Pediatric Radiology > Genitorinary > Cystic Renal Disease > Autosomal Dominant Polycystic Kidney Disease

Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is inherited in a dominant trait with variable expression. ADPKD presents in late childhood or early adulthood. US appearance may look similar to autosomal RECESSIVE polycystic kidney disease (ARPKD) initially. Macroscopic cysts will develop during the second decade and be indentified by US exam. Hepatic cysts are seen in one third of cases. Cysts can also be seen in multiple other organs. There is an association with intracranial aneurysms in approximately 20% of cases.
Enlarged bilateral polycystic kidneys on an IVP	Enlarged bilateral polycystic kidneys on an IVP
CT showing multiple cysts bilaterally	CT showing multiple cysts bilaterally
Longitudinal view of a polycystic kidney	Bilateral polycystic kidneys with renal scarring
CT showing hepatic cysts in ADPKD	Hepatic and splenic cysts in ADPKD
Enlarged bilateral polycystic kidneys on coronal T2 MRI