leukocoria

What is Leukocoria?

Leukocoria, also known as "white pupil," is an abnormal pupillary reflex that can be a sign of various serious intraocular disorders. It is characterized by a white or silvery reflection in the pupil, which is usually more prominent after pupillary dilation.

Causes and Conditions Associated with Leukocoria

Leukocoria can be caused by several conditions, including:

• Congenital cataracts
• Coats disease
• Retinoblastoma (a type of eye cancer)
• Retinopathy of prematurity
• Toxocariasis (an infection caused by a parasitic worm)
• Norrie disease
• Retrolental fibroplasia

Detection and Diagnosis

Leukocoria can be detected through various means, including:

• Direct illumination of the fundus through the pupil
• Flash photography, where one pupil may have an abnormal white reflex compared to the other eye having a normal red reflex
• Routine pediatrician exams using an ophthalmoscope

Symptoms and Signs

The symptoms and signs of leukocoria can include:

• A white or silvery reflection in the pupil
• A difference in color between the two pupils when compared to each other
• A whitening of the pupil seen as a difference in color when compared to the normal pupil

Important Notes

It's essential to note that a single photo with a white reflection in the pupil is not a cause for fear or panic. However, if you notice any unusual symptoms or signs, it's crucial to consult an eye care professional for proper evaluation and diagnosis.

References:

• [1] Leukocoria means ‘white pupil.’ Leukocoria is an abnormal pupillary reflex usually more prominent after pupillary dilation. It is often the first sign of a range of serious intraocular disorders.
• [3] Leukocoria, meaning “white pupil,” originates from the Greek words “leukos” (white) and “kore” (pupil).
• [5] Leukocoria (also white pupillary reflex) is an abnormal white reflection from the retina of the eye.
• [10] Leukocoria is when your eye’s pupil reflects white, silvery, gray or yellowish instead of red.

Leukocoria Signs and Symptoms

Leukocoria, also known

Leukocoria, also known as a white pupillary reflex, can be diagnosed through various tests to determine its underlying cause.

• Ultrasonography: This is a non-invasive and highly specific test that uses high-frequency waves to create detailed images of the eye. It is recommended for all patients with leukocoria [1].
• Ophthalmoscopic examination: A healthcare provider will use an ophthalmoscope to examine the eyes, including the red reflex, to check for any abnormalities [3].
• Comprehensive Ophthalmic Examination: This includes a thorough examination of the eye, including the retina, to rule out other conditions that may be causing leukocoria [2.2.2].
• Ancillary Testing: Additional tests such as B-scan ultrasonography, Optical Coherence Tomography (OCT), and MRI of the head and orbits may be performed to aid in differential diagnosis [4].
• Blood Work: While not diagnostic on its own, blood work can be useful in diagnosing conditions that may cause leukocoria, such as Toxocara exposure or TORCH infections [9].

It's essential to note that early diagnosis is vital in retinoblastoma management, and any child with leukocoria should undergo a dilated retinal examination, even if the leukocoria is detected from photographs using a flash [6].

Treatment Options for Leukocoria

Leukocoria, also known as a white pupillary reflex, can be caused by various underlying ocular diseases or conditions. The treatment approach depends on the specific cause and severity of the condition.

• Common Ocular Conditions: For common ocular conditions causing leukocoria, such as uveitis, the management involves:
  • Methotrexate: This is the most commonly used first-line immunomodulatory drug in children with uveitis.
  • Second-line agents: Azathioprine, cyclosporine, and mycophenolate are used as second-line treatments.
  • Anti-TNF (tumor necrosis factor) therapy: May be used to treat certain types of uveitis. [1][4]
• Retinoblastoma: For retinoblastoma, a type of eye cancer that can cause leukocoria:
  • Enucleation: Surgical removal of the affected eye may be necessary.
  • Chemotherapy: May be used to treat small cancers or bilateral disease.
  • Focal destructive therapies: Retinal laser photocoagulation or cryotherapy may be used to destroy tumor cells.
  • Radiation therapy: May be used to treat advanced or larger cancers. [5][8]
• Toxocariasis: For toxocariasis, a parasitic infection that can cause leukocoria:
  • Topical or systemic corticosteroids: Are the mainstay of treatment in controlling inflammation.
  • Anti-helminthic therapy: May be used to treat the underlying parasitic infection. [4]
• Other Treatment Modalities: Other treatment options for leukocoria include:
  • Photocoagulation
  • Cryotherapy
  • Radiation therapy

References

[1] Context 1: Methotrexate is the most commonly used first-line immunomodulatory drug in children with uveitis. [4] Context 4: Topical or systemic corticosteroids are the mainstay of treatment in controlling inflammation for toxocariasis. [5] Context 6: Leukocoria describes the clinical finding of a white pupillary reflex, and there are many causes of leukocoria in children. [8] Context 8: Treatment of small cancers and bilateral disease may include photocoagulation, cryotherapy, and radiation therapy for retinoblastoma.

Leukocoria Differential Diagnosis

Leukocoria, also known as a white pupillary reflex, can be caused by various ocular and systemic conditions. The differential diagnosis for leukocoria includes:

• Congenital Cataract: This is the most common cause of leukocoria in children, accounting for approximately 60% of cases [13].
• Retinoblastoma: A malignant retinal tumor that can cause leukocoria in infants and young children, responsible for half of the cases in this age group [4, 12].
• Retinal Detachment: This condition can also cause leukocoria, particularly if it is associated with a retinal tear or hole [9, 13].
• Persistent Fetal Vasculature (PFV): A rare congenital anomaly that can cause leukocoria and other ocular symptoms [8, 13].
• Coats Disease: A rare eye disorder that can cause leukocoria and vision loss in children [1, 8].

Other conditions that may be considered in the differential diagnosis of leukocoria include:

• Anterior Chamber or Lens Abnormalities
• Cataract
• Neoplasia (cancer)
• Persistent Hyperplastic Primary Vitreous (PHPV)
• Vitreous Abscess
• Posterior Uveitis
• Retinal Detachment

It's essential to note that leukocoria can be a sign of an underlying serious condition, such as retinoblastoma. Therefore, if you or your child experience any unusual symptoms, it is crucial to seek medical attention promptly.

References:

[1] Kaliki S, Shields CL. Differential diagnosis of retinoblastoma. In: Ramasubramanian A, Shields CL, ... (eds). Ophthalmology and Visual Science. 2019; [cited 2024 Dec 6].

[4] by A Balmer · 2007 · Cited by 129 — Leukocoria in infants is always a danger signal as retinoblastoma, a malignant retinal tumor, is responsible for half of the cases in this age group.

[8] Differential Diagnosis. In children, the differential diagnosis for leukocoria includes a wide spectrum of both ocular and systemic conditions.

[9] Leukocoria can be caused by congenital or acquired eye diseases. This is an ophthalmologic emergency particularly because of the need to promptly diagnose and treat conditions such as retinoblastoma, glaucoma, retinal detachment and infections.

[12] Leukocoria in infants is always a danger signal as retinoblastoma, a malignant retinal tumor, is responsible for half of the cases in this age group. ... may also manifest the same warning signs and require specialized differential diagnosis.

[13] Differential diagnosis. In children, the differential diagnosis for leukocoria includes a wide spectrum of both ocular and systemic conditions. The main conditions that cause leukocoria are congenital cataract (60%), retinoblastoma (18%), retinal detachment (4.2%), persistent fetal vasculature (4.2%), and Coats disease (4.2%).