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Medullary Sponge Kidney Treatment & Management

  • Author: Amit K Ghosh, MD, DM, FACP, MBA; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
 
Updated: Mar 06, 2014
 

Approach Considerations

Asymptomatic adults with medullary sponge kidney

Advise patients about the benign nature of medullary sponge kidney, and inform them about the possibility of renal calculi and urinary tract infections (UTIs) due to the condition. Obtaining periodic urinalysis and abdominal radiographs is recommended, although guidelines for the frequency of radiologic surveillance in asymptomatic adults are unclear.

Asymptomatic children with medullary sponge kidney

In asymptomatic children with medullary sponge kidney, conduct regular surveillance for Wilms tumor and other abdominal tumors.

Patients with medullary sponge kidney and recurrent nephrolithiasis

Patients are advised to drink plenty of fluids in order to have a daily urinary output in excess of 2L. A 24-hour urine collection for potential kidney stone risk factors (eg, calcium, citrate, uric acid, magnesium, sodium, oxalate, phosphate) can be very helpful in treating the metabolic factors contributing to nephrolithiasis.[1, 10]

Patients with medullary sponge kidney and distal RTA

Adjust the dosage and timing of potassium citrate supplementation to increase the urinary pH to a maximum of 7.0-7.2. Overalkalinization can lead to calcium phosphate precipitation and stone formation.

Patients with medullary sponge kidney and UTI

Medullary sponge kidney in these patients must be treated aggressively until the urine is clear. Proteus infection can lead to the formation of struvite stones and requires aggressive antibacterial therapy.

Prevention of stone formation

As previously mentioned, Fabris et al advised that patients with medullary sponge kidney be screened using 24-hour urine collection to evaluate for stone risk factors (ie, hypocitraturia, hypercalciuria, hyperuricosuria, hyperoxaluria).[8] Patients who demonstrate abnormality in any of these test results should be considered for treatment with potassium citrate to prevent stone formation. The starting dose of potassium citrate is 20 mEq/day and should be adjusted to keep a urinary citrate level of 450 mg/day and a urinary pH level of less than 7.5.

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Surgery

Patients with medullary sponge kidney and symptomatic nephrolithiasis can be treated with extracorporeal shock wave lithotripsy (ESWL), percutaneous surgery, or ureteroscopy. Partial nephrectomy must be performed in severe cases with segmental renal involvement.

In rare cases in which patients with medullary sponge kidney are suffering from severe urosepsis, unilateral nephrectomy is performed.

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Consultations

Urologic consultation may be essential in patients with medullary sponge kidney presenting with recurrent nephrolithiasis or acute urinary tract obstruction. Patients with congenital abnormalities associated with medullary sponge kidney may also need urologic surveillance.

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Diet

Patients with medullary sponge kidney and hypercalciuria should avoid a high-protein diet. Patients are advised to drink plenty of fluids in order to generate a daily urinary output in excess of 2L.

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Contributor Information and Disclosures
Author

Amit K Ghosh, MD, DM, FACP, MBA Professor of Medicine, Mayo Medical School; Consultant in General Internal Medicine, Mayo Clinic

Amit K Ghosh, MD, DM, FACP, MBA is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, Minnesota Medical Association, Society of General Internal Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Karthik Ghosh, MD Consultant, Associate Professor of Medicine, Department of Internal Medicine, Mayo Medical School

Karthik Ghosh, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Acknowledgements

Eleanor Lederer, MD Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD is a member of the following medical societies: American Association for the Advancement of Science, American Federation for Medical Research, American Society for Biochemistry and Molecular Biology, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation, and Phi Beta Kappa

Disclosure: Dept of Veterans Affairs Grant/research funds Research

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. Fick GM, Gabow PA. Hereditary and acquired cystic disease of the kidney. Kidney Int. 1994 Oct. 46(4):951-64. [Medline].

  2. 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. [Medline].

  3. 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. [Medline].

  4. 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. [Medline].

  5. Levine E, Hartman DS, Meilstrup JW, et al. Current concepts and controversies in imaging of renal cystic diseases. Urol Clin North Am. 1997 Aug. 24(3):523-43. [Medline].

  6. Palubinskas AJ. Renal pyramid structure opacification in excretory urography and its relation to medullary sponge kidney. Radiology. 1963 Dec. 81:963-70. [Medline].

  7. Higashihara E, Nutahara K, Tago K, et al. Medullary sponge kidney and renal acidification defect. Kidney Int. 1984 Feb. 25(2):453-9. [Medline].

  8. 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. [Medline].

  9. Lang EK, Macchia RJ, Thomas R, et al. Improved detection of renal pathologic features on multiphasic helical CT compared with IVU in patients presenting with microscopic hematuria. Urology. 2003 Mar. 61(3):528-32. [Medline].

  10. McPhail EF, Gettman MT, Patterson DE, Rangel LJ, Krambeck AE. Nephrolithiasis in Medullary Sponge Kidney: Evaluation of Clinical and Metabolic Features. Urology. 2011 Oct 17. [Medline].

 
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IVU-like, volume-rendered (VR) computed tomography (CT) scan 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.
Unenhanced coronal, volume-rendered (VR) computed tomography (CT) scan of the kidneys demonstrates 2 small calculi in the midportion 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 scans. Image courtesy of Dr. Terri J. Vrtiska, Consultant, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
Table 1. Clinical Features of Medullary Sponge Kidney and Etiologic Correlation
Frequency Clinical Findings Etiology
Common* Nephrolithiasis (calcium oxalate, calcium apatite) Hypercalciuria



Increased oxalate concentration



Tubular acidification defects



Hypocitraturia



Hematuria (gross 10-20%, microscopic) Acute pelvic obstruction



UTI, renal stones, or absence of both



UTI Sterile pyuria common even in absence of stones



Presence of renal stones



Rare Chronic kidney disease Repeated urinary obstruction



Repeated pyelonephritis due to urease-producing organisms (Proteus)



*Asymptomatic
Table 2. Radiologic Appearance in Medullary Sponge Kidney
Radiologic Test Appearance
Plain radiograph Normal or enlarged kidney



Medullary nephrocalcinosis indicated by isolated, single or multiple precaliceal concretions or clusters of grapelike calcifications



Excretory urogram* Papillary blush



Faint pyramidal striation



Papillary streaking or brushlike appearance



Precaliceal tubular dilatation filled with contrast



Papillary blush and multiple precaliceal dilatation



Bouquet of flowers



CT scan Papillary calcification



Hyperdense papilla



Ectasia of precaliceal tubules



*Findings limited to medullary pyramids
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