Cronkhite–Canada syndrome is a type of non-hereditary hamartomatous polyposis syndrome characterized by rash, alopecia, and watery diarrhea.
There is a recognized male predilection. Patients typically are middle age, in their 60th 1.
Patients typically present with watery diarrhea and protein losing enteropathy and associated nail atrophy, brownish skin pigmentation, and alopecia 3.
Characterized by numerous hamartomatous polyps in the digestive tract, with predominant involvement of the stomach, large intestine and, to a lesser extent, small bowel. The exact etiology is unknown and there is no recognized familial occurrence. Unlike other polyposis syndromes, it is not associated with a malignancy.
Polyps are similar to those of juvenile polyposis except that the mucosa among CCS polyps is edematous and inflammation of the lamina propria usually present; in contrast, histologically the mucosa among juvenile polyps is normal 4.
On barium studies, this syndrome is characterized by polyposis involving the entire gastrointestinal tract. Gastric and colonic polyposis are both present in 100% of patients with this diagnosis. Small bowel involvement is seen in 50% of patients.
History and etymology
Initially described by L. W Cronkhite and W. J Canada in 1955 2.
- 1. Harned RK, Buck JL, Sobin LH. The hamartomatous polyposis syndromes: clinical and radiologic features. AJR Am J Roentgenol. 1995;164 (3): 565-71. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Cronkhite LW, Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N. Engl. J. Med. 1955;252 (24): 1011-5. Pubmed citation
- 3. Brant WE, Helms CA. Fundamentals of Diagnostic Radiology. Lippincott Williams & Wilkins. (2007) ISBN:0781761352. Read it at Google Books - Find it at Amazon
- 4. Burke AP, Sobin LH. The pathology of Cronkhite-Canada polyps. A comparison to juvenile polyposis. Am. J. Surg. Pathol. 1989;13 (11): 940-6. Pubmed citation