In utero repair of myelomeningocele - experimental pathophysiology, initial clinical experience, and outcomes
In utero repair of myelomeningocele - experimental pathophysiology, initial clinical experience, and outcomes
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DOI：
10.1001/archsurg.138.8.872
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发表时间：
2003-08-01
影响因子：
--
通讯作者：
Harrison, MR
中科院分区：
文献类型：
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作者：
Farmer, DL;von Koch, CS;Harrison, MR
假设：实验工作提出的可能性，在子宫内修复脊髓脊膜膨出（MMC）可能会改善下肢，膀胱和肠功能，改善Arnold-Chiari畸形，并减少产后shunting.Design的需要：我们以前开发的胎羊模型，以创建和扭转下肢损伤和Arnold-Chiari畸形在子宫内。然后，我们运用我们丰富的经验，胎儿手术，包括胎儿内窥镜（fetoscopic）手术操作，开发技术MMC repair.Setting：三级转诊center.Patients：所有患者治疗1998年和2002年之间产前诊断的MMC。无论是胎儿镜MMC修复，胎儿镜修补，或有限的产妇子宫切开术和显微外科3层胎儿MMC repairmentwere performed.Main Outcome Measures：分娩时的胎龄，生存率，神经功能的结果，并需要脑室分流在1 year.Results：完整的胎儿镜修复完成1胎儿。另外2只胎仔接受了部分胎仔镜检查。其余10例患者行母体有限子宫切除术和显微外科三层胎儿MMC修复：4例。13例患者死亡，11例活产胎儿的平均胎龄为31周。5/9例在1岁时需要脑室分流。在2例患者中，与产前超声检查相比，下肢功能改善了2个以上椎体水平。5 10例患者谁住了3周以上，需要产后伤口修订7天内birth.Conclusions：胎儿镜修复，虽然可行，但尚未产生最佳的手术效果。妊娠22周前开放手术修补在生理上是合理的，技术上也是可行的。三分之一的患者在1岁时似乎不需要分流，但远端神经功能的改善不太清楚。此外，胎儿死亡率与此程序有关。我们的研究结果补充了宾夕法尼亚州费城儿童医院和田纳西州纳什维尔的范德比尔特大学的研究小组发表的数据。一项由美国国立卫生研究院赞助的前瞻性随机试验正在这三个中心进行，以比较胎儿修复和产后修复。
Hypothesis: Experimental work raises the possibility that in utero repair of myelomeningocele (MMC) may improve lower extremity, bladder, and bowel function, ameliorate the Arnold-Chiari malformation, and decrease the need for postnatal shunting.Design: We previously developed fetal lamb models to create and reverse lower extremity damage And the Arnold-Chiari malformation in utero. We then applied our extensive experience with fetal surgery, including fetal endoscopic (fetoscopic) surgical manipulation, to develop techniques for MMC repair.Setting: A tertiary referral center.Patients: All patients treated between 1998 and 2002 for a prenatally diagnosed MMC.Interventions:. Either fetoscopic MMC repair, fetoscopic patch repair, or limited maternal hysterotomy and microsurgical 3-layered fetal MMC repair was performed.Main Outcome Measures: Gestational age at delivery, survival, neurologic outcome, and need for ventricular shunting at 1 year.Results: Complete fetoscopic repair was accomplished in 1 fetus. Two other fetuses underwent partial fetoscopic procedures. The remaining 10 patients underwent limited maternal hysterotomy and microsurgical 3-layered fetal MMC re pair: Four of. 13 patients died, and the mean gestational age at delivery of 11 fetuses born alive was 31 weeks. Five of 9 required ventricular shunting by age I year. In 2 patients, lower extremity function improved by more than 2 vertebral levels compared with prenatal ultrasonography. Five of 10 patients who lived longer than 3 weeks required postnatal wound revision within 7 days after birth.Conclusions: Fetoscopic repair, although feasible, does not yet yield optimal surgical results. Open surgical repair before 22 weeks' gestation is physiologically sound and technically feasible. One third of patients appear to be spared the need for a shunt at age 1 year, but improvement in distal neurologic function is less clear. Additionally, fetal mortality is associated with this procedure. Our results complement the data, published by groups at Children's Hospital of Philadelphia, in Pennsylvania, and Vanderbilt University, Nashville, Tenn. A National Institutes of Health-sponsored prospective randomized trial is now underway at these 3 centers to compare fetal repair with postnatal repair.