Clinical characteristics and outcomes of biliary atresia in the eastern province of Saudi Arabia: 26-years single-center experience

Objective: This study aimed to evaluate the clinical characteristics and treatment outcomes of biliary atresia (BA) in the Eastern Province of Saudi Arabia. Methods: A retrospective analysis was performed on data collected from August 2023 to January 2024 for patients who received an intraoperative BA diagnosis in the last 26 years. Perioperative data, clinical presentation, investigations, Kasai portoenterostomy (KPE) course, complications, and outcomes were collected. Primary outcomes included BA clinical features, diagnosis, and KPE timing impact on KPE success, postoperative steroid use, and native liver survival (NLS). Results: Eight children (three females, five males) were included. Seven were Saudi. All exhibited jaundice in the initial presentation. Three patients had G6PD deficiency, one had sickle cell trait, one had alpha thalassemia, one had hemophilia B, and one had microcytic hemolytic crisis. The average age at presentation was 36 ± 16.5 days. Six patients underwent KPE and three were eligible for liver transplant. The average age at the time of the KPE was 50.6 ± 8.18 days, and all received steroids afterward. None of the patients cleared their jaundice at 6 months. Two patients remain alive, the rest were lost to follow-up. Conclusions: KPE before 60 days and post-operative steroids did not influence KPE success, highlighting the potential need for earlier detection and intervention. This research revealed a high prevalence of hematological diseases in this population.