Clinical outcomes of liver transplantation in Caroli syndrome: a retrospective analysis

To evaluate the clinical outcomes of liver transplantation (LT) in patients with Caroli syndrome. We retrospectively analyzed clinical data from 20 patients diagnosed with Caroli syndrome who underwent LT at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024. Data included baseline characteristics, surgical parameters, complications, and survival. Follow-up concluded in February 2025. These 20 cases accounted for 1.23% (20/1623) of all LTs during the study period. Thirteen patients received living donor liver transplants (LDLT), and seven received deceased donor liver transplants (DDLT). The cohort included 15 males and 5 females, aged 3–56 years (median 23.3). Common preoperative conditions included cirrhosis (n = 18), portal hypertension (n = 17), polycystic kidney disease (n = 16), and splenomegaly (n = 17). The median APACHE II score was 14; the predicted mortality averaged 26.0%. The median interval from the first major complication (e.g., recurrent cholangitis or decompensation) to LT was 12 months (IQR 4.5–21). No patients underwent cross-match or auxiliary LT; three had concurrent partial splenectomy. Postoperative complications included abdominal hemorrhage (n = 3) and seizures (n = 1). Chronic renal failure occurred in six patients during follow-up, and four experienced acute rejection (three recurrent). Compared with adults, pediatric patients had a higher proportion of females (p = 0.001), while chronic renal failure was more common in adults preoperatively (p = 0.010). Four patients (20%) died: one from heart failure (day 8), one from sudden death (6 months), one from pulmonary infection (1 year), and one from unknown causes (5.2 years). Median follow-up was 74.6 months (range 7-125). One-, three-, and five-year survival rates were 90%, 85%, and 78.5%, respectively. 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.