1Department of Neurosurgery and.
2Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota.
Neurosurg Focus. 2019 Oct 1;47(4):E13. doi: 10.3171/2019.7.FOCUS19434.
Open prenatal myelomeningocele (MMC) repair is typically associated with reversal of in utero hindbrain herniation (HBH) and has been posited to be associated with a reduction in both postoperative prenatal and immediate postnatal hydrocephalus (HCP) risks. However, the long-term postnatal risk of HCP following HBH reversal in these cases has not been well defined. The authors describe the results of a long-term HCP surveillance in a cohort of patients who underwent prenatal MMC repair at their institution.
A retrospective review of all prenatal MMC repair operations performed at the Mayo Clinic between 2012 and 2017 was conducted. Pertinent data regarding the clinical courses of these patients before and after MMC repair were summarized. Outcomes of interest were occurrences of HBH and HCP and the need for intervention.
A total of 9 prenatal MMC repair cases were identified. There were 7 cases in which MRI clearly demonstrated prenatal HBH, and of these 86% (6/7) had evidence of HBH reversal after repair and prior to delivery. After a mean postnatal follow-up of 20 months, there were 3 cases of postnatal HCP requiring intervention. One case that failed to show complete HBH reversal after MMC repair required early ventriculoperitoneal shunting. The other 2 cases were of progressive, gradual-onset HCP despite complete prenatal HBH reversal, requiring endoscopic third ventriculostomy with choroid plexus cauterization at ages 5 and 7 months.
Although prenatal MMC repair can achieve HBH reversal in a majority of well-selected cases, the prevention of postnatal HCP requiring intervention appears not to be predicated on this outcome alone. In fact, it appears that in a subset of cases in which HBH reversal is achieved, patients can experience a progressive, gradual-onset HCP within the 1st year of life. These findings support continued rigorous postnatal surveillance of all prenatal MMC repair patients, irrespective of postoperative HBH outcome.
开放性产前脊髓脊膜膨出（MMC）修复术通常与胎儿后脑疝（HBH）的逆转相关，并被认为与术后产前和即刻产后脑积水（HCP）风险的降低相关。然而，在这些病例中，HBH 逆转后长期的产后 HCP 风险尚未得到很好的定义。作者描述了他们所在机构的一组接受产前 MMC 修复的患者进行长期 HCP 监测的结果。
对梅奥诊所 2012 年至 2017 年间进行的所有产前 MMC 修复手术进行了回顾性审查。总结了这些患者在 MMC 修复前后的临床过程相关的重要数据。感兴趣的结果是 HBH 和 HCP 的发生以及干预的需求。
共确定了 9 例产前 MMC 修复病例。有 7 例 MRI 明确显示产前 HBH，其中 86%（6/7）在修复后和分娩前有 HBH 逆转的证据。在平均 20 个月的产后随访后，有 3 例需要干预的产后 HCP。1 例 MMC 修复后未完全显示 HBH 逆转的病例需要早期脑室-腹腔分流术。另外 2 例尽管有完全的产前 HBH 逆转，但表现为进行性、逐渐发作的 HCP，需要在 5 个月和 7 个月龄时行内镜第三脑室造瘘和脉络丛烧灼术。
尽管大多数精选病例中产前 MMC 修复可实现 HBH 逆转，但预防需要干预的产后 HCP 似乎不仅仅取决于这一结果。事实上，在一些 HBH 逆转的病例中，患者在生命的第一年可能会经历进行性、逐渐发作的 HCP。这些发现支持对所有接受产前 MMC 修复的患者进行严格的产后监测，无论术后 HBH 结果如何。
