Leukocoria

Specialty: Ophthalmology.

• white pupillary reflex
• white pupil
• absence of red ocular reflex

Why it occurs

• Retinoblastoma (most common intraocular neuroepithelial malignant tumor in childhood)
• Unilateral or bilateral congenital cataract
• Persistence of hyperplastic primary vitreous (hyperplastic primary vitreous syndrome)
• Coats disease (unilateral exudative retinal telangiectasias)
• Advanced retinopathy of prematurity with complete tractional retinal detachment
• Ocular toxocariasis (retinal granuloma due to migration of Toxocara canis larva)

Initial workup

Exploration of the red Brückner reflex using direct ophthalmoscope at short distance; Examination of the fundus under complete pharmacological mydriasis and sedation if necessary, using indirect ophthalmoscopy to examine the periphery; Ocular ultrasound in modes A and B (useful to detect intraocular calcifications pathognomonic of retinoblastoma and evaluate retinal detachments); Magnetic Resonance (MRI) of orbits and brain without and with gadolinium to assess intracranial or metastatic extension.

Standard management

• There is no applicable topical ophthalmic pharmacological management. The treatment of retinoblastoma is performed in highly complex ocular oncology units and includes systemic chemotherapy or intra-arterial chemotherapy directed through the ophthalmic artery using antineoplastic drugs such as Melphalan, Carboplatin and Topotecan.