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Liver transplantation for fulminant hepatic failure in infancy: a single center experience
Annette Strauss1, Enke Grabhorn, Marijke Sornsakrin
1Department of Pediatrics, Pediatric Hepatology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Insights
Liver transplantation for fulminant hepatic failure (FHF) in children yields good outcomes, with high patient and graft survival rates. Early referral and improved diagnostics are crucial for better FHF management and treatment success.
Area of Science:
- Pediatric Hepatology
- Transplant Surgery
- Critical Care Medicine
Background:
- Fulminant hepatic failure (FHF) in children presents significant challenges due to unknown causes and high mortality.
- Outcomes for FHF vary between transplant centers, highlighting the need for specialized care.
- Delayed transfer to transplant centers impacts patient management and prognosis.
Purpose of the Study:
- To evaluate the outcomes of liver transplantation (Ltx) for FHF in pediatric patients at a single center.
- To analyze the diagnostic challenges and timing of referral in FHF cases.
- To compare Ltx outcomes with medical management for FHF.
Main Methods:
- Retrospective analysis of 30 pediatric liver transplant recipients for FHF out of 83 total FHF cases.
- Review of diagnostic data, time to transfer, surgical techniques used, and patient outcomes.
- Comparison of survival rates between Ltx and medically treated FHF patients.
Main Results:
- High patient survival (93.4%) and graft survival (83.4%) at one year post-Ltx.
- Diverse surgical techniques enabled timely transplants: split (n=18), living donor (n=9), whole organ (n=2), reduced liver (n=1).
- Medically treated FHF patients had an 83% survival rate; severe post-Ltx complications included aplastic anemia and mitochondrial depletion syndrome.
Conclusions:
- Liver transplantation for pediatric FHF offers favorable survival rates.
- Early referral to specialized centers and enhanced diagnostic tools are essential for timely FHF detection and management.
- Despite challenges like delayed diagnosis and transfer, Ltx is a viable and effective treatment for pediatric FHF.
Abstract:
FHF is characterized by a high percentage of unknown causes leading to acute liver failure and furthermore by an increased morbidity and mortality prior to and post-Ltx. In different transplant centers, the reasons leading to FHF differ significantly as well as outcome. We report our single center experience with 30 pediatric patients receiving a liver transplant for FHF, out of a total of 83 children presenting with FHF. The time to transfer patients to the transplant center after the diagnosis of FHF was long, with a median of 14 days (Ltx group) and 12 days (controls), respectively. In nearly half of the patients (n = 14) in the Ltx group, we were not able to establish an exact diagnosis prior to Ltx: 50% suffered from encephalopathy, and 13 patients were treated in the intensive care unit prior to transplant. Because of the availability of different surgical techniques, all children received a timely transplant [split (n = 18), living donor (n = 9), whole organ (n = 2), and reduced liver (n = 1)]. Patient survival was 93.4%, and graft survival was 83.4% for at least one yr follow-up. Severe complications following Ltx included three cases with aplastic anemia and one child suffering from systemic mitochondrial depletion syndrome. The survival of patients treated medically was 83%. We conclude that a strong focus should be made on early referral to a specialized center and on improvement of diagnostic tools to timely detect the underlying reason for FHF. Results following Ltx for FHF are good.
