Liver Transplantation in Childhood: A 2-Year Single Center Experience

Abstract

Objective: Liver transplantation is currently the most treatment for fulminant hepatitis, end-stage liver failure, hepatocellular carcinoma, and liver-originated metabolic diseases in children. With technological advances, improvements in surgical techniques and immunosuppressive therapy protocols have increased 1-year survival rates to 80%-90%. Our center successfully performs both living donor and cadaveric liver transplants in children. This study retrospectively analyzed the preoperative and postoperative data of 72 pediatric patients who underwent liver transplantation between July 2022 and July 2024.

Methods: We included 72 patients who underwent liver transplantation between July 1, 2022, and July 1, 2024. Cases were evaluated based on demographic data, liver failure etiology, and postoperative complications.

Results: Of the cases, 37 were female (58%) and 35 male (42%), with a mean age of 6.6 years (ranging from 5 months to 17 years and 11 months). Indications included biliary atresia (25), autoimmune hepatitis (9), cryptogenic cirrhosis (7), PFIC (7), congenital hepatic fibrosis (1), Caroli disease (2), Wilson's disease (4), Alagille syndrome (3), hepatocellular carcinoma (2), primary hyperoxaluria type 1 (2), Crigler Najjar syndrome type 1 (3), Budd-Chiari syndrome (1), glycogen storage disease type 3 (1), portal vein thrombosis (1), and acute fulminant hepatitis (4). Mean PELD score for patients under 12 years was 18 (range 0-37), and MELD score for patients over 12 years was 19.3 (range 11-40). A total of 69 patients received orthotopic liver transplantation from living donors. Two patients received combined liver and kidney transplants, and 1 received a cadaveric liver transplant. Donors included 40 females and 32 males. Left lobe transplants were performed in 58 patients, and right lobe in 14. Immunosuppression included Tacrolimus + MMF in 70 patients and Cyclosporine in 2. Postoperative complications included biliary anastomosis stenosis (3), bile leakage (2), hepatic vein thrombosis (1), portal vein thrombosis (4), intestinal perforation secondary to Bogota syndrome (5), PRES syndrome due to Tacrolimus toxicity (1), primary graft dysfunction (1), and postoperative bleeding (2). Within the first month, 12 patients (16.6%) died, and 3 (4%) died between 1 month and 1 year postoperatively. The most common early cause of death was sepsis and multiorgan failure. One patient developed chronic rejection but recovered with steroid immunosuppression without the need for re-transplantation.

Conclusion: Liver transplantation is a high-risk procedure requiring lifelong medication and follow-up. It is, however, the most effective treatment method for several severe pediatric liver conditions.