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伴有脊髓脊膜膨出的附肢:一个挑战既往认知的罕见病例。

Accessory limb with myelomeningocele: a rare case challenging previously held beliefs.

作者信息

Parks Christopher, Mugamba John

机构信息

Alder Hey Hospital, Liverpool, UK,

出版信息

Childs Nerv Syst. 2014 Dec;30(12):2127-8. doi: 10.1007/s00381-014-2452-1. Epub 2014 Jun 1.

Abstract

INTRODUCTION

There have been previous reports of intra-scapular limbs associated with a closed spina bifida and this has led to a causative theory. It is thought that these dysraphic appendages could not occur with defects of primary neurulation.

CASE

The authors present a rare case of this abnormality associated with a large open myelomeningocele in a 6-day-old infant presenting to a paediatric neurosurgical hospital in Uganda. The appendage was removed and the spina bifida closed. There was significant stigma associated with such abnormality in this region.

DISCUSSION

The first reported co-existence of these two lesions challenges previously held beliefs regarding the embryological origin of intra-scapular dysraphic appendage.

摘要

引言

此前已有关于与隐性脊柱裂相关的肩胛内肢体的报道,这引发了一种病因理论。人们认为,这些神经管闭合不全附属物不会伴随原发性神经管形成缺陷而出现。

病例

作者报告了一例罕见的此类异常病例,该病例发生在一名6天大的婴儿身上,伴有巨大开放性脊髓脊膜膨出,该婴儿被送往乌干达一家儿科神经外科医院。附属物被切除,脊柱裂被闭合。在该地区,这种异常情况存在明显的污名化现象。

讨论

这两种病变首次被报道同时存在,对先前关于肩胛内神经管闭合不全附属物胚胎起源的观点提出了挑战。

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