The Kasai portoenterostomy: when is it too late?

BACKGROUND/PURPOSE Kasai portoenterostomy is recommended as the primary initial therapy for extrahepatic biliary atresia if the procedure can be performed within 10 to 12 weeks of life. The optimal management for infants with delayed presentation of biliary atresia remains controversial. The purpose of this study was to determine the success rate and outcome for patients who underwent a "late" Kasai portoenterostomy. METHODS The authors conducted a retrospective review of the medical records of all patients with biliary atresia who underwent a Kasai portoenterostomy at their institution from 1986 to 1999 (n = 31). The authors analyzed success rates compared with age at the time of the Kasai procedure and the association with patient demographics. Surgical success was defined as achievement of a total serum bilirubin < or = 2 mg/dL. Long-term follow-up assessments included the need for liver transplantation and patient survival rate. RESULTS The demographics of this study cohort showed a predominance of African-Americans, 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of success compared with subject age at the time of the initial portoenterostomy showed that 52% (13 of 25) had successful Kasai procedure at 0 to 75 days, compared with 83% success rate (5 of 6) at age 76 days or older (P = .359). Liver transplantation was performed in 16 of 31 patients (45%). Overall survival rate for the entire cohort is 23 of 31 (74%), whereas 12 of 31 (39%) are currently alive without a liver transplant. CONCLUSION These data suggest that there is no contraindication to performing a Kasai portoenterostomy for biliary atresia in children over 75 days of age.