Ten-year progression of Caroli's disease with portal hypertension despite endoscopic and pharmacological therapy: Implications for liver transplantation-A case report.

Caroli's disease is a rare congenital disorder of the intrahepatic bile ducts that may be complicated by congenital hepatic fibrosis, portal hypertension, and recurrent variceal bleeding. We report the 10-year clinical course of a 29-year-old woman with Caroli's disease and portal hypertension who was managed in the Department of Gastroenterology. At initial presentation in 2015, she had splenomegaly, severe esophagogastric fundal varices, preserved liver biochemistry, Child-Pugh class A disease, and a low model for end-stage liver disease score. Symptoms improved after nonselective beta-blocker therapy; however, longitudinal follow-up showed persistent portal hypertension, recurrent gastrointestinal bleeding, and progressive impairment of hepatic reserve. From 2023 onward, she experienced recurrent hematemesis and melena despite sequential endoscopic variceal ligation and beta-blocker therapy, including conversion from propranolol to carvedilol. During the most recent admission on May 25, 2024, laboratory and imaging findings indicated progressive cholestasis, portal hypertension, Child-Pugh class B disease, and a model for end-stage liver disease score of 9.9. After multidisciplinary team evaluation, liver transplantation was considered clinically indicated, and the patient was activated on the transplant waiting list. This case demonstrates the limitations of long-term endoscopic and pharmacological therapy in Caroli's disease complicated by portal hypertension and supports timely transplant referral before advanced hepatic decompensation occurs.