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产前和产后脊髓脊膜膨出修补术后脑积水和后脑疝的详细分析:单机构报告。

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

机构信息

Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

The Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

出版信息

Neurosurgery. 2020 May 1;86(5):637-645. doi: 10.1093/neuros/nyz302.

Abstract

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.

摘要

背景

《脊髓脊膜膨出管理研究》(MOMS)表明,与产后闭合相比,胎儿脊髓脊膜膨出(fMMC)闭合可改善脑积水和后脑疝。

目的

报告单机构在 MOMS 后时代的经验结果,包括脑积水缺失和后脑疝缓解情况。

方法

对接受胎儿/产后脊髓脊膜膨出闭合治疗的 MOMS 后患者进行单中心回顾性研究。主要结局包括脑脊液(CSF)引流状态和后脑疝缓解。如果患儿的治疗转至其他机构,通过电话联系家属获取结局信息。使用多个产前和产后变量进行单变量/多变量分析。

结果

2011 年 1 月至 2016 年 5 月,回顾了 62 例产后和 119 例胎儿脊髓脊膜膨出闭合患者家属的数据。在产后组中,80.6%需要 CSF 引流,而胎儿组中这一比例为 38.7%(P<.01)。胎儿修复后脑疝缓解率为 81.5%,而产后组为 32.6%(P<.01)。在胎儿组中,119 例患者中有 6 例(5.0%)胎儿/早产儿死亡。胎儿组 CSF 引流和后脑疝缓解的风险差异分别下降了 42.0%(95%CI -55.2 至 -28.8)和增加了 48.9%(95%CI 33.7 至 64.1)。两组的单变量分析均表明,产前心房直径、额枕角比和后脑疝缓解与临床脑积水的缺失显著相关。与产后组相比,胎儿组的脑积水治疗明显延迟(10 个月 vs 13.8 天)。

结论

本研究表明胎儿脊髓脊膜膨出闭合在 CSF 动力学方面具有优势。

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