67 Caroli’s Disease

Clinical Presentation

A 2 1/2-year-old boy was followed since prenatal ultrasonographic diagnosis of abdominal abnormalities.

Figure 67A

Figure 67B

Radiologic Findings

Ultrasonographic examination (transverse scan of the liver) shows extensive saccular dilatation of intrahepatic biliary tree with the “dot sign” (7A, arrow). Renal enlargement (14.7 cm long) with hyperechoic parenchyma is noted (Fig. 67B).

Figure 67C Differential diagnosis: simple liver cyst on routine ultrasound of an infant.

Figure 67D Differential diagnosis: extensive hepatic involvement on ultrasound of a 16-year-old girl with abdominal lymphangioma.

Figure 67E Differential diagnosis: CT of amebic abscess of liver dome in a child with cough and febrile illness.

Figure 67F Coronal MRI (inversion recovery) demonstrating saccular dilatation with high signal intensity within the liver from Caroli’s disease. There also is bilateral renal enlargement of autosomal recessive polycystic kidney disease.

Diagnosis

Caroli’s disease

Differential Diagnosis

Recurrent pyogenic cholangitis

Primary sclerosing cholangitis

Biliary tract obstruction

Mesenchymal hamartoma of the liver

Developmental hepatic cyst (Fig. 67C)
Dysontogenetic cyst
Lymphangioma of liver (Fig. 67D)
Liver abscess (amebic) (Fig. 67E)
Hydatid cyst (Echinococcus granulosus)

Figure 67G Sonogram of a 12-year-old patient with gastrointestinal bleeding, splenomegaly, and congenital cystic renal disease. The dot sign is pathognomonic of Caroli’s disease.

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