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Living related liver transplantation in children
K Tanaka1, S Uemoto, Y Tokunaga
1Second Department of Surgery, Faculty of Medicine, Kyoto University, Japan.
Insights
Living related liver transplantation (LRLT) in children offers a promising solution to liver shortages, with high survival rates in elective cases. Careful surgical planning and complication management are key for successful pediatric liver transplants.
Area of Science:
- Pediatric Surgery
- Hepatology
- Transplantation Immunology
Background:
- End-stage liver disease (ESLD) in children necessitates liver transplantation.
- Cadaveric liver donation faces significant shortages, particularly for pediatric recipients.
- Living related liver transplantation (LRLT) presents a viable alternative to address this disparity.
Purpose of the Study:
- To evaluate the outcomes of 37 living related liver transplantations (LRLT) in pediatric patients with end-stage liver disease.
- To assess the efficacy of immunosuppression protocols and surgical techniques in LRLT.
- To identify factors influencing graft and patient survival, and post-transplant complications.
Main Methods:
- Retrospective review of 37 pediatric LRLT cases over 27 months.
- Graft procurement from parental donors (mothers and fathers).
- Immunosuppression using FK506 and low-dose steroids; microsurgery for vascular reconstruction.
Main Results:
- Overall survival rate of 90% in elective LRLT and 57% in emergency cases.
- Successful management of vascular stenosis (hepatic and portal veins) via balloon dilatation.
- No hepatic artery thrombosis post-microsurgery introduction.
- Postoperative infections and EBV-associated lymphoma were significant causes of mortality.
- Acute rejection episodes were effectively controlled with FK506 and steroids, with no rejection in ABO-identical cases.
- Children with moderate growth retardation showed catch-up growth, while severe retardation persisted.
Conclusions:
- LRLT is a safe and effective option for pediatric end-stage liver disease, significantly improving survival rates.
- Meticulous surgical techniques, especially microsurgery, and comprehensive management of complications are critical for successful outcomes.
- LRLT plays a vital role in supplementing cadaveric liver donations for children.
- Growth outcomes are dependent on the severity of pre-transplant growth retardation.
Abstract:
We reviewed 37 living related liver transplantations (LRLT) performed by our department during the last 27 months on children with end-stage liver disease. The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were made from the left lateral segment (19), left lateral segment with partial S4 (11), left lobe (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total hepatectomy with preservation of the inferior vena cava. FK506 and low-dose steroids were used for immunosuppression. The survival rate was 90% (27/30) in elective cases and 57% (4/7) in emergency cases. Six recipients had functioning grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in 3 cases at 3 months after LRLT and was successfully treated by balloon dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and was also safely dilated. We incurred no hepatic artery thrombosis after introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were lethal. Three patients with ABO-blood group compatible grafts and one with an incompatible graft developed acute rejection, which was controlled in evey case by steroid bolus and/or increasing the dose of FK506. There were no definite episodes of rejection in ABO-identical cases. Children with moderate growth retardation (> or = -1.5 SD of normal growth) caught up in growth soon after LRLT, but those with severe retardation (<-1.5 SD) were slow to attain age-normal height. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT. LRLT is a promising option for alleviating the shortage of livers for pediatric transplantation and may be regarded as an independent modality to supplement cadaver donation.