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Clinical Summary

Leukocoria is an abnormal white pupillary reflex and one of the primary signs of retinoblastoma in a child, usually less than 3 years old. The “white pupil” is often noticed in photographs using a flash. Strabismus may also be a presenting sign. Metastatic disease usually occurs within 12 months of presentation, so prompt treatment is important. Survival with timely diagnosis and treatment is greater than 95%. A more common cause of leukocoria are cataracts, which form as a slow clouding of the lens due to various etiologies, leading to decrease in vision. They account for much of blindness worldwide, as well as one-third of visual impairment. Symptoms can vary and include blurry vision, faded colors, double vision, halos around lights, and difficulty with bright lights or seeing at night. The poor vision leads to increased risk of falling and depression. Etiologies of cataracts include aging (most common), trauma, radiation exposure, neonatal, genetics, iatrogenic, or following eye surgery. Most cataracts are diagnosed based on comprehensive eye exam, where slit lamp plays a key role.

Management and Disposition

Urgent referral to an ophthalmologist is recommended for a child with leukocoria to rule out retinoblastoma and to assess for other causes. Incidental diagnosis of cataract requires no action in the ED. Decreasing exposure to ultraviolet B and quitting smoking are key for prevention or limiting worsening of cataracts, as well as possible oral supplementations. Once a cataract affects quality of life as a result of poor vision, outpatient surgery is the mainstay of treatment.


Leukocoria. Leukocoria (white pupil) is seen in this child with retinoblastoma. (Photo contributor: Lawrence B. Stack, MD.)


Cataract. Bilateral congenital cataracts are seen in this child. (Photo contributor: Lawrence B. Stack, MD.)


  1. A white pupillary reflex in a child is suggestive of retinoblastoma, whereas a blue-gray pupil is seen in congenital cataract.

  2. Once a cataract is discovered, attempt to identify the etiology to slow progression and implement preventative interventions if possible.

  3. In addition to performing a comprehensive examination in your ED (eg, visual acuity, slit lamp, pressures), assess how the cataract impacts quality of life, including risk of fall and depression. Available social support for the patient is important to consider.


Cataract. A white corneal opacity consistent with a cataract is demonstrated. (Photo contributor: Kevin J. Knoop, MD, MS.)

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