Leukocoria (White Pupil): Pediatric Ophthalmology Emergency

White Pupillary Reflex: Never "Wait and See"

Leukocoria—white pupillary reflex instead of red—signals obstruction of the visual axis or abnormal retinal reflection. **Always investigate urgently.**

Differential Diagnosis (By Age & Frequency)

Condition	Typical Age	Key Features	Urgency
Retinoblastoma	0–5 yrs (peak 18 mo)	Most common primary intraocular malignancy. 40% hereditary. Leukocoria + strabismus.	EMERGENT (life/eye threat)
Congenital cataract	Birth–infancy	Lens opacity. May be isolated or syndromic. Dense = deprivation amblyopia.	URGENT (weeks)
Coats disease	Males 6–10 yrs	Telangiectatic vessels → exudation → retinal detachment. Unilateral.	URGENT
Persistent Fetal Vasculature (PFV/PHPV)	Birth	Retrolental membrane, microphthalmia, cataract, glaucoma. Unilateral.	URGENT
Retinopathy of Prematurity (ROP)	Preterm (<31 wks / <1500g)	Abnormal retinal vascularization. Screening protocol mandated.	Per screening schedule
Toxocariasis	Children (geophagia)	Granuloma, vitreous inflammation, traction. Dog/cat exposure.	URGENT
Retinal detachment (pediatric)	Variable	Trauma, high myopia, Stickler, Marfan, post-cataract.	EMERGENT
Congenital glaucoma	Birth–infancy	Epiphora, photophobia, buphthalmos, corneal edema → leukocoria late.	EMERGENT
Familial Exudative Vitreoretinopathy (FEVR)	Childhood	Peripheral avascular retina → traction/detachment. AD/AR/X-linked.	URGENT

Adult Causes

Advanced cataract, vitreous hemorrhage, asteroid hyalosis, intraocular lens reflection (pseudophakic), melanoma.

Immediate Steps

Do not delay. Leukocoria in a child = same-day pediatric ophthalmology referral.
Dilated fundus exam ± B-scan ultrasound (if media opacity).
MRI orbits/brain (retinoblastoma staging, optic nerve evaluation).
Genetic counseling (retinoblastoma, FEVR, PFV).

Photographic Leukocoria

White reflex in flash photos (especially asymmetric) is a common detection route. **One white pupil in photos = exam now.** False positives: angle, cataracts, optic disc — but never assume.

Prognosis

Retinoblastoma: >95% survival in high-income countries; globe salvage depends on stage.
Cataract/PFV/Coats: Visual outcome depends on timing of intervention and amblyopia management.

Bottom Line

Leukocoria is a sign, not a diagnosis. It demands immediate specialist evaluation. "Wait and see" is never appropriate.