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Outcome of biliary atresia in Malaysia: a single-centre study
Way-Seah Lee1, Pei-Fan Chai, Kean-Seng Lim
1Departments of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Malaysia. leews@um.edu.my
Insights
Outcomes for biliary atresia (BA) surgery in Malaysian children show 37% survival with native liver at 2 years. Overall survival was impacted by limited liver transplantation (LT) access, highlighting the need for earlier diagnosis and intervention.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatal Care
Background:
- Biliary atresia (BA) is a rare neonatal liver disease requiring timely surgical intervention.
- Outcomes vary significantly based on referral timing and access to advanced treatments like liver transplantation (LT).
Purpose of the Study:
- To evaluate the surgical outcomes for children diagnosed with biliary atresia (BA) at a Malaysian tertiary pediatric center.
- To determine survival rates with native liver and after liver transplantation (LT) at two years of age.
Main Methods:
- A prospective study was conducted from 1996 to 2005 at the University of Malaya Medical Centre (UMMC), Kuala Lumpur.
- Fifty-seven infants with BA were analyzed, focusing on referral age, surgical procedure (Kasai procedure/hepatoportoenterostomy - HPE), and survival outcomes at two years.
Main Results:
- The median age at referral was 62 days; 48 patients underwent HPE at a median age of 70 days.
- At two years, 37% of patients who had HPE survived with their native liver.
- Overall survival, including LT, was 40%, with two of four LT recipients surviving.
Conclusions:
- The 2-year native liver survival rate following HPE for BA in Malaysia is comparable to international benchmarks.
- Limited access to timely liver transplantation (LT) negatively impacts overall survival rates for BA patients.
- Increased awareness among healthcare professionals regarding early BA detection and referral is crucial for improving patient outcomes.
Aim:
This study aimed to determine the outcome of the operation of children with biliary atresia (BA) at a tertiary paediatric referral centre in Malaysia.
Methods:
A prospective study on all patients with BA referred to the University of Malaya Medical Centre (UMMC), Kuala Lumpur, from 1996 to 2005 was conducted. Survival with native liver, liver transplantation (LT) or death at 2 years of age was determined.
Results:
The median age at referral of the 57 patients with BA seen at University of Malaya Medical Centre was 62 days. Kasai procedure was not performed in nine patients who were all referred late (median age of referral 180 days). The median age at hepatoportoenterostomy (HPE) of the remaining 48 patients was 70 days. A total of 53 (93%) patients had type 3 BA, while only 1 (2%) patient had BA splenic malformation. At 2 years, the survival rate with native liver for the 48 patients who had HPE was 37%, while the overall survival (native liver and LT) rate was 40%. Two of the four patients who had LT survived with a liver graft at 2 years.
Conclusions:
The 2-year survival with native liver following corrective surgery for BA in UMMC, Malaysia, compares favourably with other international figures, but the overall survival rate was adversely affected by a lack of timely LT. The outcome of BA in Malaysia may be further improved by increasing the awareness among child-health professionals on the importance early referral for appropriate surgery in infants suspected of having BA.