Protein-Losing Hypertrophic Gastropathy, Menetrierʼs Disease, Successfully Managed With Long-Acting Octreotide

Abstract

Protein-losing gastro-enteropathies are characterized by excessive loss of protein into the gastrointestinal tract. Ménétrier's disease (MD) is a rare disease characterized by extreme foveolar hyperplasia with glandular atrophy in a patient with gross enlargement of gastric folds or ruggae. Little is understood about the pathogenesis of this disease and treatment has mostly consisted of dietary therapy (low-fat, high-protein, medium-chain triglyceride diet.) This case describes a patient with MD successfully managed with Octreotide until he developed intussusception of a large gastric adenoma which required an emergent gastrectomy. A 52 yo AA male was diagnosed with MD after evaluation of a large gastric mass/thickened fold incidentally noted on imaging. An EGD confirmed thickened and large gastric folds. Gastric biopsies demonstrated dilated hyperplastic foveolar glands with stromal edema making the diagnosis of MD (Figure 1, 2). His course was complicated by several hospitalizations for DVTs treated with anticoagulation, severe intra-abdominal hemorrhage due to a complicated LVP, complicated pancreatic fluid collections, volume over load, and severe symptomatic anemia. Labs revealed a hemoglobin of 2.6 g/dL (MCV 58), ferritin 8 ng/ml, iron of < 5 mcg/dL, and Albumin of 0.9 g/dL. For several weeks his Albumin persistently ranged between 0.9 - 1.3 g/dL. Further evaluation revealed an elevated alpha-1-antitrypsin stool clearance >59 ml/day. He was started on octreotide 100mg BID SQ x6 months and subsequently transitioned to long acting Octreotide 20mg IM q4weeks. His albumin increased from 0.9 g/dL to 2.9 g/dL over the following 12-months. His anemia, microcytosis, and iron deficiency normalized. All of his clinical manifestations of hypoalbuminemia resolved and he regained significant muscle mass. Unfortunately, almost 19-months after making the diagnosis of MD he developed a gastric outlet obstruction due to a large fungating gastric adenoma filling and markedly distending the duodenum necessitating an emergent gastrectomy. He recovered well from surgery and continues to do well upon on follow up. Our case demonstrates the potential role of Octreotide as treatment or bridge to surgery for management of patients with protein losing gastropathies as well as as a gastric outlet obstruction from a gastric adenoma, an uncommon complication of this rare disease.Figure: Endoscopic appearance of the stomach showing mucosa edema and polypoid changes of the gastric fundus and body.Figure: Gastrectomy is performed. A, Grossly, there is hypertrophy of the fundal and body mucosa with sparing of the gastric antral mucosa; B-D, histologically, the body and fundal mucosa shows edematous, mildly inflamed lamina propria with hyperplastic.