Prenatally diagnosed portocaval shunt and postnatal outcome: a case report.
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.
Department of Fetal Medicine, John Radcliffe Hospital, Oxford, UK. nicky.manning@orh.nhs.uk
Nicky
N
Manning
Lawrence
L
Impey
David
D
Lindsell
Kokila
K
Lakhoo
D000328:Adult; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging*; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology; D011247:Pregnancy; D011256:Pregnancy Outcome; D016216:Ultrasonography, Prenatal; D014471:Umbilical Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging* / Q000196:embryology