Surgical Outcome in Biliary Atresia

In Brief Objective: The exact nature of the relationship between age at Kasai portoenterostomy (KP) for biliary atresia (BA) and outcome is controversial. Methods: Single-center retrospective analysis of consecutive cases (January 1994–December 2005) undergoing KP, using 2 dichotomous measures of outcome (clearance of jaundice to <20 μmol/L and native liver survival at 2 years post-KP). Outcome was analyzed by age cohort (<30 days, 30–40 days, etc) and cumulatively. Data are quoted as median (interquartile range). Statistical analysis was by extended Fisher r × c analysis. P < 0.05 was regarded as significant. Results: Two hundred twenty-five infants with BA were divided into 3 groups based on possible etiology: isolated BA (n = 177), biliary atresia splenic malformation (BASM) syndrome (n = 28), and cystic BA (n = 23). Three infants had both BASM and a cyst. Age at KP was significantly greater in those with isolated BA [58 (48–61)] compared with both BASM [47 (39–59); P = 0.02] and cystic BA [47 (39–54); P = 0.02]. Overall, 127 (56%) cleared their jaundice and 145 (65%) survived to 2 years with their native liver. Children with isolated BA showed no statistical difference by age cohort for clearance of jaundice (P = 0.75) or for native liver survival (P = 0.14). In contrast, there was a marked detrimental effect of age at KP for both BASM and cystic BA groups (P = 0.02). Conclusion: There is a marked detrimental effect of age at KP on groups with a presumed “developmental” origin, not seen in the majority with isolated BA. Age cohort analysis was used to determine the effect of age at Kasai portoenterostomy in 225 infants with biliary atresia. This showed that those groups with a presumed developmental origin (ie, biliary atresia splenic malformation syndrome and cystic biliary atresia) had a marked detrimental effect from increasing age, not seen in the majority with isolated biliary atresia.