Pediatric Medullary Sponge Kidney Workup

Author: Howard Trachtman, MD; Chief Editor: Craig B Langman, MD more...

Updated: Mar 27, 2012

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Laboratory Studies
Imaging Studies
Other Tests
Procedures
Histologic Findings
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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 (ie, 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 medullary sponge kidney 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 syndrome. 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.

Proceed to Treatment & Management

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.

Specialty Editor Board

Uri S Alon, MD Director of Bone and Mineral Disorders Clinic and Renal Research Laboratory, Children's Mercy Hospital of Kansas City; Professor, Department of Pediatrics, Division of Pediatric Nephrology, University of Missouri-Kansas City School of Medicine

Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

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, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, Children's Memorial Hospital

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: NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

References

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].
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].
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. Jul 2010;5(7):1205-10. [Medline].
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].
Pritchard MJ. Medullary sponge kidney: causes and treatments. Br J Nurs. Aug 12-Sep 8 2010;19(15):972-6. [Medline].
[Best Evidence] McPhail EF, Gettman MT, Patterson DE, Rangel LJ, Krambeck AE. Nephrolithiasis in medullary sponge kidney: evaluation of clinical and metabolic features. Urology. Feb 2012;79(2):277-81. [Medline].
[Best Evidence] Lorenz EC, Lieske JC, Vrtiska TJ, et al. Clinical characteristics of potential kidney donors with asymptomatic kidney stones. Nephrol Dial Transplant. Aug 2011;26(8):2695-700. [Medline]. [Full Text].
Fabris A, Bernich P, Abaterusso C, Marchionna N, Canciani C, Nouvenne A. Bone disease in medullary sponge kidney and effect of potassium citrate treatment. Clin J Am Soc Nephrol. Dec 2009;4(12):1974-9. [Medline].
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. Sep 2010;5(9):1663-8. [Medline].
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].
Avner ED. Medullary sponge kidney. In: Greenber A, Cheung AK, Coffman TM, et al, eds. NKF Primer on Kidney Disease. 1997.
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].
Patriquin HB, O'Regan S. Medullary sponge kidney in childhood. AJR Am J Roentgenol. Aug 1985;145(2):315-9. [Medline].
[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].

Unenhanced coronal volume-rendered (VR) CT image of the kidneys demonstrates 2 small calculi in the mid portion of the right kidney and 2 small calculi in the lower pole of the left kidney (arrowheads). A large low-density lesion in the lower pole of the right kidney and a small low-density lesion in the upper pole of the left kidney (short arrows) were shown to represent benign simple renal cysts on the contrast enhanced CT images. Image courtesy of Dr. Terri J. Vrtiska, Consultant, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

CT image of both kidneys demonstrates brushlike densities throughout multiple papillae of both kidneys consistent with renal tubular ectasia. Correlation of the stone disease with the ectatic tubules is diagnostic of medullary sponge kidney. Image courtesy of Dr. Terri J. Vrtiska, Consultant, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

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