Johnson Mark P, Sutton Leslie N, Rintoul Natalie, Crombleholme Timothy M, Flake Alan W, Howell Lori J, Hedrick Holly L, Wilson R Douglas, Adzick N Scott
Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadephia, PA 19104, USA.
Am J Obstet Gynecol. 2003 Aug;189(2):482-7. doi: 10.1067/s0002-9378(03)00295-3.
The study was undertaken to evaluate short-term clinical outcomes with antenatal myelomeningocele (MMC) repair.
Retrospective review of 50 fetuses that underwent open fetal MMC closure was performed. Inclusion criteria included less than 26 weeks' gestation, thoracic to S1 level defect, absent clubfoot deformity, Arnold-Chiari malformation, ventriculomegaly less than 17 mm, normal karyotype, and no other anomalies.
Perinatal survival was 94% (47/50 fetuses). Mean age at delivery was 34 weeks 3 days. All fetuses demonstrated reversal of hindbrain herniation. Forty-three percent of the 50 fetuses have required ventriculoperitoneal shunting compared with 100% thoracic, 88% lumbar, and 68% sacral (85% overall) in 297 historic controls. Better-than-predicted leg function was seen in 57% of thoracic and lumbar level lesion patients.
Early experience with fetal MMC repair suggests a decreased need for ventriculoperitoneal shunting, arrest or slowing of progressive ventriculomegaly, and consistent resolution of hindbrain herniation. However, further long-term follow-up is needed to evaluate neurodevelopment and bladder and bowel function.
本研究旨在评估产前脊髓脊膜膨出（MMC）修复的短期临床结局。
对50例行开放性胎儿MMC闭合术的胎儿进行回顾性研究。纳入标准包括孕周小于26周、胸段至S1水平缺损、无马蹄内翻足畸形、阿诺德-奇阿利畸形、脑室扩大小于17mm、核型正常且无其他异常。
围产期存活率为94%（47/50例胎儿）。平均分娩年龄为34周3天。所有胎儿均表现为后脑疝复位。50例胎儿中有43%需要行脑室腹腔分流术，而297例历史对照中胸段病变者为100%、腰段病变者为88%、骶段病变者为68%（总体为85%）。57%的胸段和腰段病变患者腿部功能好于预期。
胎儿MMC修复的早期经验表明，脑室腹腔分流术需求减少、进行性脑室扩大停止或减缓以及后脑疝持续消退。然而，需要进一步长期随访以评估神经发育及膀胱和肠道功能。
