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Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma |
Ruchi Sharma, Abid Suddle, Alberto Quaglia, Praveen Peddu, John Karani, Thomas Satyadas and Nigel Heaton |
London, United Kingdom
Author Affiliations: Institute of Liver Studies (Sharma R, Suddle A, Quaglia A and Heaton N); Department of Radiology (Peddu P and Karani J), King's College Hospital, London SE5 9RS; Central Manchester University Hospital, Manchester Royal Infirmary, Manchester M13 9WL, United Kingdom (Satyadas T)
Corresponding Author: Ruchi Sharma, MBBS, MS (Gen Surg), MRCS, Department of Hepatopancreaticobiliary Surgery, Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, United Kingdom (Tel: +447702348683; Email: drruchisharma@ymail.com) |
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Abstract Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management. Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1 (complete) shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.
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