The Cronkhite-Canada syndrome consists of many hamartomatous polyps in the stomach, small intestine, and colon that resemble juvenile polyp’s retention.
The syndrome is a rare condition, and medical experts almost always see it occur to adults (the average age being around the late 50’s).
However, there are some reports of cases with younger people getting affected by this disease.
The Cronkhite-Canada syndrome is so rare that, worldwide, medical experts had reported fewer than 500 cases the past decades.
Patients suffering from this fatal disorder usually display watery diarrhea, abdominal discomfort, and anorexia; death occurs from starvation.
The syndrome is different from other diseases accompanied by polyposis because of the late start, lack of family history and its ectodermal-only features.
The treatment changed over time. At first doctors resorted to surgery to get rid of the polyps, it seemed to help in relieving some of the symptoms of Cronkhite-Canada syndrome.
But polyps usually reappeared or they were too many for the doctors.
Nowadays, medical experts facilitate the removal of the organ or polyps segment by the use of overfeeding, corticosteroids and anti-inflammatory medicines.
The etiopathogenesis of the disease is currently unknown; there is no practical treatment with a scientific base and, although experts had seen some cases in which patients show significant signs of response to empiric therapies, the prognosis is usually poor.
Medical experts are still unsure if an early diagnosis could make the forecast any better.
Some doctors are still dealing with some new cases of this mysterious and infrequent disease, and they have no medical treatment response.
That is the reason why researchers are seeking to improve the casuistry, as well as adding to the available information about the disease, to promote, through higher knowledge and diffusion, the search for an effective treatment.
Many people deem this syndrome as something both unusual and exceptional. It can feature the presence of hereditary nonpolyposis gastrointestinal, alopecia, hyperpigmentation and nail dystrophy.
Another sign typical to this condition is the occurrence of diarrhea and weight loss. The appearance of polyps which are hamartomatous (focal malformations that resemble neoplasms in the tissue of their origin) and usually develop in the stomach and intestine.
Although polyps do not usually have a malignant capacity, the prognosis is typically adverse, and the treatment is most times ineffective.
The polyps that cover the walls of the stomach, small intestine, and colon cause malabsorption, which means the body can’t properly absorb nutrients from food.
It has a worldwide distribution and although it has been relating in a wide age range (31-86 years), more than 80% of patients, in a review of 55 patients had more than 50 years.
Approximately 15% of the people with Cronkhite-Canada syndrome eventually develop cancer of the colon.
Clinical manifestations follow a sequential order. First, diarrhea, followed by weight loss and the edemas. Ectodermal changes occur later but sometimes may precede symptoms.
Diarrhea is the primary symptom and is manifested in the form of 5-7 liquid stools per day, usually without blood, although in isolated cases rectal bleeding has been the cause of death.
Although its pathogenesis is unknown, some medical experts had suggested bacterial overgrowth leads to the condition; other researchers see immune deficiency and lousy absorption as potential causes of the syndrome.
Weight loss is typical and on average is between 8 and 10 kilos. Abdominal pain is a symptom that varies in rate and intensity; it usually occurs as abdominal discomfort or colic.
People with Cronkhite-Canada syndrome may have abnormally low levels of protein in the blood, which causes weakness, nausea, vomiting, loss of taste and paresthesia.
On physical examination, the nail changes are the most common finding. Hair loss may precede other manifestations years.
Although youth polyps are not premalignant, they have described some cases as a form of carcinoma, usually in the colon or rectum.
The primary treatment is an asymptomatic type of electrolyte replacement. Some medical experts have also used anabolic steroids and dubious therapeutic effects.
Doctors only resort to surgical resection of the affected segment when complications (such as bleeding, intussusception, and prolapse) occur. Antibiotics have used in cases of bacterial overgrowth.
Recently it has reported a treated by a combination of H1 and H2 antihistaminícios (loratadine and ranitidine), cromolyn sodium, prednisone and ciprofloxacin with the disappearance of polyps and a general improvement of the patient’s case.