Late form (typically adult) of nephronophthisis (see this topic) without extrarenal signs. It is difficult to distinguish from juvenile nephronophthisis. The kidneys are of normal size and cysts appear at the corticomedullary junction.

Renal failure occurs around the age of 20 years.

Anesthetic implications:

check blood pressure, renal and hepatic function. monitoring of blood glucose

References : 

-         Bonsib SM. 
The classification of renal cystic diseases and other congenital malformations of the kidney and urinary tract. 
Arch Pathol Lab Med 2010; 134:554-68.

-        Avni F. 
Renal cystic diseases in children: new concepts. 
Pediatr Radiol 2010; 40: 939-46.

Updated: October 2018