title: A histopathological study of liver and biliary remnants in the long-term survivors (>10 years) of cases of biliary atresia.
abstract: BACKGROUND
  Biliary atresia (BA) is a destructive process affecting both extra- and intra-hepatic bile ducts leading to fibrosis and obliteration of the biliary tree and cirrhosis usually within 2 years. Factors influencing the outcome of portoenterostomy (PE) have not been clearly defined.
  
  
  OBJECTIVE
  Our aim was to identify children with no evidence of liver disease 10 years or more after PE and to compare the pathology of liver and biliary remnants in this group with those associated with poor outcome.
  
  
  METHODS
  Prospective observational study.
  
  
  METHODS
  Wedge biopsies of liver and portal remnants, taken at the time of PE, where available, were reviewed. The parameters studied were - presence of large bile ducts (>150 μ), degree of fibrosis and bile duct proliferation (BDP), presence of ductal plate malformation (DPM) and age at operation.
  
  
  METHODS
  Fisher's exact test with Freeman Halton extension for univariate analysis and Logistic regression analysis as multivariate analysis.
  
  
  RESULTS
  Of 68 cases operated between 1995 and 2001, 14 patients survived >10 years and 54 were associated with poor outcome. Large ducts were significantly more in survivors (70% vs. 26%, P = 0.02). DPM was not seen in any of the survivors and was present in 24% of poor outcome group. Fibrosis and BDP were also significantly less among the survivors (P < 0.001, P = 0.03, respectively). The mean ages at operation in the two groups were 66.8 and 89.6 days, respectively.
  
  
  CONCLUSIONS
  From this study, we feel that lower degree of fibrosis and BDP, absence of DPM, presence of large ducts and younger age at operation were associated with better long-term outcome. Of these, degree of fibrosis was the most significant factor.
authors.forename: Sabuj Ghana
authors.initials: SG
authors.lastname: Mukhopadhyay
authors.forename: Paromita
authors.initials: P
authors.lastname: Roy
authors.affiliation: Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
authors.forename: Uttara
authors.initials: U
authors.lastname: Chatterjee
authors.forename: Chhanda
authors.initials: C
authors.lastname: Datta
authors.forename: Mala
authors.initials: M
authors.lastname: Banerjee
authors.forename: Sugato
authors.initials: S
authors.lastname: Banerjee
authors.forename: Ashoke Kumar
authors.initials: AK
authors.lastname: Basu
authors.forename: Manojit
authors.initials: M
authors.lastname: Ganguli
mesh_terms: D001656:Biliary Atresia / Q000473:pathology* / Q000601:surgery*; D001659:Biliary Tract / Q000473:pathology*; D001706:Biopsy; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000473:pathology; D008297:Male; D011171:Portoenterostomy, Hepatic; D017741:Survivors; D016896:Treatment Outcome