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Cronkhite-Canada Syndrome

Last updated Aug. 15, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Cronkhite-Canada Syndrome (CCS) is a rare, progressive disease characterized by diffuse, benign polyps in the digestive tract. Most cases of CCS involve individuals over the age of 50 years.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Allergic Granulomatous Angiitis of Cronkhite-Canada
  • Canada-Cronkhite Disease
  • Polyposis, Skin Pigmentation, Alopecia, and Fingernail Changes

What is Cronkhite-Canada Syndrome? (Definition/Background Information)

  • Cronkhite-Canada Syndrome (CCS) is a rare, progressive disease characterized by diffuse, benign polyps in the digestive tract. Most cases of CCS involve individuals over the age of 50 years
  • The cause of Cronkhite-Canada Syndrome is currently unknown; it is not believed that this condition has a genetic component
  • The primary treatment of the condition is currently symptomatic, since the underlying cause of Cronkhite-Canada Syndrome is unknown. The prognosis of this syndrome is usually poor, with a 5-year mortality of 55%, despite supportive treatment

Who gets Cronkhite-Canada Syndrome? (Age and Sex Distribution)

  • Cronkhite-Canada Syndrome usually occurs in individuals between the ages of 31 and 85 years old; mostly in adults over the age of 50 years
  • Cronkhite-Canada Syndrome affects females slightly more than males (in a 1.5:1 ratio). However, in Japan, males are affected much more than females (a 2:1 ratio exists). Approximately two-thirds of all CCS cases are from Japan
  • The remaining cases have been reported from the United States and Western Europe, where Caucasians are affected. Caucasians in North America and other parts of Europe may also be vulnerable to the condition

What are the Risk Factors for Cronkhite-Canada Syndrome? (Predisposing Factors)

  • Currently, physical and mental stress is believed to be the most significant risk factor of Cronkhite-Canada Syndrome
  • There is no evidence to suggest that CCS is a genetic disorder nor is there a family history of the disorder

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cronkhite-Canada Syndrome? (Etiology)

The underlying cause of Cronkhite-Canada Syndrome is unknown. However, it is thought to occur sporadically and is not believed to be of genetic origin.

What are the Signs and Symptoms of Cronkhite-Canada Syndrome?

The signs and symptoms of Cronkhite-Canada Syndrome include:

  • Alopecia (loss of hair)
  • Abdominal pain and cramps
  • Hyperpigmentation (large patches of dark spots on the skin or nails due to increased melanin)
  • Nausea and vomiting
  • Weight loss
  • Onychodystrophy (loss of fingernails)
  • Diarrhea
  • Excessive flatulence (passing gas)
  • Ageusia (loss of taste)
  • The presence of intestinal polyps

How is Cronkhite-Canada Syndrome Diagnosed?

A diagnosis of Cronkhite-Canada Syndrome may involve:

  • A complete physical examination with thorough medical history evaluation
  • Barium enema
  • Fluoroscopy of the gastrointestinal tract
  • Endoscopic procedures, such as:
    • Colonoscopy
    • Duodenoscopy
    • Gastroscopy
  • CT scan of the abdomen
  • Histopathological studies conducted on polypoid lesions: The specimen is examined under a microscope by a pathologist to arrive at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cronkhite-Canada Syndrome?

The complications of Cronkhite-Canada Syndrome may include:

  • Malnutrition due to malabsorption
  • Gastrointestinal bleeding; bleeding in the GI tract with anemia
  • Infection of the gastrointestinal tract
  • Rectal prolapse
  • Intussusception (one part of the intestine sliding into another adjacent part)
  • Stomach and colon cancer: Approximately 15% of the individuals with Cronkhite-Canada Syndrome ultimately develop colon/stomach cancer

How is Cronkhite-Canada Syndrome Treated?

Consistently effective treatment for Cronkhite-Canada Syndrome is currently unavailable. This in part is due to the rarity of the condition. However, any combination of the following treatment methods may be used:

  • Dietary supplementation, which has been found to be necessary, but rarely leads to a complete remission of the disease
  • Antibiotics, which may be taken to treat malabsorption caused by intestinal bacterial overgrowth
  • Corticosteroid drugs, which may help decrease intestinal inflammation
  • Anabolic steroids
  • Acid suppression therapy
  • Cromolyn
  • Administration of zinc
  • Eradication of Helicobacter pylori

Many individuals are also believed to require symptomatic treatment for diarrhea and abdominal pain. Additionally, due to the widespread nature of Cronkhite-Canada Syndrome and the increased risk of complications, surgery is only and usually performed to treat complications.

How can Cronkhite-Canada Syndrome be Prevented?

  • Due to its rarity, the underlying cause of Cronkhite-Canada Syndrome is not yet completely understood. Hence, there is no clear knowledge of how this condition may be prevented
  • Active research is currently being performed to identify certain abnormalities and potential causes responsible for Cronkhite-Canada Syndrome. This has the possibility to lead to improved treatment methods and preventive techniques
  • For individuals diagnosed with this syndrome, the healthcare providers may suggest recurring examinations of the stomach, colon, and rectum

What is the Prognosis of Cronkhite-Canada Syndrome? (Outcomes/Resolutions)

  • The prognosis of Cronkhite-Canada Syndrome is typically poor. Even with treatment, the 5-year mortality rate of this syndrome is about 55%
  • Although rare, children affected by CCS (Infantile or Juvenile Cronkhite-Canada Syndrome) have a less favorable prognosis than adults
  • Approximately 15% of all individuals affected by the condition have a predisposition to stomach cancer or colon cancer
  • Since Cronkhite-Canada Syndrome is a rare disorder, optimal screening procedures have not been developed. However, yearly endoscopic observation is usually practiced

Additional and Relevant Useful Information for Cronkhite-Canada Syndrome:

  • An internist Leonard Wolsey Cronkhite and a radiologist Wilma Jeanne Canada (both Americans) first described Cronkhite-Canada Syndrome in 1955
  • In the past 50 years, fewer than 500 cases have been reported worldwide. Primarily occurring in Japan, it has also been seen in other regions such as the United States and Western Europe

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: June 21, 2015
Last updated: Aug. 15, 2018