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Medullary Sponge Kidney Clinical Presentation

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

History

Patients with medullary sponge kidney often are asymptomatic. Not infrequently, the diagnosis is made during radiologic investigations, including abdominal radiography and excretory pyelography, performed for other clinical situations.[5, 6]

Hematuria is frequent, and gross hematuria may be present in 10-20% of cases. Gross hematuria usually results from pelvic obstruction due to stones. Microscopic hematuria may be present with or without UTI. (See Table 1, below.)

Renal stones also result in UTI, urinary obstruction, and nephrocalcinosis. The stones in medullary sponge kidney are usually composed of calcium phosphate (apatite) and calcium oxalate. (See Table 1, below.)

UTI is common in medullary sponge kidney, with and without nephrolithiasis. Sterile pyuria also is common. Patients with medullary sponge kidney have more UTIs than other patients with nephrolithiasis, and the incidence of urinary infection is higher in women than in men. (See Table 1, below.)

Recurrent nephrolithiasis is a major complication of medullary sponge kidney. The most common presenting symptom of this complication is renal colic, often accompanied by hematuria. (See Table 1, below.)

Table 1. Clinical Features of Medullary Sponge Kidney and Etiologic Correlation (Open Table in a new window)

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

Medullary sponge kidney can occur in conjunction with other congenital abnormalities, as follows:

  • Anodontia
  • Autosomal dominant polycystic kidney
  • Beckwith-Wiedemann syndrome
  • Caroli syndrome
  • Congenital hemihypertrophy - As many as 10% of patients with hemihypertrophy have medullary sponge kidney, and as many as 25% of patients with medullary sponge kidney have hemihypertrophy
  • Congenital pyloric stenosis
  • Distal renal tubular acidosis
  • Ehlers-Danlos syndrome
  • Horseshoe kidney
  • Marfan syndrome
  • Parathyroid adenomas
  • Renal artery stenosis
  • Ureteral duplication

When associated with medullary sponge kidney, Beckwith-Wiedemann syndrome (high birth weight, macroglossia, omphalocele, visceromegaly, mental retardation, cysts in the adrenal cortex, enlarged kidneys, medullary sponge kidney, and hemihypertrophy) has a high tumor rate, especially Wilms tumor, adrenal gland cancer, and hepatoblastoma.

If any suggestion of a history of medullary sponge kidney in other family members exists, further investigations may be indicated to unravel the genetic pattern of transmission.

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Physical Examination

The following may be encountered on physical examination:

  • No physical findings are usually present except for hematuria
  • Renal colic may occur
  • Costovertebral angle tenderness occurs in cases of pyelonephritis or ureteral obstruction by a calculus
  • Hemihypertrophy is present in 25% of cases
  • Other signs of associated congenital disorders may be present (as listed in the History section)
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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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