Long-term Outcome and Prognostic Factors after Kasai Operation for Biliary Atresia

ABSTRACT

BACKGROUND: Kasai portoenterostomy has been the standard operative procedure in cases of biliary atresia (BA) since Kasai reported the first successful operative case in 1959. However, BA is still one of the pediatric surgical lesions that does not always show satisfactory operative results. METHODS: To evaluate the long-term results and the prognostic factors influencing the survival with the Kasai portoenterostomy for BA, we reviewed 80 BA patients treated at Department of Pediatric Surgery, Seoul National University Hospital from 1980 to 1990. Kaplan-Meier method and Log-rank test were used for statistical analysis. RESULTS: Overall 5-year and 10-year survival rates were 42% and 37%, respectively. Thirty-one patients underwent surgery at ages under 60 days, 31 at ages between 60 and 90 days, 11 at ages between 90 and 120 days and 7 at ages beyond 120 days. There were no statistically significant differences in survival rates among the age groups. In the liver biopsy, minimal hepatic fibrosis was noted in 2 patients, moderate fibrosis in 13, severe fibrosis in 25 and cirrhosis in 5. There were no statistically significant differences in the survival rates according to the degree of hepatic fibrosis but there was a statistically significant difference between patients with cirrhosis and without cirrhosis (p= .03). The size of the bile ductules at the porta hepatis was less than 50 micrometers in 18 patients, between 50 and 100 micrometers in 19 patients, between 100 and 200 micrometers in 4 patients and greater than 200 micrometers in 3 patients. There were no statistically significant differences in the survival rates according to the size of the bile ductules at the porta hepatis. Out of the 12 long-term survivors, survival of more than 10 years, 5 patients had abnormal liver function and 7 patients showed clinical manifestations of portal hypertension. CONCLUSIONS: Kasai portoenterostomy offers reasonable long-term survival and should be the initial procedure for biliary atresia, but a significant portion of the long-term survivors has abnormal liver functionand portal hypertension. Continuous and careful follow-up is needed not to miss the proper time for liver transplantation. Liver cirrhosis at the time of the operation was the only statistically significant prognostic factor influencing the survival.