Autosomal Dominant Polycystic Kidney: Macroscopic Features

Macroscopic Features in Autosomal Dominant Polycystic Kidney Disease (ADPKD): Renal involvement in ADPKD is bilateral but may show asymmetry (as seen here) or asynchrony in 15-20% of cases. In the early stages of the disease, the kidneys maintain their reniform shape and appear normal or only slightly enlarged. There may be isolated small cysts in the cortex and medulla separated by normal parenchyma. With PKD1 mutations, almost 65% of patients have cysts by the age of 10 years and 90% develop cysts by the age of 20 years. Over time, the cysts expand in size and number and completely replace the renal parenchyma. (the discussion of macroscopic features continues in the next image).