Caroli syndrome and autosomal recessive polycystic kidney disease

Q: Which classical abnormality is seen in liver? show answer

A: Intrahepatic cystic areas, which appear to communicate with biliary tree - diagnosis of Caroli's disease is almost certain. (c.f. von Meyerburg hamartomas will be relatively smaller than these cysts)

Q: Which other autosomal recessive condition is noted in the case? show answer

A: Autosomal recessive polycystic kidney is also seen.

Q: Considering the age of patient and the CT images, what seems to be the primary concern among two conditions? show answer

A: While ARPKD is the primary concern in neonatal or early childhood presentation, hepatic fibrosis dominates in juvenile presentation. As seen in this case, features of portal hypertension are quite obvious with massive splenomegaly and left lobe hypertrophy.

Q: According to Todani classification, which type of Choledochal cyst it can be classified as? show answer

A: Type V