Ménétrier Disease

• • Giant hypertrophy of the gastric rugae with excessive loss of protein from the thickened mucosa into the gut, with resulting hypoproteinemia

Epidemiology

• • High, normal, or low acid secretion

  • Excessive loss of protein from the thickened mucosa into the gut, with resulting hypoproteinemia

  • In children, the disease characteristically is self-limited and benign

  • There is an increased risk of adenocarcinoma of the stomach in adults with Ménétrier disease

  • Associated with Helicobacter pylori infection

Symptoms and Signs

• • Diarrhea

  • Indigestion

  • Anorexia

  • Weight loss

  • Skin rash

  • Edema from hypoproteinemia

  • Symptomatic anemia

Laboratory Findings

• • Hypoproteinemia

  • Anemia

Imaging Findings

• • The hypertrophic rugae present as enormous filling defects on upper GI contrast studies and are frequently misinterpreted as carcinoma

  • Hypertrophic rugae apparent on upper GI endoscopy

• • Radiographic or endoscopic evidence of hypertrophic gastric rugae with hypoproteinemia is strongly suggestive of diagnosis

Rule Out

• • Adenocarcinoma

• • Upper GI contrast radiographic study

  • Upper GI endoscopy

  • Serum protein, Hct

• • Goal is to reduce protein loss

Surgery

• • Total gastrectomy

Indications

• • Rarely indicated for severe intractable hypoproteinemia, anemia, or inability to exclude cancer

Medications

• • Protein leak may respond to atropine, hexamethonium bromide, eradication of H pylori, H2 blocking agents, or omeprazole

Treatment Monitoring

• • Endoscopic surveillance for development of adenocarcinoma

Complications

• • Hypoproteinemia

Prognosis

• • Despite medical management, gastric abnormalities and hypoproteinemia may persist

References