Objective

To assess our initial experience with prenatal restoration of hindbrain herniation following in utero repair of myelomeningocele (MMC).

Patients and Methods

Three consecutive patients with prenatally diagnosed MMC (between January 1, 2018 and September 30, 2018) were managed with open in utero surgery. As per institutional review board approval and following a protocol designed at the Mayo Clinic Maternal & Fetal Center, fetal intervention was offered between 19 0/7 and 25 6/7 weeks of gestation. Prenatal improvement of hindbrain herniation was the declared restorative end point. Obstetrical and perinatal outcomes were also assessed.

Results

Diagnosis of MMC was confirmed upon referral between 20 and 21 weeks’ gestation by using fetal ultrasound and magnetic resonance imaging. In all cases reported here, the spinal defect was lumbosacral with evidence of hindbrain herniation. Open in utero MMC repair was performed between 24 and 25 weeks’ gestation with no notable perioperative complications. Postprocedure fetal magnetic resonance imaging performed 6 weeks after in utero repair documented improvement of hindbrain herniation. Deliveries were at 37 weeks by cesarean section without complications. Most recent postnatal follow-ups were unremarkable at both 11 months (baby 1) and 3 months of age (baby 2), with mild ventriculomegaly. Antenatal and postnatal follow-up of baby 3 at 1 month of age was also unremarkable.

Conclusion

Our study highlights the prenatal restoration of hindbrain herniation following in utero MMC repair in all cases presented here as an example of a prenatal regenerative therapy program in our institution.

中文翻译：

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梅奥诊所产前再生治疗计划的一部分，在胎儿脊髓膜囊膨出症的后脑疝的子宫修复中。

目的

评估我们在子宫内脊髓膜膨出术（MMC）子宫修复后产后恢复后脑疝的初步经验。

患者和方法

连续三名产前诊断为MMC的患者（2018年1月1日至2018年9月30日之间）接受子宫内开放手术治疗。根据机构审查委员会的批准，并按照梅奥诊所母婴中心设计的方案，在妊娠19 0/7到25 6/7周之间提供胎儿干预。宣布的恢复终点是产后后脑疝的改善。还评估了产科和围产期结局。

结果

在胎儿妊娠20至21周之间转诊时，通过使用胎儿超声和磁共振成像确认了MMC的诊断。在这里报道的所有病例中，脊柱缺损均为腰s部伴有后脑突出的证据。宫内开放式MMC的修复在妊娠24至25周之间进行，没有明显的围手术期并发症。子宫内修复6周后进行的胎儿胎儿磁共振检查表明，后脑疝的改善。剖宫产分娩的时间为37周，无并发症。最近的产后随访在11个月（婴儿1）和3个月大（婴儿2）均无明显变化，轻度脑室大。1个月大的婴儿3的产前和产后随访也无异常。

结论

我们的研究强调了此处提出的所有情况下子宫内MMC修复后后脑疝的产前恢复，这是我们机构进行的产前再生治疗计划的一个示例。