Chen Karen S, Joyeux Luc, Corroenne Romain, Munoz Jessian L, Whitehead William E, McClugage Samuel, Martinez Mesha, Krings Timo, Kan Peter, Weiner Howard L, Hollier Larry H, Sutton Caitlin D, Altman Carolyn A, Belfort Michael A, Huisman Thierry A G M, Cortes Magdalena Sanz
From the Department of Radiology (KSC, MM, TAGMH), Department of Obstetrics and Gynecology (LJ, RC, JLM, MAB, MSC), Department of Surgery (LJ, WEW, SM, HW, LHH), Department of Pediatrics (CAA), Department of Anesthesiology (CDS), Texas Children's Fetal Center (KSC, LJ, RC, JLM, WEW, SM, HW, LH, CDS, CAA, MAB, TAGMH, MSC), Texas Children's Hospital/Baylor College of Medicine, Houston, TX, USA; Department of Radiology (TK), Lahey Hospital and Medical Center, Burlington, MA, USA; Department of Neurological Surgery (PK), University of Texas Medical Branch, Galveston, TX, USA.
AJNR Am J Neuroradiol. 2025 Aug 20. doi: 10.3174/ajnr.A8962.
Vein of Galen malformation (VOGM) is a rare fetal arteriovenous shunt with presentations ranging from asymptomatic infancy to high-output cardiac failure and death. Prenatal percutaneous embolization is being explored in fetuses predicted to be at high risk for death in the Neonatal Intensive Care Unit (NICU). The purpose of this study is to (1) evaluate the reliability of previously reported measurements and (2) identify any novel imaging markers predictive of NICU mortality in a cohort of VOGM patients managed at our institution.
This was a single-center retrospective cohort study of fetuses and neonates with isolated VOGM evaluated at our hospital from 2016-2024. Patients with fetal and/or neonatal MRI had assessment of the narrowest mediolateral diameter of the straight or falcine sinus (SS-MD), ventriculomegaly, pseudofeeders, cerebral ischemia, and hydrops. Indexed combined cardiac output and tricuspid regurgitation severity were evaluated echocardiographically. Advanced post-processing MRI techniques were used to calculate the VOGM varix volume, brain parenchymal volume, and varix-to-brain volume ratio (VBR). Receiver Operating Characteristic (ROC) curves were used to determine cutoff values for predicting NICU mortality. All variables predicting NICU mortality were adjusted for age at MRI and delivery using regression analysis.
Fourteen cases with pre-embolization MRIs were identified (6 fetal and 8 neonatal). NICU mortality was 29% (4/14). Survivors and non-survivors had significantly different SS-MD (6.6 vs 11.0 mm, p=0.007) and VOGM varix volume (4,145 vs. 12,758 mm3, p=0.014) respectively, however after adjusting for age at MRI and delivery, these differences were no longer statistically significant. However, VBR significantly differed between survivors and non-survivors (1.4% vs 7.7%, p=0.008, respectively) even after adjusting for age at MRI and delivery (p=0.038). A VBR >4.18% was 100% sensitive and 90% specific in predicting NICU mortality (AUC=0.95, p= 0.011) with a positive likelihood ratio of 10.
Previously reported imaging findings did not predict NICU mortality from VOGM in this cohort after adjusting for age at MRI and delivery. However, a VBR >4.18% strongly predicted NICU mortality in our cohort, suggesting that this novel parameter may help to identify VOGM patients who could benefit from fetal intervention.
VOGM = Vein of Galen Malformation, NICU = neonatal intensive care unit, AUC = area under the curve, VBR = ratio of VOGM varix volume to brain parenchyma volume, SS-MD = mediolateral width of the straight or falcine sinus at its narrowest dimension, GA = gestational age, dGA = gestational age at delivery, mGA = gestational age at MRI, mPMA = post-menstrual age at MRI, CCI = combined cardiac index, MCA = middle cerebral artery, TR = tricuspid regurgitation, TOTAL = Tracheal Occlusion to Accelerate Lung Growth, CDH = congenital diaphragmatic hernia, ROC = receiver operating characteristic.
大脑大静脉畸形（VOGM）是一种罕见的胎儿动静脉分流，表现范围从无症状婴儿期到高输出量心力衰竭及死亡。对于预计在新生儿重症监护病房（NICU）有高死亡风险的胎儿，正在探索产前经皮栓塞术。本研究的目的是：（1）评估先前报道测量值的可靠性；（2）在我们机构管理的一组VOGM患者中确定任何预测NICU死亡率的新影像学标志物。
这是一项单中心回顾性队列研究，研究对象为2016年至2024年在我院接受评估的患有孤立性VOGM的胎儿和新生儿。对有胎儿和/或新生儿MRI检查的患者评估直窦或大脑镰窦最窄的内外侧直径（SS-MD）、脑室扩大、假性供血动脉、脑缺血和水肿。通过超声心动图评估心输出量指数和三尖瓣反流严重程度。使用先进的MRI后处理技术计算VOGM瘤样扩张体积、脑实质体积和瘤样扩张与脑体积比（VBR）。受试者工作特征（ROC）曲线用于确定预测NICU死亡率的临界值。使用回归分析对所有预测NICU死亡率的变量按MRI检查时年龄和分娩时年龄进行校正。
确定了14例栓塞前有MRI检查的病例（6例胎儿和8例新生儿）。NICU死亡率为29%（4/14）。幸存者和非幸存者的SS-MD（6.6对11.0mm，p=0.007）和VOGM瘤样扩张体积（4145对12758mm³，p=0.014）分别有显著差异，然而在校正MRI检查时年龄和分娩时年龄后，这些差异不再具有统计学意义。但是，即使在校正MRI检查时年龄和分娩时年龄后（p=0.038），幸存者和非幸存者之间的VBR仍有显著差异（分别为1.4%对7.7%，p=0.008）。VBR>4.18%在预测NICU死亡率方面敏感性为100%，特异性为90%（AUC=0.95，p=0.011），阳性似然比为10。
在校正MRI检查时年龄和分娩时年龄后，先前报道的影像学表现不能预测该队列中VOGM患者的NICU死亡率。然而，VBR>4.18%在我们的队列中强烈预测NICU死亡率，表明这个新参数可能有助于识别可能从胎儿干预中获益的VOGM患者。
VOGM = 大脑大静脉畸形，NICU = 新生儿重症监护病房，AUC = 曲线下面积，VBR = VOGM瘤样扩张体积与脑实质体积之比，SS-MD = 直窦或大脑镰窦最窄处的内外侧宽度，GA = 胎龄，dGA = 分娩时胎龄，mGA = MRI检查时胎龄，mPMA = MRI检查时月经后年龄，CCI = 心输出量指数，MCA = 大脑中动脉，TR = 三尖瓣反流，TOTAL = 气管闭塞促进肺生长，CDH = 先天性膈疝，ROC = 受试者工作特征
