Cronkhite - canada syndrome

(Leonard Wolsey Cronkhite, Jr., born 1919, American internist and Wilma Jeanne Canada, 20th century, American radiologist), rare nonhereditary disorder, characterized by generalized gastrointestinal polyps, diarrhoea and malabsorption. It is associated with a variety of ectodermal abnormalities such as alopecia, hyperpigmentation of the skin and onychodystrophy. The polyps are juvenile-type inflammatory lesions without premalignant potential, although gastric and colon carcinoma have been reported. Persistent steatorrhoea with anaemia and hypoproteinaemia may lead to cachexia and even death. More commonly, however, spontaneous remission of variable duration will occur. Steroid treatment may lead to regression and even disappearance of the polyps.

Radiological findings include multiple small sessile polyps mainly located in the duodenum and the distal ileum, and associated with enlarged mucosal folds, flocculation and segmentation of barium by excessive fluid secretion.

ALB