Zdravstveni dom Koper, Koper, Slovenija
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