Review Article
Natural History of Portal Cavernoma Cholangiopathy

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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.

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.

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      However, in this study follow-up period was relatively short as the natural history of PCC may extend over 2 to 3 decades. Also, the severity of lesions even in grade III were much milder than that reported in Indian series which had evaluated patients after much longer duration since diagnosis of EHPVO.61 Patients with symptomatic PCC are normally older than patients presenting with EHPVO by median of 8–14 years, which suggests that PCC is a progressive condition and that long-term obstruction24,47,51,62 and prolonged portal hypertension are needed to cause changes severe enough to produce symptoms.

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