title: The role of operative intervention in management of congenital extrahepatic portosystemic shunt.
abstract: OBJECTIVE
  Congenital extrahepatic portosystemic shunt (CEPS) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. It is still a matter of debate whether conservative or operative strategies should be used to treat symptomatic CEPS. The aim of this study was to evaluate the role of operative intervention in the management of CEPS.
  
  
  METHODS
  Between June 2004 and August 2010, 6 consecutive patients with symptomatic CEPS were treated in our department. There were 3 male and 3 female patients, with a median age of 3.5 years (range, 1-8). Their demographic, clinical, and laboratory data were analyzed. All patients were scheduled to undergo shunt ligation or liver transplantation (LT).
  
  
  RESULTS
  Living donor LT was carried out in 4 patients, and shunt ligation in 2. After a median follow-up of 25 months, all the patients are alive currently with marked relief of symptoms.
  
  
  CONCLUSIONS
  Shunt ligation or LT for symptomatic CEPS is potentially curative.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan. yuki371@jichi.ac.jp
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Sanada
authors.forename: Taizen
authors.initials: T
authors.lastname: Urahashi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ihara
authors.forename: Taiichi
authors.initials: T
authors.lastname: Wakiya
authors.forename: Noriki
authors.initials: N
authors.lastname: Okada
authors.forename: Naoya
authors.initials: N
authors.lastname: Yamada
authors.forename: Satoshi
authors.initials: S
authors.lastname: Egami
authors.forename: Shuji
authors.initials: S
authors.lastname: Hishikawa
authors.forename: Youichi
authors.initials: Y
authors.lastname: Kawano
authors.forename: Kentaro
authors.initials: K
authors.lastname: Ushijima
authors.forename: Shinya
authors.initials: S
authors.lastname: Otomo
authors.forename: Koichi
authors.initials: K
authors.lastname: Sakamoto
authors.forename: Manabu
authors.initials: M
authors.lastname: Nakata
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Yasuda
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuta
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008026:Ligation; D016031:Liver Transplantation; D008297:Male; D011168:Portal System / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D011169:Portal Vein / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D011172:Portography; D054079:Vascular Malformations / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods*