Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Cronkhite-Canada Syndrome Follow-up

  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Nov 17, 2015
 

Further Outpatient Care

See the list below:

  • Although remissions may persist for longer than 10 years, patients must be monitored for nutritional deficiencies.
  • Patients should regularly undergo upper endoscopy and colonoscopy because 10% of patients with Cronkhite-Canada syndrome develop GI (stomach, colon) cancer. Guidelines for colorectal cancer screening have been established by the American College of Gastroenterology.[27] . The authors of a large multicenter Japanese studying describing over 200 patients suggest annual surveillance colonoscopy.[2]
  • Steroid therapy, which is expected to diminish the inflammatory polyp burden, should precede endoscopic surveillance to assist in the identification of malignancy. Advanced endoscopic techniques, including narrow-band imaging, magnifying endoscopy, and dye-based contrast-enhanced techniques, have been used to improve sensitivity.[2]
Next

Further Inpatient Care

See the list below:

  • Hyperalimentation, including electrolytes, minerals, protein, vitamins, dextrose, and lipids, are given because of the clinically significant losses of nutrients and fluid from the GI tract.
  • Monitor the patient for impending surgical complications.
Previous
Next

Inpatient & Outpatient Medications

See the list below:

  • The literature does not support prophylactic therapy.
  • All therapy should be individualized for the patient's specific symptoms and complications.
Previous
Next

Transfer

See the list below:

  • Consider transferring the patient to an intensive care unit for surgical intervention, diagnostic and therapeutic GI endoscopy, and parenteral hyperalimentation.
Previous
Next

Deterrence/Prevention

See the list below:

  • Specific recommendations are not available.
  • Epidemiologic data do not suggest specific infectious etiology.
Previous
Next

Complications

See the list below:

  • Dehydration, electrolyte abnormalities, and shock
  • Kwashiorkor and various nutritional deficiencies
  • Small bowel overgrowth, systemic infections, and sepsis
  • Anemia related to GI blood losses and deficiencies
  • Edema, anasarca, and congestive heart failure
  • Thromboembolic phenomena
  • Reportedly high rates of surgical complications
  • Secondary immune deficiencies
Previous
Next

Prognosis

See the list below:

  • Early reports indicated a grave prognosis. Large series have shown that more than one half of affected patients have a remission; long-term survivors are reported.
  • Survival largely depends on adequate and sometimes prolonged intensive care.
  • Although the diagnosis depends on alopecia, nail dystrophy, or pigmentation changes, the prognosis is related to only the GI mucosal pathology.
Previous
Next

Patient Education

See the list below:

  • Patients who go into remission should promptly report any change in GI symptoms or weight loss to their physicians.
  • Regular surveillance endoscopy is important for long-term survivors.
  • Patients should be reassured that Cronkhite-Canada syndrome is neither contagious nor familial.
Previous
 
Contributor Information and Disclosures
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 Gastroenterological Association, American Academy of Pediatrics, Phi Beta Kappa, American Association for the Advancement of Science, American College of Gastroenterology, American Medical Association, New York Academy of Sciences, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

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

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.

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.

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.

References
  1. 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. [Medline].

  2. 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. [Medline].

  3. Kopáčová M, Urban O, Cyrany J, et al. Cronkhite-Canada syndrome: review of the literature. Gastroenterol Res Pract. 2013. 2013:856873. [Medline].

  4. 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. [Medline]. [Full Text].

  5. Yashiro M, Kobayashi H, Kubo N, et al. Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. Digestion. 2004. 69(1):57-62. [Medline].

  6. Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nippon Geka Hokan. 1995 Jan 1. 64(1):3-14. [Medline].

  7. 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. [Medline].

  8. 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. [Medline].

  9. 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. [Medline].

  10. 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. [Medline].

  11. Dachman AH, Buck JL, Burke AP, Sobin LH. Cronkhite-Canada syndrome: radiologic features. Gastrointest Radiol. 1989. 14(4):285-90. [Medline].

  12. 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. [Medline].

  13. 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. [Medline].

  14. Freeman K, Anthony PP, Miller DS, Warin AP. Cronkhite Canada syndrome: a new hypothesis. Gut. 1985 May. 26(5):531-6. [Medline].

  15. [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. [Medline].

  16. Cao XC, Wang BM, Han ZC. Wireless capsule endoscopic findings in Cronkhite-Canada syndrome. Gut. 2006. 55 (6):899-900. [Medline].

  17. 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. [Medline].

  18. 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. [Medline].

  19. Russell DM, Bhathal PS, St John DJ. Complete remission in Cronkhite-Canada syndrome. Gastroenterology. 1983 Jul. 85(1):180-5. [Medline].

  20. Samoha S, Arber N. Cronkhite-Canada syndrome. Digestion. 2005. 71(4):199-200. [Medline].

  21. Ward EM, Wolfsen HC. Pharmacological management of Cronkhite-Canada syndrome. Expert Opin Pharmacother. 2003 Mar. 4(3):385-9. [Medline].

  22. 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. [Medline].

  23. 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. [Medline].

  24. 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. [Medline].

  25. Suzuki R, Irisawa A, Hikichi T, Takahashi Y, Kobayashi H, Kumakawa H. Cronkhite-Canada syndrome associated with myelodysplastic syndrome. World J Gastroenterol. 2009 Dec 14. 15(46):5871-4. [Medline].

  26. 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. [Medline].

  27. Qiao M, Lei Z, Nai-Zhong H, Jian-Ming X. Cronkhite-Canada syndrome with hypothyroidism. South Med J. 2005 May. 98(5):575-6. [Medline].

  28. Yuan B, Jin X, Zhu R, Zhang X, Liu J, Wan H. Cronkhite-Canada syndrome associated with rib fractures: a case report. BMC Gastroenterol. 2010. 10:121. [Medline].

  29. Hatogai K, Hosoe N, Imaeda H, Rey JF, Okada S, Ishibashi Y. Role of enhanced visibility in evaluating polyposis syndromes using a newly developed contrast image capsule endoscope. Gut Liver. 2012 Apr. 6(2):218-22. [Medline].

  30. Karasawa H, Miura K, Ishida K, Sase T, Kobayashi T, Kinouchi M. Cronkhite-Canada syndrome complicated with huge intramucosal gastric cancer. Gastric Cancer. 2009. 12(2):113-7. [Medline].

  31. Peitl A, Vucic Peitl M, Pavlovic E, Ljubicic D. Acute brain syndrome as a consequence of the Cronkhite-Canada syndrome. Psychiatr Danub. 2005 Jun. 17(1-2):90-3. [Medline].

  32. Samet JD, Horton KM, Fishman EK, Iacobuzio-Donahue CA. Cronkhite-Canada Syndrome: Gastric Involvement Diagnosed by MDCT. Case Report Med. 2009. 2009:148795. [Medline].

  33. Tseng KC, Sheu BS, Lee JC, Tsai HM, Chiu NT, Dai YC. Application of technetium-99m-labeled human serum albumin scan to assist surgical treatment of protein-losing enteropathy in Cronkhite-Canada syndrome: report of a case. Dis Colon Rectum. 2005 Apr. 48(4):870-3. [Medline].

  34. Watari J, Morita T, Sakurai J, Yamasaki T, Okugawa T, Toyoshima F, et al. Endoscopically treated Cronkhite-Canada syndrome associated with minute intramucosal gastric cancer: an analysis of molecular pathology. Dig Endosc. 2011 Oct. 23(4):319-23. [Medline].

Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.