Prenatally diagnosed portocaval shunt and postnatal outcome: a case report

Abstract

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.

Prenatal diagnosis of congenital agenesis of the fetal portal venous system

“…However, most describe neonates or adults and were published in pediatric, radiology or pathology journals12. A detailed search of the literature yielded only four cases with a prenatal diagnosis of CAPVS13–16.…”

Clinical classification of congenital extrahepatic portosystemic shunts

“…WE SEARCHED FOR cases of CEPS in English and Japanese medical literature published between 1793 and 2009 using the key words “shunt”, “portosystemic shunt”, “congenital portosystemic shunt”, “splenorenal shunt”, “portocaval shunt”, and “congenital absence of portal vein” using Medline for international literature and Ichushi for Japanese literature 1–38 . We excluded case reports on portosystemic shunts with portal trunk (ductus venosus failed to close), secondary portal vein obstruction with liver disease (liver cirrhosis, hepatocellular carcinoma, et cetera), extra portal vein obstruction (EHPVO), and other obvious causes (trauma, infection, hypercoagulable and prothrombotic state) after birth and in those cases where the patient's profile was not described in detail.…”

“…The number of reported cases has gradually been increasing as a result of technological advances in radiological modalities, fetal diagnosis, and neonatal mass screening. To our knowledge, only 136 cases have been reported in English and Japanese medical literature 1–38 . CEPS can be anatomically classified into two groups 1,5,9 .…”

Malformations cardiaques

“…En particulier, une encéphalopathie serait très rare [421]. En fait, il semble que le pronostic soit très variable et qu'il est bon dans la majorité des cas.…”