Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2014 Feb;4(Suppl 1):S62-6.
doi: 10.1016/j.jceh.2013.08.003. Epub 2013 Aug 27.

Natural history of portal cavernoma cholangiopathy

Manoj Kumar  1 , Vivek A Saraswat  2
Affiliations
Review

Natural history of portal cavernoma cholangiopathy

Manoj Kumar et al. J Clin Exp Hepatol. 2014 Feb.

Abstract

The natural history of portal cavernoma cholangiopathy (PCC) is poorly defined and poorly understood. It develops early after acute portal vein thrombosis (PVT) if there is failure of recanalization. In PCC, the likelihood of progression of biliary abnormalities after 1 year is extremely low. The natural history of PCC is conveniently divided into asymptomatic and symptomatic stages. The majority of patients with PCC are asymptomatic and are detected incidentally on imaging. Limited data suggest that asymptomatic PCC is static or only slowly progressive in the initial stages. However, most workers agree that, overall, PCC is a slowly progressive disease. Symptomatic PCC represents a late stage in its natural history. Finding strictures with dilatation at cholangiography is associated with a higher risk of developing symptoms of PCC. Onset of symptoms is often precipitated by the development of biliary sludge or calculi and treating calculi usually relieves symptoms for prolonged periods of time. Clinical presentations include biliary pain, obstructive jaundice, acute cholangitis, acute cholecystitis, or other presentations of gallstone disease. Progressive liver dysfunction and secondary biliary cirrhosis can develop in a minority of patients.

Keywords: EHPVO, extra-hepatic portal venous obstruction; NCPF, non-cirrhotic portal fibrosis; NCPH, non-cirrhotic portal hypertension; PCC, cavernoma cholangiopathy; PH, portal hypertension; PVCT, portal vein cavernomatous transformation; PVT, portal vein thrombosis; extrahepatic portal venous obstruction; natural history; portal cavernoma cholangiopathy; prognosis.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Magnetic resonance cholangiography (MRC) in patients followed after a diagnosis of acute portal vein thrombosis (PVT) [modified from Ref 16].
Figure 2
Figure 2
Magnetic resonance cholangiography (MRC) in patients followed after a diagnosis of chronic portal vein thrombosis (PVT) [modified from Ref 16].
See this image and copyright information in PMC

References

    1. Dilawari J.B., Chawla Y.K. Extrahepatic portal venous obstruction. Gut. 1988;29:554–555. - PMC - PubMed
    1. Poddar U., Borkar V. Management of extra hepatic portal venous obstruction (EHPVO): current strategies. Trop Gastroenterol. 2011;32(2):94–102. - PubMed
    1. Bayraktar Y. Portal ductopathy: clinical importance and nomenclature. World J Gastroenterol. 2011 Mar 21;17(11):1410–1415. - PMC - PubMed
    1. Dhiman R.K., Chawla Y., Vasishta R.K. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol. 2002;17:6–16. - PubMed
    1. Bayraktar Y., Balkanci F., Kayhan B., Ozenç A., Arslan S., Telatar H. Bile duct varices or “pseudo-cholangiocarcinoma sign” in portal hypertension due to cavernous transformation of the portal vein. Am J Gastroenterol. 1992;87:1801–1806. - PubMed

LinkOut - more resources