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

Abstract Background: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal polyposis and ectodermal dysplasia. Its etiology still remains indefinite to us. Currently, the diagnosis is mainly based on typical clinical manifestations and endoscopic features. However, there is a lack of these information from Chinese CCS patients. Our study was to investigate the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literature 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 gastrointestinal (GI) manifestations were observed in all the patients, and the most common symptoms were weight loss (98.80%) and loss of appetite (93.94%). Almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus. The size of polyps varied from 0.5 cm (88.64%) to 0.6-1.0 cm (81.82%) . The polyps were various in shapes, the most of them were nodular polyps (58.14%) ,what’s more,the sessile polyps were the most common(90.24%). 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%). There is only 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various gastrointestinal symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these gastrointestinal polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the CCS patients in China.