Cronkhite-Canada syndrome
Cronkhite-Canada syndrome is a type of non-neoplastic, non-hereditary hamartomatous polyposis syndrome characterized by rash, alopecia, and watery diarrhea.
Epidemiology
There is a recognized male predilection with 3:2 male:female. Patients typically are middle age (50-60 years of age) . 75% of the approximate 500 cases in the literature were from Japan .
Clinical presentation
Patients typically present with watery diarrhea and a protein-losing enteropathy with associated nail atrophy, brownish skin pigmentation, and alopecia .
Pathology
Cronkhite-Canada syndrome is 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 malignancy.
Polyps are similar to those of juvenile polyposis syndrome except that the mucosa among Cronkhite-Canada syndrome polyps is edematous and inflammation of the lamina propria is usually present; by contrast, histologically the mucosa between juvenile polyposis syndrome polyps is normal .
Radiographic features
Fluoroscopy
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
This syndrome was initially described by American physician Leonard W Cronkhite Jr (1919-1996) and American radiology fellow Wilma Jeanne Canada (1926-2017) in 1955, both working at Massachusetts General Hospital .
Dr. Canada became Dr. Canada Diner after she married and spent her whole post-fellowship career at the University of Arkansas for Medical Sciences in Little Rock, Arkansas, retiring as Professor of Radiology in 1997 .
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