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连接终端性脊髓脊膜膨出和脊髓脂肪瘤之间的脊柱发育不良谱:两例真性终末性脂肪脊膜膨出合并全脊髓空洞症的报告。

Bridging the spinal dysraphism spectrum between terminal myelocystocele and spinal cord lipoma: a report of two cases of true terminal lipomyelocystocele with holo-cord syrinx.

机构信息

Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India.

出版信息

Childs Nerv Syst. 2024 Dec;40(12):4347-4352. doi: 10.1007/s00381-024-06620-4. Epub 2024 Sep 16.

Abstract

Terminal myelocystocele (TMC) is a rare form of spinal dysraphism which arises due to aberration in the secondary neurulation process involving the caudal cell mass. Terminal myelocystocele has been defined by Pang et al. based on essential and non-essential features. One of the non-essential features includes non dysraphic lipomas which do not tether to the neural placode. We are presenting two cases which meets all the essential criteria outlined by Pang et al. for TMC but also show the presence of a lipomatous component tethering to the neural placode, similar to a dysraphic lipoma. Through this article, we want to showcase a subset which represents "true" terminal lipomyelocystocele (TLMC), bridging the spectrum of spinal dysraphism between TMC and lipomyelomeningocele (LMM).

摘要

终端性脊髓脊膜膨出(TMC)是一种罕见的脊柱发育畸形,是由于涉及尾细胞块的二次神经胚形成过程中的异常而引起的。终端性脊髓脊膜膨出是由 Pang 等人根据必要和非必要特征定义的。非必要特征之一包括不与神经基板相连的非发育性脂肪瘤。我们介绍了两个病例,这些病例均符合 Pang 等人对 TMC 概述的所有必要标准,但也显示出存在与神经基板相连的脂肪瘤成分,类似于发育性脂肪瘤。通过本文,我们希望展示一个子集,代表“真正的”终端性脂肪脊膜脊髓膨出(TLMC),在 TMC 和脂肪脊膜脊髓膨出(LMM)之间架起了脊柱发育畸形的桥梁。

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