Review ArticleNatural History of Portal Cavernoma Cholangiopathy
Section snippets
Frequency and Clinical Presentation of Portal Cavernoma Cholangiopathy
Portal cavernoma cholangiopathy (PCC) has been reported mainly among patients with EHPVO. The frequency of PCC in patients with EHPVO is 81–100%.4, 5, 6, 7, 8, 9, 10, 11, 12 Although studies in the past have described PCC in patients with portal hypertension due to cirrhosis of liver (0–33%)8, 9, 10, 11, 12, 13, 14, 15 and idiopathic portal hypertension or non-cirrhotic portal fibrosis (9–40%)8, 15 this is misleading as the reported findings consisted of irregularities in bile ducts, mainly
Natural History Portal Cavernoma Cholangiopathy
The natural history of PCC is not known. The majority of patients (70%–95%) do not manifest with any symptoms of biliary obstruction. However, patients with symptomatic PCC are normally older than patients presenting with EHPVO by median of 8–14 years, which is suggestive of long-term obstruction12, 13, 15, 18 and indicates that PCC is a progressive condition that develops late in the course of portal hypertension.
References (28)
- et al.
Cholangiopathy associated with portal hypertension: diagnostic evaluation and clinical implications
Gastrointest Endosc
(1999) - et al.
Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study
Hepatology
(2003) - et al.
Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein
Gastrointest Endosc
(2003) - et al.
Magnetic resonance cholangiography features of biliary abnormalities due to cavernous transformation of the portal vein
Clin Imaging
(2006) - et al.
Extrahepatic portal venous obstruction
Gut
(1988) - et al.
Management of extra hepatic portal venous obstruction (EHPVO): current strategies
Trop Gastroenterol
(2011) Portal ductopathy: clinical importance and nomenclature
World J Gastroenterol
(2011 Mar 21)- et al.
Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature
J Gastroenterol Hepatol
(2002) - et al.
Bile duct varices or “pseudo-cholangiocarcinoma sign” in portal hypertension due to cavernous transformation of the portal vein
Am J Gastroenterol
(1992) - et al.
Pseudosclerosing cholangitis in extrahepatic portal venous obstruction
Gut
(1992)
Poratalbiliopathy in extrahepatic portal venous obstruction
Indian J Gastroenterol
The ‘‘pseudo-cholangiocarcinoma sign’’ in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels
Am J Gastroenterol
Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances
Acta Radiol
Portal hypertensive biliopathy (PHB) in patients with extrahepatic portal venous obstruction (EHPVO)
J Gastroenterol Hepatol
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