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
  


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