Mitts M D, Whitehead W, Corroenne R, Johnson R, Donepudi R, Espinoza J, Shamshirsaz A A, Sanz Cortes M, Belfort M A, Nassr A A
Department of Obstetrics & Gynecology, Division of Maternal-Fetal Medicine, Baylor College of Medicine & Texas Children's Hospital, Houston, TX, USA.
Department of Neurosurgery, Baylor College of Medicine & Texas Children's Hospital, Houston, TX, USA.
Ultrasound Obstet Gynecol. 2024 Jul;64(1):65-70. doi: 10.1002/uog.27585.
Prenatal open neural tube defect (ONTD) repair is performed to decrease the risk of needing treatment for hydrocephalus after birth and to preserve motor function. Some centers may not consider patients to be candidates for surgery if severe ventriculomegaly is present and there is no expected benefit in risk for hydrocephalus treatment. This study sought to compare the postnatal outcome of fetuses with ONTD and severe ventriculomegaly (ventricular width ≥ 15 mm) that underwent prenatal repair with the outcome of fetuses with severe ventriculomegaly that underwent postnatal repair and fetuses without severe ventriculomegaly (< 15 mm) that underwent prenatal repair.
This was a retrospective study of fetuses with ONTD that underwent prenatal or postnatal repair between 2012 and 2021 at a single institution. The cohort was divided into two groups based on preoperative fetal ventricular size: those with severe ventriculomegaly (ventricular width ≥ 15 mm) and those without severe ventriculomegaly (< 15 mm). Fetal ventricular size was measured by magnetic resonance imaging before surgery using the standardized approach and the mean size of the left and right ventricles was used for analysis. Motor function of the lower extremities was assessed at the time of referral by ultrasound and if flexion-extension movements of the ankle were seen it was considered as preserved S1 motor function. Postnatal outcomes, including motor function of the lower extremities at birth and the need for a diversion procedure for hydrocephalus treatment during the first year after birth, were collected and compared between groups. Multivariate regression analysis was used to adjust for potential confounders.
In this study, 154 patients were included: 145 underwent fetal surgery (101 fetoscopic and 44 open hysterotomy) and nine with severe ventriculomegaly underwent postnatal repair. Among the 145 patients who underwent fetal surgery, 22 presented with severe ventriculomegaly. Fetuses with severe ventriculomegaly at referral that underwent prenatal repair were significantly more likely to need hydrocephalus treatment by 12 months after birth than those without severe ventriculomegaly (61.9% vs 28.9%, P < 0.01). However, motor function assessment at birth was similar between both prenatal repair groups (odds ratio, 0.92 (95% CI, 0.33-2.59), P = 0.88), adjusted for the anatomical level of the lesion. The prenatal repair group with severe ventriculomegaly had better preserved motor function at birth compared to the postnatal repair group with severe ventriculomegaly (median level, S1 vs L3, P < 0.01; proportion with S1 motor function, 68.2% vs 11.1%, P < 0.01). Fetuses with severe ventriculomegaly that underwent prenatal repair had an 18.9 (95% CI, 1.2-290.1)-times higher chance of having intact motor function at birth, adjusted for ethnicity, presence of club foot at referral and gestational age at delivery, compared with the postnatal repair group. There was no significant difference in the need for hydrocephalus treatment in the first year after birth between prenatal and postnatal repair groups with severe ventriculomegaly (61.9% vs 87.5%, P = 0.18).
Although fetuses with ONTD and severe ventriculomegaly do not seem to benefit from fetal surgery in terms of postnatal hydrocephalus treatment, there is an increased chance of preserved motor function at birth. Results from this study highlight the benefit of prenatal ONTD repair for cases with severe ventriculomegaly at referral to preserve motor function. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.
进行产前开放性神经管缺陷（ONTD）修复术是为了降低出生后需要治疗脑积水的风险并保留运动功能。如果存在严重脑室扩大且脑积水治疗风险无预期获益，一些中心可能不将患者视为手术候选人。本研究旨在比较接受产前修复的患有ONTD和严重脑室扩大（脑室宽度≥15mm）的胎儿与接受产后修复的严重脑室扩大胎儿以及接受产前修复的无严重脑室扩大（<15mm）胎儿的产后结局。
这是一项对2012年至2021年在单一机构接受产前或产后修复的患有ONTD的胎儿进行的回顾性研究。根据术前胎儿脑室大小将队列分为两组：严重脑室扩大（脑室宽度≥15mm）组和无严重脑室扩大（<15mm）组。术前使用标准化方法通过磁共振成像测量胎儿脑室大小，并使用左右脑室的平均大小进行分析。在转诊时通过超声评估下肢运动功能，如果观察到踝关节屈伸运动，则认为S1运动功能保留。收集并比较两组的产后结局，包括出生时下肢运动功能以及出生后第一年内是否需要进行脑积水治疗的分流手术。使用多变量回归分析来调整潜在的混杂因素。
本研究纳入了154例患者：145例接受了胎儿手术（101例胎儿镜手术和44例开放性子宫切开术），9例严重脑室扩大患者接受了产后修复。在接受胎儿手术的145例患者中，22例存在严重脑室扩大。转诊时接受产前修复的严重脑室扩大胎儿在出生后12个月时需要脑积水治疗的可能性显著高于无严重脑室扩大的胎儿（61.9%对28.9%，P<0.01）。然而，调整病变的解剖水平后，两个产前修复组出生时的运动功能评估相似（优势比，0.92（95%CI，0.33 - 2.59），P = 0.88）。与严重脑室扩大的产后修复组相比，严重脑室扩大的产前修复组出生时运动功能保留更好（中位水平，S1对L3，P<0.01；S1运动功能比例，68.2%对11.1%，P<0.01）。调整种族、转诊时是否存在马蹄内翻足和分娩时孕周后，与产后修复组相比，接受产前修复的严重脑室扩大胎儿出生时运动功能完整的可能性高18.9（95%CI，1.2 - 290.1）倍。严重脑室扩大的产前和产后修复组在出生后第一年内需要脑积水治疗的情况无显著差异（61.9%对87.5%，P = 0.18）。
尽管患有ONTD和严重脑室扩大的胎儿在出生后脑积水治疗方面似乎未从胎儿手术中获益，但出生时保留运动功能的机会增加。本研究结果突出了产前ONTD修复术对于转诊时存在严重脑室扩大病例保留运动功能的益处。©2024国际妇产科超声学会。
