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桡侧多指畸形。

Radial Polydactyly.

机构信息

SUNY Downstate Medical Center, Brooklyn, New York.

Cedars-Sinai Medical Center, Los Angeles, California.

出版信息

JBJS Rev. 2022 May 2;10(5). doi: 10.2106/JBJS.RVW.21.00214. eCollection 2022 May 1.

Abstract

» Radial polydactyly is one of the most common congenital anomalies of the hand, with an incidence of 0.08 to 1.40 per 1,000 live births; it requires surgical treatment early in life.» Polydactyly occurs during weeks 5, 6, 7, and 8 of embryogenesis, principally due to misregulation of the sonic hedgehog protein within the developing limb bud.» The Flatt classification system of preaxial polydactyly (types I to VII), as published by Wassel, categorizes preaxial polydactyly based on osseous abnormalities, but it has substantial limitations.» For improved function and appearance, preaxial polydactyly ideally requires surgical intervention at an early age (between 1 and 2 years of age) before the development of fine motor skills.

摘要

桡侧多(趾)畸形是最常见的手部先天畸形之一,每 1000 例活产中发病率为 0.08 至 1.40;它需要在生命早期进行手术治疗。多(趾)畸形发生在胚胎发育的第 5、6、7 和 8 周,主要是由于发育中的肢芽中 sonic hedgehog 蛋白的失调。Wassel 发表的 Flatt 分类系统(I 至 VII 型)对桡侧多(趾)畸形进行分类,基于骨骼异常,但它有很大的局限性。为了改善功能和外观,桡侧多(趾)畸形理想情况下需要在精细运动技能发展之前的早期(1 至 2 岁之间)进行手术干预。

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