Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Abstract

Introduction: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: (1) In Chinese population, people aged 50–70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. (2) The diverse range of GI manifestations was observed in all the patients, and almost all the patients had at least 1 symptom of ectodermal dysplasias. (3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema, and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%). (4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). The prevalence of cancer was 5.97% in Chinese CCS patients. Conclusions: Middle-aged and elderly people are the high-risk group. Various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps. These GI polyps have a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients.