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脊髓脊膜膨出:长期神经外科治疗管理。

Myelomeningocele: Long-Term Neurosurgical Management.

机构信息

Department of Neurosurgery, ASST Niguarda Hospital, Milan, Italy.

Department of Orthopedics, ASST Niguarda Hospital, Milan, Italy.

出版信息

Adv Tech Stand Neurosurg. 2024;49:95-122. doi: 10.1007/978-3-031-42398-7_6.

Abstract

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.

摘要

开放性脊柱裂,又称脊髓脊膜膨出(MMC),是一种最具挑战性和严重的中枢神经系统出生缺陷,与生命兼容,它是由于胚胎发育过程中新生神经管的背侧融合失败所致。MMC 常伴有一系列的伴发情况,包括脑积水、Arnold-Chiari II 畸形、脑干功能障碍、脊髓空洞症、脊髓栓系综合征和脊柱侧凸。除了早期修复背部缺陷的手术外,MMC 还需要终身护理。通常需要进行几次额外的手术来改善 MMC 患者的长期预后,多学科评估对于早期识别和处理可能伴随这种病理的各种医学状况至关重要。在本章中,我们阐述了与 MMC 相关的最常见的病理实体,重点介绍了临床表现、治疗策略和随访建议。

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