S3354 Ménétrier’s Disease: A Rare Cause of Edema in Childhood

Abstract

Introduction: Ménétrier’s disease (MD) is a rare protein-losing gastropathy characterized by enlarged gastric rugal folds. Adult MD is associated with a high degree of morbidity and mortality due to potential malignant transformation and need for gastrectomy. Pediatric MD, in contrast, is typically an acute, self-limited process, frequently associated with cytomegalovirus (CMV) infection of the stomach. Case Description/Methods: A previously healthy 2-year-old boy was admitted for generalized edema, which had been preceded by a week of diarrhea. Growth and development had been normal. Initial laboratory evaluation was notable for serum albumin of 1.3 g/dL. Other labs were largely unremarkable. Cardiac, renal, and liver etiologies were excluded. Esophagogastroduodenoscopy showed a normal gastric antrum, but the remainder of the stomach had enlarged rugae with areas of ulceration and nodularity. The duodenum and esophagus appeared normal. Histology was notable for hyperplastic gastric pits and foveolae. Immunohistochemistry for CMV and Helicobacter pylori were negative, and viral culture of a gastric sample was also negative for CMV. CMV serologies showed elevated IgM and IgG levels. Additionally, a viral enteric pathogens panel was positive for sapovirus. The patient received 2 doses of albumin and antisecretory agents during admission, but his edema and hypoalbuminemia improved without treatment of CMV. Discussion: In a toddler presenting with edema and hypoalbuminemia, it is necessary to consider protein-losing gastroenteropathies on the differential. Endoscopic evaluation with biopsy is essential for diagnosis of MD. Histology may show foveolar hyperplasia with cystic dilation of pits of the gastric body with relative sparing of the gastric antrum. Full-thickness biopsy may be required to detect classic histologic findings. Evaluation for infection should be done as one-third of pediatric cases are associated with CMV. Management of pediatric MD is primarily supportive due to the self-limited nature of the condition. Treatment with ganciclovir can be considered for severe or refractory cases associated with CMV.