Medullary Sponge Kidney with Distal Renal Tubular Acidosis: A Case Report and Review of the Literature

Medullary sponge kidney (MSK) is a rare disease, with a prevalence of 0.0002 – 0.0005%, characterized by cystic dilations of the collecting ducts. Patients with MSK have the propensity for recurrent formation of kidney stones, nephrocalcinosis and urinary tract infections (UTIs). Unfamiliar to many practitioners and commonly asymptomatic, MSK patients often remain undiagnosed. In addition to a comprehensive review of the literature published to date, we report a case of how we diagnosed MSK coexisting with distal renal tubular acidosis (dRTA) in an adult patient who presented with severe hypokalemia and hyperchloremic metabolic acidosis.