Introduction: Cystic fibrosis (CF) liver disease occurs in 5–10% of individuals with CF and represents the third most common cause of death in this population. Liver transplantation (LT) is indicated in patients with CF who develop complications of portal hypertension and end-stage liver disease.
The aim of this study was to present our experience with LT in children with CF.
Methods: We retrospectively analyzed data from 25 patients under 18 years of age with CF who underwent LT between 2001 and 2024. We evaluated preoperative data, surgical factors, complications, and long-term outcomes.
Results: 2 patients underwent laparotomy due to meconium ileus in the neonatal period. Prior to LT, 3 patients required treatment for thrombocytopenia: splenectomy was performed in 2, and 1underwent splenic embolization. The median age at LT was 168 months (range: 87–216), and the median weight was 39 kg (range: 19.5–69).
21 (84%) received a deceased donor graft with resection of the retrohepatic vena cava and two end-to-end anastomoses; 4 (16%) received a living donor graft. Bile duct reconstruction with duct-to-duct anastomosis was performed in 18 (72%). During LT, 12  (48%) additionally underwent splenectomy due to splenomegaly and hypersplenism. The median cold ischemia time was 514 minutes (range: 119–696). All transplants were AB0-compatible. Abdominal closure was delayed in 16  (64%). Early surgical revision was required in 8 (32%): 2 for hepatic artery thrombosis (HAT), 3 for biliary complications, 3 for bleeding, and 1 for ileus. 6 (24%) developed pleural effusion requiring drainage, and one underwent thoracoscopic pleurodesis due to persistent effusion. 5 (20%) required hemodialysis due to acute kidney injury.
2 required re-transplantation: one due to failed revascularization after HAT (2 months post-LT), and the other due to biliary stenosis and recurrent biliary infections (15 months post-LT).
The median follow-up time was 94 months (range: 2–271). 19 (76%) are alive with good liver and kidney function. 2 patients who underwent re-transplantation died in the early postoperative period due to multiple organ dysfunction syndrome. One patient died from respiratory failure 1 month post-LT. 3 patients died due to infectious complications at 79, 192, and 279 months after LT. In the long-term follow up after LT, 11 patients (44%) had diabetes mellitus (DM). Among 6 patients with DM prior LT, only 4 required treatment after LT. 7 developed post-transplant DM (PTDM), 5 of them required insulin therapy in the immediate post-transplant period.
Conclusions: Most patients experienced good long-term outcomes despite a relatively high rate of early postoperative complications. Post-transplant diabetes mellitus remains a significant long-term complication, affecting nearly half of the recipients. Careful perioperative management and long-term follow-up are essential to optimize outcomes in this high-risk population.