Abnormal continuation of umbilical vein into extra-hepatic portal vein: Report of three cases

Anomalies of the umbilical venous system are perplexing essentially due to dissection errors and vascular connection delineation failure. Continuation of umbilical vein into the extra-hepatic portal vein is classified as group IV umbilical vein anomaly and involves the vitelline vein or its remnants. Despite this categorization most examiners ascribe fetal extra hepatic abdominal vascular abnormality as an umbilical vein anomaly. Since these anomalies involve vitelline vein, the term umbilical vein anomaly is inappropriate and should be referred to as vitelline vein abnormalities. Vitelline vein abnormalities are exceedingly rare and to the best of our knowledge only three cases have been reported prenatally. We report three cases presenting with intrauterine fetal demise and on perinatal autopsy demonstrating aneurysmally dilated group IV umbilical vein anomaly. Review of the literature, embryological basis and clinical implications of persistent vitelline vein and its varix are discussed.