Presentation
Abdominal distention.
Patient Data
Age: 5 months
Gender: Male
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Axial non-contrast
- diffuse enlargement and heterogeneous attenuation of both kidneys, with multiple small sized hypodense cystic lesions scattered in the renal parenchyma. Findings consistent with autosomal recessive polycystic kidney disease (ARPKD)
- liver enlargement with multiple linear and cystic hypodense lesions secondary to dilated intrahepatic biliary radicles, some with central dot sign. Picture of Caroli disease
- mild splenomegaly
- marked ascites
- right-sided indirect inguinal hernia reaching the scrotum contains bowel loops and fluid, with no signs of obstruction or incarceration
- slices taken through chest reveal cardiomegaly
- bilateral enlarged axillary lymph nodes
Case Discussion
- this case demonstrates the association between ARPKD and Caroli disease
- neonatal and infantile type of ARPKD are associated with hepatic fibrosis.