Central heterochromia- 25+ Causes, Types, Diagnosis, Treatment, Pictures

Central Heterochromia is a very rare condition affecting less than 1% people in the world. Due to this rarity of central heterochromia, the people with this disorder appear to be unique, and noticeable. But, many people have never heard the term central heterochromia. So, let us try to learn in detail about this condition.

What is Heterochromia?

Heterochromia is used to describe the difference in the color of a person’s eyes, skin or hair. But, most commonly it is used to describe the difference in the color of the eye in a person. Heterochromia is a term which is derived from the Greek language. As per the Greek language, “Heteros” means different, and “Chroma” means color.

There are many other terms used to describe heterochromia as synonyms. They are heterochromia iridis and heterochromia iridum. These terms are coined based on the word iris because iris is the part of the eye which imparts color to our eyes, and in this condition, there is a difference in the color of the iris of the affected person.

Have you ever heard of melanin pigment? We have people belonging to different races and skin colors based on melanin pigment in the skin. Africans have a higher amount of melanin pigment in their skin. Caucasians have lesser melanin pigment or no melanin pigment in their skin. Melanin pigment is also present in the iris of the eye and in the hair. It imparts color to all these body parts.

A person with heterochromia might have changes in the amount of melanin present within a single eye or between the two eyes. Heterochromia is not only unique to human beings, as it is also seen in certain types of animals like cats, horses, and dogs. In fact, many celebrities are popularly known for their heterochromia eyes.

Some people think that heterochromia is a disease or disorder. But, the fact is that there are no alarming symptoms or problems associated with heterochromia in the majority of people. It is just a condition with unique eye colors.

The affected person is completely healthy and has no associated health ailments or problems. But, if heterochromia is occurring in the person as part of a disorder or disease, then he might have symptoms of the disease along with heterochromia.

Heterochromia types:

You have seen what is heterochromia. Now, let us look into different types of heterochromia.

Complete heterochromia:

In this type of heterochromia, the affected person has each eye of a different color. For example, a person might have one blue eye and one black eye, or a person might have one brown eye and one black eye, etc.

Central heterochromia:

This condition is also known with the name of cat eyes. Now, you might have remembered that you have seen many people with cat eyes in your life, and you find them to be unique. In central heterochromia, there are 2 different colors within one eye or iris. But, both the eyes appear similar to each other.

In this condition, there are 2 rings or circles around the pupil which is the central hole in the iris. The inner circle around pupil has a different color, and the outer circle or ring around the pupil has a different color.

The inner circle around the iris appears as having multiple different colors with spikes. The outer ring has a single color, so it is thought to be the original color of the iris of the person. It can happen when a person is having reduced levels of melanin pigment in their irises.

Sectoral heterochromia:

It is also known as a synonym or other name, partial heterochromia. It also has 2 different colors within a single eye or iris. But, it is different from central heterochromia. In central heterochromia, there are 2 distinct rings or circles around the pupil. But in sectoral heterochromia, there are no rings or circles around the pupil. We can only see a distinct spot of different color within the iris.

Heterochromia Causes:

There are many causes of heterochromia, including the congenital problems and also the acquired ones, which cause heterochromia at a later part of life. Let us delve into them.

Congenital causes of heterochromia:

Pigment dispersion syndrome: It is abbreviated as PDS. In this condition, the pigment cells detach from the posterior part of the iris and enter the aqueous humor in the eyes. It can lead to the development of a type of glaucoma known as pigmentary glaucoma.

Sturge-Weber syndrome: In this condition, there is the development of a red colored stain called port wine stain at birth on the forehead, scalp and around the eyes. These people may also have some problems with the blood capillaries of the brain.

Melanosis oculi: It is also known as ocular melanosis. It can affect about 1 in every 5000 to 6000 people. It can lead to a malignant tumor of the uveal tract called uveal melanoma.

Congenital Horner’s syndrome: It is a disorder in which the sympathetic nervous system is affected. It leads to several symptoms in the affected person like ptosis or drooping of eyes, miosis or smaller pupils, anhydrosis or decreased sweating, loss of ciliospinal reflex and enophthalmos or eyes becoming deeper in the eye socket.

Piebaldism: In this condition, the affected person has macules of reduced pigmentation of the body, on the forehead, and the person also has a white colored forelock from birth.

Simple congenital Heterochromia: It is present since birth and the person has no other problems apart from central heterochromia of the eyes.

Waardenburg syndrome: This condition is very rare. In this condition, the person can have deafness, neurological problems, and pigmentation changes.

Tuberous sclerosis: It is a condition in which there is the development of noncancerous tumors in different parts of the body like brain, heart, lungs, skin, kidneys, etc.

Bloch Sulzberger syndrome: It is also known with the names like Bloch Siemens syndrome,  melanoblastosis cutis, incontinentia pigmenti, etc. It affects many parts of the body including skin, hair, nails, nervous system, and the teeth.

Chediak-Higashi syndrome: The immune system of the affected person is very poor and he gets recurrent infections. He also has albinism, a pigment disorder.

Conradi syndrome: It is also known with the names of Conradi Hünermann Happle syndrome,  Happle syndrome. The person has face abnormalities like flat face, spinal column abnormalities, etc.

Parry Romberg syndrome: In this condition tissues below the skin are shrunk. It leads to atrophy on one side of the face.

Von Recklinghausen disease: It is also known as neurofibromatosis type 1. In this condition, there are tumors of the skin, nerves, and also pigment changes on the skin.

Hirschsprung disease: In this condition, there is a problem with colon since birth, so it is also called congenital megacolon. The baby has difficulty in passing stools.

Acquired causes of heterochromia:

Hypochromic causes of heterochromia:

• Iris inflammation: Inflammation or swelling of iris due to iritis or uveitis can reduce the pigmentation in the iris leading to heterochromia.These people also have a risk of developing glaucoma.
• Iris atrophy: atrophy or shrinking of the cells of the iris can reduce its pigmentation. The main cause of this condition is trauma or injury.
• Acquired Horners syndrome: We have discussed the symptoms of Horner’s syndrome earlier in this article. Acquired Horners syndrome also has similar symptoms like congenital Horners syndrome, but the causes are different.
• Duane syndrome
• Posner Schlossman syndrome

Hyperchromic causes of heterochromia:

• Iris ectropion: There is a movement of the posterior pigmented epithelium of the iris over the anterior epithelium of iris. It can lead to increased pigmentation at some part of the iris due to the overlap of the pigmented epithelium of iris.
• Siderosis bulbi: If there is any foreign body which is retained in the eye and it becomes oxidized, it leads to this condition.
• Tumors of the iris: Tumours of the eye can make the eye appear to have heterochromia. Most often they make the iris to develop sectoral heterochromia.
• Injury: Injury to the eye can lead to hyperpigmentation in a certain part of the iris making it have heterochromia.
• Rubeosis iridis: In this condition, there is the development of abnormal new blood vessels on the surface of the iris.
• Drug side effects: Some drugs used to treat Glaucoma like prostaglandins, for example, latanoprost, can increase the pigmentation of the iris and lead to central heterochromia as a side effect.

Other causes of acquired heterochromia:

• Diabetes mellitus
• Eye surgeries
• Inflammation or swelling of the eye
• Bleeding in the eye

Central Heterochromia Statistics and rarity:

We have already discussed that heterochromia is a very rare condition. Very few people are affected by this condition throughout the globe. So, an exact number of people having this rare condition is not recorded. But, it is assumed that about 1 % of the people around the world have this condition.

Central heterochromia diagnosis:

Central heterochromia is common amongst all the types of heterochromia. Majority of people with this condition have acquired it genetically. In most of the people, heterochromia is a benign condition with no serious symptoms. It also doesn’t lead to any complications.

But, in some people, central heterochromia might occur as part of a syndrome or disorder present at birth. Also in some people, the genetic color of the eye can change at a later stage of life due to various reasons.

So, a detailed history and physical examination by an ophthalmologist or eye doctor are essential to rule out other causes of central heterochromia. Your doctor might also suggest any blood tests and genetic tests for studying chromosomes if necessary.

Central heterochromia Treatment:

After history, physical examination, and performing various tests if your doctor thinks that some other disorder is causing your central heterochromia, then you might need some form of treatment to treat that condition.

But, if your central heterochromia is not caused due to any reason, and it is present since birth, no specific treatment is necessary. But, if you are concerned about your appearance or looks, then you might opt for colored contact lenses to camouflage the appearance of your eyes.

Celebrities with Heterochromia:

There are many noted personalities who have different types of heterochromia. Some have central heterochromia, some have complete heterochromia, while some other people also have sectoral heterochromia. In some people, the difference in the color of the eyes might not be apparent as they might wear colored contact lenses. In some people, the difference in the color of the iris is more pronounced under the light.

Celebrities with central heterochromia:

• Olivia Wilde (actor)
• Idina Menzel (actor)
• Christopher Walken (actor)

Actors with complete heterochromia:

• Jane Seymour
• Josh Henderson
• Mila Kunis
• Alice Eve

Actors with partial heterochromia:

• Henry Cavill
• Kate Bosworth

You people have seen all the facts about central heterochromia, and also heterochromia like its causes, types, diagnosis, celebrities with heterochromia, etc. Hope you liked the article.

References:

https://www.medicalnewstoday.com