Extra-hepatic portal vein obstruction leading to portal cavernoma cholangiopathy without liver dysfunction
Keywords:Extra-hepatic portal vein obstruction, Portal cholangiopathy, Portal cavernoma, Portal vein thrombosis
In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. EHPVO leads to the cavernous transformation of the portal vein in the absence of recanalization. Portal cholangiopathy (also referred to as portal biliopathy) is common in patients with long-standing chronic PVT. It is due to compression of the large bile ducts by the venous collaterals (portal cavernoma) that form in patients with chronic PVT. Typically, symptoms of portal cholangiopathy include jaundice, biliary colic and pruritus.
Wani ZA, Bhat RA, Bhadoria AS, Maiwall R. Extrahepatic portal vein obstruction and portal vein thrombosis in special situations: Need for a new classification. Saudi J Gastroenterol. 2015;21(3):129-38.
Samant H, Asafo-Agyei KO, Garfield K. Portal vein thrombosis. StatPearls. 2021.
Chawla Y, Dilawari JB, Katariya S. Gallbladder varices in portal vein thrombosis. AJR Am J Roentgenol. 1994;162(3):643-5.
Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, Boda MI. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology. 1993;17(5):807-13.
Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut. 2007;56(7):1001-8.
Khanna R, Sarin SK. Non-cirrhotic portal hypertension–Diagnosis and management. J Hepatol. 2014;60:421-41.