title: Long-term results with the Kasai operation for biliary atresia.
abstract: OBJECTIVE
  To evaluate long-term outcome in a series of children with biliary results atresia treated by portoenterostomy.
  
  
  METHODS
  Case series of consecutive infants with biliary atresia with 10-year follow-up. Data were obtained by retrospective chart review or phone interview.
  
  
  METHODS
  A tertiary academic medical center and regional children's hospital.
  
  
  METHODS
  A consecutive series of 104 infants diagnosed with biliary atresia more than 10 years ago were evaluated. Eighty-nine had totally obliterated extrahepatic ducts, 4 had proximal hilar cysts (correctable type), and 11 had patency of the gallbladder and distal common duct.
  
  
  METHODS
  Ninety-eight patients underwent biliary reconstruction and 6 had exploration only. Seventy-four infants underwent reconstruction using a Rouxen-Y with exteriorization. The 11 infants with distal patency underwent a portocholecystostomy ("gallbladder Kasai"). The remainder had various modifications of the Kasai operation.
  
  
  METHODS
  Survival, liver function, complications, growth, and development.
  
  
  RESULTS
  The 6 patients who did not have a portoenterostomy died. Of the 98 who had a reconstruction, 63 died (mean age at death, 27 months; median, 13.4 months), 10 following liver transplantation. Twelve of the 35 survivors ultimately required liver transplants. Twenty-three children are alive more than 10 years after portoenterostomy without the need for transplantation. Two thirds have experienced some manifestation of portal hypertension (ie, variceal bleeding, hypersplenism, ascites). Nineteen patients (79%) are anicteric with normal liver synthetic function and are in an age-appropriate school grade or working and living independently.
  
  
  CONCLUSIONS
  We found that surgical correction of biliary atresia offers long-term survival for about one quarter of patients, provides palliation until liver transplantation becomes necessary, and if surgical correction is not feasible, biliary atresia is uniformly fatal. The outlook is good for those children who survived more than 10 years and justifies continued attempts to establish bile flow in infants with biliary atresia.
authors.affiliation: Department of Surgery, University of Colorado School of Medicine, Denver, USA.
authors.forename: F M
authors.initials: FM
authors.lastname: Karrer
authors.forename: M R
authors.initials: MR
authors.lastname: Price
authors.forename: D D
authors.initials: DD
authors.lastname: Bensard
authors.forename: R J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: M R
authors.initials: MR
authors.lastname: Narkewicz
authors.forename: D J
authors.initials: DJ
authors.lastname: Smith
authors.forename: J R
authors.initials: JR
authors.lastname: Lilly
mesh_terms: D000293:Adolescent; D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome