Pediatric Cronkhite-Canada Syndrome Follow-up

Updated: Oct 20, 2017
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD  more...
  • Print
Follow-up

Further Outpatient Care

Although remissions may persist for longer than 10 years, patients must be monitored for nutritional deficiencies.

Patients should  also 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. [32] . 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

Hyperalimentation, including electrolytes, minerals, protein, vitamins, dextrose, and lipids, are indicated in the face of clinically significant losses of nutrients and fluid from the GI tract.

Monitor the patient for impending surgical complications.

Previous
Next:

Inpatient & Outpatient Medications

The literature does not support prophylactic therapy.

All therapy should be individualized for the patient's specific symptoms and complications.

Previous
Next:

Transfer

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

Previous
Next:

Deterrence/Prevention

Specific recommendations are not available.

Epidemiologic data do not suggest specific infectious etiology.

Maintenace of adequate nutritional status, including normal levels of vitamins, is essential to minimize complications.

Previous
Next:

Complications

Complications include the following:

  • 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
  • Malignant transformation: After diffuse inflammatory polyps have responded to steroid therapy, other existing adenomas require endoscopic treatments, which can decrease the possibility of neoplastic transformation. [17]

Previous
Next:

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.

Previous
Next:

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.

Previous