Leukocoria literally means "white pupil." It occurs when the pupil (the round hole in the colored part of the eye) is white rather than the usual black [See figure 1].
In more obvious cases the pupil may appear white on casual observation. In other situations the pupil may appear white only in certain circumstances such as when the pupil becomes larger in a darkened room. Sometimes leukocoria is detected from photographs when one pupil has an abnormal or "white reflex" compared to the other eye having a normal "red reflex."
When light enters the eye through the pupil, the retina absorbs most of the light. A small amount of light, however, is reflected by the retina back out of the eye through the pupil. The light is reddish-orange, reflecting the color of normal retina. The red reflex is most easily seen when the observer's line of sight is very close to the direction of illumination into the eye. An example is a camera in which the flash is mounted very close to the lens resulting in photographs with red pupillary reflexes.
The red reflex is either absent or white with leukocoria. This occurs as a result of abnormal reflection of light coming out of the eye.
Ophthalmologists utilize a retinoscope to examine the red reflex from the eye and an ophthalmoscope to directly visualize the interior of the eye. Dilating eye drops are generally used to enlarge the pupil which enables a more thorough examination.
Many conditions cause leukocoria including cataract, retinal detachment, retinopathy of prematurity, retinal malformation, intraocular infection endophthalmitis), retinal vascular abnormality, and intraocular tumor (retinoblastoma).
All diseases which cause leukocoria represent a serious threat to vision and some pose a threat to life. Prompt evaluation of leukocoria by an ophthalmologist is always appropriate.
Management of leukocoria involves treatment of the underlying condition (cataract, retinal detachment, infection, etc) responsible for the white appearing pupil.
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