eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Medullary Sponge Kidney: Differential Diagnoses & Workup

Author: Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Contributor Information and Disclosures

Updated: Nov 4, 2009

Differential Diagnoses

Polycystic Kidney Disease

Other Problems to Be Considered

Calyceal diverticulum
Papillary necrosis
Other causes of nephrocalcinosis

Workup

Laboratory Studies

In patients with medullary sponge kidney (MSK) and hemihypertrophy, serial screening should be performed to exclude malignancies, including abdominal tumors. The following studies are also indicated:

  • Urinalysis
    • Assessment of urinary calcium excretion
    • Urine culture
  • A 24-hour urine collection for calcium, magnesium, and citrate: Patients with medullary sponge kidney may have high excretion of lithogenic molecules or low excretion of urinary inhibitors of stone formation.

Imaging Studies

  • Intravenous pyelography reveals radial, linear striations in the papillae or cystic collections of contrast material in ectatic collecting ducts. The result is a characteristic blushlike pattern to the papillae, the so-called "bouquet of flowers" or "paintbrush" appearance. Typical cases involve all renal papillae but medullary sponge kidney may be unilateral or may affect only a few papillae. Declining use of intravenous pyelography as an imaging technique may result in underdiagnosis of medullary sponge kidney cases.
  • Renal ultrasonography and CT scanning are unnecessary except to distinguish medullary sponge kidney from papillary necrosis or autosomal dominant polycystic kidney disease.
  • The role of MRI is unknown.

Other Tests

  • Appropriate workup is needed if MSK appears to be associated with another condition, such as hemihypertrophy or pyloric stenosis, or is part of a syndrome, such as Marfan or Ehlers-Danlos. This testing should be performed to exclude the presence of a malignancy.

Procedures

  • No additional, specific, diagnostic procedures are warranted for diagnostic evaluation.

Histologic Findings

  • Neither renal biopsy nor nephrectomy is routinely performed.

More on Medullary Sponge Kidney

Overview: Medullary Sponge Kidney
Differential Diagnoses & Workup: Medullary Sponge Kidney
Treatment & Medication: Medullary Sponge Kidney
Follow-up: Medullary Sponge Kidney
Multimedia: Medullary Sponge Kidney
References
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References

  1. 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. Feb 2006;69(4):663-70. [Medline].

  2. 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. Sep 2009;24(9):2734-8. [Medline].

  3. Harris AM, Hall B, Kriss VM, Fowlkes JL, Kiessling SG. Rabson-Mendenhall syndrome: medullary sponge kidney, a new component. Pediatr Nephrol. Dec 2007;22(12):2141-4. [Medline].

  4. Abeshouse BS, Abeshouse GA. Sponge kidney: a review of the litrature and a report of five cases. J Urol. Aug 1960;84:252-67. [Medline].

  5. Avner ED. Medullary sponge kidney. In: Greenber A, Cheung AK, Coffman TM, et al, eds. NKF Primer on Kidney Disease. 1997.

  6. 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. [Medline].

  7. Patriquin HB, O'Regan S. Medullary sponge kidney in childhood. AJR Am J Roentgenol. Aug 1985;145(2):315-9. [Medline].

  8. [Guideline] Tiselius HG, Alken P, Buck C, Gallucci M, Knoll T, Sarica K, Turk C. Guidelines on urolithiasis. Arnhem, The Netherlands: European Association of Urology (EAU); 2008 Mar. [Full Text].

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Further Reading

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Keywords

medullary sponge kidney, MSK, medullary cysts, renal cyst, Beckwith-Wiedemann syndrome, BWS, hemihypertrophy, Caroli disease, Ehlers-Danlos syndrome, Marfan syndrome, Marfan's syndrome, pyloric stenosis, kidney stones, treatment, diagnosis

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Contributor Information and Disclosures

Author

Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Uri S Alon, MD, Director of Research and Education, Department of Pediatrics, Division of Pediatric Nephrology, Children's Mercy Hospital of Kansas City; Professor, University of Missouri at Kansas City
Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Frederick J Kaskel, MD, PhD, Director of the Division and Training Program in Pediatric Nephrology, Vice Chair, Department of Pediatrics, Montefiore Medical Center and Albert Einstein School of Medicine
Frederick J Kaskel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Eastern Society for Pediatric Research, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Renal Physicians Association, Sigma Xi, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Craig B Langman, MD, The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology
Disclosure: Amgen Grant/research funds None; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
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