and Incomplete Septal Cirrhosis
Non-cirrhotic portal hypertension & incomplete septal cirrhosis
Non-cirrhotic portal hypertension & incomplete septal cirrhosis
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“Biliary Features” in NCPH (Portal Hypertensive Biliopathy)<br />
(Dhiman. Gut 2007; 56: 1001-1008)<br />
• Mainly seen as complication of extrahepatic portal vein obstruction<br />
• Can also occur in intrahepatic NCPH, usually to a lesser degree<br />
Extrahepatic Portal Vein<br />
Obstruction<br />
Frequency 94%<br />
(81- 100%)<br />
“Idiopathic” NCPH<br />
9 - 40%<br />
Clinical<br />
manifestations<br />
Pathogenesis<br />
Histological<br />
Features<br />
19% symptomatic<br />
(5-38%)<br />
Cavernomatous transformation of<br />
portal vein & formation of<br />
choledochal varices<br />
→ Bile duct compression<br />
Resemble sclerosing cholangitis<br />
Rarely symptomatic<br />
Cholestatic LFTs common<br />
Mechanism uncertain<br />
? Vascular abnormalities in small<br />
portal tracts may compromise blood<br />
supply to bile ducts via the<br />
peribiliary vascular plexus<br />
Resemble sclerosing cholangitis
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