Autosomal Recessive Polycystic Kidney: Macroscopic Features

Autosomal recessive polycystic kidney disease (ARPKD) - Macroscopic Features: The kidneys retain their reniform shape and show massive, symmetric enlargement. The external surface is generally smooth. The cut surface shows diffuse replacement of the renal parenchyma by numerous tubular cysts, 1-2 mm in diameter, radiating linearly from the medulla to the cortex, giving kidneys a spongy appearance. The cysts are formed by fusiform dilation of the collecting ducts and their long axes are oriented perpendicularly to the renal surface. The cortico-medullary junction is indistinct or obliterated. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission