Clinical Efficacy of Liver Transplantation in Caroli Syndrome: A Retrospective Analysis

Objective: To investigate the clinical efficacy of liver transplantation in treating Caroli syndrome. Methods: A retrospective analysis was conducted on the clinical data of patients diagnosed with Caroli syndrome and undergoing liver transplantation at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024. The analysis focused on baseline characteristics, surgical indicators, postoperative complications, and long-term survival rates. All patients were followed up postoperatively, with the follow-up period ending in February 2025. Results: A total of 20 patients diagnosed with Caroli syndrome underwent liver transplantation at Beijing Friendship Hospital, accounting for 1.23% (20/1623) of all liver transplant patients during the same period. Of these, 13 patients received living donor liver transplants (LDLT), and 7 received deceased donor liver transplants (DDLT). The cohort included 15 male and 5 female patients, with ages ranging from 3 to 56 years (median age 23.3). Preoperative major comorbidities included cirrhosis(18 patients), portal hypertension(17), splenomegaly(17), polycystic kidney disease(16), hypoalbuminemia(14), ascites(13), gastrointestinal bleeding(8), hepatomegaly(9), renal injury(2), pulmonary hypertension(3), hepatopulmonary syndrome(1), congenital liver fibrosis(2), and cholangitis(1). Preoperative APACHE II score ranged from 4 to 27 (mean 14.4 ±5.9), with an expected mortality rate between 5.1% and 72.5% (mean 29.4±17.3). The preoperative SOFA score ranged from 0 to 13 (mean 5.0), CLIF-SOFA score from 0 to 7 (mean 5.0), PELD score (for children under 12) from 0 to 28 (mean 15), and MELD score from 9 to 24 (mean 16.7±7.3). The graft weight ranged from 224 to 1327g (mean 492.1±331.1g), with a graft-to-recipient weight ratio (GRWR) of 0.76% to 4.90% (mean 1.7± 1.2%). The warm ischemia time ranged from 2 to 8 minutes (mean 3.4±1.7 minutes), and cold ischemia time ranged from 30 to 657 minutes (mean 233.2±209.3 minutes). None of the patients received a cross-match transplant or auxiliary liver transplantation. Three patients underwent simultaneous partial splenectomy. Postoperative complications included abdominal hemorrhage in three patients, and seizures in one patient. Comparing adult and pediatric patients, females were more frequent in the pediatric group (p=0.001), and preoperatively, chronic renal failure was more common in adults (p=0.010). There were no significant differences in other preoperative comorbidities, early postoperative complications, or late complications. Long-term postoperative complications included chronic renal failure in six patients (onset between 12.1 and 46.7 months post-surgery) and acute rejection in four patients, three of whom experienced recurrent acute rejection. Four patients (20%) died: one died from postoperative heart failure on day 8, one from unexplained sudden death at 6 months, one from pulmonary infection and respiratory failure at 1 year, and one from an unknown cause at 5.2 years. The 1-year, 3-year, and 5-year survival rates were 90%, 85%, and 78.5%, respectively. Conclusion: Liver transplantation significantly improves the survival prognosis for patients with Caroli syndrome, but attention to kidney function preservation and individualized immunosuppressive management is critical for optimal outcomes.