title: Surgical management of portal cavernoma cholangiopathy.
abstract: 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.
authors.affiliation: Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India.
authors.forename: Vibha
authors.initials: V
authors.lastname: Varma
authors.affiliation: Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Arunanshu
authors.initials: A
authors.lastname: Behera
authors.affiliation: Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Leileshwar
authors.initials: L
authors.lastname: Kaman
authors.affiliation: Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India.
authors.forename: Somnath
authors.initials: S
authors.lastname: Chattopadhyay
authors.affiliation: Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India.
authors.forename: Samiran
authors.initials: S
authors.lastname: Nundy
keywords: CBD, common bile duct; EHBRD, extrahepatic biliary radical dilatation; EHPVO, extrahepatic portal venous obstruction; ERCP, endoscopic retrograde cholangiopancreatography; GB, gall bladder; HJ, hepaticojejunostomy; IHBRD, intrahepatic biliary radical dilatation; LFT, liver function tests; NCPF, non cirrhotic portal fibrosis; NPSS, non-portosystemic shunt; PB, portal biliopathy; PCC, portal cavernoma cholangiopathy; PSS, portosystemic shunt; PTBD, percutaneous transhepatic biliary drainage; UGI, upper gastrointestinal; biliary obstruction; extrahepatic portal venous obstruction; portal cavernoma cholangiopathy; portal hypertension; portosystemic shunt surgery