Detailed Nephro-urological Management of a Case of Medullary Sponge Kidney with Distal Renal Tubular Acidosis and Obstructive Uropathy.

Medullary sponge kidney (MSK) is a rare renal malformation characterized by precalyceal tubular ectasia of the renal collecting ducts that clinically manifests as recurrent renal stones, distal renal tubular acidosis (dRTA), osteoporosis, and nephrocalcinosis. In this case report, we present the case of a woman with a severe form of MSK associated with dRTA. She had extensive nephrocalcinosis and obstructive uropathy caused by a large upper ureteric stone in the left kidney. The stone was disintegrated by flexible ureteroscopic laser lithotripsy. Her initial biochemical derangements were identified and corrected with a Polycitra-K solution and hydrochlorothiazide, leading to reduced stone load and osteopenia 1 year later.