Cronkhite-Canada syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at January 9, 2017
StartDiseasesCronkhite-Canada syndrome

Cronkhite-Canada syndrome is a condition characterized by polyps of the gastrointestinal tract as well as alopecia and problems associated with their nails. These polyps are hamartomas, which are non-malignant masses. It is not an inherited disorder. Approximately 500 cases have been documented in the medical literature, making this an exceptionally rare condition.

Contents

Definition & Facts

Cronkhite-Canada syndrome was discovered and recognized as a disorder in 1955. It is extremely rare. This condition is characterized by the presence of hamartomas. These polyps do not develop in the esophagus but otherwise can grow anywhere in the gastrointestinal tract.

This condition may cause an increased risk of colon cancer and gastric cancer due to the possibility that these polyps can become malignant. Because of the possible increased cancer risk, people with this condition are recommended to be vigilant and have frequent and extensive cancer screenings.

Cronkhite-Canada syndrome resembles other disorders such as familial adenomatous polyposis and Cowden syndrome. As such, diagnosis of this condition must rule out these other conditions.

Cronkhite-Canada is more common among men than women, and it tends to manifest in people over the age of 50. It seems to occur more often in Japan than in other locations.

Symptoms & Complaints

Abdominal pain is a persistent symptom of this condition. Symptoms of this condition include diarrhea. Diarrhea involves bowel movements which are loose and watery. The diarrhea seen in patients with Cronkhite-Canada syndrome is chronic; that is, it lasts longer than two weeks.

Alopecia is another symptom. With this symptom, a patient will display patches of hairlessness. This baldness could affect the scalp and the face. It has also been reported to affect the eyebrows and pubic hair.

There will be other symptoms pertaining to the skin including hyperpigmentation and onychatrophia. Hyperpigmentation is a symptom in which certain parts of the skin are a different color than other parts of the skin. This can take the form of vitiligo. Onychatrophia is a symptom in which the nail begins wasting away or atrophying. The nail may also be discolored.

Unexplained weight loss can also occur among those with this condition. Edema can also be a symptom, which is bodily swelling due to the buildup of fluid. Difficulty swallowing is another symptom of this condition. Pancreatitis can strike those with this condition as well.

Sometimes, this condition can cause neurological problems. Nystagmus is a symptom associated with this condition. It involves uncontrolled eye movements. Eye movements are involuntary and repetitive. This can make simple tasks like driving a car impossible or highly difficult. Seizures may also occur in association with this condition.

Causes

It is unclear what causes this syndrome, though it is not believed to be inherited or present among families. It may be associated with arsenic poisoning. There is evidence to suggest it is a type of autoimmune disease including elevated IgG4 (immunoglobulin G4) levels. It is often seen with autoimmune disorders such as systemic lupus erythematosus and rheumatoid arthritis.

It is unclear if some of the symptoms affecting the skin (hyperpigmentation, alopecia, etc) of Cronkhite-Canada syndrome are caused by malabsorption of nutrients due to the presence of polyps or if they are manifestations of the underlying syndrome. Weight loss is likely due to malabsorption that results from polyps.

Diagnosis & Tests

Cronkhite-Canada syndrome is diagnosed via the presentation of several signs: nail discoloration or atrophy, alopecia, and the presence of hamartomas in the gastrointestinal tract. The first two symptoms can be detected via a physical examination. The health care professional will take note of both fingernails and toenails that are cracked, shedding, and discolored. They will note hair loss. They will also detect skin changes such as discolored patches of skin. The patches may be darker than the rest of the skin or they may be lighter.

The diagnostic process will also involve the health care professional taking note of any edema presenting in the patient. Any unexplained weight loss will be noted, as this condition can cause severe weight loss.

Polyps in the gastrointestinal tract can be detect via various imaging studies. These include a colonoscopy and a gastroscopy. These will be conducted through the use of an endoscope, which is a thin, flexible instrument that is inserted into the body and has a camera on its end.

Another imaging examination that can assist in diagnosis is a computed tomography (CT) scan. This is a type of imaging study that produces a three-dimensional image, and it will be taken of the abdomen in order to detect polyps. The use of a barium enema may also be an option to examine the gastrointestinal tract.

A number of different laboratory examinations will also be conducted. These include stool tests, clinical urine tests, and various blood tests. Erythrocyte sedimentation rate is one type of blood test. A complete blood count will likely be taken. The patient may also be tested for Helicobacter pylori infection.

Treatment & Therapy

There is no established regimen for treating Cronkhite-Canada syndrome because it is a rare disease that has not been thoroughly studied. Current treatment approaches involve the use of corticosteroids in order to reduce inflammation in the gastrointestinal tract. Prednisone is a type of steroid that is used to treat this condition.

Antibiotics may also be used to treat a Helicobacter pylori infection or other bacterial infection that may be present in the digestive system.

Prevention & Prophylaxis

Cronkhite-Canada syndrome cannot be prevented because its causes are still poorly understood. Though the link between the polyps that characterize this condition and an increased risk for cancer is still controversial as of 2012 according to the National Institutes of Health, adopting an aggressive approach towards cancer screenings may be necessary.