Endoscopic features of Cronkhite-Canada syndrome.

Abstract

Cronkhite–Canada syndrome (CCS) is a rare protein-losing enteropathy and profound malnutrition disease with high mortality [1–3]. CCS can be challenging to differentiate from other polyposis syndromes [4]. We describe the endoscopic features of CCS before and after treatment. A 69-year-old woman presented with chronic diarrhea, body pigmentation, weight loss, and dysgeusia. Examination showed alopecia, glossitis, onycholysis, and palmar pigmentation. Her laboratory values showed abnormally low albumin (26g/dL), hemoglobin (11g/dL), and zinc (658 μg/L). Computed tomogram of the abdomen showed thickened folds and polyp-like protrusions in the stomach. We performed an upper endoscopy, which showed normal esophagus. Upon entering the stomach, multiple large inflammatory polyps covering the antrum and body were seen (▶Fig. 1). The duodenum showed mucosal edema, villous blunting, and atrophy. Similarly, colonoscopy revealed extensive large inflammatory polyps throughout the colon. The ileum appeared edematous with villous atrophy (▶Fig. 2). Biopsies performed from the gastric polyps showed mild infiltration with inflammatory cells, submucosal edema, and tortuous hyperplastic foveolar glands (▶Fig. 3). Based on the clinical features, characteristic endoscopic appearance, and histopathology findings, we diagnosed the patient to have CCS.We treated her using a tapering dose of prednisolone, azathioprine, and proton pump inhibitors. We provided oral nutritional supplements and corrected the micronutrient deficiency. After 3 months of treatment, her symptoms started to resolve. We repeated upper endoscopy and colonoscopy, which showed a regression of her gastric and colonic polyps (▶Fig. 4, ▶Fig. 5). The villous atrophy and blunting in the small bowel had reversed. We have reviewed our other similar cases, followed over an E-Videos