A rare combination of arterio-portal fistula and portosystemic shunt. Endovascular treatment

BACKGROUND: Portosystemic shunts (PSS) is a rare malformation in which there is an abnormal connection between the portal system and the systemic circulation. Arterio-portal fistulas (APF) are arterio-venous connections between the arteries of the ventral trunk system and the portal vein. Hepatopulmonary syndrome) is defined by the respiratory dysfunction due to the defect in arterial oxygenation induced by the dilatation of intrapulmonary vessels associated with liver disease. CLINICAL CASE DESCRIPTION: In our clinic, we treated endovascularly a 10.5-y.o. patient with a rare combination of congenital portosystemic shunt (CPSS) and congenital APF complicated by Hepatopulmonary syndrome. The treatment was performed in several stages. The first stage was APF occlusion. The second stage was a balloon occlusion test. The third stage was PSS occlusion. In the postoperative period, there were signs of portal hypertension (PH), that is why the anticoagulant therapy was administered which resulted in PH symptoms regression. CONCLUSION: In case of the combined arterio-portal fistula and congenital portosystemic shunt, it is necessary, first, to occlude APF, then to perform the balloon occlusion test, and then -if there are well developed intrahepatic branches of the portal vein- to perform PSS occlusion. In the postoperative period, anticoagulant therapy is recommended for preventive purposes.