Related Experiment Videos
Liver transplantation in babies and children with extrahepatic biliary atresia
1Liver Unit, Children's Hospital, Birmingham, England.
Insights
Orthotopic liver transplantation (OLT) in children with biliary atresia can be successful, even for infants under one year old. Technical advances in reduction hepatectomy allow for comparable outcomes to whole grafts, making size and age less of a contraindication.
Area of Science:
- Pediatric Surgery
- Hepatology
- Transplantation Medicine
Background:
- Biliary atresia is a leading cause of end-stage liver failure in children.
- Orthotopic liver transplantation (OLT) is a critical treatment option for these patients.
- Many children present with advanced liver disease, necessitating timely intervention.
Purpose of the Study:
- To evaluate the outcomes of OLT in children with biliary atresia, focusing on the impact of graft type (reduced vs. whole) and patient characteristics.
- To assess the feasibility and efficacy of using reduced-size grafts in infants and small children.
- To analyze early and late complications and long-term survival rates.
Main Methods:
- Retrospective review of 39 children undergoing OLT for biliary atresia between 1987 and 1991.
- Analysis of patient demographics, pre-transplant conditions, graft types (reduced segmental vs. whole), surgical techniques, complications, and survival rates.
- Specific focus on the Brisbane technique for reduced grafts and outcomes in infants under one year.
Main Results:
- Overall survival rate was 72% with 11 deaths due to primary nonfunction, sepsis, or bleeding.
- Graft loss rates were 33% for reduced grafts and 27% for whole grafts.
- Technical advances, including reduction hepatectomy (Brisbane technique), enabled successful OLT in small infants (<1 year), yielding comparable results to whole grafts.
Conclusions:
- Size and age should not be contraindications for OLT in children with biliary atresia.
- Reduction hepatectomy techniques have significantly expanded the eligibility for OLT in pediatric patients.
- OLT offers a life-saving option with good quality of life, catch-up growth, and normal development post-transplant.
Abstract:
Orthotopic liver transplantation (OLT) is a life-saving procedure for end-stage liver failure. We reviewed 39 children (24 girls, 15 boys) who received OLT for biliary atresia from 1987 to 1991. Twenty had unsuccessful portoenterostomy, 6 were referred too late for a drainage operation, and the remaining 13 achieved bile drainage but developed portal hypertension. At transplant 37 had decompensated liver disease with varices (28), ascites (24), encephalopathy (17), and gastrointestinal bleeding (12). The median weight and age at transplant were 8 kg and 12.6 months, respectively. The median waiting time was 65 days. Forty-eight grafts (30 reduced and 18 whole) were performed; graft loss was 33% and 27%, respectively. Of the 30 segmental grafts, 15 were reduced conserving the left lateral segment and hepatic vein (Brisbane technique)--13 were from the left lobe and 2 from the right lobe. The overall subject survival rate is 72%. Eleven deaths occurred: primary nonfunction (3), sepsis (3), perioperative bleed (3), and other causes (2). Early complications included: hepatic artery thrombosis (5), hepatic vein thrombosis (2), bowel perforation (3), biliary leak (3), and acute rejection (8). Later complications were chronic rejection (4) and biliary stricture requiring reconstruction (3). Follow-up at 12 months confirms good quality of life for both child and family with catch up growth and normal development. Technical advances in reduction hepatectomy have allowed us to treat small babies under 1 year with an urgent requirement for OLT, with comparable results to those obtained with whole grafts. In conclusion, in the future size and age need not be a contraindication to OLT in children with biliary atresia.