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儿科人群中的并指畸形:文献综述

Syndactyly in the Pediatric Population: A Review of the Literature.

作者信息

Mahindroo Sonal, Tabaie Sean

机构信息

Orthopedic Surgery, George Washington University School of Medicine and Health Sciences, Washington DC, USA.

Orthopedic Surgery, Children's National Hospital, Washington DC, USA.

出版信息

Cureus. 2023 Mar 14;15(3):e36118. doi: 10.7759/cureus.36118. eCollection 2023 Mar.

DOI:10.7759/cureus.36118
PMID:36937133
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10016737/
Abstract

Syndactyly is one of the most common congenital upper extremity deformities. Syndactyly can be described as either simple, involving just the skin and soft tissue, or complex, involving the phalanges. Additionally, syndactyly can be categorized as complete, involving the entire digit (including the nail fold), or incomplete, which does not involve the nail fold. Multiple familial or spontaneous genetic abnormalities can cause syndactyly, and these mutations typically involve the canonical wingless-type (WNT) pathway. Surgical repair of syndactyly is typically done between six to 18 months of age, depending on the type of syndactyly. Regardless of the classification of the syndactyly, the repair is performed before school-going age (except in the case of extremely mild or rare, extremely complex syndactyly). One or more imaging modalities are used to aid the surgeon in deciding the surgical approach for the syndactyly repair. The surgical plan must be clearly communicated with parents to manage expectations of aesthetics and function of the digits post-surgery. In brief, a syndactyly release surgery involves the creation of the web space using a geometrical design of the surgeon's choice, defatting of finger flaps, separation of the digits, and closure with absorbable sutures. However, the approach may vary depending on the patient. A "best" approach for rectifying the difference in surface area of separated versus fused digits has not yet been determined. While this was typically done using a skin graft, the use of alternative methods (most notably, using a synthetic dermal substitute or not using a graft at all and allowing the skin to heal with secondary intention) has been on the rise given the undesirable side effects of a graft. Less commonly, an external fixator can be used to expand soft tissue and skin. In the case of complete syndactyly, the Buck-Gramcko technique is most commonly used for nail flap reconstruction. Complications of the surgery include contracture, web creep, and the need for a second surgery. Thus, parents must be counseled in recognizing signs of complications.

摘要

并指畸形是最常见的先天性上肢畸形之一。并指畸形可分为单纯性,仅累及皮肤和软组织;或复杂性,累及指骨。此外,并指畸形可分为完全性,累及整个手指(包括甲皱襞);或不完全性,不累及甲皱襞。多种家族性或自发性基因异常可导致并指畸形,这些突变通常涉及经典的无翅型(WNT)信号通路。并指畸形的手术修复通常在6至18个月龄之间进行,具体取决于并指畸形的类型。无论并指畸形如何分类,修复手术均在入学年龄前进行(极轻度或罕见、极其复杂的并指畸形除外)。使用一种或多种成像方式辅助外科医生决定并指畸形修复的手术方法。必须与家长清楚沟通手术方案,以管理他们对术后手指美观和功能的期望。简而言之,并指松解手术包括使用外科医生选择的几何设计创建指蹼间隙、修剪手指皮瓣、分离手指以及用可吸收缝线缝合。然而,手术方法可能因患者而异。尚未确定纠正分离手指与融合手指表面积差异的“最佳”方法。虽然这通常使用皮肤移植来完成,但鉴于移植的不良副作用,使用替代方法(最显著的是使用合成真皮替代物或根本不使用移植,让皮肤二期愈合)的情况正在增加。较少见的情况下,可使用外固定器来扩张软组织和皮肤。对于完全性并指畸形,Buck-Gramcko技术最常用于甲皱襞重建。手术并发症包括挛缩、指蹼退缩以及需要二次手术。因此,必须向家长提供咨询,使其认识并发症的迹象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f02/10016737/621ae6fde629/cureus-0015-00000036118-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f02/10016737/5a6cd9b85ac1/cureus-0015-00000036118-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f02/10016737/621ae6fde629/cureus-0015-00000036118-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f02/10016737/5a6cd9b85ac1/cureus-0015-00000036118-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f02/10016737/621ae6fde629/cureus-0015-00000036118-i02.jpg

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本文引用的文献

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Genetic determinants of syndactyly: perspectives on pathogenesis and diagnosis.并指的遗传决定因素:发病机制和诊断的新视角。
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Outcomes of Pediatric Syndactyly Repair Using Synthetic Dermal Substitute.使用合成真皮替代物进行小儿并指修复的结果。
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