PC2167: Expanding the Differential of an Acutely Edematous Toddler: A Case of Menetrier Disease

Introduction: Menetrier disease (MD) is typically a chronic protein-losing enteropathy that most commonly develops in middle-aged men. The underlying pathology is related to a hypertrophic gastropathy, often precipitated by infectious gastritis. Features include vomiting, abdominal pain, weight loss, peripheral edema, and microcytic anemia. Though rare, MD is also reported in children with 2.5x more cases in males.

Case Description: A 13-month-old previously healthy female presented to the emergency department twice over 5 days for periorbital swelling; worsening edema was also noted in her extremities. In 5.5 weeks, her weight percentiles increased from 33rd to 70th and linear growth remained at the 25th percent. Mom denied fever, vomiting, abdominal pain, or diarrhea. Stools were without blood or mucus and daily. The patient had a robust appetite including whole milk (up to 30 ounces) daily. Work-up demonstrated hypoalbuminemia, low prealbumin, normocytic anemia, eosinophilia, thrombocytosis, and a normal urinalysis and protein-to-creatinine ratio (Table-1). Stools were negative for occult blood, positive for leukocytes, and showed elevated calprotectin and alpha-1-antitrypsin levels. Esophagogastroduodenoscopy and sigmoidoscopy were significant for scattered mucosal nodules in the pre-pyloric antrum and duodenal bulb (Figure-1), though otherwise normal with normal histology. Cytomegalovirus (CMV) testing was positive. She required 3 infusions of albumin and 1 unit packed red blood cells over an 8-day hospitalization. She was discharged on a high-protein diet. Her labs normalized and she returned to her clinical baseline over the next week without additional interventions.

Discussion: Although presenting symptomatology between adults and children with MD is similar, pediatric disease is often self-limited. CMV can be associated with both pediatric and adult MD, but rates of CMV are much higher in pediatric MD. MD has only been reported in 16 otherwise healthy infants and toddlers (4 months - < 4 years). CMV testing was positive in 14/16. All presented with prominent abdominal symptoms including vomiting and/or diarrhea. Iron deficiency anemia is a common feature of MD, but significant anemia was not identified in any of these patients.

Conclusion: Although MD is rare in children, our patient displayed a similar self-limited pattern of disease as previously described. However, she had no accompanying or preceding gastrointestinal symptoms. A low prealbumin level without other features of chronic malnutrition suggests our patient’s disease may have evolved over several weeks prior to presentation. Anemia is also not easily explained in the context of her disease. Iron deficiency has previously been noted as a possible risk factor for protein-losing enteropathy in children. Yet despite the history of excessive cow’s milk consumption, her indices were more consistent with acute blood loss versus a transient post-viral etiology. It is unclear how normocytic anemia related to her disease and presentation. Our case highlights the need for an expanded differential when evaluating the acutely edematous child.