Introduction:  

There has not been a formal study to determine the incidence of biliary atresia(BA) in Costa Rica. Only two studies mention rates of 1.5 (1983) and 0.38(1988) per 10.000 live births. Statistics are necessary to make policy/protocols since they will go through a long chronic disease or need a liver transplant(LT).  

Method:  

Medical records of patients with BA diagnosis 2010-2021 were obtained from the office of health records and statistics and revised for information. Number of live births per year and dates of death were obtained from government open data bases.   

Results:  

Between 1 and 6 patients per year were diagnosed with BA for a total of 41 and an average of 3.4. Average incidence is 5.03 in 100,000 or 1 in 19,899 live births with 1,4:1 female:male ratio.  

38(92%) patients had Kasai surgery, those who didn’t all died, 1 after LT. Twenty-two(53%) patients are alive, 18(58%) with native liver and 4 after LT. 9 patients received LT, 5 are dead, 4 are alive.  

Conclusion:  

The incidence of BA in Costa Rica is low, like that described in Canada and Europe. The female predominance and the number of patients alive with native liver in our population is like that described in most studies.  

Further study is required to determine if average age of Kasai is related to the early death of almost half of our patients, which could lead to policy and protocol changes in our health system.