Ménétrier disease

Ménétrier disease, also known as giant hypertrophic gastritis or hypoproteinemic hypertrophic gastropathy, is a form of rare idiopathic hypertrophic gastropathy.

Epidemiology

This is a rare disease with an incidence of <1 per 200,000. The distribution is modal, 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 predominance in both the juvenile and adult forms ⁷.

Clinical presentation

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. 

Pathology

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.

Location

It most commonly affects the gastric fundal region, but any part of the stomach may be involved ⁷. 

Radiographic features

Fluoroscopy

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 

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 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)

• gastrectomy

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.

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

  • infective gastritis

  • radiation gastritis

  • eosinophilic gastritis

• Zollinger-Ellison syndrome