Description
There is a gross enlargement of the kidneys with preservation of the normal renal form with a
recognisable renal pelvis. The outer surface shows multiple minute cysts while the cut surface shows
radially oriented fusiform or cylindrical cysts. This form of polycystic disease invariably leads to death
in the first few weeks or months of life. It appears to be genetically distinct from the adult form of
polycystic kidney.