Presentation
Recurrent vomiting and raised serum bilirubin. Past history of splenectomy.
Patient Data
Atrophy of segments 2, 3, and 4 of the liver with hypertrophy of the caudate lobe of the liver with a heterogeneous enhancement of liver parenchyma on portal venous phase due to chronic main portal vein thrombosis.
Multiple dilated tortuous venous channels are seen replacing the fibrotic and chronically thrombosed main portal vein - suggestive of cavernous transformation of the portal vein.
These dilated tortuous venous channels are seen surrounding the gallbladder, cystic duct, common hepatic and common bile duct, causing narrowing of the distal common bile duct and resultant dilatation of common bile duct and dilatation of intrahepatic biliary radicals (segments 6, 7, and 8).
There is also dilatation of duodenum and proximal jejunal loops secondary to a long segment jejunal stricture showing wall thickening and peripheral fat stranding.
Post splenectomy changes are seen.
Case Discussion
Portal biliopathy refers to biliary obstruction associated with cavernous transformation of portal vein secondary to portal vein thrombosis.
When the portal vein thrombosis fails to recanalize, multiple venous collaterals develop to bypass the obstructed portal vein resulting in portal cavernoma formation. These peri-biliary collateral vessels can cause extrinsic compression over the intra-hepatic or extra-hepatic bile ducts, resulting in obstructive jaundice.
Complications may include cholangitis or hemobilia.
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