title: Congenital absence of the portal vein in liver transplantation for biliary atresia.
abstract: BACKGROUND
  Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Coexistent congenital anomalies are common (25%), but the combination of polysplenia syndrome (10%) and absence of portal vein is rare.
  
  
  OBJECTIVE
  We report a case of successful reduced size liver transplantation on a 13-month-old girl with extrahepatic biliary atresia, polysplenia syndrome and congenital absence of the portal vein.
  
  
  METHODS
  Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed.
  
  
  RESULTS
  The donor portal vein was successfully anastomosed to the confluence of the superior mesenteric vein and splenic vein. The child is well with good graft function at a follow-up of 20 months.
  
  
  CONCLUSIONS
  Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.
authors.affiliation: Liver Transplant Surgical Service, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
authors.forename: P
authors.initials: P
authors.lastname: Andreani
authors.forename: P
authors.initials: P
authors.lastname: Srinivasan
authors.forename: C S
authors.initials: CS
authors.lastname: Ball
authors.forename: N D
authors.initials: ND
authors.lastname: Heaton
authors.forename: M
authors.initials: M
authors.lastname: Rela
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D011169:Portal Vein / Q000002:abnormalities*; D013154:Spleen / Q000002:abnormalities