1Department of Neurosurgery, Tulane/Ochsner Medical Center, New Orleans, Louisiana.
2Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
J Neurosurg Pediatr. 2020 Dec 4;27(2):151-159. doi: 10.3171/2020.7.PEDS20441. Print 2021 Feb 1.
Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.
神经管分裂综合征（SNS）是一种罕见的中枢神经系统先天性缺陷，与影响多个器官系统的多种异常有关。其中一种关联是与胃肠道和脊柱相通，以前被认为是神经肠瘘（NEF）。在这里，作者描述了一例女性婴儿 SNS 和 NEF 的独特病例，该婴儿采用多阶段手术修复进行治疗。三阶段手术计划包括缺陷的两阶段修复以及暂时的皮下分流，随后进行延迟脑室-腹腔分流术。该婴儿术后恢复良好，随访 5 年。报告了病例描述、手术技术和原理。此外，还利用 MEDLINE 数据库进行了系统的文献回顾。采用多学科多阶段方法治疗 SNS 合并 NEF，修复肠道缺陷、闭合神经元素并将脑脊液引流至腹膜，为未来的病例提供了一种安全可行的选择。
