What is Aniridia?
Aniridia is a rare eye condition that can occur with trauma or it can be present at birth. It describes a condition in which someone is missing parts or all of their iris. What is the iris? The iris is better known as the colored part of your eye. It controls the size of your pupil (a hole in the center of your eye that allows light to pass through). The iris expands or contracts this hole, determining the size of it. In dark conditions or at night, the iris expands to open up the pupil and allow more light in. During the daytime or in sunlight, the iris contracts the pupil to decrease the amount of light in. This plays a huge role in vision because it allows for light regulation. This directly impacts our vision and allows us to maintain clear and comfortable sight. Without the iris, one would experience extreme light sensitivity and blurry vision. The extent of the condition’s effect on the eye depends on the severity. Aniridia is typically associated with other eye conditions such as glaucoma or cataracts. It nearly always involves other underdeveloped parts of the eye as well, further limiting vision. Luckily, there are a number of treatment options available that can decrease unwanted symptoms and increase your quality of life.
Your eye doctor can determine if you have aniridia during a comprehensive eye exam. This can be done by clinical assessment using different lenses in addition to thorough case history. Diagnosis typically occurs early on in life if congenital and later on if it is trauma related. Genetic testing can be done to find out what type of aniridia one has. This can help in providing information on the type of medical conditions that may be associated. Some common symptoms of aniridia include:
- Extreme light sensitivity
- Nystagmus (involuntary eye movements)
- Strabismus (misalignment of eyes)
- Blurry vision
Cosmetic contact lenses
Cosmetic contact lenses are created with iris pigment on the front surface and a black backing on the back surface. The black back surface is used to keep light out, effectively helping out patients that experience glare and light sensitivity due to their aniridia. The lenses can have varying pupil diameters. Customization is possible to allow for the best option for each individual eye. All lenses are replaced on a year-to-year basis and require a cleaning regime just like that of a regular soft contact lens. Candidates for cosmetic contact lenses include those that have been disfigured from injury or disease, ocular albinism, and aniridia.
The benefits of cosmetic contact lenses include:
- Restoration of “normal” eye appearance
- Reduction of glare
- Reduction of light sensitivity
Types of prosthetic contact lenses
- Computer generated and printed lenses
- This option involves predetermined colors and parameters. All colors are generated by a computer and then printed. Limitations of colors and available diameters are some negative aspects of this type of lens. However, these are much easier to be replaced and are less likely to lose its color over time.
- Hand painted lenses
- Hand painted lenses allow for customized colors and diameters. This option provides the most realistic and natural look possible. This is done by hand by an artist instead of a computer. It is done by matching the painting to a photograph of your eye.
Treatments for Aniridia
Specialty contact lenses can be used to treat aniridia, allowing patients to see better and look better. This can be done by producing a more normal looking eye. Cosmetic contact lenses can be used to control the amount of light that goes into the eye, acting as an artificial iris. Cosmetic contact lenses are soft contact lenses that can be hand painted to achieve the look of a real iris. Cosmetic contact lenses can help reduce the extreme glare that patients with aniridia experience as well as sharpen vision and improve general eye appearance.
Aniridia can affect the eyes in many ways and so there are a number of treatments available:
- Specialty colored cosmetic contact lenses to improve appearance, vision, and symptoms of light sensitivity.
- Sunglasses to minimize light sensitivity
- Surgical placement of an artificial iris
- Artificial tears to maintain corneal health
- Removal of cataracts through surgery to improve vision
- Close monitoring for conditions such as glaucoma
- Low vision examination and vision rehabilitation services. A comprehensive exam with a low vision doctor can help by going over your options of assistance devices.
Your eye doctor can go over treatment options available to you and determine what your best course of action is.
Complications of Aniridia
Patients with aniridia must be followed with regular eye exams to ensure a healthy and long-lasting vision. Some complications associated with congenital aniridia include cataracts or glaucoma. Cataracts are the clouding of the lenses in the eye which can impair vision. Glaucoma involves an increase in pressure in the eye which can then cause damage to its structures. These conditions would both require their own treatment, such as surgery or eye drops. Problems with the optic nerve (nerve that sends visual information to brain) or retinal (back of eye) detachments are more likely in this condition as well.
FREQUENTLY ASKED QUESTIONS ABOUT ANIRIDIA
Is Aniridia associated with systemic health problems?
It is possible for aniridia to be associated with a syndrome or by itself. Miller syndrome describes aniridia associated with a kidney tumor. This tumor is called a Wilms tumor or nephroblastoma. WAGR syndrome stands for Wilms tumor, aniridia, GI problems, and mental retardation. These 4 conditions can be associated together. Speak to your doctor about screening for Wilms tumor and monitoring your kidney.
How often should I have an eye exam if I have aniridia?
A diagnosis of aniridia requires regular eye examinations throughout the entirety of one’s life. The frequency that the follow ups are scheduled depends on the severity of the condition.
Can I pass aniridia on to my child?
Studies have shown that there may be a genetic link to aniridia. Defects on a particular gene can cause someone to be born with this condition. If you are a carrier for this gene, your child will have about a 50% chance of having aniridia as well. However, a child can also be born with the condition even if there is no previous family history of it. Most children will be able to live a normal life and go to school naturally. However, your child may need extra support in the classroom and treatments like cosmetic lenses or magnifiers to make them more comfortable. Early intervention makes a huge difference in their quality of life.
Is there a cure for aniridia?
There is unfortunately no cure for aniridia. However, your eye doctor can provide you with options to stabilize your vision and provide you with better comfort.
Can glasses or contacts improve my vision to 20/20?
The use of contacts in aniridia is primarily for the reduction of light sensitivity and glare. It also improves the cosmetic appearance of the eyes, which helps patients psychologically. Contacts and glasses cannot fully restore the vision lost in cases of aniridia due to the complexity of the condition. Patients with aniridia may have other eye problems that reduce their vision as well.
Will I be able to drive with aniridia?
Those with aniridia always have vision problems but the degree of vision loss between each patient varies. Some patients may be legally blind while others may retain good enough vision to drive a car. Schedule your comprehensive exam and speak to your eye doctor about your ability to drive. They will be able to determine if you are eligible to get behind the wheel.
How will my child’s life change with aniridia?
Due to your child’s extreme light sensitivity, your child should utilize the most of evening outdoor play. Tinted sunglasses and hats will help with outdoor activities as well. The use of non-glare surfaces and thick curtains will assist in vision indoors. Using hand held magnifiers, telescopes, and other optical devices prescribed by a low vision optometrist can provide some relief with vision. Your child will be able to attend school normally with a little extra help from those around him/her. Aniridia will affect each individual differently depending on the severity of disease and additional complications that arise. Your eye doctor can give you a better understanding of your child’s condition during their comprehensive eye exam.