Vein of Galen Malformation.

* Abbreviations: CPAP: : continuous positive airway pressure Fio2: : fraction of inspired oxygen MFCC: : Maternal Fetal Care Clinic MRA: : magnetic resonance angiography MRI: : magnetic resonance imaging MRV: : magnetic resonance venography PGE1: : prostaglandin E1 VOGM: : vein of Galen malformation A 37-year-old gravida 3, para 1 pregnant woman was referred to the Maternal Fetal Care Center (MFCC) at Boston Children’s Hospital at 36 weeks’ gestation for the finding of vein of Galen malformation (VOGM). The patient’s pregnancy was complicated by gestational diabetes. Her surgical history was notable for adenoidectomy, a previous cesarean section, and dilation and curettage following a spontaneous abortion. Maternal medications included prenatal vitamins and glyburide. Family history was noncontributory. This pregnancy was planned and naturally conceived. The woman received appropriate prenatal care. Noninvasive prenatal screening showed her to be at low risk and cell-free fetal DNA screening was negative. She had a normal anatomy scan at 20 weeks’ gestation. Fetal ultrasonography performed at 36 weeks' gestation for evaluation of fetal growth demonstrated a prominent rounded vascular structure in the quadrigeminal cistern with turbulent flow consistent with a VOGM. Fetal echocardiography at that time revealed dilation of the right atrium and right ventricle with normal biventricular function. There was no evidence of hydrops. Imaging at 37 weeks’ gestation in the Boston Children’s Hospital MFCC included repeat comprehensive fetal echocardiography, fetal ultrasonography, and fetal brain magnetic resonance imaging (MRI). Echocardiography revealed a markedly enlarged right atrium and right ventricle with mild right ventricular dysfunction. Moderate tricuspid regurgitation was noted, the innominate vein was markedly dilated, and the superior vena cava had increased flow. The interventricular septum was dyskinetic, suggesting increased pressure within the right ventricle. There was a patent foramen ovale and a patent ductus arteriosus with right-to-left shunting throughout the cardiac cycle. No evidence of pericardial effusion or hydrops fetalis was seen. Fetal ultrasonography showed a singleton male fetus with biometry measurements, including head circumference, corresponding to estimated gestational age. Amniotic fluid volume was normal. The fetal survey demonstrated a large midline intracranial vascular structure in the expected …