Medullary sponge kidney

Changed by Ayush Goel, 10 Oct 2014
Back to revision history

Updates to Article Attributes

Body was changed:

Medullary sponge kidney (MSK) is a sporadic condition. The medullary and papillary portions of the collecting ducts are dysplastic and dilated and in most cases develop medullary nephrocalcinosis. 

Epidemiology

The incidence of medullary sponge kidney is estimated at ≈ 1≈1:5000.

Clinical presentation

Most patients with medullary sponge kidney remain asymptomatic throughout life, with the diagnosis only made incidentally when the renal tract is imaged for other reasons. 

Alternatively, patients may present with a complication (accounting for ~ 10~10% of patients), including:

Recognised associations include:

Pathology

Medullary sponge kidney represents a developmental defect affecting the formation of collecting tubules and results in cystic dilatation of medullary & papillary portions of collecting ducts.   

Radiographic features

Medullary nephrocalcinosis occurs in the majority of cases ( ≈ 80≈80%) . It may be unilateral or bilateral and affect a single or multiple pyramids. 

Plain film / CT/CT

Clusters of pyramidal medullary calcification are characteristic. Delayed post contrast CT may demontrate a "paint brush" appearance to the renal medullary regions 4.

Fluoroscopy /: IVP

The pyelogram may give a characteristic bouquet of flowers appearance 3.

Ultrasound

Ultrasound of the kidneys of patients with medullary sponge kidneys typically demonstrates echogenic medullary pyramids. This appearance is found whether or not medullary nephrocalcinosis is also present. 

Differential diagnosis

The differential diagnosis is essentially that of medullary nephrocalcinosis and other causes of multiple medullary cysts and includes:

  • -<p><strong>Medullary sponge kidney (MSK)</strong> is a sporadic condition. The medullary and papillary portions of the collecting ducts are dysplastic and dilated and in most cases develop <a href="/articles/medullary-nephrocalcinosis">medullary nephrocalcinosis</a>. </p><h4>Epidemiology</h4><p>The incidence of medullary sponge kidney is estimated at ≈ 1:5000.</p><h4>Clinical presentation</h4><p>Most patients with medullary sponge kidney remain asymptomatic throughout life, with the diagnosis only made incidentally when the renal tract is imaged for other reasons. </p><p>Alternatively, patients may present with a complication (accounting for ~ 10% of patients), including:</p><ul>
  • +<p><strong>Medullary sponge kidney (MSK)</strong> is a sporadic condition. The medullary and papillary portions of the collecting ducts are dysplastic and dilated and in most cases develop <a href="/articles/medullary-nephrocalcinosis">medullary nephrocalcinosis</a>. </p><h4>Epidemiology</h4><p>The incidence of medullary sponge kidney is estimated at ≈1:5000.</p><h4>Clinical presentation</h4><p>Most patients with medullary sponge kidney remain asymptomatic throughout life, with the diagnosis only made incidentally when the renal tract is imaged for other reasons. </p><p>Alternatively, patients may present with a complication (accounting for ~10% of patients), including:</p><ul>
  • -<a href="/articles/urolithiasis">urolithiasis</a> / <a href="/articles/urolithiasis">ureteric calculi</a>
  • +<a href="/articles/urolithiasis">urolithiasis</a>/<a href="/articles/urolithiasis">ureteric calculi</a>
  • -<li>congenital hemihypertrophy / <a href="/articles/beckwith-wiedemann-syndrome-2">Beckwith-Wiedemann syndrome</a> (rare) <sup>1</sup>
  • +<li>congenital hemihypertrophy/<a href="/articles/beckwith-wiedemann-syndrome-2">Beckwith-Wiedemann syndrome</a> (rare) <sup>1</sup>
  • -</ul><h4>Pathology</h4><p>Medullary sponge kidney represents a developmental defect affecting the formation of collecting tubules and results in cystic dilatation of medullary &amp; papillary portions of collecting ducts.   </p><h4>Radiographic features</h4><p><a href="/articles/nephrocalcinosis">Medullary nephrocalcinosis</a> occurs in the majority of cases ( ≈ 80%) . It may be unilateral or bilateral and affect a single or multiple pyramids. </p><h5>Plain film / CT</h5><p>Clusters of pyramidal medullary calcification are characteristic. Delayed post contrast CT may demontrate a "paint brush" appearance to the renal medullary regions <sup>4</sup>.</p><h5>Fluoroscopy / IVP</h5><p>The pyelogram may give a characteristic <a href="/articles/bouquet-of-flowers-appearance">bouquet of flowers appearance</a> <sup>3</sup>.</p><h5>Ultrasound</h5><p>Ultrasound of the kidneys of patients with medullary sponge kidneys typically demonstrates echogenic medullary pyramids. This appearance is found whether or not medullary nephrocalcinosis is also present. </p><h4>Differential diagnosis</h4><p>The differential diagnosis is essentially that of <a href="/articles/medullary-nephrocalcinosis">medullary nephrocalcinosis</a> and other causes of multiple medullary cysts and includes:</p><ul>
  • +</ul><h4>Pathology</h4><p>Medullary sponge kidney represents a developmental defect affecting the formation of collecting tubules and results in cystic dilatation of medullary &amp; papillary portions of collecting ducts.   </p><h4>Radiographic features</h4><p><a href="/articles/nephrocalcinosis">Medullary nephrocalcinosis</a> occurs in the majority of cases (≈80%) . It may be unilateral or bilateral and affect a single or multiple pyramids. </p><h5>Plain film/CT</h5><p>Clusters of pyramidal medullary calcification are characteristic. Delayed post contrast CT may demontrate a "paint brush" appearance to the renal medullary regions <sup>4</sup>.</p><h5>Fluoroscopy: IVP</h5><p>The pyelogram may give a characteristic <a href="/articles/bouquet-of-flowers-appearance">bouquet of flowers appearance</a> <sup>3</sup>.</p><h5>Ultrasound</h5><p>Ultrasound of the kidneys of patients with medullary sponge kidneys typically demonstrates echogenic medullary pyramids. This appearance is found whether or not medullary nephrocalcinosis is also present. </p><h4>Differential diagnosis</h4><p>The differential diagnosis is essentially that of <a href="/articles/medullary-nephrocalcinosis">medullary nephrocalcinosis</a> and other causes of multiple medullary cysts and includes:</p><ul>

ADVERTISEMENT: Supporters see fewer/no ads