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 were observed in all the patients, and 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, 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%). There is 5.97% cancer reported. 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 has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients. Key words: Cronkhite-Canada syndrome, Clinical characteristics, Endoscopy

Cronkhite-Canada syndrome with esophagus involvement and six-year follow-up: A case report

“…In a Japanese nationwide survey, the esophagus was involved in 12.3% of cases, in contrast to prior reports[ 17 ]. However, in a retrospective study of 103 Chinese cases, the ratio was 4/103, much lower than the Japanese data[ 18 ]. Multiple changes, including but not limited to polyps, may occur in the esophagus, which might not be focused on except when there are polyps.…”

“…However, the long-term efficacy and side effects of low-dose corticosteroids still require observation. In both the Japanese nationwide survey and the largest single-center cohort of Chinese patients, nearly 40% of patients failed to achieve long-term clinical remission after corticosteroid administration, and relapse occurred during or after the cessation of corticosteroid use[ 17 , 18 ]. A proportion of patients were prescribed low-dose (5-10 mg/d) corticosteroids or immunosuppressants to counteract the tendency to relapse[ 19 ].…”

“…Despite the adverse effects of corticosteroids, they are still the most important therapy with the most clinical evidence and efficacy. The widespread use of corticosteroids may have contributed to the decrease in 5-year mortality from 55% to 16% and the general improvement of outcomes[ 4 , 18 ].…”

Endoscopic features help to identify the histopathological patterns of colorectal polyps in Cronkhite–Canada syndrome

“…The gastrointestinal polyps in CCS are mostly described as nonneoplastic hamartomas, including the characteristic hamartomatous polyps, hyperplastic polyps, and inflammatory polyps in histology. To date, CCS has been reported worldwide, mostly in East Asia 2–4 . Despite that its etiology and pathogenesis remain to be clarified, CCS is generally thought to be a chronic inflammatory gastrointestinal disease and its diagnosis is established based on a comprehensive evaluation of characteristic clinical manifestations, endoscopic, radiological, and histopathological findings.…”

“…To date, CCS has been reported worldwide, mostly in East Asia. [2][3][4] Despite that its etiology and pathogenesis remain to be clarified, CCS is generally thought to be a chronic inflammatory gastrointestinal disease and its diagnosis is established based on a comprehensive evaluation of characteristic clinical manifestations, endoscopic, radiological, and histopathological findings. Intermediate-to full-dose corticosteroids is the first choice for the induction of remission in active CCS, whereas a low-dose corticosteroid (prednisone <10 mg/d) or azathioprine is usually used for the maintenance therapy.…”