Menetrier's disease in childhood: report of two cases and a review of the literature.

Two new cases plus 13 previously reported cases of Menetrier's disease in childhood are reviewed. The most common features of this illness are severe upper gastrointestinal symptoms (abdominal pain, nausea, vomiting, hematemesis) followed by the onset of generalized edema and ascites. Significant laboratory findings include hypoalbuminemia, eosinophilia, and anemia. Gastrointestinal protein loss and decreased gastric acid secretion can be documented. Upper gastrointestinal radiographs demonstrate the characteristic hypertrophic gastric rugae. Histologic features include hypertrophic tortuous gastric glands, basilar cysts, and interstitial round cell inflammation. The natural course of this disease in childhood is usually benign and self-limited. Uncommonly, the course may be severe and require gastric resection. This is in contrast to the adult form, where chronicity and severity is the rule. The etiology of this problem remains unknown. Endoscopy and biopsy are the diagnostic procedures of choice, although laparotomy may be necessary in equivocal cases. Therapy should be supportive except for those few patients who require surgery to control hemorrhage.