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British paediatric surveillance unit study of biliary atresia: outcome at 13 years
P J McKiernan1, Alastair J Baker, Carla Lloyd
1Liver Unit, The Children's Hospital NHS Trust, Birmingham, UK. Pat.Mckiernan@bch.nhs.uk
Insights
Successful Kasai portoenterostomy for biliary atresia offers good long-term outcomes, reducing the need for liver transplantation. Treatment in high-volume centers significantly improves survival rates for children with this condition.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Limited contemporary data exists on the long-term prognosis of biliary atresia in national pediatric cohorts.
- Understanding long-term outcomes is crucial for improving patient management and surgical strategies.
Observation:
- A prospective study followed 93 children diagnosed with biliary atresia in the UK and Ireland between 1993 and 1995.
- The median follow-up duration was 12 years, with a range of 0.25 to 14 years.
Findings:
- Successful Kasai portoenterostomy resulted in 80% of patients surviving without liver transplantation.
- Children treated at high-volume centers ( >5 cases/year) had a significantly better 13-year actuarial survival rate without transplantation (54% vs. 27.3%).
- Liver transplantation was required for 45% of children, with a 90% survival rate.
Implications:
- Effective Kasai portoenterostomy can prevent the need for liver transplantation before adolescence.
- Centralizing biliary atresia treatment in experienced, high-volume centers is critical for optimizing surgical success and long-term outcomes.
- These findings underscore the importance of specialized care in pediatric liver diseases.
Background:
Little information is available on contemporary, prospectively collected data on the long-term outcome of national cohorts of children with biliary atresia.
Objective:
This study aimed to describe the current outcome of a national cohort of children with biliary atresia.
Patients And Methods:
All 93 cases of biliary atresia in the United Kingdom and Ireland diagnosed between March 1993 and February 1995 were followed up prospectively.
Results:
A total of 91 children underwent Kasai portoenterostomy in 15 individual centres. Only 2 centres treated more than 5 children annually. Median age at last follow-up was 12 years (range 0.25-14). Fifteen children (16%) have died: 10 after unsuccessful portoenterostomy, 1 of sepsis after successful portoenterostomy, and 4 after liver transplantation. Forty-two (45%) underwent liver transplantation at a median age of 1 year (range 0.5-9), with 90% survival. All 41 children with failed portoenterostomy (and 2 without portoenterostomy) died or underwent liver transplantation at a median age of 0.8 years (range 0.25-6.5). When the portoenterostomy was successful, 40 of 50 patients (80%) are alive without liver transplantation. The 13-year actuarial survival without liver transplantation is 43.8% overall and is better in children treated at centres that treat more than 5 cases yearly (54% vs 27.3%, P = 0.005).
Conclusions:
If the portoenterostomy is successful, then few children with biliary atresia will need transplantation before adolescence. Children with biliary atresia should be treated in experienced centres to maximize the chance of successful surgery.