Leukocoria

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Article initiated by:

Sarah Chaudhry

All contributors:

S. Grace Prakalapakorn, MD, MPH, Sarah Chaudhry, Jonathan C. Tsui, MD, Andrew R. Lee, MD

Assigned editor:

Andrew R. Lee, MD

Review:

Assigned status Update Pending

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Definition and Terminology

Leukocoria, meaning “white pupil,” originates from the Greek words “leukos” (white) and “kore” (pupil). It refers to the reflection of white light seen upon direct illumination of the fundus through the pupil, in contrast to the usual red glow.

Leukocoria of the right eye due to retinoblastoma

Etiopathogenesis

Direct interference of the normal red reflex from opacities and abnormalities occurring anywhere from the crystalline lens through to the posterior pole can create the leukocoric reflex. For example the white retinal mass seen in retinoblastoma creates leukocoria.

Differential Diagnosis

• Tumors
  • Retinoblastoma
  • Medulloepithelioma
  • Combined Hamartoma of Retina and Retinal Pigment Epithelium
  • Retinal astrocytic hamartoma (tuberous sclerosis)
• Congenital malformations
  • Persistent Fetal Vasculature
  • Chorioretinal or optic nerve coloboma
  • Retinal fold
  • Myelinated retinal nerve fiber layer
  • Morning Glory Anomaly
  • Norrie disease
  • Incontinentia pigmenti
  • Retinal dysplasia
• Media opacities
  • Cataract
  • Corneal opacity
  • Organizing vitreous hemorrhage
• Retinal detachment
• Vascular diseases
  • Retinopathy of prematurity
  • Coats disease
  • Familial exudative vitreoretinopathy
• Inflammatory diseases
  • Ocular toxocariasis
  • Congenital toxoplasmosis
  • Cytomegalovirus retinitis
  • Herpes simplex retinitis
  • Endophthalmitis
  • Uveitis
• Trauma
  • Commotio retinae
• Refractive
  • High myopia or anisometropia
• Miscellaneous
  • Strabismus (Brückner’s phenomenon)
  • Photographic artifact
  • Pseudoleukocoria due to optic nerve reflex

Diagnosis

History

Comprehensive history should include age of onset, duration, and other ocular symptoms. Past ocular history and past medical history including prematurity, retinopathy of prematurity, trauma, arthritis, prenatal infection, and other systemic diseases should be assessed. Family history of ocular diseases including retinoblastoma, familial exudative vitreoretinopathy, and coloboma should be obtained as well.

Clinical Examination

Red Reflex

Red reflex testing recommendations from the Americn Academy of Pediatrics are as follows:

"All infants should have an examination of the red reflex of the eyes performed during the first 2 months of life by a pediatrician or other primary care clinician trained in this examination technique. This examination should be performed in a darkened room on an infant with his or her eyes open, preferably voluntarily, using a direct ophthalmoscope held close to the examiner’s eye and approximately an arm’s length from the infant’s eyes.... To be considered normal, the red reflex of the 2 eyes should be symmetrical. Dark spots in the red reflex, a blunted red reflex on 1 side, lack of a red reflex, or the presence of a white reflex (retinal reflection) are all indications for referral to an ophthalmologist."

Comprehensive Ophthalmic Examination

In cases of abnormal red reflex, a comprehensive ophthalmic examination is indicated. Anterior segment abnormalities can include microphthalmia, microcornea, persistent pupillary membrane, iris coloboma, cataract, or neovascularization or inflammation. Fundus abnormalities can include vitreous opacities, optic nerve anomalies, retinal vascular abnormalities (in ROP, Coats disease, retinoblastoma, and FEVR), and retinal masses, exudation, detachment, stalk, or fibrosis.

Ancillary Testing

Ancillary testing should be performed as indicated by other findings on the clinical examination. Ultrasonography can help detect calcification in retinoblastoma and can also diagnose findings in other diseases in the above differential diagnosis including Coats disease, PFV, and retinal detachment. Ultrasonography is particularly valuable in cases with media opacities which may prevent direct visualization of the posterior segment.

Other ancillary testing may include fluorescein angiography, optical coherence tomography, or neuroimaging such as computerized tomography or magnetic resonance imaging.