Aniridia means lack of an iris. It is a rare disorder in which the iris of the eye is partly or completely missing. The iris is the colored part of the eye. It controls the size of the pupil and the amount of light that enters the eye.
Aniridia often affects both eyes. It causes the pupil to be abnormally large. The pupil may also be oddly shaped. Aniridia can also cause problems with the cornea, lens, retina, and optic nerve. Some of these problems may be present at birth. Others may happen later in life. Problems with other parts of the eye may cause problems that are more severe than the missing iris.
Types of treatment
Your child will need regular eye exams by a pediatric ophthalmologist. This is a health care provider who specializes in treating eye problems in children. They will look for changes in your child’s vision and signs of problems.
Your child may need special glasses. The glasses protect your child’s eyes from sunlight and injury. Glasses can also help correct vision loss. Photochromatic (lenses that darken when exposed to light) or tinted glasses may be helpful. As your child gets older, they may be able to wear special soft contact lenses. The painted or tinted contact lenses help to reduce glare and improve the look of the eyes. They may also improve vision.
Your child may also need medicines, surgery, or other procedures for glaucoma or cataracts.
The FDA has approved an artificial iris implant. Some children may have surgery to replace the iris with an artificial one. It may improve the disabling glare, light sensitivity during day or night, the cosmetic appearance of the eye, and vision-related quality of life.
Artificial tear drops and ointments can help with the corneal problems seen in aniridia.
Possible complications of aniridia
It is common for children with aniridia to develop other serious problems with their eyes. They may occur soon after birth or later in life. Your child may have:
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Corneal scarring. This is damage to the tissue covering the front of the eye (cornea). It causes glare, which makes it even more difficult to see.
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Glaucoma. This is a buildup of pressure in the eye. It causes more vision problems. It usually occurs around the teen years.
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Cataracts. This is clouding of the lens. The lens may also shift out of position. These problems can make vision worse.
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Problems with the retina and optic nerve. The retina is the light-sensitive area in the back of the eye. It sends messages to the optic nerve. The optic nerve sends messages to the brain. Problems with the retina or optic nerve can reduce vision.
Living with aniridia
Make sure your child sees the eye care provider regularly. This provider will tell you how often your child needs to be checked.
It is recommended that all children with sporadic congenital aniridia be screened regularly with abdominal ultrasound screening for Wilms tumor (tumor of the kidneys) as follows:
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Every 3 months up to 5 years of age
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Every 6 months from age 5 years to 10 years
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Every 12 months from age 10 years to 16 years
Most children with aniridia can attend a regular school. But your child may need extra help in the classroom. Include the school staff so your child can get as much physical help and emotional support as possible.
You may find a support group helpful, such as:
When to contact your doctor
Contact your child’s healthcare provider right away if your child has sudden vision loss or eye pain, or any new vision symptoms.
