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

作者：Fei Hou, Haofeng Xiong, Ying Liu, Shuang Zhao, Yizhi Zhang, Kang Qian, Jingyi Liu, Wei Lin, Wei Qu, Zhi‐Gui Zeng, Zhi‐Jun Zhu, Li‐Ying Sun · 发表于：Orphanet Journal of Rare Diseases · 年份：2025 · DOI：10.1186/s13023-025-03943-6 · 被引用次数：1 · 研究领域：Genetic and Kidney Cyst Diseases、Genetic Syndromes and Imprinting、Congenital Anomalies and Fetal Surgery

OBJECTIVE: To evaluate the clinical outcomes of liver transplantation (LT) in patients with Caroli syndrome. METHODS: 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. RESULTS: 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 ch...