Approach

There is no specific treatment for medullary sponge kidney (MSK). Many patients have no symptoms, and the condition is normally benign. For patients with symptoms, treatment of MSK focuses on treating urinary tract infection (UTI), removing kidney stones, and preventing recurrent infections and stones. Hematuria is usually secondary to UTIs or stones, and the underlying causes should be investigated and treated.

With UTI

Medullary collecting duct plugs and nephrolithiasis in MSK may lead to UTI and pyelonephritis.[3] These patients require antibiotics. Fluoroquinolones are appropriate for the treatment of complicated UTIs (anatomic and functional abnormalities of the genitourinary tract), although the US Food and Drug Administration and the European Medicines Agency warn that fluoroquinolones are associated with disabling and potentially permanent side effects involving tendons, muscles, joints, nerves, and the central nervous system.[37][38][39] Other antibiotics (e.g., trimethoprim/sulfamethoxazole, nitrofurantoin) may be used based on local resistance patterns.[40] The decision whether to admit the patient for intravenous antibiotic treatment should be based on patients' symptoms and comorbidities. See Acute pyelonephritis.

The recommended treatment duration is 14 days. However, patients with uncomplicated UTI may require only a 7-day course of antibiotics.

With nephrolithiasis (renal stones)

Patients presenting with renal colic, renal stones, and MSK are managed in the same way as those without MSK.[3]

Prevention of recurrent stones may not be possible with the anatomic abnormality of MSK. However, risk factors identified through 24-hour urine stone risk profiles may be used to direct diet modification and medication.[3][41][42][43][44][45] One retrospective study showed potassium citrate not only increased urine citrate but decreased hypercalciuria in patients with metabolic stone risk factors, but the mechanisms were unclear.[45]

Fluid intake should be increased to more than 2 L/day to keep urine output high and reduce risk of both renal stones and UTI.[46] Thiazide diuretics may be used in patients with evidence of nephrocalcinosis to reduce hypercalciuria and stone formation.[47][48]

The urologic management of obstructing stones is not reviewed here. As endo-urologic practice evolves, the use of ureteroscopy and percutaneous nephrostolithotomy is supplanting extracorporeal shock wave lithotripsy.[49][50] See Nephrolithiasis.

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