Medullary sponge kidney

Xiang, Hao; Han, Jason; Ridley, William E; Ridley, Lloyd J

doi:10.1111/1754-9485.40_12784

Cited by 7 publications

(9 citation statements)

References 8 publications

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“…This scenario was confirmed by the analysis of our cohort, in which intravenous urography was not available for review and CT was mainly performed without contrast medium (for a study of kidney stones) limiting its resolution power. The use of contrast medium-enhanced CT urography (CTU) with 3D volume-rendered imaging could be useful to diagnose MSK [10,13,14]. There are two studies published about using CTU to diagnose MSK.…”

Section: Discussionmentioning

confidence: 99%

“…Intravenous urography (IVU) is still considered the gold standard for the diagnosis of MSK. This technique reveals pathognomonic images of collection of contrast medium in dilated papillary ducts, giving the appearance of a blush or linear striations in the mildest cases or of a bouquet of flowers in full-blown cases [ 1 – 4 , 6 , 8 – 10 ]. However, as in patients with nephrolithiasis urography is now less used in favor of computed tomography (CT), the chances to diagnose MSK are likely reduced [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

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Ultrasound to address medullary sponge kidney: a retrospective study

et al. 2020

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Background Medullary sponge kidney (MSK) is a rare disease characterized by cystic dilatation of papillary collecting ducts. Intravenous urography is still considered the gold standard for diagnosis. We identified a cohort of patients from our outpatient clinic with established diagnosis of MSK to outline some ultrasonographic characteristics that may help establish a diagnosis. Methods We conducted a retrospective study of patients seen between January 1st 2009 and January 1st 2019 in our clinic. Out of 4321 patients, 18 had a diagnosis of MSK. We reviewed their clinical and family history, laboratory data and imaging studies. Specifically, we focused on ultrasound imaging. Results Patients were referred to our outpatient clinic because of renal impairment (44%), family history of nephropathy (17%), nephrolithiasis or an established diagnosis of MSK (39%). Seventy-two percent of patients presented with chronic kidney disease, 22% required hemodialysis. Urinary tract infections (44%), nephrolithiasis (33%), microscopic hematuria (50%) and proteinuria (44%) were reported. Seven patients underwent computed tomography; all of them received ultrasound. Ultrasound examination showed bilateral renal cysts, usually small and located in the renal medulla, and microcalcifications located in the medulla or within the cysts. Conclusion We identified a peculiar tetrad associated with MSK: 1) hypoechoic medullary areas, 2) hyperechoic spots, 3) microcystic dilatation of papillary zone, 4) multiple calcifications (linear, small stones or calcified intracystic sediment) in each papilla. The presence of this diagnostic tetrad, added to laboratory data and clinical history, could be helpful in the differential diagnosis to identify patients with MSK.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ultrasound to address medullary sponge kidney: a retrospective study

et al. 2020

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“…Medullary sponge kidney (MSK), first described in 1939, is a renal malformation characterized by mineral deposits in the ectatic precalyceal papillary collecting ducts, including terminal ducts of Bellini and inner medullary collecting ducts [1] , [2] , [3] , [4] . Pathologically, there are two distinct features that are observed.…”

Section: Introductionmentioning

confidence: 99%

Ambiguous clear cell carcinoma in medullary sponge kidney: A case report

Chen

Cheng

2020

Asian Journal of Urology

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Medullary sponge kidney (MSK) is a characteristic renal malformation, with a relatively low incidence. Radiologically, identification of MSK is sometimes ambiguous when compared to a renal mass. Here, we report a novel renal clear cell carcinoma in MSK, and discuss our approach to treatment. We recommended that a preoperative biopsy should be performed, followed by a comprehensive discussion regarding the appropriate perioperative preparations and careful surgical techniques that should be performed for this complex disease.

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“…Medullary sponge kidney (MSK) disease is a rare condition affecting the distal nephron within the renal medulla and typically presents itself with nephrocalcinosis, distal renal tubular acidosis, and hypocitraturia 1–3. MSK is defined as dilatation of medullary and papillary portions of the collecting ducts due to cystic damage to the distal nephron 4.…”

Section: Introductionmentioning

confidence: 99%

“…This rare renal malformation has been seen with other renal manifestations including Beckwith-Wiedemann syndrome, polycystic renal disease, Wilms Tumor, Horseshoe Kidney, Rabson-Mendenhall syndrome, Cakut syndrome, and Caroli syndrome 2,3,10,11. The genetic association between MSK and Caroli’s disease (CD) is known.…”

Section: Introductionmentioning

confidence: 99%

<p>Medullary Sponge Kidney: Current Perspectives</p>

Imam¹,

Patail²,

Patail³

2019

IJNRD

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Medullary Sponge Kidney (MSK) disease is a rare congenital malformation of the distal nephron where cystic dilatation is appreciable in the collecting ducts and renal papillae. Most cases of the malformation are thought to arise from a malfunction within neurotrophic factor and tyrosine kinase interactions. Presentation and prognosis are usually indolent; however, they include urinary tract infections (UTI), nephrolithiasis and nephrocalcinosis, distal renal tubular acidosis (dRTA) and hypocitraturia. With an insidious and asymptomatic onset, MSK is a difficult renal manifestation to both diagnose and treat. Difficulty diagnosing MSK today arises from clinical settings deviating from the usage of contrast methods when assessing the urogenital tract. Many healthcare standards for kidney disorders center diagnosis around imaging techniques rather than contrast methods. This ultimately leads to a decrease in the total number of confirmed cases of MSK. Though intra-venous urogram (IVU) remains as the current gold standard to diagnose MSK, other methods such as endoscopy and Multi-detector computed tomography (MDCT) are being put into place. Endoscopic examination and renal biopsy may allow definitive diagnosis; however, such invasive methods may be considered excessive. Moving forward, differential diagnoses for MSK can be made more precisely when patients present with other renal manifestations, especially in groups at risk. These groups include patients between the age of 20 and 30, patients with other renal malformations, high sodium diet patients, hyperparathyroid patients, and patients with family history of MSK. Basic treatment is aimed at controlling stone formation by stabilizing urinary pH. Treatment for patients, especially those prone to forming stones, includes the application of potassium citrate compounds, prophylactic water and diet control, surgical intervention or lithotripsy for removal of symptomatic kidney stones.

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Medullary sponge kidney

Cited by 7 publications

References 8 publications

Ultrasound to address medullary sponge kidney: a retrospective study

Ultrasound to address medullary sponge kidney: a retrospective study

Ambiguous clear cell carcinoma in medullary sponge kidney: A case report

<p>Medullary Sponge Kidney: Current Perspectives</p>

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