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. ( J CLIN EXP HEPATOL 2014;4:S2-S14)

Spectrum of biliary abnormalities in portal cavernoma cholangiopathy (PCC) secondary to idiopathic extrahepatic portal vein obstruction (EHPVO)—a prospective magnetic resonance cholangiopancreaticography (MRCP) based study

“…1 It is defined as "abnormalities in the extrahepatic biliary system including the cystic duct and gall bladder with or without abnormalities in the first and second generation biliary ducts in a patient with portal cavernoma". 1 The presence of a portal cavernoma, typical cholangiographic changes on endoscopic or MR cholangiography and the absence of other causes of biliary changes such as bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc. are mandatory to arrive at a diagnosis.…”

“…are mandatory to arrive at a diagnosis. 1 Frequency of PCC has been reported to be much greater in patients with idiopathic extrahepatic portal vein obstruction (EHPVO) (80-100%) as compared with cirrhotic liver disease (0-33%) and non-cirrhotic portal fibrosis (9-40%). [2][3][4] In addition, pancreatic duct abnormalities were observed in a large proportion of patients with portal vein cavernous transformation.…”

Imaging and radiological interventions in extra-hepatic portal vein obstruction

“…The cavernous transformation of portal vein and resultant extra and intrahepatic biliary ductal and gall bladder complications has been termed portal cavernoma cholangiopathy (PCC) also called portal biliopathy. The development of PCC has been attributed to two main causes: (1) Compression of the pliable common bile duct (CBD) by dilated collaterals and by new vessels formed due to long standing portal thrombosis (neovascularization); and (2) Bile duct ischaemia either due to prolonged compression by collaterals, thrombosis of smaller veins draining the duct or excessive deposition of connective tissue forming a "tumor-like" cavernoma [4] . In a study by Dhiman et al [5] , 3 out of 5 patients of EHPVO showed partial resolution of biliary changes, 1 patient showed complete resolution whereas 1 patient had no reversal after PSS surgery, thus suggesting than both mechanical compression by collaterals and bile duct ischaemia can lead to cholangiopathy and both these causes are not mutually exclusive.…”

“…Symptoms of PCC are mainly due to chronic cholestasis and biliary stones and can manifest as jaundice, biliary colic or cholangitis. Such symptoms usually manifest 8-10 years after diagnosis of EHPVO, thus suggesting that PCC is a progressive condition developing after long standing obstruction [4,9] . Khuroo et al [10] reported that all patients with symptomatic portal biliopathy in their study presented almost a decade later than those patients presenting with variceal bleed.…”

Therapeutic Challenges for Symptomatic Portal Cavernoma Cholangiopathy

“…Its major consequences are represented by portal hypertension and anomalies within the biliary tree [1][2][3]. 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 first and second generation biliary ducts, in a patient with portal cavernoma in the absence of other causes [4,5]. Previously used names were portal hypertensive biliopathy, portal biliopathy or portal cholangiopathy [6].…”