Pediatric Ménétrier's Disease Triggered by Cytomegalovirus Infection: A Rare Case of Severe Hypoalbuminemia and Edema

TL;DR

A 3-year-old boy with severe edema and low albumin was diagnosed with Ménétrier's disease linked to cytomegalovirus, despite negative initial tests.

Contribution

This case highlights CMV's role in pediatric Ménétrier's disease and challenges the reliability of standard CMV detection methods.

Findings

Ménétrier's disease in a child was confirmed to be CMV-associated despite negative PCR and serology.

The patient showed clinical improvement with antiviral therapy and supportive care.

Hypogammaglobulinemia was observed, suggesting possible immunodeficiency.

Abstract

Ménétrier's disease (MD) is a rare protein-losing gastropathy characterized by hypertrophy of the gastric mucosa, particularly in the fundus and body, leading to hypoalbuminemia and peripheral edema. The etiology remains unclear, but in children, MD is frequently associated with Cytomegalovirus (CMV) infection. Pediatric cases usually have an acute, self-limiting course and may respond to supportive care.

We present a case of a 3-year-old previously healthy male who developed vomiting, fatigue, and progressive edema, including facial, limb, and testicular swelling. Laboratory evaluation revealed significant hypoalbuminemia (2.3 g/dL), hypoproteinemia, hypogammaglobulinemia, elevated triglycerides, and lymphocytosis. Abdominal ultrasound showed free fluid and dilated bowel loops. Esophagogastroduodenoscopy revealed thickened gastric folds, and biopsy confirmed MD with CMV inclusion bodies and positive CMV immunostaining, despite negative CMV PCR and serology. The patient received supportive treatment including albumin infusions, diuretics, high-protein diet, and IV ganciclovir, with clinical improvement.

This case reinforces the known association between CMV and MD in children and highlights the variability of CMV detection via PCR or serology. Although pediatric MD is often self-limiting, antiviral therapy may be indicated in severe cases or when supportive care is insufficient. The presence of hypogammaglobulinemia in this case also raises questions about potential underlying immunodeficiency. Clinicians should maintain a high index of suspicion for CMV-associated MD in children with acute-onset edema and hypoalbuminemia, even when routine viral testing is negative.

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