Ultrasound to address medullary sponge kidney: a retrospective study
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RESEARCH ARTICLE
Open Access
Ultrasound to address medullary sponge kidney: a retrospective study Isabella Pisani1* , Roberto Giacosa2, Sara Giuliotti3, Dario Moretto1, Giuseppe Regolisti1, Chiara Cantarelli1, Augusto Vaglio4,5, Enrico Fiaccadori1 and Lucio Manenti1
Abstract 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. Keywords: Renal cystic disease, Ultrasonography, Medullary sponge kidney, Nephrolithiasis, Chronic renal failure
Background Medullary sponge kidney (MSK) is a rare renal disease, characterized by ectasia and cystic dilatation of intrapapillary portions of medullary collecting ducts that give the renal medulla a “spongy” appearance at autopsy. Its prevalence in the general population is not exactly known, but is estimated to be about 1/5000 persons and, among patients with recurrent nephrolithiasis, it ranges * Correspondence: [email protected] 1 U.O. Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Dipartimento di Medicina e Chirurgia, Università di Parma, Via Gramsci 14, 43126 Parma, Italy Full list of author information is available at the end of the article
from 12 to 20% [1–7]. MSK is typically associated with nephrocalcinosis and recurrent renal stones formation, distal renal tubular acidosis, hypocitraturia, hypercalcemia, renal concentration defects and defects of the proximal tubule
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