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Title: Congenital veno-venous malformations of the liver: Widely variable clinical presentations
Authors: Witters, Peter ×
Maleux, Geert
George, Christophe
Delcroix, Marion
Hoffman, Ilse
Gewillig, Marc
Verslype, Chris
Monbaliu, Diethard
Aerts, Raymond
Pirenne, Jacques
Van Steenbergen, Werner
Nevens, Frederik
Fevery, Johan
Cassiman, David #
Issue Date: Aug-2008
Publisher: Blackwell publishing
Series Title: Journal of gastroenterology and hepatology vol:23 issue:8 pages:E390-E394
Abstract: Background and Aim: Congenital portosystemic veno-venous malformations are rare abnomalities that often remain undiagnosed. Typically they are classified by their anatomical characteristics according to Morgan (extrahepatic, Abernethy malformations type Ia,b and II) and Park (intrahepatic, types 1-4). However, their clinical presentation is less dependent on the anatomical type. Method: We reviewed the clinical characteristics of six cases drawn from our files (from 1970 to 2006). Results: One patient, a 25-year-old male, had extrahepatic shunting whereby the liver receives only arterial blood because the portal vein (PV) connects with the inferior caval vein (ICV) (Abernethy Ib); he presented with episodes of jaundice and pruritus. Three patients had extrahepatic shunting with patent intrahepatic portal veins, but with shunting of splenomesenterial blood towards the ICV (Abernethy II); these included a 66-year-old male with hepatic encephalopathy, a 17-year-old female with (porto?-)pulmonary hypertension without portal hypertension, and a 33-year-old female with epidsodes of acute pain secondary to spontaneous bleeding within a primary liver tumor. Two patients had intrahepatic shunting; these included an 8-year-old boy who was diagnosed incidentally during work-up for abnormal liver enzymes with a communication between right PV and ICV (Park type 1), and a 59-year-old male with multiple PV-ICV-shunts in several liver segments (Park, type 4) who presented with hepatic encephalopathy. Conclusion: Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.
URI: 
ISSN: 0815-9319
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Abdominal Surgical Oncology
Pneumology
Radiology
Laboratory of Abdominal Transplantation
Hepatology
Cardiovascular Developmental Biology
Laboratory of Nephrology
× corresponding author
# (joint) last author

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