ABSTRACT
Context: Biliary atresia (BA) is a destructive process affecting both extraand 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. Aims: 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. Settings and Design: Prospective observational study. Materials and 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. Statistical Analysis Used: 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. Conclusion: 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.
ABSTRACT
Context: The indicators of poor prognosis in cases of extrahepatic biliary atresia (EHBA) continue to remain controversial. Aims: To correlate the histopathological findings of wedge biopsy from liver and tissue obtained from the shaving at the porta hepatis, during hepatic portoenterostomy, with the clinical outcome. Materials and Methods: All cases of EHBA surgically treated in our hospital from 1995 to 2006 have been reviewed. Wedge biopsies of the liver and biopsies from the porta hepatis were analyzed with hemotoxylin-eosin stains and immunohistochemistry. The parameters correlated with clinical outcomes were - presence of large bile ducts ( > 150μm diameter) in the portal tissue plaque, degree of fibrosis (semi-quantitative; graded as mild, moderate and severe), presence of ductal plate malformation (DPM) and age at operation. Results: The proportions of patients with small or large ductal diameter who remained clinically controlled (serum bilirubin < 1.5mg/dl with no evidence of end stage liver failure) were 39% and 66.6% respectively (P=0.44). There was a highly significant correlation between the extent of fibrosis and clinical outcome. Mild, moderate and severe fibrosis resulted in clinical control rates of 78.5%, 34.4% and 24% respectively (P=0.001). Ductal plate malformation was seen in 15% of our cases and was uniformly associated with poor outcome. A non-significant trend towards poorer outcome was seen with increasing age at surgery. Conclusions: Histopathological correl ations with clinical outcome in EHBA have been rarely reported from the Indian subcontinent. A greater degree of fibrosis at the time of hepatic portoenterostomy and presence of ductal plate malformation is associated with a significantly poorer clinical outcome.