This condition causes the pupil of the eye to look white instead of black.

The the pupil of the human eye is normally black. In flash photographs the pupil may appear red, an effect called "red eye." This is also called the "red reflex" by health care providers, and is entirely normal.

On occasion, the pupil of the eye may appear white. This is never a normal condition and requires immediate evaluation by trained specialists (ophthalmologists, "eye doctors," not optometrists who generally prescribe lenses or glasses).

There are many different causes for white pupil, which are described below. Other conditions can also mimic white pupil. A cloudy cornea (usually, the clear part of the eye) may produce an appearance similar to a white pupil. The causes of a cloudy or white cornea are different from those of a white pupil, but are also significant and require immediate attention.

Cataracts may also cause the pupil to appear white.

• Retinoblastoma
• Congenital cataract - may be hereditary or may result from other conditions, including congenital rubella, galactosemia, retrolental fibroplasia (retinopathy of prematurity)
• Intraocular infections - infections within the eyes)
• Persistent primary hyperplastic vitreous
• Coats' disease - exudative retinopathy

If a white pupil is noted, an immediate appointment with your health care provider should be made. Pediatricians routinely screen for the appearance of a white pupil in children. If a child develops a white pupil or cloudy cornea, immediate attention is required, preferably from an ophthalmologist.

Contact your health care provider if you notice any color changes in the pupil or cornea of the eye.

The health care provider will perform a physical exam and ask questions, such as:

• When did you first notice this?
• Are both eyes affected?
• Is there any change in the vision, including a decrease in vision, blurred vision, or others?
• What other symptoms are also present?
• Is there a family history of disease such as congenital cataracts or retinoblastoma?
• Are the eyes crossing?
• Any recent eye surgery?

The physical examination will include a detailed eye examination.

The following tests may be performed:

• Slit-lamp exam
• Standard eye exam
• Visual acuity
• Ophthalmoscopy
• Other tests depending on the suspected cause, including a head CT or MRI