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

Aetiology

• Genetic: sporadic inheritance

Pathophysiology

• Dilation of collecting ducts
• In severe cases the medullary area appears like a sponge
• Cysts have calculi
• Renal failure unusual

Clinical features

• 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