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Congenital absence of the portal vein (CAPV) is a rare anomaly in the form of a portocaval shunt, whereby the intestinal and splenic venous drainage bypasses the liver and drains directly into the systemic circulation. We report a case of CAPV diagnosed prenatally after the recognition of a dilated umbilical vein draining directly into a large inferior vena cava (IVC). The IVC then drained into the right atrium of a dilated, hyperdynamic heart. The ductus venosus could not be identified. Repeated postnatal scans showed a gradual disappearance of venous lakes in the region of the porta hepatis and a clear drainage of the splenic vein to the left renal vein and the superior mesenteric vein to the IVC. From birth up to twelve months follow-up there was no evidence of liver dysfunction, encephalopathy or liver lesions.

Original publication




Journal article


Prenat Diagn

Publication Date





537 - 540


Adult, Female, Fetal Diseases, Follow-Up Studies, Humans, Infant, Newborn, Male, Portal Vein, Pregnancy, Pregnancy Outcome, Ultrasonography, Prenatal, Umbilical Veins, Vena Cava, Inferior