GI Endoscopy · 1 min read

Cronkhite Canada Syndrome (CCS)

Experienced teaching points

Clinical Pearls

  1. Cronkhite-Canada Syndrome (CCS) is a rare, non-hereditary condition characterized by diffuse gastrointestinal pseudo-polyposis paired with ectodermal triad: alopecia, hyperpigmentation, and dystrophic nail changes.
  2. Patients typically present in middle age with chronic diarrhea, profound weight loss, anorexia, and severe malabsorption complications like iron deficiency anemia and zinc deficiency.
  3. Endoscopically, CCS features prominent edematous mucosal streaks, enlarged gastric folds, and widespread polyps/pseudopolyps spanning from the stomach to the colon.

A 48-year-old woman presented with chronic diarrhea and loss of taste. On clinical exam she appeared pale, had brittle nails and had partial hair loss. She was found to have severe iron deficiency anemia.

Colonoscopy showed multiple reddish, edematous mucosal streaks and polyps and pseudopolyps ranging from 5 to about 20 mm. On EGD the stomach was characterized by enlarged mucosal folds, pseudoplyps and duodenal mucosal atrophy. Histology showed the above findings (see figure 2).

CCS is a non-hereditary syndrome of unknown etiology characterized by diffuse gastrointestinal GI pseudo-polyposis, alopecia, hyperpigmentation, dystrophic changes in finger and toe nails

Clinical presentation: diarrhea, weight loss, abdominal pain, anorexia, weakness, hematochezia, iron deficiency anemia. Onset usually in middle aged adults.

Patients with CCS occasionally gastric adenocarcinoma, and adenocarcinomas anywhere in GI tract.

Treatment is not well standardized but consist of corticosteroids, H. pylori eradication and zinc supplementation.

Clinical endoscopic image

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