Cronkhite-Canada syndrome

Cronkhite-Canada syndrome is a type of non-neoplastic, non-hereditary hamartomatous polyposis syndrome characterised by rash, alopecia, and watery diarrhoea.

Epidemiology

There is a recognised male predilection with 3:2 male:female. Patients typically are middle age (50-60 years of age) ¹. 75% of the approximate 500 cases in the literature were from Japan ⁷.

Clinical presentation

Patients typically present with watery diarrhoea and a protein-losing enteropathy with associated nail atrophy, brownish skin pigmentation, and alopecia ³.

Pathology

Cronkhite-Canada syndrome is characterised 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 aetiology is unknown and there is no recognised 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 oedematous and inflammation of the lamina propria is usually present; by contrast, histologically the mucosa between juvenile polyposis syndrome polyps is normal ⁴.

Radiographic features

Fluoroscopy

On barium studies, this syndrome is characterised 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) ⁶and American radiology fellow Wilma Jeanne Canada (1926-2017) ⁵ in 1955, both working at Massachusetts General Hospital ².

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 ⁵.