First liver transplantation for biliary atresia in children: The hidden effects of non-centralization

Area of Science:

• Pediatric Surgery
• Hepatology
• Transplantation Medicine

Background:

• Biliary atresia (BA) is a severe neonatal liver disease requiring liver transplantation (LT).
• The management pathway, including initial care center and referral timing, may impact LT outcomes.
• Optimizing care coordination is crucial for pediatric LT success.

Purpose of the Study:

• To evaluate the impact of initial medical and surgical care orientation for children with BA on LT procedures and outcomes.
• To compare outcomes between children managed at a single center versus those secondarily referred after initial management failure.

Main Methods:

• Retrospective analysis of 185 children with BA undergoing their first LT between 2006 and 2015.
• Patients were categorized into single-center management and secondarily referred groups.
• Analysis focused on disease severity, blood transfusion requirements, and overall survival rates.

Main Results:

• Secondarily referred patients experienced shorter pre-LT check-up to transplantation delays but showed higher rates of severe undernutrition (23.7% vs 11.1%).
• Single-center management was associated with better coagulation profiles (higher INR and Factor V levels) at transplantation.
• Secondarily referred patients required twice the amount of blood products (red blood cells and fresh frozen plasma).
• Overall 12-month survival rates were significantly higher in the single-center management group (92.1% vs 83.1%).

Conclusions:

• Early referral and integrated management at a specialized transplant center improve LT outcomes in children with BA.
• Single-center management is associated with better nutritional status, reduced transfusion needs, and enhanced survival post-LT.
• Optimizing the care pathway through early referral is recommended to improve pediatric liver transplantation results for BA.