What is Leukocoria (white pupillary reflex)?

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From the Guidelines

Leukocoria is defined as an abnormal white reflection from the retina of the eye, which can be related to abnormalities of the lens, vitreous, or retina, and requires immediate medical attention as it can indicate several serious eye disorders, including retinoblastoma, a rare childhood eye cancer 1.

Causes and Diagnosis

The common causes of leukocoria in children include:

  • Retinoblastoma
  • Persistent hyperplastic primary vitreous (PHPV)
  • Retinopathy of prematurity
  • Coats disease
  • Congenital cataract
  • Larval granulomatosis Diagnosis of these conditions is based on the clinical history, fundoscopic examination, and ocular US performed by the ophthalmologist, and may not require additional imaging 1.

Importance of Early Detection

Early detection of leukocoria is crucial, particularly for retinoblastoma, where prompt treatment significantly improves outcomes and can save both vision and life 1.

Imaging Evaluation

In situations where a malignant intraocular or intraorbital mass (retinoblastoma) is suspected, MRI of the orbits with and without IV contrast is useful in the initial evaluation, and is best performed in association with the MRI of the head 1.

From the Research

Definition of Leukokoria

  • Leukokoria is an abnormal pupillary light reflection that usually results from an intraocular abnormality and is seen most often in children 2.
  • It is a significant symptom that can be caused by various conditions, including retinoblastoma, persistent hyperplastic primary vitreous (PHPV), retinopathy of prematurity (ROP), Coats disease, toxocaral endophthalmitis, and retinal astrocytoma.

Causes of Leukokoria

  • Retinoblastoma is the most common cause of leukokoria, accounting for approximately half of all cases in children 2.
  • Other causes of leukokoria include:
    • Persistent hyperplastic primary vitreous (PHPV) (28% of cases) 2.
    • Retinopathy of prematurity (ROP) (5% of cases) 2.
    • Coats disease (16% of leukokoria) 2.
    • Toxocaral endophthalmitis (16% of leukokoria) 2.
    • Retinal astrocytoma (3% of leukokoria) 2.

Association with Retinoblastoma

  • Leukokoria is a common presenting symptom of retinoblastoma, a malignant tumor of immature retinoblasts 2, 3, 4, 5, 6.
  • Retinoblastoma is the most common eye cancer in children, and leukokoria is often the first sign of the disease 3.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Differential diagnosis of leukokoria: radiologic-pathologic correlation.

Radiographics : a review publication of the Radiological Society of North America, Inc, 1994

Research

Chemotherapy for retinoblastoma.

Ophthalmology clinics of North America, 2005

Research

Treatment of intraocular retinoblastoma with vincristine and carboplatin.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2003

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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