Medication Summary

A generalized algorithm for treating CCS, such as one that addresses the use of any specific pharmacologic agent or nonstandard dosages, has not been established. Therapy is always supportive and includes vigorous fluid and electrolyte replacement and maintenance of macronutrient and micronutrient requirements. Antibiotics are given orally to treat small-bowel overgrowth or parenterally to treat systemic infections. Broad-spectrum coverage should be provided to patients with immunocompromise. Corticosteroids (30-49 mg prednisolone/day) have been widely used in Japan and have proven to be effective in about 80% of patients. Care should be used to document endoscopic remission of polyposis to avoid early relapse.^[2]Other immunosuppressive agents (calcineurin inhibitors, azathioprine, anti-TNG-alpha agents) have been used as steroid-sparing agents or in steroid-refractory patients.

Class Summary

These drugs are presumably used for their anti-inflammatory effect. They elicit anti-inflammatory and immunosuppressive properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Hydrocortisone (Solu-Cortef)

View full drug information

Decreases inflammation by suppressing migration of polymorphonuclear (PMN) leukocytes and reversing increased capillary permeability. Data to support use sparse and anecdotal. Corticosteroids often used in combination with other agents. Documentation of spontaneous remissions further obscures picture. Standard corticosteroid dosage, as in other GI inflammatory conditions, is empiric but prudent starting point.

Prednisone (Deltasone, Orasone)

View full drug information

Used for immunosuppression and anti-inflammatory effects. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Data to support use sparse and anecdotal. Corticosteroids often used in combination with other agents. Documentation of spontaneous remissions further obscures picture. Standard corticosteroid dosage, as in other GI inflammatory conditions, is empiric but prudent starting point.

Follow-up

Ruymann FB. Juvenile polyps with cachexia. Report of an infant and comparison with Cronkhite-Canada syndrome in adults. Gastroenterology. 1969 Oct. 57(4):431-8. [QxMD MEDLINE Link].
Watanabe C, Komoto S, Tomita K, Hokari R, Tanaka M, Hirata I, et al. Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey. J Gastroenterol. 2015 Jul 28. [QxMD MEDLINE Link].
Kopáčová M, Urban O, Cyrany J, et al. Cronkhite-Canada syndrome: review of the literature. Gastroenterol Res Pract. 2013. 2013:856873. [QxMD MEDLINE Link].
Kato K, Ishii Y, Mazaki T, Uehara T, Nakamura H, Kikuchi H, et al. Spontaneous Regression of Polyposis following Abdominal Colectomy and Helicobacter pylori Eradication for Cronkhite-Canada Syndrome. Case Rep Gastroenterol. 2013 Jan. 7(1):140-6. [QxMD MEDLINE Link]. [Full Text].
Yashiro M, Kobayashi H, Kubo N, et al. Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. Digestion. 2004. 69(1):57-62. [QxMD MEDLINE Link].
Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nippon Geka Hokan. 1995 Jan 1. 64(1):3-14. [QxMD MEDLINE Link].
Vernia P, Marcheggiano A, Marinaro V, et al. Is Cronkhite-Canada Syndrome necessarily a late-onset disease?. Eur J Gastroenterol Hepatol. 2005 Oct. 17(10):1139-41. [QxMD MEDLINE Link].
Patil V, Patil LS, Jakareddy R, Verma A, Gupta AB. Cronkhite-Canada syndrome: a report of two familial cases. Indian J Gastroenterol. 2013 Mar. 32(2):119-22. [QxMD MEDLINE Link].
Hizawa K, Nakamori M, Yao T, Matsumoto T, Iida, M. A Case of Cronkhite-Canada Syndrome with Colorectal Adenomas: Effect of the Nonsteroidal Anti-Inflammatory Drug Sulindac. Am J Gastroenterol. August 2007. 102 (8):1831-2. [QxMD MEDLINE Link].
Riegert-Johnson DL, Osborn N, Smyrk T, Boardman LA. Cronkhite-Canada Syndrome Hamartomatous Polyps are Infiltrated with IgG4 Plasma Cells. Digestion. 2007/05. 75 (2-3):96-97. [QxMD MEDLINE Link].
Dachman AH, Buck JL, Burke AP, Sobin LH. Cronkhite-Canada syndrome: radiologic features. Gastrointest Radiol. 1989. 14(4):285-90. [QxMD MEDLINE Link].
Slavik T, Montgomery EA. Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease. J Clin Pathol. 2014 Oct. 67 (10):891-7. [QxMD MEDLINE Link].
Kilcheski T, Kressel HY, Laufer I, Rogers D. The radiographic appearance of the stomach in Cronkhite-Canada syndrome. Radiology. 1981 Oct. 141(1):57-60. [QxMD MEDLINE Link].
Goto A, Mimoto H, Shibuya C, Matsunami E. Cronkhite-Canada syndrome: an analysis of clinical features and follow-up studies of 80 cases reported in Japan. Nippon Geka Hokan. 1988 Nov 1. 57(6):506-26. [QxMD MEDLINE Link].
Freeman K, Anthony PP, Miller DS, Warin AP. Cronkhite Canada syndrome: a new hypothesis. Gut. 1985 May. 26(5):531-6. [QxMD MEDLINE Link].
[Guideline] Dominic OG, McGarrity T, Dignan M, Lengerich EJ. American College of Gastroenterology Guidelines for Colorectal Cancer Screening 2008. Am J Gastroenterol. 2009 Oct. 104(10):2626-7; author reply 2628-9. [QxMD MEDLINE Link].
Zong Y, Zhao H, Yu L, Ji M, Wu Y, Zhang S. Case report-malignant transformation in Cronkhite-Canada syndrome polyp. Medicine (Baltimore). 2017 Feb. 96 (6):e6051. [QxMD MEDLINE Link].
Cao XC, Wang BM, Han ZC. Wireless capsule endoscopic findings in Cronkhite-Canada syndrome. Gut. 2006. 55 (6):899-900. [QxMD MEDLINE Link].
Nakayama M, Muta H, Somada S, Maeda T, Mutoh T, Shimizu K. Cronkhite-Canada syndrome associated with schizophrenia. Intern Med. 2007. 46(4):175-80. [QxMD MEDLINE Link].
Daniel ES, Ludwig SL, Lewin KJ, et al. The Cronkhite-Canada Syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine (Baltimore). 1982 Sep. 61(5):293-309. [QxMD MEDLINE Link].
Nonomura A, Ohta G, Ibata T, et al. Cronkhite-Canada syndrome associated with sigmoid cancer case report and review of 54 cases with the syndrome. Acta Pathol Jpn. 1980 Sep. 30(5):825-45. [QxMD MEDLINE Link].
Russell DM, Bhathal PS, St John DJ. Complete remission in Cronkhite-Canada syndrome. Gastroenterology. 1983 Jul. 85(1):180-5. [QxMD MEDLINE Link].
Samoha S, Arber N. Cronkhite-Canada syndrome. Digestion. 2005. 71(4):199-200. [QxMD MEDLINE Link].
Ward EM, Wolfsen HC. Pharmacological management of Cronkhite-Canada syndrome. Expert Opin Pharmacother. 2003 Mar. 4(3):385-9. [QxMD MEDLINE Link].
Anderson RD, Patel R, Hamilton JK, Boland CR. Cronkhite-Canada syndrome presenting as eosinophilic gastroenteritis. Proc (Bayl Univ Med Cent). 2006 Jul. 19(3):209-12. [QxMD MEDLINE Link].
Heinzow HS, Domschke W, Meister T. Innovative video capsule endoscopy for detection of ubiquitously elongated small intestinal villi in Cronkhite-Canada syndrome. Wideochir Inne Tech Maloinwazyjne. 2014 Mar. 9 (1):121-3. [QxMD MEDLINE Link].
Bettington M, Brown IS, Kumarasinghe MP, de Boer B, Bettington A, Rosty C. The challenging diagnosis of Cronkhite-Canada syndrome in the upper gastrointestinal tract: a series of 7 cases with clinical follow-up. Am J Surg Pathol. 2014 Feb. 38 (2):215-23. [QxMD MEDLINE Link].
Flannery CM, Lunn JA. Cronkhite-Canada Syndrome: an unusual finding of gastro-intestinal adenomatous polyps in a syndrome characterized by hamartomatous polyps. Gastroenterol Rep (Oxf). 2015 Aug. 3 (3):254-7. [QxMD MEDLINE Link].
Watanabe-Okada E, Inazumi T, Matsukawa H, Ohyama M. Histopathological insights into hair loss in Cronkhite-Canada syndrome: Diffuse anagen-telogen conversion precedes clinical hair loss progression. Australas J Dermatol. 2013 May 29. [QxMD MEDLINE Link].
Sweetser S, Ahlquist DA, Osborn NK, Sanderson SO, Smyrk TC, Chari ST, et al. Clinicopathologic features and treatment outcomes in Cronkhite-Canada syndrome: support for autoimmunity. Dig Dis Sci. 2012 Feb. 57(2):496-502. [QxMD MEDLINE Link].
Yamakawa K, Yoshino T, Watanabe K, Kawano K, Kurita A, Matsuzaki N, et al. Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports. BMC Gastroenterol. 2016 Oct 6. 16 (1):123. [QxMD MEDLINE Link].
Qiao M, Lei Z, Nai-Zhong H, Jian-Ming X. Cronkhite-Canada syndrome with hypothyroidism. South Med J. 2005 May. 98(5):575-6. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Simon S Rabinowitz, MD, PhD, FAAP Professor of Clinical Pediatrics, Vice Chairman, Clinical Practice Development, Pediatric Gastroenterology, Hepatology, and Nutrition, State University of New York Downstate College of Medicine, The Children's Hospital at Downstate

Simon S Rabinowitz, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Gastroenterology, American Gastroenterological Association, American Medical Association, New York Academy of Sciences, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nonyelum Erica Ebigbo, MBBS Resident Physician, Department of Pediatrics, Richmond University Medical Center

Nonyelum Erica Ebigbo, MBBS is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Chris A Liacouras, MD Director of Pediatric Endoscopy, Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania School of Medicine

Chris A Liacouras, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Kanika Puri, MD Resident Physician, Department of Pediatrics, State University of New York Downstate Medical Center, Brooklyn

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor Dr Minoj Sheth to the development and writing of this article.