What Is Leukocoria?

Leukocoria occurs when the usually black pupil at the center of the eye is white or abnormally colored when direct light is shined on it. Simply put, “leukocoria” means “white pupil.”

Parents or other family members may often detect this abnormal pupil in children. It may be noticed in a darkened room or sometimes spotted in a photograph, where instead of the typical "red reflex," the eye with leukocoria appears white or another color.

This article examines the symptoms, causes, diagnostic factors, and treatments associated with leukocoria. If your child develops this condition, here's what to know from the start.

Symptoms

Normally, light in the eye travels through the pupil and is mostly absorbed by the retina, the light-sensing layer at the back of the eye. But a small amount is reflected back out through the pupil. This is known as a red reflex for the reddish-orange color reflected. This may often be caught by flash photography and referred to as red eye.

In cases of leukocoria, however, the pupil in one or both eyes may look white, yellow, or just plain pale instead.

Sometimes a child’s leukocoria cannot be detected in a photograph, given that new photography software often eliminates eye reflections. And at other times, if a photo is taken at certain angles, the pupil may occasionally appear white even when there is no leukocoria.

However, if you notice something abnormal, you should tell your healthcare provider about it.

Causes

An off-color pupil can occur for a variety of reasons. Here are some of the conditions that can cause leukocoria:

• Retinoblastoma (tumor): This may account for about half of U.S. leukocoria cases and is considered the most common eye malignancy (cancer) in children.
• Cataract (clouding of the lens of the eye): In congenital (present at birth) cases, this lens abnormality can commonly cause a blue-gray pupil.
• Prenatal infections: A white reflex may occur with what has been dubbed TORCH syndrome infections. "TORCH" stands for "toxoplasmosis, other infections, rubella, cytomegalovirus, and herpes."
• Retinal detachment: If the retina comes loose from the back of the eye, it can produce an abnormal red reflex.
• Coats disease: This rare disease causes abnormal blood vessel formation in the retina. It has been known to cause a yellow pupillary reflex.
• Retinopathy of prematurity (ROP): With this condition of improper growth of blood vessels in the retina of premature infants, the pupil may appear white due to fibrous tissue or retinal detachment.
• Strabismus (eyes do not move in tandem): This is something that commonly may be at the root of red reflex testing (a test to see how light is reflected by the pupil) that differs between the two eyes. If it is not promptly treated, it may cause amblyopia (lazy eye, the eyes do not move together), in which the weaker eye never develops clear vision. Also, in about 20% of cases, it can be related to retinoblastoma,
• Anisometropia: This is a condition in which there is a big difference in vision between the eyes. It can sometimes account for asymmetrical red reflex results.

In cases in which leukocoria is detected no matter what is the likely cause, this should be immediately evaluated by an ophthalmologist (a physician specializing in medical and surgical eye care).

Diagnosis

Leukocoria is commonly screened for during well-child visits. To do this, the healthcare provider will use what's known as an ophthalmoscope to view the inside of the eye.

If leukocoria is identified, the healthcare provider will refer the child to an ophthalmologist to determine specifically what may be causing it.

The practitioner will take an in-depth history. Some factors that may provide important clues are:

• How old the child is when the leukocoria occurs: A condition such as retinoblastoma is more likely to occur in a very young child, while Coats disease tends to occur in older kids. Retinoblastoma has genetic risk factors, so a family history of it should be considered.
• How long the leukocoria has persisted: To find this out, try scanning back through old photos and see if you begin to notice a white reflex at a certain point.
• What else is happening with the eyes: Healthcare providers will look for signs pointing to infection, such as redness, pain, or blurred vision.
• If the eye recently had a trauma: An abnormal red reflex can be caused by something such as a vitreous hemorrhage (bleeding in the eye).
• If the child's mother had exposure to cats or dogs during pregnancy: Exposure to cats could cause toxoplasmosis, while exposure to dogs could lead to toxocariasis, both of which are TORCH syndrome infections.
• If the child was born prematurely: This could make them at risk for retinopathy of prematurity.
• If the child has arthritis: Arthritis may cause a dense form of uveitis (inflammation of the uvea in the center of the eye) that may look similar to retinoblastoma.

Treatment

For cases of leukocoria, after the diagnosis is made, it is important to begin treatment promptly. However, there is not one universal treatment here. The approach taken is linked to whatever the underlying cause of the leukocoria appears to be.

For instance, if a cataract seems to be causing the discolored pupil, it needs to be removed. If a tumor is found, it needs to be removed and treated. Likewise, treatment for any underlying infection should be promptly begun.

Summary

Cases of leukocoria involve a white or other abnormally colored pupil instead of a black one. This often is spotted by a family member noticing an unusual white area in one or both eyes of a child, possibly in a photograph.

This should be immediately reported to your healthcare provider, who will need to check for a variety of conditions that are associated with leukocoria. Treatment will hinge on the underlying cause.

A Word From Verywell

If leukocoria is detected in your child's eye, keep in mind that there can be a variety of reasons for this. While all of them should be taken seriously from the start, some cases may easily be remedied and won't necessarily jeopardize your child's vision.

But do promptly bring this to your practitioner's attention so that all possibilities can be checked and treated, if necessary.