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Published on: November 7, 2020
[Current status of and issues in pediatric liver transplantation in Japan]
1Department of Transplant Surgery, Jichi Medical University, Shimotsuke, Japan.
Insights
Pediatric living donor liver transplantation (PLDLT) survival rates have improved significantly. However, outcomes for fulminant hepatic failure and hepatoblastoma require further multidisciplinary treatment strategies.
Area of Science:
- Hepatology and Transplant Surgery
- Pediatric Gastroenterology
Context:
- Pediatric living donor liver transplantation (PLDLT) is a critical intervention for end-stage liver disease in children.
- Annual PLDLT volume in Japan is approximately 140 cases, with biliary atresia as the primary indication.
- Significant advancements in surgical techniques, immunosuppression, and infection control have improved outcomes over the last two decades.
Purpose:
- To review the current status and outcomes of pediatric living donor liver transplantation in Japan.
- To identify challenges and propose strategies for improving patient prognosis in specific indications like fulminant hepatic failure and hepatoblastoma.
Summary:
- The 5-year patient survival rate post-PLDLT has reached 84%, demonstrating considerable progress.
- Increased PLDLTs for fulminant hepatic failure and metabolic diseases show improved prognosis due to optimal surgical timing.
- Survival remains poor for unknown origin fulminant hepatic failure and hepatoblastoma, necessitating multidisciplinary approaches.
- Postoperative management includes regular imaging for complications (e.g., vascular stenosis, biliary stricture) and protocol biopsies for graft fibrosis.
Impact:
- Early diagnosis and interventional radiology can effectively manage mid/long-term complications.
- Protocol biopsies guide immunosuppressive therapy to prevent graft fibrosis and ensure long-term graft function.
- Establishing multidisciplinary treatment regimens is crucial for improving outcomes in challenging PLDLT cases.
Abstract:
Approximately 140 pediatric living donor liver transplantations (PLDLTs) are performed annually in Japan. The most common indication for PLDLT is biliary atresia. The 5-year patient survival rate after PLDLT has reached 84% with advances in surgical techniques, immunosuppressive therapy, and antiinfection therapy in the past two decades. Recently, although the number of PLDLTs performed for the treatment of fulminant hepatic failure and metabolic disease has increased, the optimal timing of surgery for each disease has led to better patient prognosis. However, patient survival rates after fulminant hepatic failure of unknown origin and hepatoblastoma are still poor in comparison with other indications. A multidisciplinary treatment regimen including postoperative medical therapy therefore needs to be established for these conditions. In the outpatient clinic, regular and continuing imaging examination is indispensable for the diagnosis of mid/long-term complications such as vascular anastomotic stenosis and biliary stricture in addition to standard liver function tests. These complications can be treated with interventional radiology if diagnosed in the early stage. Protocol biopsy is also important to confirm graft fibrosis over the long term after PLDLT. Patients with graft fibrosis should receive more potent immunosuppressants to maintain permanent graft function.
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