BCNatal | Fetal Medicine Research Center (Hospital Clínic and Hospital Sant Joan de Déu), University of Barcelona, Barcelona, Spain (Drs Trigo, Eixarch, and Gratacos); Fetal Therapy Program, Hospital Israelita Albert Einstein, Sao Paulo, Brazil (Drs Trigo, and Lapa); Fetal Therapy Program, Hospital Infantil Sabará, Sao Paulo, Brazil (Drs Trigo, and Lapa).
BCNatal | Fetal Medicine Research Center (Hospital Clínic and Hospital Sant Joan de Déu), University of Barcelona, Barcelona, Spain (Drs Trigo, Eixarch, and Gratacos); Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain (Drs Eixarch, and Gratacos); Centre for Biomedical Research on Rare Diseases, Barcelona, Spain (Drs Eixarch, and Gratacos).
Am J Obstet Gynecol MFM. 2023 Jun;5(6):100932. doi: 10.1016/j.ajogmf.2023.100932. Epub 2023 Mar 16.
Open spina bifida is associated with central nervous system anomalies such as abnormal corpus callosum and heterotopias. However, the impact of prenatal surgery over these structures remains unclear.
This study aimed to describe longitudinal changes of central nervous system anomalies before and after prenatal open spina bifida repair and to evaluate their relationship with postnatal neurologic outcomes.
Retrospective cohort study of fetuses with open spina bifida who underwent percutaneous fetoscopic repair from January 2009 to August 2020. All women had presurgical and postsurgical fetal magnetic resonance imaging, at an average of 1 week before and 4 weeks after surgery, respectively. We evaluated defect characteristics in the presurgical magnetic resonance images; and fetal head biometry, clivus supraocciput angle, and the presence of structural central nervous system anomalies, such as abnormalities in corpus callosum, heterotopias, ventriculomegaly, and hindbrain herniation, in both presurgical and postsurgical magnetic resonance images. Neurologic assessment was performed using the Pediatric Evaluation of Disability Inventory scale in children who were 12 months or older, covering 3 different sections, namely self-care, mobility, and social and cognitive function.
A total of 46 fetuses were evaluated. Presurgery and postsurgery magnetic resonance imaging were performed at a median gestational age of 25.3 and 30.6 weeks, with a median interval of 0.8 weeks before surgery, and 4.0 weeks after surgery. There was a 70% reduction in hindbrain herniation (100% vs 32.6%; P<.001), and a normalization of the clivus supraocciput angle after surgery (55.3 [48.8-61.0] vs 79.9 [75.2-85.4]; P<.001). No significant increase in abnormal corpus callosum (50.0% vs 58.7%; P=.157) or heterotopia (10.8% vs 13.0%; P=.706) was observed. Ventricular dilation was higher after surgery (15.6 [12.7-18.1] vs 18.8 [13.7-22.9] mm; P<.001), with a higher proportion of severe ventricular dilation after surgery (≥15mm) (52.2% vs 67.4%; P=.020). Thirty-four children underwent neurologic assessment, with 50% presenting a global optimal Pediatric Evaluation of Disability Inventory result and 100% presenting a normal social and cognitive function. Children with optimal global Pediatric Evaluation of Disability Inventory presented a lower rate of presurgical anomalies in corpus callosum and severe ventriculomegaly. When analyzed as independent variables to global Pediatric Evaluation of Disability Inventory scale, the presence of abnormal corpus callosum and severe ventriculomegaly showed an odds ratio of 27.7 (P=.025; 95% confidence interval, 1.53-500.71) for a suboptimal result.
Prenatal open spina bifida repair did not change the proportion of abnormal corpus callosum nor heterotopias after surgery. The combination of presurgical abnormal corpus callosum and severe ventricular dilation (≥15 mm) is associated with an increased risk of suboptimal neurodevelopment.
开放性脊柱裂与中枢神经系统异常有关，如胼胝体异常和异位。然而，产前手术对这些结构的影响仍不清楚。
本研究旨在描述开放性脊柱裂产前修复前后中枢神经系统异常的纵向变化，并评估其与产后神经结局的关系。
回顾性队列研究，纳入 2009 年 1 月至 2020 年 8 月期间接受经皮胎儿镜修复的开放性脊柱裂胎儿。所有女性均在术前和术后平均 1 周和 4 周分别进行胎儿磁共振成像。我们评估了术前磁共振图像中的缺陷特征；并评估了胎儿头部生物测量、枢椎颅顶角和结构中枢神经系统异常的存在，如胼胝体异常、异位、脑室扩张和后脑疝，在术前和术后磁共振图像中均进行了评估。对于 12 个月或以上的儿童，使用残疾儿童评估量表（Pediatric Evaluation of Disability Inventory scale）进行神经评估，涵盖了自我护理、运动和社会认知功能 3 个不同的部分。
共评估了 46 例胎儿。术前和术后磁共振成像分别在中位妊娠 25.3 和 30.6 周进行，术前中位间隔为 0.8 周，术后中位间隔为 4.0 周。后脑疝减少了 70%（100%比 32.6%；P<.001），术后枢椎颅顶角正常化（55.3[48.8-61.0]比 79.9[75.2-85.4]；P<.001）。异常胼胝体（50.0%比 58.7%；P=.157）或异位（10.8%比 13.0%；P=.706）的比例无显著增加。术后脑室扩张更高（15.6[12.7-18.1]比 18.8[13.7-22.9]mm；P<.001），术后严重脑室扩张（≥15mm）的比例更高（52.2%比 67.4%；P=.020）。34 例儿童接受了神经评估，50%的儿童获得了全球最佳残疾儿童评估量表结果，100%的儿童获得了正常的社会和认知功能。具有全球最佳残疾儿童评估量表结果的儿童，其术前胼胝体和严重脑室扩张异常的发生率较低。当作为独立变量分析全球残疾儿童评估量表时，异常胼胝体和严重脑室扩张的存在的优势比为 27.7（P=.025；95%置信区间，1.53-500.71），表明结果不佳的风险增加。
产前开放性脊柱裂修复并未改变术后胼胝体异常或异位的比例。术前异常胼胝体和严重脑室扩张（≥15mm）的组合与神经发育不良的风险增加有关。
