SHM Converge Scientific Abstract ePoster Gallery-Take the Caroli: Asymptomatic Caroli's Disease in an Elderly Patient With Cystic Renal Disease

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Northwell Health is New York State’s largest health care provider and private employer, committed to improving community health through high-quality clinical care, medical education, biomedical research, health promotion, and care for everyone regardless of ability to pay. It operates 23 hospitals and more than 700 outpatient facilities, with over 13,600 affiliated physicians.<br /><br />The case report describes a 69-year-old woman with a history of hemoglobin S/C disease, hypertension, and chronic kidney disease who was admitted after a fall. Imaging done during her evaluation incidentally revealed cystic lesions in both the liver and kidneys. CT showed cystic abnormalities, and MRI demonstrated innumerable kidney and liver cysts arranged in an intrahepatic biliary pattern, with interval progression since 2014. She was diagnosed with asymptomatic Caroli’s disease, likely associated with autosomal dominant polycystic kidney disease (ADPKD).<br /><br />The report reviews key background points: Caroli’s disease is a rare disorder involving cystic dilation of intrahepatic bile ducts, usually presenting before age 30, and can be associated with ADPKD. Symptoms may range from none to serious biliary complications such as cholangitis, cholelithiasis, and hepatic abscesses. Because liver cysts are common in ADPKD, liver involvement should be considered when renal cysts suggest ADPKD.<br /><br />Teaching points emphasize that Caroli’s disease can coexist with PKD and that patients may be at risk for biliary complications. The case also highlights how underlying ADPKD may be missed when kidney disease is attributed to other conditions.

Asset Subtitle

Michael Attanasi

Meta Tag

Author List Ava-Dawn Gabbidon MD, Cassie Wang, Michael Attanasi

Category Clinical Vignettes

Concept Caroli Disease

Concept Intrahepatic Bile Ducts

Concept String of Beads Pattern

Concept Polycystic Liver Disease

Concept Cholangitis

Distinguished Non-Finalist

Presenter Organization North Short University Hospital/Long Island Jewish Medical Center

Presenting Author Michael Attanasi

Track Adult

Keywords

Northwell Health

Caroli disease

autosomal dominant polycystic kidney disease

ADPKD

liver cysts

kidney cysts

intrahepatic bile ducts

chronic kidney disease

biliary complications

case report

Caroli Disease

Intrahepatic Bile Ducts

String of Beads Pattern

Polycystic Liver Disease

Cholangitis