Medullary sponge kidney - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Medullary sponge kidney (MSK) is a rare, developmental abnormality characterized by ectatic (dilatory) or cystic malformations in the medullary collecting ducts of the kidney resulting in medullary cysts.
Most patients are asymptomatic and the condition may be diagnosed based on incidental findings following radiologic investigation for other reasons.
May be present in 3% to 20% of patients with renal stones.
May present with flank pain, recurrent urinary tract infections (UTIs), hematuria (gross and microscopic), nephrolithiasis, and nephrocalcinosis.
No specific treatment, and for many patients the condition is normally benign. For patients with symptoms, treatment focuses on treating UTI, removing kidney stones, and preventing recurrent infections and stones.

Computed tomography (CT) urogram showing papillary blush with calculi within dilated collecting tubules (arrows)

From Maw AM, et al. Am J Kidney Dis. 2007 Jul;50(1):146-50, with permission

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Computed tomography (CT) urogram showing papillary blush with calculi within dilated collecting tubules (arrows)
Endoscopic images of renal papilla from medullary sponge kidney (MSK) stone formers. The affected papillae are characterized by a rounding of contours and enlargement, which creates a billowy appearance (a and b). As part of the general rounding of contours, the papillary tips are blunted. These papillae also show sites of white (panel a, arrowhead) and yellow (a, arrows) plaque, and dilated opening of ducts of Bellini with (c, double arrow) and without deposits (a, asterisk). An occasional calyceal stone is noted (c). The unaffected papillae possess a normal morphology as seen in the compound papillum in (d) and single papillum in (e) (case 5); note numerous sites of white plaque (arrowheads) and an attached stone (double arrowhead). The unique morphology of affected papillae of MSK patients can be characteristic of all papillae (diffuse pattern) of a kidney or only some of the papillae (segmental pattern)

Citations

Key Articles

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.[Abstract]
Imam TH, Patail H, Patail H. Medullary sponge kidney: current perspectives. Int J Nephrol Renovasc Dis. 2019;12:213-8.[Abstract][Full Text]
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.[Abstract]
Maw AM, Megibow AJ, Grasso M, et al. Diagnosis of medullary sponge kidney by computed tomographic urography. Am J Kidney Dis. 2007 Jul;50(1):146-50.[Abstract]
Pearle MS, Goldfarb DS, Assimos DG, et al; American Urological Association. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug(reviewed and validity confirmed 2019);192(2):316-24.[Abstract][Full Text]
Türk C, Neisius A, Petrik A, et al. EAU guidelines on urolithiasis. 2022 [internet publication].[Full Text]

Other Online Resources

NIDDK: medullary sponge kidney

Referenced Articles

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