ArticleCureus2026
Recurrent Fever and Biliary Ectasia Leading to the Diagnosis of Caroli Syndrome: A Diagnostic Challenge.
Article in Cureus, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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5 authors.
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Abstract
Caroli syndrome (CS) is a rare congenital fibropolycystic liver disorder characterized by non-obstructive intrahepatic bile duct dilatation and congenital hepatic fibrosis, often masquerading as fever of unknown origin. A 38-year-old Hispanic male presented with four weeks of intermittent high-grade fevers and right upper quadrant pain following recent travel to Mexico. An extensive infectious workup was unrevealing, while inflammatory markers were markedly elevated despite normal liver enzyme levels. Cross-sectional imaging revealed multiple intrahepatic cystic dilatations with biliary ectasia, and liver biopsy confirmed congenital hepatic fibrosis, establishing the diagnosis of CS. He defervesced rapidly on empirical broad-spectrum antibiotics. CS should be considered in patients with recurrent unexplained fevers and biliary abnormalities, even when liver enzyme levels are normal, to enable timely diagnosis and surveillance for long-term complications.
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