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Dong J, Ma TS, Tu JF, Chen YW. Surgery for Cronkhite-Canada syndrome complicated with intussusception: A case report and review of literature. World J Gastrointest Surg 2022; 14:200-210. [PMID: 35317544 PMCID: PMC8908339 DOI: 10.4240/wjgs.v14.i2.200] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2021] [Revised: 10/15/2021] [Accepted: 01/20/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps, skin pigmentation, hair loss, and fingernail/toenail dystrophy. Intussusception is a serious condition with an occurrence rate of 5% in adults, which is mainly caused by intestinal tumors or other intestinal occupations. CASE SUMMARY A 57-year-old woman was admitted to our hospital due to abdominal distension and pain for the past year. Her nausea and vomiting symptoms had been aggravated for the past month. Previous transoral enteroscopy results one year prior showed chronic erosive gastritis protuberans, duodenitis, and jejunitis. She had sparse body hair and brown pigmentation on the skin of her hands and bilateral anterior tibias. The nails of both hands were pale and lacked luster, and the fingernail of her ring finger was longitudinally cracked. Gastroscopy showed extensive diffuse polypoid lump changes in the gastric body and antrum, of 0.5-3 cm in size. Colonoscopy showed multiple polypoid mucosal bulges in the terminal ileum and multiple polyps (0.3-5 cm) throughout the colon. The patient was diagnosed with CCS and underwent partial excision of the polyps, but she refused hormone therapy. One month later, the patient complained of nausea and vomiting, accompanied by abdominal pain and inability to pass gas or stool. Contrast-enhanced computed tomography of the abdomen showed gastrointestinal polyposis and ileocecal intussusception. She underwent stomach and bowel surgery. CONCLUSION CCS, as a rare disease with poor prognosis, should be treated aggressively. Systematic steroids, immunosuppressive agents, and biological agents were not applied; thus, the patient's symptoms quickly progressed, and intussusception occurred. She had to undergo surgery. Improved compliance may lead to a better prognosis.
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Affiliation(s)
- Jie Dong
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - Tian-Shi Ma
- Cancer Center, Department of Pathology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - Jiang-Feng Tu
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - You-Wei Chen
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
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Wu ZY, Sang LX, Chang B. Cronkhite-Canada syndrome: from clinical features to treatment. Gastroenterol Rep (Oxf) 2020; 8:333-342. [PMID: 33163187 PMCID: PMC7603875 DOI: 10.1093/gastro/goaa058] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 03/04/2020] [Accepted: 04/16/2020] [Indexed: 02/07/2023] Open
Abstract
Cronkhite–Canada syndrome (CCS) is a rare acquired polyposis with unknown etiology. To date, >500 cases have been reported worldwide. CCS is typically characterized by gastrointestinal symptoms, such as diarrhea and skin changes (e.g. alopecia, pigmentation, and nail atrophy). Endoscopic features include diffuse polyps throughout the entire gastrointestinal tract, except for the esophagus. Pathological types of polyps in CCS mainly include inflammatory, hyperplastic, hamartomatous, and adenomatous polyps. CCS can be complicated by many diseases and has a canceration tendency with a high mortality rate. Moreover, there is no uniform standard treatment for CCS. A review of the reported cases of CCS is presented herein, with the goal of improving our understanding of this disease.
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Affiliation(s)
- Ze-Yu Wu
- Department of Gastroenterology, First Hospital of China Medical University, Shenyang, Liaoning, P. R. China
| | - Li-Xuan Sang
- Department of Geriatrics, First Hospital of China Medical University, Shenyang, Liaoning, P. R. China
| | - Bing Chang
- Corresponding author. Department of Gastroenterology, First Affiliated Hospital of China Medical University, No. 155, Nanjing North Street, Shenyang, Liaoning 110001, P. R. China. Tel: +86-24-83283765; Fax: +86-24-83283764;
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Chen X, Li BX, Zhu LP, Ma S, Zhao JW, Zhong WL, Wang BM. Cronkhite-Canada syndrome: A rare polyposis. Shijie Huaren Xiaohua Zazhi 2019; 27:977-983. [DOI: 10.11569/wcjd.v27.i16.977] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Cronkhite-Canada syndrome (CCS) is a rare non-genetic disease whose clinical manifestations are diffuse polyps of the gastrointestinal tract, finger-nail malnutrition, alopecia, skin pigmentation, diarrhea, weight loss, and taste disorders. The majority of CCS cases have a poor prognosis and often have life-threatening complications. The etiology of and appropriate treatments for CCS are still in the exploratory stage. This paper aims to improve the understanding of CCS and explore its best treatments by summarizing the common causes, clinical manifestations, endoscopic features, and treatment options of CCS.
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Affiliation(s)
- Xin Chen
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Bian-Xia Li
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Lan-Ping Zhu
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Shuang Ma
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Jing-Wen Zhao
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Wei-Long Zhong
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
| | - Bang-Mao Wang
- Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
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Yuan W, Tian L, Ai FY, Liu SJ, Shen SR, Wang XY, Liu F. Cronkhite-Canada syndrome: A case report. Oncol Lett 2018; 15:8447-8453. [PMID: 29805581 PMCID: PMC5950510 DOI: 10.3892/ol.2018.8409] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2016] [Accepted: 12/12/2017] [Indexed: 12/20/2022] Open
Abstract
Cronkhite-Canada syndrome (CCS) is a rare non-inherited condition characterized by gastrointestinal (GI) hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, weight loss and diarrhea. The etiology is most likely autoimmune and diagnosis is based on patient history, physical examination, endoscopic findings of GI polyposis and histology. The disease is very rare; thus far more than 500 cases of CCS have been reported globally. A 58-years-old male with CCS was reported in the present case study. The patient experienced a history of diarrhea and hematochezia for 4 months, with abdominal pain for 1 month and additional nail and toenail loss for half a month. The clinical, endoscopic and histological data confirmed the diagnosis.
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Affiliation(s)
- Wei Yuan
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China.,Department of Hepatology, The First Affiliated Hosipital of Hu-Nan University of Chinese Medicine, Changsha, Hunan 410007, P.R. China
| | - Li Tian
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
| | - Fei-Yan Ai
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
| | - Shao-Jun Liu
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
| | - Shou-Rong Shen
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
| | - Xiao-Yan Wang
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
| | - Fen Liu
- Department of Gastroenterology, Hunan Key Laboratory of Non-Resolving Inflammation and Cancer, The Third Xiangya Hospital of Central South University, Changsha, Hunan 410013, P.R. China
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Chakrabarti S. Cronkhite-Canada Syndrome (CCS)-A Rare Case Report. J Clin Diagn Res 2015; 9:OD08-9. [PMID: 25954656 DOI: 10.7860/jcdr/2015/11919.5700] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2014] [Accepted: 02/05/2015] [Indexed: 12/18/2022]
Abstract
Cronkhite-Canada syndrome (CCS) is an extremely rare non-inherited condition characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, weight loss and diarrhoea. The aetiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; approximately 450 cases of CCS have been reported worldwide. The author reports a case of CCS in an elderly Indian male.
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Affiliation(s)
- Subrata Chakrabarti
- Post Graduate Trainee, Department of General Medicine, Ipgmer , Kolkata, India
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Slavik T, Montgomery EA. Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease. J Clin Pathol 2014; 67:891-7. [PMID: 25004941 DOI: 10.1136/jclinpath-2014-202488] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Cronkhite–Canada syndrome is a rare gastro-enterocolopathy of uncertain aetiology first described almost 60 years ago. It is characterised by diffuse gastrointestinal polyposis sparing only the oesophagus, ectodermal abnormalities and an unpredictable but often fatal clinical course. The disease may demonstrate extremely diverse clinical and endoscopic features, which often leads to a delay in diagnosis. A high index of suspicion and recognition of the characteristic histological findings frequently facilitate a correct diagnosis, but the distribution of the gastrointestinal pathology and its microscopic features may be atypical. The pathologist thus requires a thorough knowledge of both the typical and many atypical faces of this disease, for which various documented therapies often still prove ineffective. Close correlation with clinical findings, including any pertinent ectodermal abnormalities, and careful examination of biopsies derived from polypoid and endoscopically spared mucosa will ensure a timely and correct diagnosis in patients with this enigmatic syndrome.
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Kopáčová M, Urban O, Cyrany J, Laco J, Bureš J, Rejchrt S, Bártová J, Tachecí I. Cronkhite-Canada syndrome: review of the literature. Gastroenterol Res Pract 2013; 2013:856873. [PMID: 24369458 PMCID: PMC3863521 DOI: 10.1155/2013/856873] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2013] [Accepted: 10/30/2013] [Indexed: 12/19/2022] Open
Abstract
Cronkhite-Canada syndrome is a rare disease characterised by diffuse polyposis of the gastrointestinal tract, diarrhoea, weight loss, abdominal pain, cutaneous hyperpigmentation, dystrophic changes of fingernails, and alopecia. The etiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; about 450 cases have been described in the literature so far. We present a review of the literature with our own picture documentation of this rare condition.
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Affiliation(s)
- Marcela Kopáčová
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Ondřej Urban
- Department of Gastroenterology, Vitkovice Hospital, Zalužanského 1192/15, 703 00 Ostrava-Vítkovice, Czech Republic
| | - Jiří Cyrany
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Jan Laco
- The Fingerland Department of Pathology, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Jan Bureš
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Stanislav Rejchrt
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Jolana Bártová
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
| | - Ilja Tachecí
- 2nd Department of Medicine, Charles University in Praha, Faculty of Medicine at Hradec Králové, University Teaching Hospital, Sokolska 581, 500 05 Hradec Králové, Czech Republic
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Yuan B, Jin X, Zhu R, Zhang X, Liu J, Wan H, Lu H, Shen Y, Wang F. Cronkhite-Canada syndrome associated with rib fractures: a case report. BMC Gastroenterol 2010; 10:121. [PMID: 20955587 PMCID: PMC2972238 DOI: 10.1186/1471-230x-10-121] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2010] [Accepted: 10/18/2010] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Cronkhite-Canada syndrome (CCS) is a rare multiple gastrointestinal polyposis. Up till now, many complications of CCS have been reported in the literature, but rib fracture is not included. CASE PRESENTATION We report a case of a 58-year-old man who was admitted to our hospital with a 6-month history of frequent diarrhea, intermittent hematochezia and a weight loss of 13 kg. On admission, physical examination revealed alopecia of the scalp, hyperpigmentation of the hands and soles, and dystrophy of the fingernails. Laboratory data revealed hypocalcaemia and hypoproteinemia. Esophagogastroduodenoscopy, video capsule endoscopy and colonoscopy revealed various sizes of generalized gastrointestinal polyps. Histological examination of the biopsy specimens obtained from the stomach and the colon showed adenomatous polyp and inflammatory polyp respectively. Thus, a diagnosis of CCS was made. After treatment with corticosteroids for 24 days and nutritional support for two months, his clinical condition improved. Two months later, he was admitted to our hospital for the second time with frequent diarrhea and weight loss. The chest radiography revealed fractures of the left sixth and seventh ribs. Examinations, including emission computed tomography, bone densitometry test, and other serum parameters, were performed, but could not identify the definite etiology of the rib fractures. One month later, the patient suffered from aggravating multiple rib fractures due to the ineffective treatment, persistent hypocalcaemia and malnutrition. CONCLUSIONS This is the first case of a CCS patient with multiple rib fractures. Although the association between CCS and multiple rib fractures in this case remains uncertain, we presume that persistent hypocalcaemia and malnutrition contribute to this situation, or at least aggravate this rare complication. Besides, since prolonged corticosteroid therapy will result in an increased risk of osteoporotic fracture, CCS patients who accept corticosteroid therapy could be potential victims of rib fracture.
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Affiliation(s)
- Bosi Yuan
- Department of Gastroenterology, Jinling Hospital, Jiangsu province, China
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