Mann L K, Pandiri S, Agarwal N, Northrup H, Au K S, Grundberg E, Bergh E P, Austin M T, Patel R, Miller B, Zhu S, Feinberg J S, Lai D, Tsao K, Fletcher S A, Papanna R
UTHealth Houston Fetal Institute, Division of Fetal Intervention, Department of Obstetrics, Gynecology & Reproductive Sciences, McGovern Medical School, UTHealth Houston, Houston, TX, USA.
Department of Pediatrics, McGovern Medical School, UTHealth Houston, Houston, TX, USA.
Ultrasound Obstet Gynecol. 2025 Aug 23. doi: 10.1002/uog.70035.
The binary classification of spina bifida lesions as myelomeningocele (with sac) or myeloschisis (without sac) belies a spectrum of morphologies. This study aimed to characterize types of spina bifida lesion and correlate them with preoperative presentation and postoperative outcome.
This was a secondary analysis of a prospective cohort of patients referred for fetal spina bifida who underwent fetoscopic repair between 2020 and 2023 at a single center. We evaluated lesion morphology on high-resolution intraoperative images and videos to categorize lesions based on placode exposure and nerve root stretching. The reproducibility of lesion classification was assessed using the Kappa statistic (κ) to measure interrater agreement between a primary and a secondary reviewer, and among two additional reviewers. Preoperative characteristics analyzed included largest ventricular diameter, tonsillar herniation level, movement of the lower extremities and lesion dimensions. Outcomes collected included duration of surgery, need for patch-based skin closure, gestational age at delivery, occurrence of preterm prelabor rupture of membranes (PPROM), length of stay in the neonatal intensive care unit (NICU) and neonatal cerebrospinal fluid (CSF) diversion.
Of 60 lesions repaired during the study period, 57 had high-quality images available and were included in the analysis. We distinguished five types of lesion that differed according to the extent of nerve root stretching and neural placode exposure. The primary and secondary reviewers classified lesions with almost perfect agreement (κ = 0.854). Fetal characteristics that differed between lesion types at preoperative evaluation included lesion volume (P < 0.001), tonsillar herniation (P = 0.005) and head circumference percentile (P = 0.001). Lesion level, prevalence of talipes and movement of the lower extremities did not differ according to lesion type. Regarding surgical and perinatal outcomes, need for patch-based skin closure (P < 0.001), gestational age at delivery (P = 0.002) and length of stay in the NICU (P = 0.001) differed according to lesion type, whereas the rates of PPROM, CSF leakage at birth and CSF diversion in the NICU did not. On linear regression analysis, the severity of ventriculomegaly was associated with lesion type, but not with tonsillar herniation level.
There is a distinct phenotypic spectrum in open spina bifida. The severity of ventriculomegaly is associated with lesion type, rather than tonsillar herniation level. Our findings have expanded the classification of spina bifida to reveal a spectrum that warrants further study. © 2025 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
脊柱裂病变分为脊髓脊膜膨出（有囊）或脊髓裂（无囊）这种二元分类掩盖了一系列形态学特征。本研究旨在描述脊柱裂病变的类型，并将其与术前表现及术后结果相关联。
这是一项对2020年至2023年在单一中心接受胎儿镜修复的胎儿脊柱裂前瞻性队列患者的二次分析。我们在高分辨率术中图像和视频上评估病变形态，以根据神经板暴露和神经根拉伸对病变进行分类。使用Kappa统计量（κ）评估病变分类的可重复性，以测量一名主要审阅者和一名次要审阅者之间以及另外两名审阅者之间的评分者间一致性。分析的术前特征包括最大脑室直径、扁桃体疝水平、下肢活动情况和病变尺寸。收集的结果包括手术持续时间、基于补片的皮肤闭合需求、分娩时的孕周、胎膜早破（PPROM）的发生率、新生儿重症监护病房（NICU）的住院时间以及新生儿脑脊液（CSF）分流情况。
在研究期间修复的60个病变中，57个有高质量图像并纳入分析。我们区分出五种根据神经根拉伸程度和神经板暴露情况不同的病变类型。主要和次要审阅者对病变的分类几乎完全一致（κ = 0.854）。术前评估时不同病变类型之间存在差异的胎儿特征包括病变体积（P < 0.001）、扁桃体疝（P = 0.005）和头围百分位数（P = 0.001）。病变水平、马蹄内翻足的患病率和下肢活动情况根据病变类型并无差异。关于手术和围产期结果，基于补片的皮肤闭合需求（P < 0.001）、分娩时的孕周（P = 0.002）和NICU住院时间（P = 0.
