title: Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma.
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.
authors.affiliation: Department of Hepatopancreaticobiliary Surgery, Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK. drruchisharma@ymail.com.
authors.forename: Ruchi
authors.initials: R
authors.lastname: Sharma
authors.forename: Abid
authors.initials: A
authors.lastname: Suddle
authors.forename: Alberto
authors.initials: A
authors.lastname: Quaglia
authors.forename: Praveen
authors.initials: P
authors.lastname: Peddu
authors.forename: John
authors.initials: J
authors.lastname: Karani
authors.forename: Thomas
authors.initials: T
authors.lastname: Satyadas
authors.forename: Nigel
authors.initials: N
authors.lastname: Heaton
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D017115:Catheter Ablation; D004621:Embolization, Therapeutic; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009536:Niacinamide / Q000031:analogs & derivatives / Q000627:therapeutic use; D010671:Phenylurea Compounds / Q000627:therapeutic use; D011169:Portal Vein / Q000002:abnormalities*; D000077157:Sorafenib; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis*
chemical_list: D000970:Antineoplastic Agents; D010671:Phenylurea Compounds; D009536:Niacinamide; D000077157:Sorafenib