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Outcome of biliary atresia among Saudi children: A tertiary care center experience
Sinan Holdar1, Badr Alsaleem1, Ali Asery1
1Division of Pediatric Gastroenterology, Department of Pediatric Sub-Specialities, The Children's Specialized Hospital, King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia.
Insights
Biliary atresia (BA) is uncommon in Saudi Arabia, with late diagnosis and poor outcomes without liver transplantation (LT). Early referral and intervention are crucial for improving survival rates in pediatric BA cases.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Epidemiology
Background:
- Biliary atresia (BA) is a leading cause of pediatric end-stage liver disease and liver transplantation (LT) in Western countries.
- Limited data exists on the epidemiology and outcomes of BA in Saudi Arabia.
- This study aimed to characterize BA in the Saudi pediatric population.
Purpose of the Study:
- To determine the clinical and epidemiological characteristics of biliary atresia (BA) in Saudi children.
- To evaluate the outcomes of BA management, including Kasai portoenterostomy (KPE) and liver transplantation (LT).
Main Methods:
- Retrospective review of infantile cholestasis cases from 2008 to 2015.
- Data collection included clinical, biochemical, imaging, and histopathological characteristics.
- Primary outcomes were successful KPE (jaundice resolution) and native liver survival.
Main Results:
- 21 cases of BA diagnosed (4.7% of infantile cholestasis).
- Median age at presentation to specialists was 65 days; KPE performed at 73 days.
- Successful KPE in 33% (4/12); 4-year native liver survival was 14.3%; overall survival was 81%.
Conclusions:
- Biliary atresia (BA) is an uncommon cause of infantile cholestasis in Saudi Arabia.
- Late referral to pediatric gastroenterologists and poor outcomes without LT characterize BA in this population.
- Further research is needed to improve early diagnosis and management strategies.
Background/Aims:
Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population.
Patients And Methods:
We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution of jaundice (total serum bilirubin <20 μmol/L) and (2) survival with native liver.
Results:
Over the study period, we evaluated 450 cases of infantile cholestasis. In all, 21 cases (11 males) were diagnosed with BA (4.7%). BA cases were first seen by pediatric gastroenterologists at a median age of 65 days. KPE was performed in 12 cases at a median age of 73 days. Successful KPE was achieved in four cases (33%). Five of the remaining eight cases had LT and three died before LT. Nine of the 21 BA cases were denied KPE and had primary LT at median 8 months of age. The native liver 4-year survival rate was 14.3% and the overall survival rate was 81%.
Conclusion:
BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT.
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