Detailed Analysis of Hydrocephalus and Hindbrain Herniation After Prenatal and Postnatal Myelomeningocele Closure: Report From a Single Institution

Detailed Analysis of Hydrocephalus and Hindbrain Herniation After Prenatal and Postnatal Myelomeningocele Closure: Report From a Single Institution
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DOI:
10.1093/neuros/nyz302
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发表时间:
2020-05-01
期刊:
影响因子:
4.8
通讯作者:
Adzick, N. Scott
Adzick, N. Scott
中科院分区:
医学1区
文献类型:
--
作者:
Flanders, Tracy M.;Heuer, Gregory G.;Adzick, N. Scott

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背景技术背景:脊髓脊膜膨出的治疗研究(MOMS)表明,胎儿脊髓脊膜膨出(fMMC)闭合导致改善脑积水和后脑脑疝,与产后闭合相比。目的:报告单一机构在后MOMS时代的经验结果,关于脑积水的缺失和后脑脑疝的解决。方法:对接受胎儿/出生后脊髓脊膜膨出闭合术的MOMS后患者亚组进行了单中心回顾性研究。主要结局包括脑脊液(CSF)分流状态和后脑疝消退。如果护理转移到外部机构,则通过电话联系家庭以获取结果信息。单变量/多变量分析进行了产前和产后variables.RESULTS:从2011年1月至2016年5月,数据来自62个产后和119 fMMC关闭患者的家庭。在产后组中,80.6%的病例需要CSF改道,而胎儿病例的这一比例为38.7%(P <0.01)。胎儿期修补后后脑疝消退率为81.5%,而出生后为32.6%(P <0.01)。在胎儿组中,6/119例(5.0%)患者发生胎儿/早产儿死亡。在胎儿组中,CSF分流和后脑疝消退的风险差异分别降低42.0%(95% CI -55.2至-28.8)和增加48.9%(95% CI 33.7至64.1)。在两组的单变量分析中,产前心房直径、额枕角比和后脑疝的消退与临床脑积水的不存在显著相关。胎儿组的治疗脑积水显着延迟相比,出生后组(10个月与13.8天)。结论:本研究表明,fMMC关闭CSF动力学方面的好处。
BACKGROUND: The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure.OBJECTIVE: To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution.METHODS: A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables.RESULTS: From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P < .01). Hindbrain herniation resolution occurred in 81.5% fetal repairs compared to 32.6% postnatal (P < .01). In the fetal group, fetal/premature neonatal demise occurred in 6/119 (5.0%) patients. There was a 42.0% decrease (95% CI -55.2 to -28.8) and 48.9% increase (95% CI 33.7 to 64.1) in risk difference for CSF diversion and hindbrain herniation resolution, respectively, in the fetal group. On univariate analysis for both groups, prenatal atrial diameter, frontal-occipital horn ratio, and hindbrain herniation resolution were significantly associated with the absence of clinical hydrocephalus. The treatment of hydrocephalus was significantly delayed in the fetal group compared to the postnatal group (10 mo vs 13.8 d).CONCLUSION: This study demonstrates the benefits of fMMC closure with regard to CSF dynamics.