Medullary Sponge Kidney

Congenital disorder of the kidneys characterized by cystic dilatation of the collecting tubules in one or both kidneys

Aetiology

• Sporadic inheritance

• Dilation of collecting ducts

Pathophysiology

• In severe cases the medullary area appears like a sponge

• Cysts have calculi

• Renal failure unusual

Clinical presentation

• Mostly asymptomatic - discovered e.g. during an investigation of haematuria

• Symptomatic patients typically present as middle-aged adults with renal colic, kidney stones, nephrocalcinosis and/or recurrent urinary tract infections

Investigations

• Excretion urography

Management

• Usually aggressive treatment is not necessary - maintain adequate fluid intake to decrease risk of kidney stones