Contemporary management and outcome of myelomeningocele: the Rotterdam experience
Contemporary management and outcome of myelomeningocele: the Rotterdam experience
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DOI：
10.3171/2019.7.focus19447
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发表时间：
2019-10-01
影响因子：
4.1
通讯作者：
de Jong, Tjeerd H. R.
中科院分区：
文献类型：
--
作者：
Spoor, Jochem K. H.;Gadjradj, Pravesh S.;de Jong, Tjeerd H. R.
脊髓脊膜膨出（MMC）是最常见的脊柱裂形式，对出生时患有这种疾病的婴儿的生活质量有终身影响。近几十年来，胎儿手术已经从一种实验性治疗发展成为世界上许多中心的标准护理。在这项研究中，作者的目的是提供目前的管理和结果的婴儿MMC在他们的机构管理的概述。这就提供了一个中心特定的历史队列，以便与未来产前治疗的MMC病例进行比较。方法：这是一项回顾性的单机构队列研究，纳入了2000年1月1日至2018年6月1日在Erasmus MC进行的所有连续MMC病例。结果数据包括缺损闭合（位置、时间和手术参数）、脑积水管理、II型Chiari畸形（CMTII）管理、脊髓栓系的发生率和结果、运动结果和尿失禁。结果93例患者以腰骶部病变为主。2例患者在随访期间死亡。脑积水占84%，脑室腹腔分流再手术率为71%。12%的脊髓栓系患者在平均8岁时接受手术治疗。3例CMTII患者行减压手术。63%的特殊教育与脑积水有显著相关性（p < 0.015）。19%的患者能够独立行走，47%的患者不能行走。75%的患者出现尿失禁，4%的患者出现大便失禁。结论：本研究提供了作者中心目前MMC结果的概述，并将作为一个历史队列，用于与未来几年在该中心进行的胎儿手术病例进行比较。除了手术解栓率相对较低外，作者的结果数据与文献中的结果相当。脑积水在产后治疗的MMC患者中非常普遍；在这项研究和许多文献中，脑积水与认知功能低下有关。胎儿手术治疗MMC的一半需要分流治疗的选择组MMC妊娠，构成一个主要指征，我们接受过渡到胎儿手术中心。开放式产前手术对MMC胎儿的益处已经得到了很好的证实，但关于脊髓栓系的长期数据仍有待进一步研究。
OBJECTIVE 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.METHODS 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.RESULTS 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.CONCLUSIONS 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.