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无脑儿畸形:神经放射学和外科特征。病例报告及文献综述。

Iniencephaly: neuroradiological and surgical features. Case report and review of the literature.

作者信息

Erdinçler P, Kaynar M Y, Canbaz B, Koçer N, Kuday C, Ciplak N

机构信息

Department of Neurosurgery, Cerrahpaşa Medical Faculty, Istanbul University, Turkey.

出版信息

J Neurosurg. 1998 Aug;89(2):317-20. doi: 10.3171/jns.1998.89.2.0317.

DOI:10.3171/jns.1998.89.2.0317
PMID:9688130
Abstract

Iniencephaly is a rare congenital anomaly characterized by spina bifida of the cervical vertebrae, fixed retroflexion of the head on the cervical spine, and occipital bone defect. There are only five reports of surviving patients with iniencephaly. The authors report the case of a newborn who presented with iniencephaly and an encephalocele that were surgically treated in our service. Neurological examination of the patient yielded normal results except for a moderate psychomotor retardation. The neuroradiological and surgical findings of the case suggested that the trigger of the anomaly was the occipital bone defect and rachischisis of the posterior vertebral arches.

摘要

无脑儿后凸是一种罕见的先天性异常,其特征为颈椎脊柱裂、头部在颈椎上固定性后屈以及枕骨缺损。仅有五例无脑儿后凸存活患者的报告。作者报告了一例新生儿,该患儿患有无脑儿后凸和脑膨出,并在我院接受了手术治疗。除中度精神运动发育迟缓外,该患者的神经学检查结果正常。该病例的神经放射学和手术结果表明,异常的触发因素是枕骨缺损和后椎弓脊柱裂。

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Iniencephaly: neuroradiological and surgical features. Case report and review of the literature.无脑儿畸形:神经放射学和外科特征。病例报告及文献综述。
J Neurosurg. 1998 Aug;89(2):317-20. doi: 10.3171/jns.1998.89.2.0317.
2
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Surviving against the odds: exploring the clinical and radiological features of iniencephaly compatible with life. Illustrative case.战胜重重困难存活:探索与生命相容的无脑儿的临床和放射学特征。病例说明。
J Neurosurg Case Lessons. 2024 Mar 11;7(11). doi: 10.3171/CASE2414.
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Iniencephaly Clausus: A New Case With Clinical and Imaging Findings.
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J Pediatr Neurosci. 2015 Apr-Jun;10(2):181-4. doi: 10.4103/1817-1745.159211.
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Iniencephaly: Radiological and pathological features of a series of three cases.无脑儿后凸:三例系列病例的放射学和病理学特征
J Neurosci Rural Pract. 2014 Oct;5(4):389-93. doi: 10.4103/0976-3147.139994.
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Neural tube defects--disorders of neurulation and related embryonic processes.神经管缺陷——神经胚形成及相关胚胎发育过程的紊乱。
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Iniencephaly and long-term survival: a rare case report.无脑儿与长期生存:一例罕见病例报告
Childs Nerv Syst. 2007 Jun;23(6):719-21. doi: 10.1007/s00381-007-0309-6. Epub 2007 Mar 16.