Cronkhite-Canada Syndrome Treatment & Management

Updated: Feb 22, 2019
  • Author: Melba Estrella, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Because of the unknown etiology, treatment for Cronkhite-Canada syndrome (CCS) remains predominantly symptomatic. Controlled therapeutic trials have not been possible because of the rarity of the disease. Remissions may occur spontaneously.

The primary goal of the treatment is to correct fluid, electrolyte, and protein loss, and to regulate stool frequency. These measures help improve the patient's general condition. Most patients need symptomatic treatment for abdominal pain.

The most effective treatment is combination therapy composed of systemic corticosteroids together with an antiplasmin, an elemental diet, and hyperalimentation (nutritional supplements). Antibiotics are used to correct intestinal bacterial overgrowth syndrome. The indication for corticosteroids is gastrointestinal inflammation; however, its origin is not clear. Yamakawa et al reported two cases of steroid-resistant Cronkhite-Canada syndrome that were effectively treated with cyclosporine. Dramatic improvement of clinical symptoms and endoscopic findings were observed in both cases. [20] Another case in which multiple treatments, including steroids, azathioprine, adalimumab, and cyclosporine, failed was treated with sirolimus, with apparent clinical and endoscopic remission of the disease. [66] One case has been reported to be treated with rituximab. [34]

Reports describe clinical response in Cronkhite-Canada syndrome to tumor necrosis factor (TNF) inhibitors, with improvement of symptoms, weight gain, and polyp regression. These findings reinforce the theory of this syndrome’s inflammatory etiology. [11, 29, 67, 68, 69] Immunosuppression in Cronkhite-Canada syndrome patients should be cautiously performed, owing to the potential accelerated malignancy risk.

Nutritional supplementation includes oral and/or intravenous fluids, electrolytes, vitamins, minerals, amino acids, albumins, and lipids.

Transfusions are sometimes required for severe anemia or acute blood loss.

In one reported case with elevated gastric acid secretion, the patient responded very well to ranitidine therapy. [70] Other medications used are sulfasalazine and metronidazole.

After H pylori eradication, gastric polypectomy can be of value. [17]

Emergent re-admission of patients with Cronkhite-Canada syndrome may occur because of significant diarrhea, gastrointestinal bleeding, intussusception, prolapse of gastric polyp-bearing mucosa, and thromboembolic episodes due to dehydration.

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Surgical Care

At present, surgery is needed only for complications of Cronkhite-Canada syndrome (CCS), such us prolapse, bowel obstruction, and malignancy. Other authors suggest that surgical intervention should also be reserved for patients who are not responsive to conservative methods.

Surgical intervention carries a significant risk in weakened patients with Cronkhite-Canada syndrome. Careful consideration must be given to the clinical course of Cronkhite-Canada syndrome before administration of systemic corticosteroids.

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Consultations

Gastroenterologist consultation always is required to establish number, localization, and size of polypoid lesions.

Consultation with a surgeon can help determine if surgical intervention is necessary.

Acute brain syndrome requires psychiatrist consultation.

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Long-Term Monitoring

Some Cronkhite-Canada syndrome (CCS) patients remain asymptomatic after the hospital treatment and do not require further therapy. Prolonged corticosteroid therapy is necessary in other patients.

Controlling the macronutrient and micronutrient balance, periodically performing endoscopic examination of the gastrointestinal tract, and testing for occult blood presence in the stool are recommended.

It is essential to explain that patients must be reliable with follow-up care and regular examination of the gastrointestinal tract. Large polyps (>1 cm in diameter) require biopsy and pathologic examination.

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