Fetal extra-abdominal umbilical vein varix: Two cases of a rare malformation

Fetal umbilical vein varix is a rare malformation of the umbilical cord. Most of the varices arise intraabdominally and there have been multiple studies about the diagnosis and management of fetal intra-abdominal umbilical vein varices. However fetal extra-abdominal umbilical vein varices are much rarer and up to date there are no clear criteria for the diagnosis or the clinical management of the condition. We saw two cases within a month in our ultrasound department in 2022 and had a favorable outcome in both pregnancies. In the first case the diagnosis was only established at 39 weeks of gestation, and we performed a cesarean section the next day. The second case was diagnosed at 31 weeks of gestation. The patient was admitted to hospital, and we administered Betamethasone for RDS prophylaxis. At 34 weeks of gestation there were fetal heartbeat abnormalities, and a cesarean section was performed. We also reviewed the literature of previous cases and our two cases included, so far only 19 cases of fetal extra-abdominal umbilical vein varix have been described.