What is the management of Mènètrier disease?

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Management of Ménétrier Disease

The management of Ménétrier disease should focus on treating underlying causes, controlling symptoms, and monitoring for complications, with surgical intervention reserved for refractory cases.

Disease Overview

Ménétrier disease is a rare acquired disorder characterized by:

  • Giant hypertrophic gastric mucosal folds in the fundus and body of the stomach
  • Protein-losing gastropathy leading to hypoalbuminemia
  • Increased mucus secretion
  • Hypochlorhydria (reduced stomach acid)
  • Possible edema due to protein loss

Diagnostic Approach

Clinical Presentation

  • Abdominal pain
  • Nausea and vomiting
  • Postprandial fullness
  • Weight loss
  • Peripheral edema due to hypoalbuminemia

Diagnostic Testing

  • Upper endoscopy with biopsy (gold standard)
    • Shows characteristic giant gastric folds
    • Histology reveals foveolar hyperplasia, cystic dilation of pits, and reduced parietal and chief cells
  • Laboratory tests
    • Hypoalbuminemia
    • Possible anemia
  • MRI may be considered for patients with asymmetric findings to rule out other conditions 1

Management Algorithm

1. Test and Treat for Associated Infections

  • Test for Helicobacter pylori
    • Eradicate if positive (standard triple or quadruple therapy)
  • Test for Cytomegalovirus (CMV), especially in pediatric cases
    • Treat with appropriate antiviral therapy if positive

2. Medical Management

  • Supportive care
    • Albumin infusions for severe hypoalbuminemia
    • Nutritional support
  • Acid suppression therapy
    • H2 receptor antagonists
    • Proton pump inhibitors
  • Consider targeted therapies
    • Cetuximab (anti-EGFR monoclonal antibody) for refractory cases
    • Somatostatin analogs may be beneficial 1

3. Patient Education and Lifestyle Modifications

  • Dietary counseling
    • Low-sodium diet may help reduce edema
    • High-protein diet to compensate for protein loss
  • Regular follow-up and monitoring

4. Surveillance

  • Regular endoscopic surveillance due to increased risk of gastric cancer
    • 8.9% of patients develop gastric cancer within 10 years of diagnosis 2
  • Monitor albumin levels and nutritional status

5. Surgical Management

  • Gastrectomy (partial or total) for:
    • Refractory symptoms not responding to medical therapy
    • Severe protein loss
    • Suspicion of malignancy
    • Intractable bleeding
  • Gastrectomy is considered curative 1, 3

Prognosis and Follow-up

  • Mortality is higher in patients with Ménétrier disease compared to controls
    • 72.7% 5-year survival rate
    • 65.0% 10-year survival rate 2
  • Pediatric cases often have better prognosis with spontaneous remission, especially when associated with CMV infection 4
  • Regular follow-up is essential due to increased risk of gastric cancer

Special Considerations

Pediatric Patients

  • Often associated with CMV infection
  • Generally self-limiting with spontaneous remission
  • Different clinical course compared to adult form 4

Risk Factors

  • Smoking has been associated with Ménétrier disease
  • No significant association with alcohol use or inflammatory bowel disease 2

Clinical Pearls and Pitfalls

  • Pearl: Consider Ménétrier disease in patients with unexplained hypoalbuminemia and gastric symptoms
  • Pitfall: Mistaking Ménétrier disease for gastric malignancy on imaging alone; endoscopy with biopsy is essential
  • Pearl: Pediatric cases often resolve spontaneously, especially when associated with CMV
  • Pitfall: Failure to monitor for development of gastric cancer, which occurs at higher rates than in the general population

References

1
Research

Menetrier's Disease.

Current treatment options in gastroenterology, 2008

2
Research

Survival Times of Patients With Menetrier's Disease and Risk of Gastric Cancer.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2021

3
Research

[Ménétrier disease--a rare disease of the stomach].

Zentralblatt fur Chirurgie, 1993

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Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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