Highlights & Basics
- Medullary sponge kidney (MSK) is a rare, developmental abnormality characterized 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 radiologic investigation for other reasons.
- May be present in 3% to 20% of patients with renal stones.
- May present with flank pain, recurrent urinary tract infections (UTIs), hematuria (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.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Computed tomography (CT) urogram showing papillary blush with calculi within dilated collecting tubules (arrows)
Endoscopic images of renal papilla from medullary sponge kidney (MSK) stone formers. The affected papillae are characterized by a rounding of contours and enlargement, which creates a billowy appearance (a and b). As part of the general rounding of contours, the papillary tips are blunted. These papillae also show sites of white (panel a, arrowhead) and yellow (a, arrows) plaque, and dilated opening of ducts of Bellini with (c, double arrow) and without deposits (a, asterisk). An occasional calyceal stone is noted (c). The unaffected papillae possess a normal morphology as seen in the compound papillum in (d) and single papillum in (e) (case 5); note numerous sites of white plaque (arrowheads) and an attached stone (double arrowhead). The unique morphology of affected papillae of MSK patients can be characteristic of all papillae (diffuse pattern) of a kidney or only some of the papillae (segmental pattern)
Citations
Forster JA, Taylor J, Browning AJ, et al. A review of the natural progression of medullary sponge kidney and a novel grading system based on intravenous urography findings. Urol Int. 2007;78(3):264-9.[Abstract]
Imam TH, Patail H, Patail H. Medullary sponge kidney: current perspectives. Int J Nephrol Renovasc Dis. 2019;12:213-8.[Abstract][Full Text]
Gambaro G, Feltrin GP, Lupo A, et al. Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s. Kidney Int. 2006 Feb;69(4):663-70.[Abstract]
Maw AM, Megibow AJ, Grasso M, et al. Diagnosis of medullary sponge kidney by computed tomographic urography. Am J Kidney Dis. 2007 Jul;50(1):146-50.[Abstract]
Pearle MS, Goldfarb DS, Assimos DG, et al; American Urological Association. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug(reviewed and validity confirmed 2019);192(2):316-24.[Abstract][Full Text]
Türk C, Neisius A, Petrik A, et al. EAU guidelines on urolithiasis. 2022 [internet publication].[Full Text]
1. Pyrah LN. Medullary sponge kidney. J Urol. 1966 Mar;95(3):274-83.[Abstract]
2. Forster JA, Taylor J, Browning AJ, et al. A review of the natural progression of medullary sponge kidney and a novel grading system based on intravenous urography findings. Urol Int. 2007;78(3):264-9.[Abstract]
3. Imam TH, Patail H, Patail H. Medullary sponge kidney: current perspectives. Int J Nephrol Renovasc Dis. 2019;12:213-8.[Abstract][Full Text]
4. Janjua MU, Long XD, Mo ZH, et al. Association of medullary sponge kidney and hyperparathyroidism with RET G691S/S904S polymorphism: a case report. J Med Case Rep. 2018 Jul 9;12(1):197.[Abstract][Full Text]
5. Pesce C, Colombo B, Nicolini E, et al. Medullary sponge kidney with severe renal function impairment: a case report [in Italian]. Pediatria Med Chir. 1995 Jan-Feb;17(1):65-7.[Abstract]
6. Kinoshita H. Clinical studies on medullary sponge kidney evaluated from urolithiasis [in Japanese]. Nippon Hinyokika Gakkai Zasshi. 1990 Mar;81(3):372-9.[Abstract]
7. Jungers P, Joly D, Barbey F, et al. ESRD caused by nephrolithiasis: prevalence, mechanisms, and prevention. Am J Kidney Dis. 2004 Nov;44(5):799-805.[Abstract]
8. Mayall GF. The incidence of medullary sponge kidney. Clin Radiol. 1970 Apr;21(2):171-4.[Abstract]
9. Palubinskas AJ. Medullary sponge kidney. Radiology. 1961 Jun;76:911-9.[Abstract]
10. Gambaro G, Feltrin GP, Lupo A, et al. Medullary sponge kidney (Lenarduzzi-Cacchi-Ricci disease): a Padua Medical School discovery in the 1930s. Kidney Int. 2006 Feb;69(4):663-70.[Abstract]
11. Parks JH, Coe FL, Strauss AL. Calcium nephrolithiasis and medullary sponge kidney in women. N Engl J Med. 1982 May 6;306(18):1088-91.[Abstract]
12. Pisani I, Giacosa R, Giuliotti S, et al. Ultrasound to address medullary sponge kidney: a retrospective study. BMC Nephrol. 2020 Oct 12;21(1):430.[Abstract][Full Text]
13. Ria P, Fabris A, Dalla Gassa A, et al. New non-renal congenital disorders associated with medullary sponge kidney (MSK) support the pathogenic role of GDNF and point to the diagnosis of MSK in recurrent stone formers. Urolithiasis. 2017 Aug;45(4):359-62.[Abstract]
14. Torregrossa R, Anglani F, Fabris A, et al. Identification of GDNF gene sequence variations in patients with medullary sponge kidney disease. Clin J Am Soc Nephrol. 2010 Jul;5(7):1205-10.[Abstract]
15. Little M, Georgas K, Pennisi D, et al. Kidney development: two tales of tubulogenesis. Curr Top Dev Biol. 2010;90:193-229.[Abstract]
16. Mezzabotta F, Cristofaro R, Ceol M, et al. Spontaneous calcification process in primary renal cells from a medullary sponge kidney patient harbouring a GDNF mutation. J Cell Mol Med. 2015 Apr;19(4):889-902.[Abstract][Full Text]
17. Fabris A, Lupo A, Ferraro PM, et al. Familial clustering of medullary sponge kidney is autosomal dominant with reduced penetrance and variable expressivity. Kidney Int. 2013 Feb;83(2):272-7.[Abstract][Full Text]
18. Diouf B, Ka EH, Calender A, et al. Association of medullary sponge kidney disease and multiple endocrine neoplasia type IIA due to RET gene mutation: is there a causal relationship? Nephrol Dial Transplant. 2000 Dec;15(12):2062-3.[Abstract][Full Text]
19. Higashihara E, Nutahara K, Tago K, et al. Medullary sponge kidney and renal acidification defect. Kidney Int. 1984 Feb;25(2):453-9.[Abstract][Full Text]
20. Lahme S, Bichler K-H, Lang F, et al. Metabolic evaluation of patients suffering from medullary sponge kidney. In: Borghi L, Meschi T, Briganti A, et al, eds. Proceedings of the 8th European Symposium on Urolithiasis. Parma, Italy: Editoriale Bios; 1999:599-601.
21. Osther PJ, Mathiasen H, Hansen AB, et al. Urinary acidification and urinary excretion of calcium and citrate in women with bilateral medullary sponge kidney. Urol Int. 1994;52(3):126-30.[Abstract]
22. Carboni I, Andreucci E, Caruso MR, et al. Medullary sponge kidney associated with primary distal renal tubular acidosis and mutations of the H+-ATPase genes. Nephrol Dial Transplant. 2009 Sep;24(9):2734-8.[Abstract][Full Text]
23. Yagisawa T, Kobayashi C, Hayashi T, et al. Contributory metabolic factors in the development of nephrolithiasis in patients with medullary sponge kidney. Am J Kidney Dis. 2001 Jun;37(6):1140-3.[Abstract]
24. Pabico RC, Mckenna BA, Freeman RB. Renal tubular dysfunction in patients with cystic disease of the kidneys. Urology. 1998 May;51(5A suppl):156-60.[Abstract]
25. Evan AP, Worcester EM, Williams JC Jr, et al. Biopsy proven medullary sponge kidney: clinical findings, histopathology, and role of osteogenesis in stone and plaque formation. Anat Rec (Hoboken). 2015 May;298(5):865-77.[Abstract]
26. O'Neill M, Breslau NA, Pak CY. Metabolic evaluation of nephrolithiasis in patients with medullary sponge kidney. JAMA. 1981 Mar 27;245(12):1233-6.[Abstract]
27. Jungers P, Lacour E, Daudon M. Nephrolithiasis in patients with medullary sponge kidney. In: Borghi L, Meschi T, Briganti A, et al, eds. Proceedings of the 8th European Symposium on Urolithiasis. Parma, Italy: Editoriale Bios; 1999:589-91.
28. McPhail EF, Gettman MT, Patterson DE, et al. Nephrolithiasis in medullary sponge kidney: evaluation of clinical and metabolic features. Urology. 2012 Feb;79(2):277-81.[Abstract]
29. Gambaro G, Goldfarb DS, Baccaro R, et al. Chronic pain in medullary sponge kidney: a rare and never described clinical presentation. J Nephrol. 2018 Aug;31(4):537-42.[Abstract]
30. Gaunay GS, Berkenblit RG, Tabib CH, et al. Efficacy of multi-detector computed tomography for the diagnosis of medullary sponge kidney. Curr Urol. 2018 Mar;11(3):139-43.[Abstract][Full Text]
31. Maw AM, Megibow AJ, Grasso M, et al. Diagnosis of medullary sponge kidney by computed tomographic urography. Am J Kidney Dis. 2007 Jul;50(1):146-50.[Abstract]
32. Koraishy FM, Ngo TT, Israel GM, et al. CT urography for the diagnosis of medullary sponge kidney. Am J Nephrol. 2014;39(2):165-70.[Abstract][Full Text]
33. Ekstrom T, Engfeldt B, Lagergren C, et al. Medullary sponge kidney: a roentgenologic, clinical, histopathological and biophysical study. Stockholm: Almquist and Wiksell; 1959.
34. Darmady EM, MacIver AG. Renal cystic disease. In: Crowford T, ed. Renal pathology. London: Butterworth and Co.; 1980:93-121.
35. Pearle MS, Goldfarb DS, Assimos DG, et al; American Urological Association. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug(reviewed and validity confirmed 2019);192(2):316-24.[Abstract][Full Text]
36. Türk C, Neisius A, Petrik A, et al. EAU guidelines on urolithiasis. 2022 [internet publication].[Full Text]
37. US Food and Drug Administration. FDA Drug Safety Communication: FDA updates warnings for oral and injectable fluoroquinolone antibiotics due to disabling side effects. 8 March 2018 [internet publication].[Full Text]
38. European Medicines Agency. Quinolone- and fluoroquinolone-containing medicinal products. 19 March 2019 [internet publication].[Full Text]
39. Medicines and Healthcare products Regulatory Agency (UK). Fluoroquinolone antibiotics: new restrictions and precautions for use due to very rare reports of disabling and potentially long-lasting or irreversible side effects. 21 March 2019 [internet publication].[Full Text]
40. Bader MS, Hawboldt J, Brooks A. Management of complicated urinary tract infections in the era of antimicrobial resistance. Postgrad Med. 2010 Nov;122(6):7-15.[Abstract]
41. Fabris A, Bernich P, Abaterusso C, et al. Bone disease in medullary sponge kidney and effect of potassium citrate treatment. Clin J Am Soc Nephrol. 2009 Dec;4(12):1974-9.[Abstract]
42. Barcelo P, Wuhl O, Servitge E, et al. Randomized double-blind study of potassium citrate in idiopathic hypocitraturic calcium nephrolithiasis. J Urol. 1993 Dec;150(6):1761-4.[Abstract]
43. Ettinger B, Tang A, Citron JT, et al. Randomized trial of allopurinol in the prevention of calcium oxalate calculi. N Engl J Med. 1986 Nov 27;315(22):1386-9.[Abstract]
44. Borghi L, Schianchi T, Meschi T, et al. Comparison of two diets for the prevention of recurrent stones in idiopathic hypercalciuria. N Engl J Med. 2002 Jan 10;346(2):77-84.[Abstract][Full Text]
45. Fabris A, Lupo A, Bernich P, et al. Long-term treatment with potassium citrate and renal stones in medullary sponge kidney. Clin J Am Soc Nephrol. 2010 Sep;5(9):1663-8.[Abstract]
46. Borghi L, Meschi T, Amato F, et al. Urinary volume, water and recurrences in idiopathic calcium nephrolithiasis: a 5-year randomized prospective study. 1996 Mar;155(3):839-43.[Abstract]
47. Laerum E, Larsen S. Thiazide prophylaxis of urolithiasis: a double-blind study in general practice. Acta Med Scand. 1984;215(4):383-9.[Abstract]
48. Finkielstein VA, Goldfarb DS. Strategies for preventing calcium oxalate stones. CMAJ. 2006 May 9;174(10):1407-9.[Abstract][Full Text]
49. Geavlete P, Nita G, Alexandrescu E, et al. The impact of modern endourological techniques in the treatment of a century old disease - medullary sponge kidney with associated nephrolithiasis. J Med Life. 2013;6(4):482-5.[Abstract][Full Text]
50. Sun H, Zhang Z, Yuan J, et al. Safety and efficacy of minimally invasive percutaneous nephrolithotomy in the treatment of patients with medullary sponge kidney. Urolithiasis. 2016 Oct;44(5):421-6.[Abstract]
51. Assimos D, Krambeck A, Miller NL, et al. Surgical management of stones: American Urological Association/Endourological Society Guideline, part I. J Urol. 2016 Oct;196(4):1153-60.[Abstract][Full Text]
52. Taylor EN, Stampfer MJ, Mount DB, et al. DASH-style diet and 24-hour urine composition. Clin J Am Soc Nephrol. 2010 Dec;5(12):2315-22.[Abstract][Full Text]
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