Medullary Sponge Kidney Treatment & Management

Updated: Aug 11, 2020
  • Author: Amit K Ghosh, MD, DM, FACP, MBA; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Treatment

Approach Considerations

Asymptomatic adults with medullary sponge kidney

Advise patients about the benign nature of medullary sponge kidney, and inform them about the possibility of renal calculi and urinary tract infections (UTIs) due to the condition. Obtaining periodic urinalysis and abdominal radiographs is recommended, although guidelines for the frequency of radiologic surveillance in asymptomatic adults are unclear.

Asymptomatic children with medullary sponge kidney

In asymptomatic children with medullary sponge kidney, conduct regular surveillance for Wilms tumor and other abdominal tumors.

Patients with medullary sponge kidney and recurrent nephrolithiasis

Patients are advised to drink plenty of fluids in order to have a daily urinary output in excess of 2L. A 24-hour urine collection for potential kidney stone risk factors (eg, calcium, citrate, uric acid, magnesium, sodium, oxalate, phosphate) can be very helpful in treating the metabolic factors contributing to nephrolithiasis. [2, 13]

Patients with medullary sponge kidney and distal RTA

Adjust the dosage and timing of potassium citrate supplementation to increase the urinary pH to a maximum of 7.0-7.2. Overalkalinization can lead to calcium phosphate precipitation and stone formation.

Patients with medullary sponge kidney and UTI

Medullary sponge kidney in these patients must be treated aggressively until the urine is clear. Proteus infection can lead to the formation of struvite stones and requires aggressive antibacterial therapy.

Prevention of stone formation

As previously mentioned, Fabris et al recommended that patients with medullary sponge kidney be screened using 24-hour urine collection to evaluate for stone risk factors (ie, hypocitraturia, hypercalciuria, hyperuricosuria, hyperoxaluria). [10] Patients who demonstrate abnormality in any of these test results should be considered for treatment with potassium citrate to prevent stone formation.

The starting dose of potassium citrate is 20 mEq/day. The dosage should be adjusted to maintain a urinary citrate level of 450 mg/day and a urinary pH of less than 7.5.

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Surgery

Patients with medullary sponge kidney and symptomatic nephrolithiasis can be treated with extracorporeal shock wave lithotripsy (ESWL), percutaneous surgery, or ureteroscopy. Partial nephrectomy must be performed in severe cases with segmental renal involvement.

In rare cases in which patients with medullary sponge kidney are suffering from severe urosepsis, unilateral nephrectomy is performed.

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Consultations

Urologic consultation may be essential in patients with medullary sponge kidney presenting with recurrent nephrolithiasis or acute urinary tract obstruction. Patients with congenital abnormalities associated with medullary sponge kidney may also need urologic surveillance.

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Diet

Patients with medullary sponge kidney and hypercalciuria should avoid a high-protein diet. Patients are advised to drink plenty of fluids in order to generate a daily urinary output in excess of 2 L.

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