Pediatric Cronkhite-Canada Syndrome Treatment & Management

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

Medical Care

  • Neither evidence-based medicine nor systematic investigations of medical or surgical interventions are available.[11] Reported therapies include supportive care, hyperalimentation, antibiotics, corticosteroids, acid suppression, cromolyn, anabolic steroids, surgery, administration of zinc, and eradication of H pylori with standard therapy as well as combinations of these therapies. Each approach is successful in some cases. A short communication has described the successful use of NSAIDs to regress Cronkhite-Canada syndrome (CCS) polyps, as has been described in patients with hereditary polyposis adenoma-to-carcinoma syndromes.[5]
  • A published expert opinion described successful management recommendations that included treating GI symptoms, aggressive nutritional support, and vigilant monitoring for complications requiring emergent surgery. Many anecdotal reports support the use of corticosteroid and, less commonly, anabolic steroids if deterioration continues. Antibiotics are indicated for sepsis, peritonitis, and possible small-bowel overgrowth.
  • Abdominal pain, anorexia, and Hemoccult-positive stools may be related to gastroduodenal mucosal erosions that respond to acid suppression and sucralfate. Flatulence of abdominal pain secondary to distension is addressed by restricting disaccharides, especially lactose, and decreasing small-bowel flora. Dramatic increases in abdominal pain are secondary to perforation or intussusception.
  • The degree of malabsorption present dictates the form of nutritional therapy. Elimination diets or antiperistaltic agents attenuate diarrhea. Mild malnutrition is reversed with replacement of nutrients, the above measures, and treatment of small bowel overgrowth. Specific antibiotic regimens are not suggested. Elemental diets and parenteral hyperalimentation are required with progressive compromise.
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Surgical Care

  • Reports describe patients whose conditions were diagnosed only after surgical resection and others who achieved symptomatic remission after the polypoid tissue was removed. Because of the generalized nature of Cronkhite-Canada syndrome and the possibility of operative complications in an elderly and compromised patient population, surgery is usually reserved to treat complications (eg, bleeding ulcers, perforation, intussusception, malignancy).
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Consultations

  • The team that successfully cares for a patient with Cronkhite-Canada syndrome includes a nutritional support group, a surgeon, and a gastroenterologist able to perform diagnostic and therapeutic endoscopy.
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Diet

  • As outlined above, dietary requirements evolve as the mucosal involvement varies. Preservation of an adequate nutritional status is essential to minimize morbidity.
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Activity

  • The overall clinical condition of the patient determines the level of physical activity. Prophylactic dietary or activity modifications are not recommended as prevention for Cronkhite-Canada syndrome.
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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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