Pediatric Cronkhite-Canada Syndrome Treatment & Management

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

Approach Considerations

Cronkhite-Canada syndrome is a unique condition that affects the GI tract and the skin, nails, and hair. The morbidity is related to malabsorption and the potential for GI malignancy. The vast majority of patients are middle-aged Asian adults with few reports in younger patients.

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Medical Care

Neither evidence-based medicine nor systematic investigations of medical or surgical interventions are available. [30] Reported therapies are based on small nonrandomized series, retrospective surveys and expert opinions. These include supportive care, hyperalimentation, antibiotics, corticosteroids, acid suppression, cromolyn, anabolic steroids, surgery, and combinations of these therapies. Isolated anecdotal reports have described administration of zinc other immune suppressives, and eradication of H pylori. Each approach has been reported to be successful in some cases. A short communication has described the successful use of NSAIDs to regress CCS polyps, as has been described in patients with hereditary polyposis adenoma-to-carcinoma syndromes. [18]

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 and a recent review support the use of corticosteroids, immunosuppressives, [16] and, less commonly, anabolic steroids if deterioration continues. A multicenter comprehensive study indicated that about 90% of the Japanese patients received corticosteroids and that 80% of those had a good early response 30-49 mg/d). Most nonresponders were able to have a good response to other agents, such as calcineurin inhibitors, octreotide, azathioprine, and anti-TNF alpha agents. [2]

In a case series, two patients had no clinical response to steroids but marked improvement in both clinical symptoms and polyposis after beginning cyclosporine. The authors, in their analysis of medical literature, found that out of 57 patients for which outcomes have been described, 48 patients (84%) responded to steroids whereas 9 patients (16%) did not. Out of these 9 cases, 5 achieved remission with immunosuppresive therapy (calcineurin inhibitors for 4 patients and infliximab for 1). [31]

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.

One report suggest that after medical therapy has been initiated, remission of diarrhea usually occurs first (51 d), followed by reversal of dysgeusia (84 d), and then the ectodermal changes (97 d). Decrease in size and number of polyps takes considerably longer (almost 250 d). [2]  The attenuation of polyps often results in a nodular mucosa with a cobblestone appearance. [12]

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Surgical Care

Some patients were diagnosed only after surgical resection and others have 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).

Among 36 Japanese patients, 37 operations were performed. The indications were for 19 colon cancers, 7 gastric cancers, 3 gastroduodenal adenomas, 4 intussusception cases, 2 GI bleeding cases, 1 gastric gastrointestinal stromal tumor, and 1 severe enteric protein loss case. No mortalities were reported in this series, and the mean duration of posttreatment survival was 5 years (range of 0-14 years). [2]  

Newer imaging techniques such as the capsule endoscopy and technetium Tc 99m–labeled human serum albumin nuclear medicine scans can enable surgeons to identify specific regions of the bowel that have either suspicious lesions or more active malabsorption. These hold promise for directing limited resections and improving overall outcomes.

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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

Parenteral or enteral nutritional therapy has been used in about half of the Japanese patients who received corticosteroids. No difference was reported in early response between patients receiving this form of therapy and those who did not. [2]

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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Long-Term Monitoring

The goals of therapy are to restore nutritional adequacy and prevent complications. The most significant problem to avoid is CCS-related cancer. The experience in Japan has demonstrated that sustained improvement demonstrated by serial endoscopies correlated with a decreased risk of cancer and mortality. Among 66 patients with sustained endoscopic remission, only one new case of cancer was reported. However, among 42 patients who did not achieve long-term response, the risk of cancer was 40%, and the risk of CCS-related death was 16% risk. [2]

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