How much do you know about this gastrointestinal polyposis? – Cronkhite-Canada syndrome

Introduction

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Cronkhite-Canada Syndrome (CCS) is a non-hereditary disease with clinical manifestations such as multiple polyps in the gastrointestinal tract, alopecia, nail dystrophy, skin pigmentation and diarrhea, which are rare in clinical practice . The disease was first reported in 1955 and named CCS by Jarnum and Jensen in 1966, but its etiology is still unclear. Therefore, no specific treatment has been established at this time.

This article mainly starts with a case of CCS to introduce the relevant knowledge of this disease.

Case introduction

Female patient, 78 years old, Previously diagnosed with irritable bowel syndrome, he presented with 6 months of progressive abdominal pain, gastroesophageal reflux, loss of taste, diarrhea (up to 10 times per day), intermittent transrectal blood loss, and weight loss of 15 kg. Physical examination revealed nail dystrophy. Subsequent esophagogastroduodenoscopy revealed multiple hyperplastic polyps in the stomach (Figure 1), and the gastric antrum was completely replaced by polyps (Figure 2).

Figure 1

Fig.2

Colonoscopy revealed diffuse vascular changes, hyperemia, and nodular mucosal areas with numerous inflammatory and adenomatous polyps of varying sizes (Figures 3 and 4). Capsule endoscopy also revealed multiple polyps throughout the small bowel.

Figure 3

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Figure 4

Based on clinical history, the patient was diagnosed with CCS and received 6-week tapering high-dose prednisone followed by intermittent courses of budesonide 9 mg/d plus rifaximin. After treatment, the patient’s symptoms completely resolved.

Understanding Cronkhite-Canada Syndrome

as above As stated, CCS is a rare non-genetic disorder of unknown etiology. Gastrointestinal symptoms are the main presentation, including abdominal pain and diarrhea, but also with frequent nausea and acid regurgitation, anorexia, dysgeusia, or fatigue. Some patients have skin pigmentation on the back, the back of the hands, and the feet, and some have nail dystrophy, which affects one or more fingers (toes). In addition, some patients may develop edema, especially in the eyelids and extremities.

The diagnosis of CCS is based on history, physical examination, endoscopic findings of gastrointestinal polyps, and histopathology. Digestive endoscopy is the most direct means of examination of this disease.

Currently, there is no effective treatment for CCS. Commonly used treatment methods include conservative medical treatment and surgical treatment, such as corticosteroids and anabolic steroids , proton pump inhibitors (PPIs), H2 receptor antagonists, nutritional support, sodium cromoglycate, immunosuppressants, antibiotics, surgery, 5-aminosalicylic acid, rituximab, Helicobacter pylori eradication and these therapies The combination.

References:

[1] Liu Y, Zhang L, Yang Y, et al. Cronkhite-Canada syndrome: report of a rare case and review of the literature[J]. J Int Med Res. 2020 May;48(5):300060520922427.

[2] Hai Jing, Luo Hesheng. Cronkhite-Canada syndrome literature Review[J].Journal of Gastroenterology and Hepatology, 2020, 29(09):1061-1064.

[3] Wang Hongxia, Yan Bin, Zhou Zhenghua, et al. Analysis of clinical diagnosis and treatment of 13 cases of Cronkhite-Canada syndrome [J]. World Chinese Journal of Digestion, 2019, 27(09):570-575.

[4] Kwon J, Fluxa-Cardenas D, Francis D. Cronkhite-Canada Syndrome[J]. Clin Gastroenterol Hepatol. 2021 Sep 4:S1542-3565(21)00934-4.