Incidence and outcome of biliary atresia in the United Arab Emirates: A multicenter national study

Abstract

Background:
While extensive research provides valuable insights into biliary atresia (BA) globally, there remain gaps in region-specific data, such as West Asia. We aim to evaluate the incidence and survival of patients with BA in the United Arab Emirates.


Methods:
We retrospectively reviewed the records of all patients diagnosed with BA in all paediatric tertiary hospitals from 2011 to 2021.


Results:
50 patients were identified, 11 were excluded due to insufficient information to confirm a diagnosis of BA. The final cohort was 39 patients. The overall incidence of biliary atresia was 3.75 per 100,000 live births. The incidence rates varied over the years, with the lowest recorded incidence in 2011 at 1.19 per 100,000 live births and the highest in 2017 at 7.16 per 100,000 live births. The median age at Kasai portoenterostomy procedure was 52 days (CI: 42–60, range: 6–142) The survival with native liver was 50% at 12 months, and 42.1% at 5 and 10 years post-operatively. The overall survival with native or transplant liver was 88.2% at 5 and 10 years.


Conclusion:
While the incidence of BA is variable worldwide, the incidence in the United Arab Emirates in amongst the lowest reported. The role of early diagnosis and specialized surgical intervention is of importance for patient survival with native liver. The overall patient survival is further enhanced by availability of well-structured liver transplantation programs.