Outcome of early hepatic portoenterostomy for biliary atresia

Background: The outcome of the hepatic portoenterostomy (Kasai) procedure f or biliary atresia is improved when it is performed before 90 days of age. However, it is not known whether intervention before 30 days is better than intervention between 30 and 90 days. Methods: The authors reviewed the records of all patients seen by the Pedia tric Gastroenterology Service at St. Louis Children's Hospital from 1984-19 99 to ascertain the outcome of patients who underwent Kasai procedure befor e or after 30 days of age. Results: Of 92 patients with biliary atresia treated at St. Louis Children' s Hospital over 15 years, 9 underwent the Kasai procedure before 30 days of age. Liver transplantation was necessary in 77.8% of these patients at a m ean age of 11.0 +/- 4.26 months, as compared with 53.4% at 32.14 +/- 7.14 m onths for the remainder of the patients who underwent the procedure after 3 0 days of age. Conclusions: Although these data suggest that outcomes are worse for patien ts who undergo the procedure before 30 days of age, they may reflect a diff erence in the pathogenesis of biliary atresia that brings it to clinical at tention earlier and may provide further evidence that biliary atresia is a phenotype for a number of distinct underlying disease processes.