A striking flail chest: a rare manifestation in Cronkhite-Canada syndrome

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Intest Res. 2018;.ir.2018.00132
Publication date (electronic) : 2018 December 14
doi : https://doi.org/10.5217/ir.2018.00132
1Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
2Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
Correspondence to Ji Li, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing 100730, China. Tel: +86-10-6915-5019, Fax: +86-10-6915-5019, E-mail: liji0235@pumch.cn
Co-Correspondence to Jiaming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing 100730, China. Tel: +86-10-6915-5019, Fax: +86-10-6915-5019, E-mail: qianjiaming1957@126.com
Received 2018 September 23; Revised 2018 October 5; Accepted 2018 October 8.

Question: A 59-year-old male patient was admitted to the department of gastroenterology for intermittent diarrhea for 4 years, anorexia and weight loss. He had hyperpigmentation of skin (Fig. A) and oral mucosa, alopecia and nail dystrophy (Fig. A). He had no family history of colon cancer or gastrointestinal polyps. A flail chest (bilateral chest wall collapse) (Fig. B) and paradoxical breathing were noticed on physical examination. Laboratory tests showed severe deficiency of 25 hydroxyvitamin D (7.4 μg/L), elevated parathyroid hormone (92 pg/mL), normal serum calcium and alkaline phosphatase. The chest CT scan revealed bilateral, multiple rib fractures (right rib, 2–11; left rib, 2–11). A gastroduodenoscopy and a colonoscopy revealed hundreds of polymorphic polyps lining the stomach (Fig. C), duodenum and the whole colon (Fig. D). Histopathology of this patient showed hyperplastic polyps in antrum and colon and villoustublar adenoma in colon. What is the diagnosis of this patient?

Answer to the Images: Cronkhite-Canada Syndrome

A clinic-pathologic diagnosis of Cronkhite-Canada syndrome (CCS) was made based on the above findings.

CCS is a rare, non-hereditary disease characterized by widespread gastrointestinal polyposis and ectodermal abnormalities [1]. The diagnosis of CCS should be based on characteristic clinical, endoscopic, radiologic and histologic findings [2]. Gastrointestinal symptoms and ectodermal abnormalities were prominent in this patient. Endoscopy showed typically CCS polyps that distributed among the entire gastrointestinal tract except oesophagus [3]. It is necessary to differentiate CCS from other gastrointestinal polyposis syndromes such as Peutz-Jeghers syndrome, juvenile polyposis syndrome, Gardner syndrome and Turcot syndrome when polyposis of gastrointestinal tract is noted under endoscopy [4]. The diagnosis of CCS is usually delayed due to low incidence and unrecognized phenotype. Although not considered as a malignant disorder, the mortality of CCS is high, mainly due to complications such as gastrointestinal bleeding, malabsorption and rectal prolapse [2]. In this case, the patient suffered from severe osteoporosis and osteomalacia due to the deficiency of vitamin D. To the best of our knowledge, flail chest caused by CCS has not been described in the literature up to now.

Considering the high mortality of CCS due to diverse complications, it is important to achieve early diagnosis and appropriate treatment. Thus, we suggest gastroenterologists be cognizant of the typical manifestations and diagnostic methods to better identify CCS. Gastroenterologists should also be aware of the potential danger of CCS-related osteoporosis and osteomalacia for better disease management.

Notes

FINANCIAL SUPPORT

This work was supported by specialized scientific research fund project for healthcare industry (201002020) and Teaching Quality Project of Peking Union Medical College (2017zlgc0110).

CONFLICT OF INTEREST

No potential conflict of interest relevant to this article was reported.

AUTHOR CONTRIBUTION

Conceptualization: SL, JL. Methodology: formal analysis: funding acquisition: JMQ, JL. Project administration: JMQ. Visualization: YY. Writing-original draft: SL. Writing-review and editing: GCR, YY, JL. Approval of final manuscript: all authors.

References

1. Zhao R, Huang M, Banafea O, et al. Cronkhite-Canada syndrome: a rare case report and literature review. BMC Gastroenterol 2016;16:23. doi: 10.1186/s12876-016-0436-1.
2. Chakrabarti S. Cronkhite-Canada syndrome (CCS): a rare case report. J Clin Diagn Res 2015;9:OD08–OD09.
3. Slavik T, Montgomery EA. Cronkhite-Canada syndrome six decades on: the many faces of an enigmatic disease. J Clin Pathol 2014;67:891–897.
4. Xue LY, Hu RW, Zheng SM, Cui DJ, Chen WX, Ouyang Q. Cronkhite-Canada syndrome: a case report and review of literature. J Dig Dis 2013;14:203–206.

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