Pediatric Cronkhite-Canada Syndrome Follow-up

  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Jan 11, 2010
 

Further Inpatient Care

  • 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.
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Further Outpatient Care

  • 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 more than 10% of patients with Cronkhite-Canada syndrome (CCS) develop GI (stomach, colon) cancer. Guidelines for colorectal cancer screening have been established by the American College of Gastroenterology.[12]
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Inpatient & Outpatient Medications

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

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

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

  • 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
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Prognosis

  • 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.
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Patient Education

  • 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.
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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 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 and Nutrition, Phi Beta Kappa, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Manoj Sheth, MD  Chief Resident, Department of Pediatrics, Richmond University Medical Center of Long Island

Manoj Sheth, MD is a member of the following medical societies: Indian Medical Association and Indian Orthopedic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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

Disclosure: Nothing to disclose.

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, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

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, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

References

  1. Ruymann FB. Juvenile polyps with cachexia. Report of an infant and comparison with Cronkhite-Canada syndrome in adults. Gastroenterology. Oct 1969;57(4):431-8. [Medline].

  2. 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].

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

  4. Vernia P, Marcheggiano A, Marinaro V, et al. Is Cronkhite-Canada Syndrome necessarily a late-onset disease?. Eur J Gastroenterol Hepatol. Oct 2005;17(10):1139-41. [Medline].

  5. 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].

  6. 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].

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

  8. Kilcheski T, Kressel HY, Laufer I, Rogers D. The radiographic appearance of the stomach in Cronkhite-Canada syndrome. Radiology. Oct 1981;141(1):57-60. [Medline].

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

  10. 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. Nov 1 1988;57(6):506-26. [Medline].

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

  12. [Guideline] Dominic OG, McGarrity T, Dignan M, Lengerich EJ. American College of Gastroenterology Guidelines for Colorectal Cancer Screening 2008. Am J Gastroenterol. Oct 2009;104(10):2626-7; author reply 2628-9. [Medline].

  13. 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). Sep 1982;61(5):293-309. [Medline].

  14. 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. Sep 1980;30(5):825-45. [Medline].

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

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

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

 
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