Enlarged periportal network of veins, initially millimetric in caliber, within which hepatopetal portal blood circulates. It results from a chronic thrombotic occlusion of the extrahepatic portal system.

The cavernoma is generally caused by a combination of a local cause (umbilical vein catheterism in the neonatal period, omphalitis, abdominal trauma) and a prothrombotic disease (myeloproliferative syndrome, G20210A mutation of the thrombin gene, antiphospholipid syndrome, antithrombin 3, protein C or protein S deficiency, factor V Leiden mutation). The biliary consequences of cavernoma are linked to the compression of the main bile duct by varicose veins, and are usually asymptomatic.

Clinical signs are related to the extrahepatic portal hypertension: hematemesis due to rupture of esogastric varices, splenomegaly with hypersplenism, rectorrhagia due to rupture of anorectal varices, growth retardation in children, etc.

Anesthetic implications: 

see isolated portal hypertension; hemorrhagic surgery

References:

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Updated: April 2024