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Medullary sponge kidney is a disease characterized by precaliceal dilatation of the renal tubules. Clinical manifestations include recurrent kidney stones, urinary tract infections, hematuria, and back pain. Biological abnormalities, such as hypocitraturia, hypercalciuria, and hyperoxaluria, are most commonly associated with active and recurrent lithiasis. Diagnosis is challenging and based on imaging, formerly intravenous urography and now multidetector computed tomography urography or ureteroscopy. The pathophysiology is not well understood, with hypotheses suggesting disturbances in renal organogenesis. The genetic origin and congenital nature of the disease are suspected to be underlying causes. Treatment is primarily symptomatic and includes prophylactic dietary guidelines common to all nephrolithiasis patients. The natural course of the disease is unknown. Further data are needed to better characterize these patients.