Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Mayo Clinic College of Medicine, Rochester, MN; Center for Regenerative Medicine, Mayo Clinic College of Medicine, Rochester, MN.
Division of Pediatric Neurosurgery, Department of Surgery, Mayo Clinic College of Medicine, Rochester, MN.
Mayo Clin Proc. 2020 Apr;95(4):738-746. doi: 10.1016/j.mayocp.2019.10.039.
To assess our initial experience with prenatal restoration of hindbrain herniation following in utero repair of myelomeningocele (MMC).
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.
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.
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.
评估我们在子宫内修复脊髓脊膜膨出后对后脑疝进行产前修复的初步经验。
3 例连续的产前诊断为脊髓脊膜膨出的患者（2018 年 1 月 1 日至 2018 年 9 月 30 日）接受了开放性子宫内手术治疗。根据机构审查委员会的批准，并遵循梅奥诊所母婴胎儿中心设计的方案，胎儿干预在妊娠 19 周零 7 天至 25 周零 6 天之间进行。产前改善后脑疝是宣布的修复终点。还评估了产科和围产期结局。
通过胎儿超声和磁共振成像在转诊时于 20 至 21 周妊娠时确诊脊髓脊膜膨出。在这里报告的所有病例中，脊柱缺陷均为腰骶部，伴有后脑疝。开放性子宫内脊髓脊膜膨出修复术在 24 至 25 周妊娠时进行，无明显围手术期并发症。子宫内修复后 6 周进行的术后胎儿磁共振成像显示后脑疝改善。通过剖宫产在 37 周分娩，无并发症。最近的产后随访在 11 个月（婴儿 1）和 3 个月（婴儿 2）时均无异常，仅有轻度脑室扩大。婴儿 3 在 1 个月龄时的产前和产后随访也无异常。
我们的研究强调了在子宫内修复脊髓脊膜膨出后，这里报告的所有病例都可以进行产前后脑疝修复，这是我们机构产前再生治疗计划的一个例子。
