Cronkhite-Canada Syndrome: Report of Three Cases and Literature Review

Abstract

Cronkhite-Canada syndrome (CCS) is currently considered to be a non-genetic disorder and a relatively rare clinical condition that is characterized endoscopically by multiple polyps in the gastrointestinal tract and clinically manifested by gastrointestinal symptoms, skin pigmentation, hair loss, and nail loss. In this paper, we analyzed the clinical characteristics and treatment procedures of three patients diagnosed with CCS at Qilu Hospital of Shandong University from August 2012 to September 2021 and reviewed the relevant literature. The diagnosis of CCS is based on medical history, physical examination, endoscopic findings of gastrointestinal polyps, and histopathology, with gastrointestinal endoscopy being the most direct means of examination. All three patients presented endoscopically with diffuse multiple polyps in the gastrointestinal tract, had hair and nail loss and were free of HP infection. Moreover, case 1 and case 3 both had reduced serum albumin levels; case 2 and case 3 were negative for antinuclear antibodies (ANA) , case 1 was not checked for ANA. Glucocorticoids and proton pump inhibitors (PPI) were the main pharmacological treatments for CCS. Case 1 was not treated with glucocorticoids, but only PPI and supportive treatment, and later developed bloody stools and intestinal necrosis and underwent total gastrectomy for gastric cancer 6 years after the onset of the disease. Cases 2 and 3 were improved after being treated with prednisone and PPI. Early diagnosis, glucocorticoid-based pharmacotherapy, regular gastrointestinal endoscopy, and new treatment options may contribute to a better prognosis, and the reduction in possibilities of missed diagnosis or misdiagnosis of CCS via improving clinicians' knowledge and understanding of the disease.

Keywords

• intestinal polyposis|disease attributes|therapy|case reports