title: Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver.
abstract: Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery.
authors.affiliation: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Radha K
authors.initials: RK
authors.lastname: Dhiman
authors.affiliation: Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Vivek A
authors.initials: VA
authors.lastname: Saraswat
authors.affiliation: Service d'Hépatologie, Hôpital Beaujon, Clichy, France.
authors.forename: Dominique C
authors.initials: DC
authors.lastname: Valla
authors.affiliation: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Yogesh
authors.initials: Y
authors.lastname: Chawla
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 Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India.
authors.forename: Vibha
authors.initials: V
authors.lastname: Varma
authors.affiliation: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Swastik
authors.initials: S
authors.lastname: Agarwal
authors.affiliation: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Ajay
authors.initials: A
authors.lastname: Duseja
authors.affiliation: Department of Gastroenterology, Army Hospital (R&R), New Delhi, India.
authors.forename: Pankaj
authors.initials: P
authors.lastname: Puri
authors.affiliation: Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
authors.forename: Naveen
authors.initials: N
authors.lastname: Kalra
authors.affiliation: Muzaffarnagar Medical College, Muzaffarnagar, India.
authors.forename: Chittapuram S
authors.initials: CS
authors.lastname: Rameshbabu
authors.affiliation: Institute of Liver and Biliary Sciences, New Delhi, India.
authors.forename: Vikram
authors.initials: V
authors.lastname: Bhatia
authors.affiliation: Department of Gastroenterology, Jaswant Rai Speciality Hospital, Meerut, India.
authors.forename: Malay
authors.initials: M
authors.lastname: Sharma
authors.affiliation: Institute of Liver and Biliary Sciences, New Delhi, India.
authors.forename: Manoj
authors.initials: M
authors.lastname: Kumar
authors.affiliation: Department of Surgical Gastroenterology & Liver Transplantation, Indraprastha Apollo Hospital, New Delhi, India ; Department of Gastroenterology and Hepatology, Indraprastha Apollo Hospital, New Delhi, India.
authors.forename: Subhash
authors.initials: S
authors.lastname: Gupta
authors.affiliation: Department of Surgical Gastroenterology & Liver Transplantation, Indraprastha Apollo Hospital, New Delhi, India ; Department of Gastroenterology and Hepatology, Indraprastha Apollo Hospital, New Delhi, India.
authors.forename: Sunil
authors.initials: S
authors.lastname: Taneja
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 Gastroenterology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, India.
authors.forename: Showkat A
authors.initials: SA
authors.lastname: Zargar
authors.affiliation: Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India.
authors.forename: Samiran
authors.initials: S
authors.lastname: Nundy
authors.affiliation: Department of Gastroenterology, S.C.B. Medical College, Cuttack, India.
authors.forename: Shivaram P
authors.initials: SP
authors.lastname: Singh
authors.affiliation: Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Subrat K
authors.initials: SK
authors.lastname: Acharya
authors.affiliation: Department of Gastroenterology, Inver Clyde Royal Hospital, Greenock, UK.
authors.forename: Jang B
authors.initials: JB
authors.lastname: Dilawari
keywords: CBD, common bile duct; CHD, common hepatic duct; CT, computed tomography; EHPVO, extrahepatic portal venous obstruction; ERC, endoscopic retrograde cholangiography; EUS, endoscopic ultrasound; GRADE, Grading of Recommendations, Assessment, Development and Evaluation; INASL, Indian National Association for Study of the Liver; MRC, magnetic resonance cholangiography; MRI, magnetic resonance imaging; NCPF, non-cirrhotic portal fibrosis; PSS, portosystemic shunt; PVT, portal vein thrombosis; UDCA, ursodeoxycholic acid; USG, ultrasound; cholestasis; extrahepatic portal venous obstruction; gallbladder varices; obstructive jaundice; portal hypertensive biliopathy