Description
Ménétrier disease, also known as hyperplastic hypersecretory gastropathy, is a rare disorder characterised by the presence of enlarged gastric folds, increased mucous production with resultant protein loss, and decreased gastric acid secretion.
Pathogenesis
The pathogenesis of Ménétrier disease is not fully understood, but it’s believed to involve an overproduction of transforming growth factor alpha (TGF-α) leading to hypertrophy of the gastric mucosa, particularly the rugae of the stomach body and fundus.
Subtypes
No specific subtypes of Ménétrier disease are recognised.
Epidemiology, Risk Factors & Associations
- Ménétrier disease is extremely rare and affects both sexes equally.
- Association with CMV and H. pylori infection has been noted, especially in paediatric cases.
Clinical Features
- Symptoms include upper abdominal pain, nausea, vomiting, and peripheral oedema due to hypoalbuminaemia from protein loss.
- Signs may include an enlarged, doughy abdomen and weight loss.
Complications
- Severe protein-losing gastropathy can lead to oedema and ascites.
- Increased risk of gastric carcinoma.
Pathological Features
Histopathology
- Macroscopic: Thickened gastric folds, predominantly in the fundus and body of the stomach.
- Microscopic: Foveolar hyperplasia with cystic dilation of the glandular structures.
Serology
- Decreased serum albumin due to protein loss.
Biochemistry
- Hypochlorhydria due to decreased acid production.
Radiological Features
General Features
- Thickening of the gastric folds, especially in the fundus and body.
CT
- Non-contrast: Non-specific, may show thickening of the gastric folds.
- Contrast-enhanced: Thickened, enhancing gastric folds.
MRI
- T1WI: Non-specific, may show thickening of the gastric folds.
- T2WI: Non-specific, may show thickening of the gastric folds.
- T1 C+: Enhancing thickened gastric folds.
- DWI/ADC: Non-specific.
Diagnosis
Diagnosis is confirmed by upper endoscopy showing thickened gastric folds and biopsy showing foveolar hyperplasia. The presence of hypochlorhydria and hypoalbuminaemia can support the diagnosis.
Differential Diagnosis
- Gastric carcinoma: May also present with thickened gastric folds, but usually has an irregular or ulcerated surface and is often associated with lymphadenopathy.
- Gastritis and peptic ulcer disease: Can cause thickening of gastric folds, but the clinical presentation and endoscopic findings differ.
Management
- Treatment is symptomatic and includes dietary modification and acid suppression.
- For severe disease, partial or total gastrectomy may be needed.