Dystopia canthorum

Many of us wonder what dystopia canthorum could be. Not many are aware about this syndrome and what exactly it means and what is the cause for the same. Let’s read further to check what is it all about?

Waardenburg syndrome comprises of many hereditary disorders, like the abnormal pigmentation of color, hearing loss and difference in the facial features.

Dr. Petrus Waardenburg reported a syndrome of dystopia canthorum. It is also called as Telecanthus.  In this situation the facial feature is a little deformed, where the eyes are not spaced normally. They are far from each other however, the pupils are placed properly. Due to it one’s eyes appear too far (widely spaced) though they are not far.

People affected by it often have white forelock and iris heterochromia. Which means that the hair growth on the forehead is white and the color of the eyes are different. And some may be partial in individual irises or may be the entire iris in one eye matching the pattern of iris. Mostly half the portion of the iris is brilliant blue.

In Dystopia canthorum due to the prominent nasal root and increased intercantal distance it may result to hypertelorism which means excessive width between two body organs.

Inheritance is typical for the Waardenburg syndrome. These are transmitted as autosomal dominant with interfamilial and intrafamilal variability. The major cause for this is mutation in multiple genes.

Any other malfunction related to dystopia canthorum may be due to involvement of micro deletions or contiguous gene defects.

The individuals affected with it may suffer from neural tube defects, cleft lip and palate. It also results in limb abnormalities and Hirsch sprung disease.

This syndrome is not known from any racial or ethnic predilection. It affects male and female with same frequency. It can be diagnosed in children at an early age however; in some individuals it may be undiagnosed until people learn about it.

Physical appearance of dystopia canthorum is complete or segmental heterochromia and Brilliant sapphire blue eyes.

There is a 50% chance in each pregnancy having an affected offspring of it. The features can be widely different within families and it is impossible to predict if the offspring would be less or more affected than the parents.

In some cases surgery may be required to repair the severe cases. In some cases the neural tube defects, cleft lip or palate or the Hirsch sprung disease also needs a surgery.

It requires no special diet. It also does not restrict any activities. Currently for dystopia canthorum drug therapy is not a component of the standard of care.

Further impatient care may be needed for patients who are undergoing the surgery for repair of neural tube defects, cleft lip or palate, limb abnormalities, and Hirsch sprung disease.