Medullary Sponge Kidney : Epidemiology & Clinical

Epidemiology & Clinical Features: Medullary sponge kidney (MSK) is usually bilateral and is slightly more common in males. It is uncommon, with an estimated frequency of 1 in 5000. The kidneys are normal in size and function. MSK is usually asymptomatic and may remain undiagnosed for life. It becomes symptomatic when complicated by nephrolithiasis, hematuria and infection, usually between 3rd and 5th decades of life. Pediatric cases have been reported. Most cases are sporadic; however, there is evidence of a genetic predisposition in about 5% of cases. MSK has been reported in association with congenital hemihypertrophy, Beckwith-Wiedemann syndrome, Ehler-Danlos syndrome, Wilms tumor, and multiple endocrine neoplasia type II.