Laparoscopic cholecystectomy (LC) in patients with extrahepatic portal vein obstruction causing portal cavernoma (PC) is considered high risk because of portosystemic collateral veins in the hepatocystic triangle. The literature is limited to isolated case reports. We describe our experience of LC in patients with PC.

Data of patients with PC who underwent LC for symptomatic gallstones or related complications was reviewed. Patients with simultaneous cholecystectomy with splenorenal shunt and open cholecystectomy were excluded. Pre-operative evaluation consisted of complete blood count, international normalisation ratio and liver function tests, ultrasound of the abdomen with Doppler, contrast-enhanced computerised tomography, magnetic resonance cholangiopancreatography and esophagogastroscopy as indicated. A standard four-port LC was performed. The technical principles followed were to avoid injury to the collateral veins, liberal use of energy sources and division of dominant collateral veins between clips.

Seven adult patients including three females underwent LC. Three patients had thrombosis of previous surgical shunt with persistent PC. The remaining four patients did not have any indication for shunt surgery. Successful LC was performed in six patients. The median duration of surgery was 170 (130-250 min). Blood transfusion was not required. All the patients had uneventful post-operative recovery. The histopathology of gall bladder consists of acute cholecystitis in three patients and chronic cholecystitis in four.

LC is feasible in patients with PC at a centre with experience in both laparoscopic and portal hypertension surgeries. Excellent outcome with low rate of conversion to open surgery can be achieved.

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.

Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures (hepaticojejunostomy or choledechoduodenostomy).