Case Author(s): Jeff Chesnut, D.O. and Henry Royal, M.D. , 6/24/99 . Rating: #D2, #Q4

Diagnosis: Caroli Disease

Brief history:

6 year old boy with renal failure.


Flow images from a hepatobiliary scintigram are shown.

View main image(hs) in a separate image viewer

View second image(hs). Function images from a hepatobiliary scintigram.

View third image(us). Ultrasound of the liver.

View fourth image(ct). CT slice through the liver.

Full history/Diagnosis is available below

Diagnosis: Caroli Disease

Full history:

6 year old boy diagnosed with renal failure. Ultrasound demonstrated multiple hepatic cysts.


Tc-99m Mebrofenin


The initial images demonstrate relatively homogenous uptake throughout the liver. At approximately 25 minutes, filling of the gallbladder and small bowel begins. In addition, the liver begins to appear more heterogenous, with more of an overall "lumpy-bumpy" appearance. This is more marked in the left hepatic lobe.

The multiple areas of biliary dilatation on the hepatobiliary scintigram correspond to cystic-appearing regions on the patient's previous computed tomography and ultrasound studies, consistent with the diagnosis of Caroli disease.


This child has autosomal-recessive polycystic kidney disease. Caroli disease, the saccular dilatation of biliary ducts is part of the spectrum of this syndrome. In general, the worse the hepatic cysts, the better the renal cysts and vice-versa. Patients with Caroli disease develop bile stasis resulting in biliary stones and an increased rate of infection.


CT scan through the kidneys demonstrate several small renal cysts.

View followup image(ct). CT scan through the kidneys.

ACR Codes and Keywords:

References and General Discussion of Hepatobiliary Scintigraphy (Anatomic field:Gasterointestinal System, Category:Normal, Technique, Congenital Anomaly)

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Case number: hs017

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