Rare, developmental abnormality characterised by ectatic (dilatory) or cystic malformations in the medullary collecting ducts of the kidney resulting in medullary cysts.
Most patients are asymptomatic and the condition may be diagnosed based on incidental findings following radiological investigation for other reasons.
May be present in 3% to 25% of patients with renal stones.
May present with flank pain, recurrent urinary tract infections (UTIs), haematuria (gross and microscopic), nephrolithiasis, and nephrocalcinosis.
No specific treatment, and for many patients the condition is normally benign. For patients with symptoms, treatment focuses on treating UTI, removing kidney stones, and preventing recurrent infections and stones.
Medullary sponge kidney (MSK) is a rare, developmental abnormality that can occur in 1 or both kidneys, characterised by ectatic or cystic malformations in the medullary collecting ducts. The medullary cysts can range from 1 to 7 mm in diameter. Their presence gives the medulla a sponge-like appearance. Most patients are asymptomatic.
History and exam
Key diagnostic factors
- presence of risk factors
Other diagnostic factors
- previous nephrolithiasis
- flank pain
- costovertebral angle tenderness
- urinary urgency
- increased frequency of urination
- groin pain
- Beckwith-Wiedemann's syndrome
- Caroli's disease
- congenital hepatic fibrosis
- Ehlers-Danlos' syndrome
- family history of polycystic kidney disease
- chronic kidney disease
- female sex
1st investigations to order
- urine culture
- urea and creatinine
- serum electrolytes
- abdominal radiography
- renal ultrasound
- intravenous urography (IVU)
- non-contrast CT scan
- CT urogram
Investigations to consider
- 24-hour urine monitoring
- stone analysis
with urinary tract infection (UTI)
Michael J. Choi, MD
Associate Professor of Medicine
Clinical Director of Nephrology
Johns Hopkins University School of Medicine
MJC declares that he has no competing interests.
David S. Goldfarb, MD
Division of Nephrology
New York University School of Medicine
New York VA Medical Center
DSG declares that he has no competing interests.
Dr Michael J. Choi and Dr David S. Goldfarb would like to gratefully acknowledge Dr Amaka Edeani, a previous contributor to this monograph. AE declares that she has no competing interests.
Khashayar Sakhaee, MD
Internal Medicine Physician
UT Southwestern Medical Center
KS declares that he has no competing interests.
Eric N. Taylor, MD, MSc
Assistant Professor of Medicine
Harvard Medical School
Brigham and Women's Hospital
ENT declares that he has no competing interests.
- Distal renal tubular acidosis
- Renal papillary necrosis
- Tuberculosis of kidney
- Guidelines on urolithiasis
- Surgical management of stones: AUA/Endourological Society guideline
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