title: Biliary atresia with choledochal cyst: implications for classification.
abstract: OBJECTIVE
  To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature.
  
  
  METHODS
  Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations."
  
  
  RESULTS
  A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8).
  
  
  CONCLUSIONS
  BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required.
authors.affiliation: Division of Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Aleixo M
authors.initials: AM
authors.lastname: Muise
authors.forename: Dan
authors.initials: D
authors.lastname: Turner
authors.forename: Eytan
authors.initials: E
authors.lastname: Wine
authors.forename: Peter
authors.initials: P
authors.lastname: Kim
authors.forename: Margaret
authors.initials: M
authors.lastname: Marcon
authors.forename: Simon C
authors.initials: SC
authors.lastname: Ling
mesh_terms: D001656:Biliary Atresia / Q000145:classification* / Q000453:epidemiology* / Q000601:surgery; D015529:Choledochal Cyst / Q000453:epidemiology*; D015897:Comorbidity; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation