Hepatic barrage to high-flow, intra-hepatic arteroportal fistulas requiring combined interventional approach

Transjugular intra-hepatic porto-systemic shunt (TIPS) is a proven strategy for the management of portal hypertension (PH) complications. Here, we report on a complex case of haemorrhagic shock due to the rupture of gastro-oesophageal varices in the context of PH originally sustained by idiopathic, likely congenital, high-flow intrahepatic arterioportal fistulas (IAPFs) preceded by extensive bowel ischaemia. While the occlusion of the IAPFs potentially controlled the steal of arterial splanchnic blood into the portal circulation, it failed to manage PH related bleeding, necessitating the placement of a salvage TIPS. Porta-caval pressure gradient persisted markedly increased after IAPFs occlusion, indicating an independent intra-hepatic component causing PH. Moreover, hepatic histology demonstrated a pre-sinusoidal/sinusoidal barrage response of the hepatic parenchyma secondary to long-standing IAPFs, causing the onset of an intra-hepatic component of PH. For these reasons, the combined interventional approach led to resolution of the refractory portal hypertensive bleeding, avoiding fatal evolution of diffuse bowel infarction.