Citation, DOI & article data
Ménétrier disease, also known as giant hypertrophic gastritis or hypoproteinemic hypertrophic gastropathy, is a form of rare idiopathic hypertrophic gastropathy.
Rare disease with an incidence of <1 per 200,000. Bimodal in distribution, it usually occurs in children younger than 10 years and the second peak occurs in adulthood between 30 and 60 years of age with an average age at diagnosis of 55 years 1. There is a male preponderance in both the juvenile and adult forms 7.
Achlorhydria, hypoproteinemia and edema comprise the classic triad. It is thought to be caused by protein-losing enteropathy. Acid production may be compromised. Hypoproteinemia may lead to ascites and pleural effusions. It is also characterized by excessive mucus production 10.
The exact etiology 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.
It most commonly affects the gastric fundal region, but any part of the stomach may be involved 7.
Upper GI fluoroscopy findings include 10:
markedly enlarged and tortuous folds in the fundus and body, especially along the greater curvature, with sparing of the antrum
barium is diluted due to mucus hypersecretion, leading to impaired mucosal coating
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 convolutions of brain 7. Gastric wall thickness is normal between folds 10.
Treatment and prognosis
high-protein diet to correct hypoproteinemia
proton pump inhibitors (PPI) to treat attendant gastritis
EGFR monoclonal antibody (cetuximab)
History and etymology
It is named after Pierre Ménétrier (1859–1935), a French pathologist who described certain pathologic gastric changes associated with the condition in 1888 3,9.
Imaging differential considerations for diffuse rugal thickening include
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- 9. Coffey R & Tanksley J. Pierre Ménétrier and His Disease. Trans Am Clin Climatol Assoc. 2012;123:126-33; discussion 133. PMC3540591 - Pubmed
- 10. Jeffrey Klein, Emily N. Vinson, Clyde A. Helms et al. Brant and Helms' Fundamentals of Diagnostic Radiology. (2018) ISBN: 9781496367396 - Google Books