Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.

Hepatogastric fistula is very rare. We report a case of hepatogastric fistula as a complication of pyogenic liver abscess. A 40-year-old man presented with upper abdominal pain and high-grade fever of 2 weeks. Evaluation revealed multiple liver abscesses. On an empirical diagnosis of pyogenic liver abscess, he was treated with antibiotics. During hospital stay he developed intermittent large quantity bilious vomiting. Gastroduodenoscopy and contrast-enhanced CT of the abdomen showed rupture of left lobe liver abscess into the stomach. As expectant management failed to resolve the abscess, endoscopic retrograde papillotomy and stenting of common bile duct was performed. After endoscopic stenting, symptoms subsided. Imaging repeated after 2 weeks of endoscopic stenting showed resolving abscess. He was discharged and is doing well on regular follow-ups. We conclude that hepatogastric fistula can be managed by endoscopic stenting as bile flow through the stent hastens resolution and healing of the fistula.

The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5-38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure.