Endoscopic features of Cronkhite–Canada syndrome

Cronkhite–Canada syndrome (CCS) is a rare protein-losing enteropathy and profound malnutrition disease with high mortality [1] [2] [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 (26 g/dL), hemoglobin (11 g/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 extended time (3 years), that were treated with long-term immunosuppressants ([Video 1]). The patients have remained asymptomatic during the follow-up time.

Fig. 1 Upper endoscopy at diagnosis: a multiple large inflammatory polyps in the gastric antrum; b edematous duodenal mucosa with villous atrophy.

Fig. 2 Colonoscopy at diagnosis: a large inflammatory polyps carpeting the colon; b villous atrophy in the ileum.

Fig. 3 Biopsy from the gastric polyp: a mild infiltration with inflammatory cells with submucosal edema; b tortuous foveolar gland hyperplasia.

Fig. 4 Upper endoscopy after 3 months of treatment: a decrease and disappearance of inflammatory gastric polyps; b reversal of villous blunting in the duodenum.

Fig. 5 Colonoscopy after 3 months of treatment: a disappearance of colonic inflammatory polyps; b improvement in villous blunting and edema.

Video 1 Endoscopic features of Cronkhite–Canada syndrome.

In conclusion, recognizing the distinct and specific endoscopic features of CCS may allow it to be diagnosed and differentiated from other polyposis syndromes ([Video 1]). Uniquely, in CCS, the multiple large inflammatory polyps may be reversed with treatment [3] [5].

Endoscopy_UCTN_Code_CCL_1AB_2AC_3AB

Publication History

Article published online:
14 January 2021

• References

• 1 Jarnum S, Jensen H. Diffuse gastrointestinal polyposis with ectodermal changes. A case with severe malabsorption and enteric loss of plasma proteins and electrolytes. Gastroenterology 1966; 50: 107-118
• 2 Riegert-Johnson DL, Osborn N, Smyrk T. et al. Cronkhite–Canada syndrome hamartomatous polyps are infiltrated with IgG4 plasma cells. Digestion 2007; 75: 96-97
• 3 Daniel ES, Ludwig SL, Lewin KJ. et al. The Cronkhite–Canada syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine (Baltimore) 1982; 61: 293-309
• 4 Burke AP, Sobin LH. The pathology of Cronkhite–Canada polyps: a comparison to juvenile polyposis. Am J Surg Pathol 1989; 13: 940-946
• 5 Chadalavada R, Brown DK, Walker AN. et al. Cronkhite–Canada syndrome: sustained remission after corticosteroid treatment. Am J Gastroenterol 2003; 98: 1444-1446