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三重神经管缺陷——颅裂合并颅前和颅尾脊柱裂——人类神经管多部位闭合的现存证据。

Triple neural tube defect--cranium bifidum with rostral and caudal spina bifida--live evidence of multi-site closure of the neural tube in humans.

作者信息

Tekkök Ismail H

机构信息

Mersin University School of Medicine, Turkey.

出版信息

Childs Nerv Syst. 2005 Apr;21(4):331-5. doi: 10.1007/s00381-004-1027-y. Epub 2004 Sep 29.

Abstract

OBJECTIVE

The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date.

CASE REPORT

The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17x15x15-cm(3) parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11x11x8-cm(3) thoracolumbar myelomeningocele. Hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome.

CONCLUSIONS

Current neural tube closure theories and models are reviewed in an attempt to better understand this extremely unusual coexistence. The multi-site closure model is clearly more useful in our understanding of NTDs.

摘要

目的

单个儿童同时存在三种神经管缺陷(NTD)是一种罕见事件。文献回顾显示有9例已发表的“双重”NTD病例,但迄今为止尚未报道过“三重”NTD病例。

病例报告

本文介绍了一名患有三种不同神经管缺陷的两岁男孩的罕见病例。该男孩有一个17×15×15立方厘米的顶枕部脑膨出、一个小的颈段脊髓脊膜膨出和一个11×11×8立方厘米的胸腰段脊髓脊膜膨出。神经管缺陷伴有脑积水和Chiari II畸形。所有三个病变均接受了手术治疗,美容效果良好,神经功能预后满意。

结论

对当前的神经管闭合理论和模型进行了回顾,以期更好地理解这种极其罕见的共存情况。多部位闭合模型显然对我们理解神经管缺陷更有用。

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