Biliary atresia and the Kasai portoenterostomy: Never say never?

Background/Purpose. Kasai portoenterostomy is recommended as the primary in itial therapy for extrahepatic biliary atresia if the procedure can be perf ormed within 10 to 12 weeks of life. The optimal management for infants wit h delayed presentation of biliary atresia remains controversial. The purpos e of this study was to determine the success rate and outcome for patients who underwent a "late" Kasai portoenterostomy. Methods. The authors conduct ed a retrospective review of the medical records of all patients with bilia ry atresia who underwent a Kasai portoenterostomy at their institution from 1986 to 1999 (n = 31). The authors analyzed success rates compared with ag e at the time of the Kasai procedure and the association with patient demog raphics. Surgical success was defined as achievement of a total serum bilir ubin less than or equal to 2 mg/dL. Long-term follow-up assessments include d the need for liver transplantation and patient survival rate. Results: Th e demographics of this study cohort showed a predominance of African-Americ ans. 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of success compa red with subject age at the time of the initial portoenterostomy showed tha t 52% had successful Kasai procedure at 0 to 75 days, compared with 83% suc cess rate at age 76 days or older (P = .359), Liver transplantation was per formed in 16 of 31 patients (45%). Overall survival rate for the entire coh ort 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 contrain dication to performing a Kasai portoenterostomy for biliary atresia in chil dren over 75 days of age.