Farmer Diana L, von Koch Cornelia S, Peacock Warwick J, Danielpour Moise, Gupta Nalin, Lee Hanmin, Harrison Michael R
Departments of Surgery and Neurosurgery and the Fetal Treatment Center, University of California, San Francisco, CA 94143-0570, USA.
Arch Surg. 2003 Aug;138(8):872-8. doi: 10.1001/archsurg.138.8.872.
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.
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.
A tertiary referral center.
All patients treated between 1998 and 2002 for a prenatally diagnosed MMC.
Either fetoscopic MMC repair, fetoscopic patch repair, or limited maternal hysterotomy and microsurgical 3-layered fetal MMC repair was performed.
Gestational age at delivery, survival, neurologic outcome, and need for ventricular shunting at 1 year.
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 repair. 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 1 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.
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.
实验研究提出了一种可能性，即胎儿期修复脊髓脊膜膨出（MMC）可能改善下肢、膀胱和肠道功能，改善阿诺德 - 奇亚里畸形，并减少出生后分流的需求。
我们之前开发了胎羊模型，用于在子宫内造成并逆转下肢损伤和阿诺德 - 奇亚里畸形。然后，我们运用在胎儿手术方面的丰富经验，包括胎儿内镜（胎儿镜）手术操作，来开发脊髓脊膜膨出修复技术。
一家三级转诊中心。
1998年至2002年间所有因产前诊断为脊髓脊膜膨出而接受治疗的患者。
进行胎儿镜下脊髓脊膜膨出修复术、胎儿镜下补片修复术，或有限的母体子宫切开术及显微外科三层胎儿脊髓脊膜膨出修复术。
分娩时的孕周、存活率、神经学结局以及1岁时脑室分流的需求。
1例胎儿完成了完全胎儿镜修复。另外2例胎儿接受了部分胎儿镜手术。其余10例患者接受了有限的母体子宫切开术及显微外科三层胎儿脊髓脊膜膨出修复术。13例患者中有4例死亡，11例存活出生胎儿的平均分娩孕周为31周。9例中有5例在1岁前需要脑室分流。与产前超声检查相比，2例患者的下肢功能改善超过2个椎体节段。10例存活超过3周的患者中有5例在出生后7天内需要进行产后伤口修复。
胎儿镜修复虽然可行，但尚未产生最佳手术效果。妊娠22周前进行开放性手术修复在生理上合理且技术上可行。三分之一的患者在1岁时似乎无需分流，但远端神经功能的改善尚不清楚。此外，该手术与胎儿死亡率相关。我们的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范德比尔特大学研究小组发表的数据。美国国立卫生研究院资助的一项前瞻性随机试验目前正在这3个中心进行，以比较胎儿期修复与出生后修复。
