粪腔外翻与两例有限背髓裂病变的关系:1例报告及文献复习

Abdulrahman Jafar Sabbagh, R. Moshref, Faisal Asiri, A. Basurrah, Abdulrahman Arafah, J. Kamal
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引用次数: 0

摘要

背景:局限性背髓裂(LDM)一词由Pang于1993年提出。它涉及初级神经发育过程中背神经褶皱的不完全闭塞。另一方面,泄殖腔外翻是最复杂的内胚层异常之一,通常与脊柱异常有关;因此,OEIS复合体这个术语被创造出来(脐膨出、泄殖腔外翻、肛门闭锁和脊柱畸形复合体)。病例描述:我们报告了一名诊断为肛肠外翻的儿童同时存在两个LDM病变(起源于L2和S2水平)。最初,产前超声检测到腰椎而不是骶骨病变。患者于4岁时在术中神经生理监测(IOM)下手术解栓,本文报道其一年的随访。结论:脊髓畸形必须对脊髓腔外翻病例进行检查。LDM很少见,需要仔细诊断。应对整个神经轴进行MRI检查,以排除相关情况,包括多发性。手术应在IOM下进行,以避免长期并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Association between cloacal exstrophy and two limited dorsal myeloschisis lesions: A case report and literature review
Background: The term limited dorsal myeloschisis (LDM) was coined by Pang in 1993. It involves incomplete obliteration of the dorsal neural fold in primary neurulation. On the other hand, Cloacal Exstrophy is one of the most complex endodermal anomalies and is usually associated with spinal anomalies; hence, the term OEIS complex was coined (Omphalocele, Exstrophy of the cloaca, Imperforate anus, and Spinal deformities complex). Case Description: We report the coexistence of two LDM lesions in tandem (originating at L2 and S2 levels) in a child diagnosed with cloacal exstrophy. Initially, prenatal ultrasound detected the lumbar but not the sacral lesion. The patient was surgically untethered under intraoperative neurophysiologic monitoring (IOM) at four years of age, and this paper reports his one-year follow-up. Conclusion: Cases of cloacal exstrophy must always be investigated for spinal cord malformations. LDM is rare and requires careful diagnosis. MRI should be done for the whole neuroaxis to rule out associated conditions, including multiplicity. Surgery should be done under IOM to avoid long-term complications.
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