Long-Term Outcome of Integrative Care for Biliary Atresia: 17 Years of Experience in a Single Center
Masashi Kadohisa1, Yukihiro Inomata, Keita Shimata
1>From the Department of Transplantation and Pediatric Surgery, Kumamoto University, Kumamoto and the Department of Hepatobiliary Pancreatic and Transplantation/Pediatric Surgery, Kyoto University, Kyoto, Japan.
Insights
Biliary atresia treatment combining Kasai portoenterostomy and living donor liver transplant shows good outcomes. Continued improvements in Kasai portoenterostomy and timely transplants are vital for better results in pediatric liver disease.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia significantly impacts infant liver health.
- Integrated treatment strategies, including Kasai portoenterostomy and liver transplantation, have improved patient prognosis.
- A unified pediatric surgical team performing both procedures offers a unique approach to managing biliary atresia.
Purpose of the Study:
- To review the clinical features and outcomes of patients with biliary atresia treated with Kasai portoenterostomy and living donor liver transplant.
- To evaluate the effectiveness of a combined surgical approach for biliary atresia.
- To assess long-term survival rates and identify key factors in biliary atresia management.
Main Methods:
- Retrospective review of 41 patients with biliary atresia who underwent Kasai portoenterostomy between August 2000 and March 2017.
- Analysis of patient data including age at surgery, jaundice clearance, native liver survival, and need for subsequent living donor liver transplant.
- Evaluation of outcomes for patients undergoing living donor liver transplant, including age, weight, indications, and survival rates.
Main Results:
- Kasai portoenterostomy achieved jaundice clearance in 56.1% of patients.
- Native liver survival rates at 1, 5, 10, and 20 years were 51.2%, 46.3%, 43.0%, and 38.3%, respectively.
- Living donor liver transplant was performed in 24 patients, with a 15-year overall survival rate of 90.9% for those transplanted in infancy/early childhood.
Conclusions:
- Integrated management of biliary atresia by a single surgical team performing Kasai portoenterostomy and living donor liver transplant leads to favorable long-term outcomes.
- Optimizing the Kasai portoenterostomy technique is essential for improving early results.
- Timely living donor liver transplantation remains a critical component for achieving the best possible survival in biliary atresia patients.
Objectives:
Integration of the treatment for biliary atresia, including Kasai portoenterostomy and liver transplant, has had an important role in the marked improvement of the prognosis. We reviewed our experience with Kasai portoenterostomy and living donor liver transplant, both performed by the same pediatric surgical team and described the clinical features and outcomes of patients with biliary atresia.
Materials And Methods:
We reviewed 41 patients with biliary atresia who underwent Kasai portoenterostomy at Kumamoto University from August 2000 to March 2017.
Results:
The median age at Kasai portoenterostomy was 63 days (IQR, 54-75 days). Jaundice clearance was achieved by 23 patients (56.1%) at any time during the postoperative course. The 1-year, 5-year, 10-year, and 20-year native liver survival rates were 51.2%, 46.3%, 43.0%, and 38.3%, respectively. Of 41 patients, 24 eventually underwent living donor liver transplant. Of these, 22 patients underwent transplant during infancy or early childhood; their median age and body weight at living donor liver transplant were 6.5 months (IQR, 5.3-8.0 months) and 7.0 kg (IQR, 6.4-8.0 kg), respectively. The most common indication for living donor liver transplant was jaundice-induced liver cirrhosis; other indications included portal hyper-tension and recurrent cholangitis. The 15-year overall survival rate of 22 patients was 90.9%.
Conclusions:
Patients with biliary atresia treated by our team capable of both Kasai portoenterostomy and living donor liver transplant have shown good long-term outcomes. However, further improvement of Kasai portoenterostomy and timely living donor liver transplant remain crucial.
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