1Department of Neurosurgery.
2Department of Neurosurgery, Leiden University Medical Center, Leiden, The Netherlands.
Neurosurg Focus. 2019 Oct 1;47(4):E3. doi: 10.3171/2019.7.FOCUS19447.
Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases.
This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence.
A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence.
This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.
脊膜脊髓膨出（MMC）是最常见的神经管缺陷，对患有这种疾病的婴儿的生活质量产生终身影响。近几十年来，胎儿手术已从一种实验性治疗方法发展为世界上许多中心的标准治疗方法。在这项研究中，作者旨在概述他们所在机构管理和治疗 MMC 婴儿的现状和结果。这为与未来接受产前治疗的 MMC 病例进行比较提供了一个特定中心的历史队列。
这是一项回顾性的单中心队列研究，包括 2000 年 1 月 1 日至 2018 年 6 月 1 日期间在伊拉斯谟医学中心接受治疗的所有连续 MMC 病例。结果数据包括缺陷（位置、时间和手术参数）的闭合、脑积水管理、Chiari 畸形 II 型（CMTII）管理、脊髓栓系的发生率和结果、运动结果以及控尿情况。
共纳入 93 例腰骶部病变患者。2 例患者在随访期间死亡。84%存在脑积水，脑室-腹腔分流术再手术率为 71%。12%的患者因脊髓栓系而行手术，平均年龄为 8 岁。3 例患者因 CMTII 行减压手术。63%的患者接受特殊教育与脑积水显著相关（p<0.015）。19%的患者能够独立行走，47%的患者不能行走。75%的患者获得了尿控，4%的患者出现了粪便失禁。
本研究概述了作者所在中心目前的 MMC 结果，并将作为历史队列，与未来几年中心接受胎儿手术治疗的病例进行比较。除了相对较低的脊髓松解手术率外，作者的结果数据与文献中的数据相当。在接受产后治疗的 MMC 患者中，脑积水的发病率很高；在本研究和大多数文献中，脑积水与认知功能低下相关。胎儿手术治疗 MMC 对半选择性 MMC 妊娠分流术的需求减半，这是我们向胎儿手术中心过渡的主要指征。开放性产前手术治疗 MMC 的胎儿获益已得到充分证实，但人们急切地等待着关于脊髓栓系的长期数据。
