Abstract
Background Caroli’s disease is an inherited disorder characterized by dilatation of intrahepatic bile ducts
and congenital hepatic fibrosis. Caroli’s disease is complicated by choledocholithiasis, cholangitis, and portal
hypertension.
Case presentation A 28-year-old Palestinian female with 7-year history of Caroli’s disease complicated by liver
cirrhosis, portal hypertension, and esophageal varices presented with hypersplenism and thrombocytopenia. She
was managed with splenic artery embolization, which improved her platelet count. The splenic artery embolization
was complicated by postembolization syndrome, and she was treated with antibiotics and supportive care. Two
months later, she died owing to liver encephalopathy and spontaneous bacterial peritonitis.
Conclusion This case highlights the role of splenic artery embolization in managing hypersplenism associated
with Caroli’s disease. Despite improvement in platelet count, post-embolization syndrome remains a significant risk.
Early liver transplantation should be considered in patients with advanced Caroli’s syndrome to improve long-term
outcomes.
Keywords Caroli’s syndrome, Caroli’s disease, Hypersplenism, Splenic artery embolization, Portal hypertension, Case
report

Title

Journal of Medical Case Reports

Publisher

BMC

Publisher Country

United Kingdom

Publication Type

Prtinted only

Volume

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Year

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Pages

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