Medullary Sponge Kidney - Lieberman's eRadiologyeradiology.bidmc.harvard.edu/LearningLab/genito/Mohan.pdf · Medullary Sponge Kidney ... of recurrent nephrolithiasis, presents to

Michael Mohan, HMS-IVGillian Lieberman, MD

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Hiding in Plain Sight: Hiding in Plain Sight: MedullaryMedullary Sponge KidneySponge Kidney

Michael Mohan, Harvard Medical School Year IVMichael Mohan, Harvard Medical School Year IVGillian Lieberman, MDGillian Lieberman, MD

September 17, 2010September 17, 2010


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Our patient: HistoryOur patient: History

Ms. C, a 49Ms. C, a 49--yearyear--old woman with a history old woman with a history of recurrent of recurrent nephrolithiasisnephrolithiasis, presents to the , presents to the emergency department with intermittent emergency department with intermittent flank pain.flank pain.She is She is afebrileafebrile and has no significant and has no significant findings on physical exam. Labs are findings on physical exam. Labs are notable for microscopic notable for microscopic hematuriahematuria..You suspect renal stone disease.You suspect renal stone disease.


33

Please continue for discussion…

What imaging would you What imaging would you order to further evaluate our order to further evaluate our

patient?patient?


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Choice of imaging in suspected Choice of imaging in suspected nephrolithiasisnephrolithiasis

NoncontrastNoncontrast CT:CT:–– The current standard of careThe current standard of care–– 95% sensitive and 98% specific for stone disease95% sensitive and 98% specific for stone disease–– Can look for dilation of the collecting system (Can look for dilation of the collecting system (hydronephrosis/hydroureterhydronephrosis/hydroureter) which may signal ) which may signal

partial or complete obstruction; may also allow diagnosis of a ppartial or complete obstruction; may also allow diagnosis of a passed stoneassed stone–– Accurate detection of stone size and location allows estimation Accurate detection of stone size and location allows estimation of likelihood of spontaneous of likelihood of spontaneous

passagepassage–– May see other May see other intraabdominalintraabdominal pathology in the differential diagnosispathology in the differential diagnosis

Ultrasound:Ultrasound:–– May be first line test in pregnant women or patients in whom May be first line test in pregnant women or patients in whom biliarybiliary and/or gynecologic and/or gynecologic

pathology are high on the differential pathology are high on the differential –– Excellent for diagnosing obstruction (Excellent for diagnosing obstruction (hydronephrosishydronephrosis, , hydroureterhydroureter, and , and perirenalperirenal fluid); fluid);

however, there is a time laghowever, there is a time lag–– Difficult to see Difficult to see ureteralureteral stonesstones

Abdominal plain film:Abdominal plain film:–– Will not see radiolucent stones Will not see radiolucent stones –– Cannot evaluate for obstructionCannot evaluate for obstruction–– Not used when CT is available except in patients with recurrent Not used when CT is available except in patients with recurrent radiopaqueradiopaque stonesstones

Intravenous Intravenous urogramurogram (IVU):(IVU):–– Previous gold standard, now replaced by Previous gold standard, now replaced by noncontrastnoncontrast CT which is more sensitive, avoids CT which is more sensitive, avoids

need for contrast, is more rapid, and can identify alternative dneed for contrast, is more rapid, and can identify alternative diagnosesiagnoses


55

Please continue to see the images…

You have read about the benefits and You have read about the benefits and drawbacks of different imaging modalities for drawbacks of different imaging modalities for suspected suspected nephrolithiasisnephrolithiasis. For our patient, the . For our patient, the

choice of imaging made in the emergency choice of imaging made in the emergency department was department was noncontrastnoncontrast CT.CT.


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Our patient: Please evaluate the Our patient: Please evaluate the noncontrastnoncontrast abdominal CTabdominal CT

PACS, BIDMCAxial C-

CT

PACS, BIDMCAxial C-

CT


77

Our patient: Please evaluate the Our patient: Please evaluate the noncontrastnoncontrast abdominal CTabdominal CT

PACS, BIDMCCoronal C-

CTPACS, BIDMCSagittal

C-

CT


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You have had a chance to You have had a chance to evaluate our patientevaluate our patient’’s abdominal s abdominal CT. CT. Please continue to see the conclusions of the radiologists

who read the film…


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Our patient: Official read of Our patient: Official read of noncontrastnoncontrast abdominal CTabdominal CT

No No hydronephrosishydronephrosisNo cysts or soft No cysts or soft tissue massestissue masses1.8 cm stone1.8 cm stone in the in the left renal pelvis, left renal pelvis, nonobstructingnonobstructingBilateral Bilateral nephrocalcinosisnephrocalcinosis (calcium deposition in (calcium deposition in renal renal perenchymaperenchyma and tubules)and tubules) PACS, BIDMC

Left kidney on axial C-

CT


1010

Q: In what part of our patientQ: In what part of our patient’’s s kidneys is the kidneys is the calcinosiscalcinosis??

PACS, BIDMCRight kidney on axial C-

CTPACS, BIDMC

Left kidney on axial C-

CT


1111

Let us review renal anatomy to Let us review renal anatomy to help illustrate the location of our help illustrate the location of our

patientpatient’’s s nephrocalcinosisnephrocalcinosis……


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Renal anatomyRenal anatomy

Kidneys and Urinary Tract. Kidneys and Urinary Tract. MedcyclopaediaMedcyclopaedia..http://www.medcyclopaedia.com/library/radiology/chapter25/25_2.ahttp://www.medcyclopaedia.com/library/radiology/chapter25/25_2.aspx.spx.

Internal Kidney Structure. Internal Kidney Structure. TutorVista.comTutorVista.com. http://. http://www.tutorvista.comwww.tutorvista.com/topic/internal/topic/internal--kidneykidney--structure.structure.


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Our patientOur patient’’s s nephrocalcinosisnephrocalcinosis is is localized to the medulla. Please localized to the medulla. Please

continue to view our patientcontinue to view our patient’’s s images next to a diagram of images next to a diagram of

renal anatomy to illustrate the renal anatomy to illustrate the medullarymedullary location of our location of our

patientpatient’’s s nephrocalcinosisnephrocalcinosis……


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Our patient: Our patient: MedullaryMedullary calcinosiscalcinosis on axial CTon axial CT

Internal Kidney Structure. Internal Kidney Structure. TutorVista.comTutorVista.com. http://. http://www.tutorvista.comwww.tutorvista.com/topic/internal/topic/internal--kidneykidney--structure.structure. PACS, BIDMCAcial

C-

CT


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Our patient: Our patient: MedullaryMedullary calcinosiscalcinosis on plain filmon plain film

Internal Kidney Structure. Internal Kidney Structure. TutorVista.comTutorVista.com. http://. http://www.tutorvista.comwww.tutorvista.com/topic/internal/topic/internal--kidneykidney--structure.structure.

PACS, BIDMCFrontal abdominal plain film


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Our patient: Our patient: MedullaryMedullary calcinosiscalcinosis on on plain filmplain film




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Our patient: Our patient: MedullaryMedullary calcinosiscalcinosis on abdominal CT with contraston abdominal CT with contrast

PACS, BIDMCSagittal

C+ CTPACS, BIDMCCoronal C+ CT


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You have seen images of You have seen images of our patientour patient’’s s medullarymedullary calcinosiscalcinosis (also called (also called

medullarymedullary nephrocalcinosisnephrocalcinosis). ). What is the differential What is the differential

diagnosis for this condition?diagnosis for this condition?

Please continue for a discussion…


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MedullaryMedullary nephrocalcinosisnephrocalcinosis: : Differential diagnosisDifferential diagnosis

HyperparathyroidismHyperparathyroidismMedullaryMedullary sponge kidneysponge kidneyDistal renal tubular acidosisDistal renal tubular acidosisLess commonly:Less commonly:–– MilkMilk--alkali syndromealkali syndrome–– HypervitaminosisHypervitaminosis DD–– SarcoidosisSarcoidosis–– OthersOthers PACS, BIDMC

Frontal abdominal plain film


2020

We have seen the differential We have seen the differential diagnosis for diagnosis for medullarymedullary calcinosiscalcinosis. .

Our patient has no electrolyte Our patient has no electrolyte abnormalities and her abnormalities and her

nephrocalcinosisnephrocalcinosis is longstanding. is longstanding. She has a diagnosis of She has a diagnosis of medullarymedullary

sponge kidney (MSK)sponge kidney (MSK)..

Please continue to learn more about medullary sponge kidney…


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MedullaryMedullary Sponge Kidney: FactsSponge Kidney: Facts

Irregular dilatations of the Irregular dilatations of the medullarymedullary and papillary collecting ducts (cortex is and papillary collecting ducts (cortex is spared)spared)Congenital but family history usually Congenital but family history usually absent; exact etiology unknownabsent; exact etiology unknownCommon but frequency unknown because Common but frequency unknown because usually benign and discovered incidentally usually benign and discovered incidentally 70% bilateral at diagnosis, usually affects 70% bilateral at diagnosis, usually affects all papillae of an affected kidneyall papillae of an affected kidney


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MedullaryMedullary Sponge Kidney: Clinical Sponge Kidney: Clinical ManifestationsManifestations

Usually asymptomatic and found Usually asymptomatic and found incidentally on abdominal imagingincidentally on abdominal imagingUrinary stasis and perhaps impaired Urinary stasis and perhaps impaired calcium calcium resorptionresorption predispose to predispose to nephrolithiasisnephrolithiasis–– MSK found in 12MSK found in 12--20% of patients with stones20% of patients with stonesStasis in cysts predisposes to urinary tract Stasis in cysts predisposes to urinary tract infections (infections (UTIsUTIs))May give microscopic or gross May give microscopic or gross hematuriahematuria


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MedullaryMedullary Sponge Kidney: Sponge Kidney: Management and PrognosisManagement and Prognosis

Treatment is symptomatic:Treatment is symptomatic:–– Drink fluids to prevent stonesDrink fluids to prevent stones–– Treat Treat UTIsUTIsPrognosis is good Prognosis is good –– renal function renal function unaffected unless recurrent stones or unaffected unless recurrent stones or UTIsUTIs


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You have learned about the You have learned about the clinical presentation and clinical presentation and

management of management of medullarymedullary sponge sponge kidney. Please continue to learn kidney. Please continue to learn

about the characteristic features of about the characteristic features of medullarymedullary sponge kidney on sponge kidney on various imaging modalitiesvarious imaging modalities……


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Companion patients 1 and 2: Appearance of Companion patients 1 and 2: Appearance of MSK on plain filmMSK on plain film

MedullaryMedullary calcinosiscalcinosis or clusters of or clusters of pericalycealpericalyceal stonesstones (in the (in the medullarymedullary tubules)tubules)Appearance on plain film is nonspecificAppearance on plain film is nonspecific

Tanagho

EA, McAninch

JW: Smiths’s General Urology, 17th

Edition:http://www.accessmedicine.com


Fauci

AS et al.: Harrison’s Principles of Internal Medicine, 17th

Edition.http://www.accessmedicine.com


Companion patient 1 Companion patient 2


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HyperechoicHyperechoic medullarymedullary region due to calcificationsregion due to calcificationsPyramids often dilated but pelvis and calyces normalPyramids often dilated but pelvis and calyces normalAlso nonspecificAlso nonspecific

Khan AN, MacDonald S, Chandramohan

M, Chandramohan

H. Nephrocalcinosis

Imaging. July 10, 2008.Online at http://emedicine.medscape.com/article/379449-imaging

Transabdominal

ultrastound

Companion patient 3: Appearance Companion patient 3: Appearance of MSK on ultrasoundof MSK on ultrasound

Companion patient 3


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PyelogramPyelogram phase most useful phase most useful –– shows collecting systemshows collecting systemAccumulation of contrast in distended collecting ducts of Accumulation of contrast in distended collecting ducts of medullarymedullary pyramids seen as pyramids seen as ““brushbrush”” or or ““blushblush”” appearanceappearance radiating outward from calycesradiating outward from calyces

Dunnick

RN, McCallum RW, Sandler CM: Textbook of Uroradiology. Williams & Wilkins, 1991. Frontal view intravenous urogram

Rose, BD, Pathophysiology of Renal Disease, 2nd

Ed, McGraw-Hill, 1987.Frontal view intravenous urogram

Companion patients 4 and 5: Appearance of Companion patients 4 and 5: Appearance of MSK on intravenous MSK on intravenous urogramurogram

Companion patient 4 Companion patient 5


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Without contrast: Without contrast: medullarymedullary calcinosiscalcinosis or or pericalycealpericalyceal stonesstonesWith contrast: may see characteristic With contrast: may see characteristic ““blushblush”” pattern pattern from from ectaticectatic collecting ducts collecting ducts

Fauci

AS et al.: Harrison’s Principles of Internal Medicine, 17th

Edition: http://www.accessmedicine.comAxial C-

CT

Companion patient 6: Appearance Companion patient 6: Appearance of MSK on CTof MSK on CT

Companion patient 6

http://www.accessmedicine.com/


2929

You have learned about the You have learned about the characteristic imaging features of characteristic imaging features of medullarymedullary sponge kidney. Let us sponge kidney. Let us return now to our patient Ms. C to return now to our patient Ms. C to

see how she is doingsee how she is doing……


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Our patient: Clinical courseOur patient: Clinical course

No obstructing stone found on imagingNo obstructing stone found on imagingHowever, she was found to have a UTI, However, she was found to have a UTI, with 50,000with 50,000--100,000 colonies/100,000 colonies/mLmL Pseudomonas Pseudomonas aeruginosaaeruginosa on urine cultureon urine culturePredisposed to Predisposed to UTIsUTIs by by medullarymedullary sponge sponge kidney, has had many kidney, has had many UTIsUTIs in the pastin the pastCurrently undergoing treatment with Currently undergoing treatment with antibiotics antibiotics


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ConclusionsConclusions

NoncontrastNoncontrast CT is generally the first line CT is generally the first line test for suspected test for suspected nephrolithiasisnephrolithiasisMedullaryMedullary sponge kidney is common and sponge kidney is common and predisposes to recurrent stones as well as predisposes to recurrent stones as well as UTIsUTIsMedullaryMedullary sponge kidney is often sponge kidney is often diagnosed when diagnosed when medullarymedullary calcinosiscalcinosis is is found incidentallyfound incidentallyOther common causes of Other common causes of medullarymedullary calcinosiscalcinosis are hyperparathyroidism and are hyperparathyroidism and renal tubular acidosisrenal tubular acidosis


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AcknowledgementsAcknowledgements

Dr. Gillian Lieberman, Course DirectorDr. Gillian Lieberman, Course DirectorDr. Rich Dr. Rich RanaRana, Senior Resident in , Senior Resident in RadiologyRadiologyEmily Hanson, Educational CoordinatorEmily Hanson, Educational CoordinatorLarry Larry BarbarasBarbaras, Webmaster, Webmaster


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ReferencesReferencesBaumgartenBaumgarten DA, Francis IR, DA, Francis IR, CasalinoCasalino DD, Arellano RS, Curry NS, DD, Arellano RS, Curry NS, DigheDighe M, M, FulghamFulgham P, Israel P, Israel GM, GM, LeyendeckerLeyendecker JR, JR, PapanicolaouPapanicolaou N, Prasad S, N, Prasad S, RamchandaniRamchandani P, Remer EM, P, Remer EM, ShethSheth S. ACR S. ACR Appropriateness Criteria: Acute onset flank pain Appropriateness Criteria: Acute onset flank pain –– suspicion of stone disease. suspicion of stone disease. American College American College of Radiologyof Radiology. (2008) Online at . (2008) Online at Acr.orgAcr.org..CurhanCurhan GC, Aronson MD, Preminger GM, Goldfarb S, OGC, Aronson MD, Preminger GM, Goldfarb S, O’’Leary MP, Post TW. Diagnosis and acute Leary MP, Post TW. Diagnosis and acute management of suspected management of suspected nephrolithiasisnephrolithiasis in adults. in adults. UpUp--toto--Date OnlineDate Online, April 30 2010., April 30 2010.DalrympleDalrymple NC, NC, VergaVerga M, Anderson KR, M, Anderson KR, BoveBove P, Covey AM, P, Covey AM, RosenfieldRosenfield AT, Smith RC. The value AT, Smith RC. The value of unenhanced helical computerized tomography in the management of unenhanced helical computerized tomography in the management of acute flank pain. of acute flank pain. J J UrolUrol 1998 Mar;159(3):7351998 Mar;159(3):735--40.40.DharDhar M, M, DenstedtDenstedt JD. Imaging in diagnosis, treatment, and followJD. Imaging in diagnosis, treatment, and follow--up of stone patients. up of stone patients. Adv Adv Chronic Kidney Chronic Kidney DisDis 2009 Jan;16(1):392009 Jan;16(1):39--47.47.DunnickDunnick RN, McCallum RW, Sandler CM. RN, McCallum RW, Sandler CM. Textbook of Textbook of UroradiologyUroradiology. (1991) Williams and Wilkins. . (1991) Williams and Wilkins. McAninchMcAninch JW. JW. ““Chapter 31. Disorders of the Kidneys.Chapter 31. Disorders of the Kidneys.”” In In TanaghoTanagho EA, EA, McAninchMcAninch JW: JW: Smith's Smith's General UrologyGeneral Urology, 17, 17thth Edition. (2007) Edition. (2007) Accessmedicine.comAccessmedicine.com..NovellineNovelline RA. RA. Fundamentals of RadiologyFundamentals of Radiology, 6, 6thth Edition. (2004) Harvard University Press.Edition. (2004) Harvard University Press.Rose, BD. Rose, BD. Pathophysiology of Renal DiseasePathophysiology of Renal Disease, 2, 2ndnd Edition. (1987) McGrawEdition. (1987) McGraw--Hill.Hill.Rose BD, Goldfarb S, Post TW. Rose BD, Goldfarb S, Post TW. MedullaryMedullary Sponge Kidney. Sponge Kidney. UpUp--toto--Date OnlineDate Online, February 9 2010., February 9 2010.SalantSalant DJ, Patel PS. DJ, Patel PS. ““Chapter 278. Polycystic Kidney Disease and Other Inherited TubulChapter 278. Polycystic Kidney Disease and Other Inherited Tubular ar Disorders.Disorders.”” In In FauciFauci AS, AS, BraunwaldBraunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, E, Kasper DL, Hauser SL, Longo DL, Jameson JL, LoscalzoLoscalzo J: J: Harrison's Principles of Internal MedicineHarrison's Principles of Internal Medicine, 17, 17thth Edition. (2008) Edition. (2008) Accessmedicine.comAccessmedicine.com. . WatnickWatnick S, Morrison G. S, Morrison G. ““Chapter 22. Kidney Disease.Chapter 22. Kidney Disease.”” In In McPheeMcPhee SJ, SJ, PapadakisPapadakis MA, Tierney LM, MA, Tierney LM, Jr.: Jr.: Current Medical Diagnosis and Treatment 2010Current Medical Diagnosis and Treatment 2010. (2010) . (2010) Accessmedicine.comAccessmedicine.com..

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Medullary Sponge Kidney - Lieberman's eRadiologyeradiology.bidmc.harvard.edu/LearningLab/genito/Mohan.pdf · Medullary Sponge Kidney ... of recurrent nephrolithiasis, presents to