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Medullary sponge kidney is formation of diffuse, bilateral medullary cysts caused by abnormalities in pericalyceal terminal collecting ducts.
The cause of medullary sponge kidney is unknown, but genetic transmission occurs in < 5% of cases.
Most patients are asymptomatic, and the disorder usually remains undiagnosed. It predisposes to calculus formation and UTI, so the most common presenting symptoms are the following:
Medullary sponge kidney is benign, and long-term prognosis is excellent. Obstruction by renal calculi may transiently reduce GFR and increase serum creatinine.
Diagnosis
The diagnosis is suspected in patients with recurrent calculi or UTIs or on the basis of incidental radiographic findings. Urinalysis typically shows evidence of incomplete distal renal tubular acidosis (overt metabolic acidosis is rare) and decreased urine-concentrating ability in patients without symptomatic polyuria.
Diagnosis is generally confirmed by CT, but IVU can be used. Ultrasonography is not helpful because cysts are small and located deep in the medulla.
Treatment
Treatment is indicated only for UTIs and for recurrent calculus formation. Thiazide diuretics (eg, hydrochlorothiazide 25 mg po bid) and high fluid intake inhibit calculus formation and may reduce incidence of obstructive complications in patients with recurrent calculi.
Last full review/revision July 2009 by Drew C. Cutler, MD
Content last modified July 2009
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