Leukocoria: the white pupil
- Clinical approach
A white pupil in a child, in the exam or in a flash photo, can be a retinal cancer. It is always referred urgently to ophthalmology.
What it is
Leukocoria is a white, yellow or pink reflex from the pupil instead of the normal red reflex. In children the first diagnosis to exclude is retinoblastoma, the commonest primary eye cancer of childhood, which is curable when found early. Other causes are congenital cataract, retinopathy of prematurity, Coats disease, persistent fetal vasculature, ocular toxocariasis and retinal detachment.
Red flags
- Any leukocoria in a child, including one seen only by the parents in a photograph.
- A new squint (strabismus) in a young child: retinoblastoma can present this way.
- Family history of retinoblastoma: children of survivors or with an affected sibling need screening from birth.
- A red, painful eye or a large eye with leukocoria: advanced disease or raised pressure.
- Leukocoria in an infant with a dense cataract: risk of permanent amblyopia within weeks.
Step-by-step approach
- Ask when and how the white reflex was seen, and look at the family's photographs.
- Ask about prematurity, family history of eye cancer or cataract, and contact with dogs or soil.
- Do the red reflex test in a dim room with the ophthalmoscope held at arm's length, both eyes at once.
- Check for strabismus, the size of each eye and the fixation of each eye.
- Refer urgently; do not delay to arrange imaging.
Causes of leukocoria in children
| Cause | Typical child | Clue |
|---|---|---|
| Retinoblastoma | Under 3 years, one or both eyes | White mass with calcification on ultrasound |
| Congenital cataract | From birth | Lens opacity; may be inherited or from infection in pregnancy |
| Retinopathy of prematurity | Born very preterm | Retinal detachment in advanced stages |
| Coats disease | Boys, usually one eye | Yellow exudate under the retina |
| Persistent fetal vasculature | From birth, one small eye | Fibrovascular stalk behind the lens |
| Ocular toxocariasis | Older child, contact with puppies | Retinal granuloma |
Keys to the differential
- Retinoblastoma is caused by loss of both copies of the RB1 gene; the hereditary form is bilateral and earlier, and carries a risk of later sarcomas.
- A normal red reflex test at birth and at each well-child visit is the screening test for both retinoblastoma and congenital cataract.
- Strabismus in a child always needs a dilated fundus exam to exclude a tumour or cataract.
Next step / Initial management
- Any leukocoria: urgent referral to paediatric ophthalmology, seen within days.
- Suspected retinoblastoma: examination under anaesthesia, ocular ultrasound and MRI of the orbits and brain; avoid CT where possible because of radiation risk in hereditary disease; never biopsy the eye.
- Dense congenital cataract: surgery within the first weeks of life, then optical correction and amblyopia treatment.
- Retinoblastoma family history: genetic counselling and screening exams from birth.
Test yourself
Think of the answer, then tap the question to see it.
What is the first diagnosis to exclude in a child with leukocoria?
Retinoblastoma, the commonest primary intraocular cancer of childhood.
Why is a biopsy avoided when retinoblastoma is suspected?
Because opening the eye can seed the tumour outside it; the diagnosis is made by examination under anaesthesia and imaging.
Why must a dense congenital cataract be operated on in the first weeks of life?
Because the blurred image during the critical period causes deprivation amblyopia that becomes permanent.
Elsewhere in DurgiPedia
Connections
Educational content; not a clinical guideline and no substitute for assessment by a health professional. For sudden visual loss, severe eye pain, a chemical burn or an eye injury, go to an emergency department at once.
Sources: Leukocoria (StatPearls Publishing (NCBI Bookshelf), 2023); Retinoblastoma (StatPearls Publishing (NCBI Bookshelf), 2025); Exudative Retinitis (Coats Disease) (StatPearls Publishing (NCBI Bookshelf), 2023).
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