Department of Gynecology and Obstetrics, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Japan Society for the Promotion of Science, Tokyo, Japan.
Congenit Anom (Kyoto). 2021 Jan;61(1):4-8. doi: 10.1111/cga.12397. Epub 2020 Nov 5.
Umbilical vein varix is associated with a high incidence of fetal anomalies and perinatal complications. There are two types of umbilical vein varix: fetal intra-abdominal and extra-abdominal. Herein, a case is reported of severe fetal anemia with extra-abdominal umbilical vein varix. A 33-year-old primigravida was referred to our hospital for fetal growth restriction, fetal cardiomegaly, and decreased fetal movements at 26 weeks' gestation. A Doppler assessment showed an elevated middle cerebral artery peak systolic velocity at 2.2 MoM, suggesting fetal anemia. Umbilical vein varix had caused intermittent turbulent flow, provoking hemolytic anemia. Intrauterine transfusion improved fetal circulatory failure and anemia and prolonged gestational period. At 33 weeks' gestation, the patient underwent cesarean delivery due to nonreassuring fetal status. Pathological analysis revealed focal loss of vascular smooth muscle of the umbilical vein. Extra-abdominal umbilical vein varix has been reported in 14 cases including this case. The antenatal diagnosis rate is reported to be 79%; fetal heartbeat abnormalities and fetal deaths were reported as 50% and 14%, respectively. Eighty-six percent of patients had intra-umbilical cord thrombosis, but currently this is the only case of hemolytic anemia. Furthermore, extra-abdominal umbilical vein varix may present as fetal hydrops with anemia. During ultrasound examination of fetal anemia, umbilical cord screening should be performed with caution.
脐静脉静脉瘤与胎儿畸形和围产儿并发症的高发生率相关。脐静脉静脉瘤有两种类型：胎儿腹内型和腹外型。本文报告了一例因腹外型脐静脉静脉瘤导致严重胎儿贫血的病例。一位 33 岁的初产妇因胎儿生长受限、胎儿心胸比增大和胎动减少，于 26 孕周就诊于我院。多普勒评估显示中动脉收缩峰速度升高 2.2MoM，提示胎儿贫血。脐静脉静脉瘤导致间歇性湍流，引发溶血性贫血。宫内输血改善了胎儿循环衰竭和贫血，延长了孕周。在 33 孕周时，因胎儿情况不稳定，患者行剖宫产术。病理分析显示脐静脉局部血管平滑肌丧失。包括本例在内，共报告了 14 例腹外型脐静脉静脉瘤病例。产前诊断率为 79%；胎儿心跳异常和胎儿死亡的发生率分别为 50%和 14%。86%的患者存在脐带内血栓，但目前这是唯一一例溶血性贫血。此外，腹外型脐静脉静脉瘤可能表现为伴有贫血的胎儿水肿。在胎儿贫血的超声检查中，应谨慎进行脐带筛查。
