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Slovenska pediatrija 2021; 28: 10-15

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Review article


Paola Trupac
Zdravstveni dom Koper, Koper, Slovenija

Petja Fister
Klinični oddelek za neonatologijo, Pediatrična klinika, Univerzitetni klinični center in Katedra za pediatrijo, Medicinska fakulteta Ljubljana, Ljubljana, Slovenija


Congenital portosystemic venous shunts (CPSS) are a rare group of heterogeneous developmental malformations of the portal and systemic circulation causing blood from the portal system to bypass liver metabolism through various vascular junctions and flow directly into the systemic circulation. They can be divided into two groups comprising intrahepatic shunts, with persistent ductus venosus, and extrahepatic shunts. The anomalous flow of splanchnic blood with undegraded metabolites directly into the systemic circulation may lead to severe complications: neonatal cholestasis, benign and malignant liver tumours, hepato-pulmonary syndrome, porto-pulmonary hypertension and encephalopathy. Small intrahepatic shunts may regress spontaneously in the first two years of life. Other permanent intrahepatic and extrahepatic shunts should be closed in one or two steps by minimally invasive interventional radiological closure methods or by surgical procedure. Extrahepatic type 1 shunts always require liver transplantation.

Key words: portosystemic shunts, intrahepatic shunt, extra-hepatic shunt, hepato-pulmonary syndrome, neonate