split-thickness skin graft should be applied to the defects. For complex deformities involving several digits and associated with postural or osseous malformation, several operative procedures may be required. The surgeon must be vigilant to note and correct the sequelae resulting from recurrent contractures and imbalances associated with growth.

摘要

并指畸形是一种常见的先天性异常,与影响手指、手部、手臂甚至整个身体的其他异常有关。过去它被视为一个单一的实体。然而,实验胚胎学、形态发生学研究及其他来源提供的最新信息表明,至少存在两种不同的实体:原发性并指是由于正常情况下最终导致手指分成离散部分的事件顺序受到干扰,继发性并指是由于原始表面紧密接触导致相邻手指重新粘连。原发性并指可能是新发的,也可能是突变现象的表现或作为一种基因控制的综合征出现。继发性并指是一般修复或愈合过程中相邻部位机械粘连的结果。导致部分手指截肢的初始损伤可能受基因控制,也可能不受基因控制;粘连是偶然发生的。并指畸形的治疗取决于畸形的复杂程度。对于简单的皮肤并指,外科医生应做锯齿状切口,并为指蹼底部提供一个近端带蒂的矩形皮瓣,通常在患儿2岁之前进行。应将全厚或中厚皮片移植到缺损处。对于涉及多个手指且伴有姿势或骨骼畸形的复杂畸形,可能需要进行多次手术。外科医生必须警惕注意并纠正与生长相关的反复挛缩和失衡所导致的后遗症。

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