In many cases, the symptoms appear in the following order:
The ectodermal changes occur after a few weeks or a few months. The first and major complaint from patients is that of periodic or constant pain in the upper or lower abdomen. The intensity varies greatly from mild and localized to generalized and severe . The pain comes with recurrent watery diarrhea or chronic diarrhea and in some cases, melena.
The watery bowel movements may be reported 7 times or more each day. In some cases, stool volume as high as 4-6 L/d are reported. Following the diarrhea is progressive weight loss.
Anorexia have been reported in some patients and loss of smell and change in taste sensations have equally been reported
Other gastrointestinal symptoms are nausea and vomiting and this is more readily seen in female patients.
Typically, the patients experience hair loss and swallowing difficulties have been reported as well. The hair loss happens in tandem across the extremities, pubic areas, axillae, face, eyebrows and scalp. However, some patients have presented loss of hair in only the scalp area. Alopecia takes place very fast with reports of total hair loss with a few days common .
Other ectodermal changes that happen in many cases include hyperpigmentation of the skin, vitiligo, discoloured and ragged fingernails (nail dystrophy) which often results in onycholysis. Some neurologoc symptoms have been reported as well and these include sensory neuropathy, syncope, seizures, vestibular disturbances (ie, gaze-evoked nystagmus, dysequilibrium) and sensory neuropathy.
Hematology tests are carried out to check for hematocrit, red blood cell count, hemoglobin and depressed white blood cell count.
Serum protein tests aid in checking hypoalbuminemia, hypoproteinamia and increase in alpha 1 globulin level.
Endoscopic tests procedures show polyps lesions of the sessile or semipedunculated type within the stomach, colon, Ileum and duodenum. The endoscopic procedures to be carried out include gastroscopy, colonoscopy and sigmoidoscopy.
Treatment remains predominantly symptomatic due to the unknown etiology. Management aims at correcting the fluid, electrolyte, and protein loss.
Cronkhite-Canada syndrome is considered a progressive disease with a variable course and poor prognosis.
Newer findings have disputed this hypothesis however as hair and nail changes often do not show signs of improvement even with improved nutrition .
There are other conditions which show multiple harmartomatous polyps of the digestive system. These include Cowden disease, juvenile polyposis and Peutz-Jeghers syndrome. Related polyposis conditions include MUTYH, Birt–Hogg–Dubé syndrome, attenuated familial adenomatous polyposis and familial adenomatous polyposis .
The CCS has a worldwide distribution but 75% of reports come from Japan. The Cronkhite Syndrome is a rare disorder because by the end of 2002, only 467 cases have been reported in literature across the world with 354 of those coming from groups in Japan.
The CCS is regarded as a progressive condition which has poor prognosis and runs a variable course and is dependent mainly on the control of electrolyte balance and protein . Regardless of therapy used, the mortality rate is more than 50%. Co-existing gastrointestinal bleeding, the possibility of intussusception and malignant changes in the polyps all lead to an increase in mortality rate.
The first two patients who were described to have the Cronkhite-Canada syndrome died of starvation 7-8 months after the beginning of symptoms. There have been reported cases of spontaneous remission after nutritional support. In children, the prognosis is often less optimistic than what is obtainable amongst adults .
Although majority of cases come from Japan, there is no data to prove any racial predisposition. Therefore the condition is treated as a worldwide condition.
Sex-wise, the male to female ratio is 1.5-1.31 approximately so the condition is believed to affect both sexes equally.
The Cronkhite-Canda syndrome often sets in between middle age and old age with average age being 55 years. The age rang at which this condition is seen mostly is 31-86 years of age. The disease may stay asymptomatic and therefore it may not be diagnosed for quite a while. Many patients are aged 50 and above by the time of presentation. There have been very scanty reports regarding cases of infantile Cronkhite-Canada Syndrome .
There is no evidence to show the existence of familial predisposition. There is equally the possibility of afflicted patients and offspring that are asymptomatic. The most important risk factors for the CCS are mental stress and physical stress .
This stress brings about a local inflammatory reaction since it acts on the gastrointestinal mucosa.
Two weeks after the start of taking oral thyroxine, some patients with the CCS develop ectodermal lesions. At cessation of treatment, the cutaneous signs improved.
The gastrointestinal lesions in CCS are polyps of the harmatomatous type and these histologically show pseudopolypoid-inflammatory changes. Malabsorption is believed to be what leads to the cutaneous symptoms. Changes that occur in the ectorderm do not happen in tandem with other activities of the disease and so it improved in some cases despite gut dysfunction .
There are no guidelines for prevention of Cronkhite-Canda syndrome.
Cronkhite-Canada syndrome (CCS) is a rare non-inherited disorder that was reported for the first time in 1955 by Leonard W. Cronkhite Jr and Wilma J. Canada .
The condition occurs sporadically. It was seen for the first time as a separate condition within 2 female patients who have onychodystrophy, alopecia, cutaneous pigmentation and generalized gastrointestinal polyps.
If diagnosed for this condition, one way to deal with it is to judiciously stick to medical regimes reporting anything unusual as soon as possible.