Autosomal recessive polycystic kidney disease associated with Caroli disease
Presentation
Abdominal distention.
Patient Data
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 types of ARPKD are associated with hepatic fibrosis