Ménétrier disease
Updates to Article Attributes
Ménétrier disease (MD) is a form of rare idiopathic hypertrophic gastropathy.
Epidemiology
Rare disease with incidence < 1/200.000 per 200,000. Bimodal in distribution, children often < 10 years, adult forms 30-60 years, peak 55 years. Male preponderance both in juvenile and adult form 7.
Pathology
The exact aetiology is not well known. The juvenile form has been linked to cytomegaloviral infection and usually resolves spontaneously. The adult form tends to progress with time 7 and TGF-A activation of the EGFR receptor has been implicated.
The characteristic of the disease is gastric mucosal hypertrophy, which may cause the rugae to resemble convolutions of the brain 2. Rugal thickening is predominantly caused by the expansion of the epithelial cell compartment of the gastric mucosa
Location
It most commonly affects the gastric fundal region also any part of the stomach may be involved 7.
Clinical presentation
Achlorhydria, hypoproteinaemia and oedema comprise the classic triad. It is thought to be caused by protein-loss in the digestive system, protein-losing enteropathy. Acid production may be compromised. Hypoproteinaemia may lead to ascites and pleural effusions.
Radiographic features
Fluoroscopy
Usually characterised by giant rugal folds.
CT
On contrast-enhanced CT images, the thickened rugae appear as areas of thickened mucosa that project into the gastric lumen to a degree that may resemble convolution of brain 7.
Treatment
- high protein diet to correct hypoproteinaemia
- PPI to treat attendant gastritis
- EGFR monocloncal antibody (Cetuximab)
- gastrectomy
Differential diagnosis
Imaging differential considerations for diffuse rugal thickening include
- gastric lymphoma / may also occur as part of post transplant lymphoproliferative disorder affecting the stomach.
- forms of gastritis
History and etymology
It is named after Pierre Ménétrier, a French pathologist, who described certain pathologic gastric changes associated with the condition in 1888 3.
-<p><strong>Ménétrier disease (MD)</strong> is a form of rare idiopathic hypertrophic gastropathy.</p><h4>Epidemiology</h4><p>Rare disease with incidence < 1/200.000. Bimodal in distribution, children often < 10 years, adult forms 30-60 years, peak 55 years. Male preponderance both in juvenile and adult form <sup>7</sup>.</p><h4>Pathology</h4><p>The exact aetiology is not well known. The juvenile form has been linked to cytomegaloviral infection and usually resolves spontaneously. The adult form tends to progress with time <sup>7 </sup>and TGF-A activation of the EGFR receptor has been implicated. </p><p>The characteristic of the disease is gastric mucosal hypertrophy, which may cause the rugae to resemble convolutions of the brain <sup>2</sup>. Rugal thickening is predominantly caused by the expansion of the epithelial cell compartment of the gastric mucosa</p><h5>Location</h5><p>It most commonly affects the gastric fundal region also any part of the stomach may be involved<sup> 7</sup>. </p><h4>Clinical presentation</h4><p>Achlorhydria, hypoproteinaemia and oedema comprise the classic triad. It is thought to be caused by protein-loss in the digestive system, protein-losing enteropathy. Acid production may be compromised. Hypoproteinaemia may lead to ascites and pleural effusions. </p><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>Usually characterised by giant rugal folds. </p><h5>CT</h5><p>On contrast-enhanced CT images, the thickened rugae appear as areas of thickened mucosa that project into the gastric lumen to a degree that may resemble convolution of brain <sup>7</sup>. </p><h4>Treatment</h4><ul>- +<p><strong>Ménétrier disease (MD)</strong> is a form of rare idiopathic hypertrophic gastropathy.</p><h4>Epidemiology</h4><p>Rare disease with incidence < 1 per 200,000. Bimodal in distribution, children often < 10 years, adult forms 30-60 years, peak 55 years. Male preponderance both in juvenile and adult form <sup>7</sup>.</p><h4>Pathology</h4><p>The exact aetiology is not well known. The juvenile form has been linked to cytomegaloviral infection and usually resolves spontaneously. The adult form tends to progress with time <sup>7 </sup>and TGF-A activation of the EGFR receptor has been implicated. </p><p>The characteristic of the disease is gastric mucosal hypertrophy, which may cause the rugae to resemble convolutions of the brain <sup>2</sup>. Rugal thickening is predominantly caused by the expansion of the epithelial cell compartment of the gastric mucosa</p><h5>Location</h5><p>It most commonly affects the gastric fundal region also any part of the stomach may be involved<sup> 7</sup>. </p><h4>Clinical presentation</h4><p>Achlorhydria, hypoproteinaemia and oedema comprise the classic triad. It is thought to be caused by protein-loss in the digestive system, protein-losing enteropathy. Acid production may be compromised. Hypoproteinaemia may lead to ascites and pleural effusions. </p><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>Usually characterised by giant rugal folds. </p><h5>CT</h5><p>On contrast-enhanced CT images, the thickened rugae appear as areas of thickened mucosa that project into the gastric lumen to a degree that may resemble convolution of brain <sup>7</sup>. </p><h4>Treatment</h4><ul>
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