Technical overview of pediatric liver transplantation for congenital absence of the portal vein: a collective review of six cases at a single center

Congenital absence of the portal vein (CAPV) is a rare vascular anomaly in which mesenteric venous blood bypasses the liver and drains directly into systemic circulation. Liver transplantation (LT) is considered for patients who develop severe complications or do not respond to medical therapy. Because splanchnic venous anatomy varies markedly among patients with CAPV, portal vein (PV) reconstruction must be customized to individual vascular patterns. We reviewed surgical approaches to PV reconstruction in six pediatric LT cases with CAPV reported from a single institution. Patients ranged in age from 3 years 5 months to 12 years at the time of LT. Portocaval shunts (PCS) were classified as extrahepatic in five cases and intrahepatic in one case. Portal hypertension was identified in three patients. Sources of portal inflow included the PCS vein, native extrahepatic PV trunk, enlarged PV collateral vein, pericholedochal collateral vein, and confluence of the mesenteric-splenic vein. All patients recovered without vascular complications and remained alive, with follow-up ranging from 3 to 11 years. In conclusion, CAPV is frequently associated with complex vascular anomalies. Careful preoperative and intraoperative assessment is essential, and PV reconstruction must be individually tailored with anatomically appropriate techniques to achieve successful outcomes.