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开放性脊柱裂的宫内修复:来自拉丁美洲脊柱裂联盟的历史方面、当前可及性和临床结果。

Open intrauterine repair of spina bifida aperta: Historical aspects, current availability, and clinical outcomes from the Latin American Spina Bifida Consortium.

机构信息

FETALMED-Maternal-Fetal Diagnostic Center, Santiago, Chile.

Clinica Las Condes, Santiago, Chile.

出版信息

Prenat Diagn. 2021 Jul;41(8):933-941. doi: 10.1002/pd.5994. Epub 2021 Jul 1.

Abstract

OBJECTIVE

To determine the historical aspects, current availability, and clinical outcomes of open intrauterine repair of spina bifida aperta (IRSBA) in Spanish-speaking Latin American countries.

METHODS

Cases were collected from centers with at least 2 years of experience and a minimum of 10 open IRSBA interventions by December 2020. Clinical variables were compared to the results of the Management of Myelomeningocele Study (MOMS) trial.

RESULTS

Clinical experience with 314 cases from seven centers was reviewed. Most cases (n = 189, 60.2%) were performed between 24 and 25.9 weeks' gestation. Delivery at less than 30 weeks' gestation occurred in 36 cases (11.5%) and the overall perinatal mortality rate was 5.4% (17 of 314). The rate of maternal complications was low, including the need for blood transfusion (n = 3, 0.9%) and dehiscence or a thin uterine scar (n = 4, 1.3%). No cases of maternal death were recorded. Fifteen neonates required additional surgical repair of the spinal defect (4.8%) and 63 of 167 infants (37.7%) required a cerebrospinal fluid diversion procedure. Only two of the seven centers reported preliminary experience with fetoscopic IRSBA.

CONCLUSIONS

Clinical experience and outcomes were within the expected results reported by the MOMS trial. There is still very limited experience with fetoscopic IRSBA in this part of the world.

摘要

目的

确定西班牙语拉丁美洲国家开放性神经管缺陷(IRSBA)宫内修复的历史背景、当前可用性和临床结局。

方法

收集至少有 2 年经验且至少有 10 例开放性 IRSBA 干预的中心的病例。将临床变量与神经管缺陷管理研究(MOMS)试验的结果进行比较。

结果

回顾了来自七个中心的 314 例临床经验。大多数病例(n=189,60.2%)在 24 至 25.9 孕周进行。36 例(11.5%)在小于 30 孕周分娩,围产期总死亡率为 5.4%(314 例中有 17 例)。母亲并发症发生率较低,包括需要输血(n=3,0.9%)和子宫切口裂开或变薄(n=4,1.3%)。无产妇死亡病例。15 例新生儿需要进一步的脊柱缺陷修复手术(4.8%),167 例婴儿中有 63 例(37.7%)需要脑脊液分流术。只有 7 个中心中的 2 个报告了胎儿镜 IRSBA 的初步经验。

结论

临床经验和结局与 MOMS 试验报告的预期结果相符。在世界的这一地区,胎儿镜 IRSBA 的经验仍然非常有限。

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