Childhood Optic Neuritis Clinical Presentation

Updated: Nov 02, 2021
  • Author: Honey H H Herce, MD; Chief Editor: Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC  more...
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Presentation

History

In general, obtaining an accurate history from children may be difficult. Young children may not notice unilateral visual loss and may not report bilateral visual loss until their behavior indicates visual loss to parents or teachers. A review of systemic symptoms should be aimed at detecting recent vaccinations, infections, or vasculitis.

History of present illness (HPI) findings

Optic neuritis produces a subacute loss of vision, reaching its maximum deficit in a few days to 2-3 weeks. Unilateral optic neuritis may progress to bilateral optic neuritis (< 2 weeks is considered bilateral simultaneous optic neuritis; 2-12 weeks is considered bilateral recurrent optic neuritis). [1] In many cases, recovery is already underway at 2-3 weeks.

Visual symptoms reflect the expected deficit observed in any optic neuropathy, as follows:

  • Loss of visual acuity: Children often experience greater visual acuity deficits. [10]
  • Change in color perception
  • Change in brightness sense
  • Loss of portions of the visual field

Headache is common in children with optic neuritis.

Periorbital pain, especially if it worsens with eye movements, supports a diagnosis of optic neuritis.

Neurologic symptoms may include the following:

  • Prior resolved neurologic symptoms imply a recurrent process, such as MS
  • Ongoing neurologic symptoms may indicate MS, acute disseminated encephalomyelitis, or neuromyelitis optica.
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Physical

Decreased visual acuity: Visual acuity is not the most sensitive indicator of optic nerve injury and may be normal; however, most children with optic neuritis have some loss of visual acuity. Occasionally, patients with optic neuritis have severe loss of vision that includes no light perception. [1]

Dyschromatopsia: A deficit in color vision is a more sensitive indicator of optic nerve injury, and, when checked, a deficit is expected that is out of proportion to any loss of visual acuity. Patients report red color desaturation. [1]

Afferent pupil defect: In unilateral cases of optic neuritis, an afferent pupil defect should be present. In bilateral cases of optic neuritis, this sign is less reliable unless the 2 nerves are asymmetrically affected.

Subjective light brightness difference between the 2 eyes is also common in asymmetric or unilateral cases of optic neuritis.

Visual field defects occur from a few hours to days, reaching maximum loss within several days. [1] . The most common visual field defect is a central or cecocentral scotoma, which occurs in 50% of children who are able to undergo formal visual field testing. [4, 23]

Papillitis: In contrast to adults with optic neuritis, in whom 65% of cases are retrobulbar, optic disc swelling may be seen in up to 73% of children with optic neuritis. [4]

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Causes

See the list below:

  • Immune mediated

    • In children, as many as 85% of cases of optic neuritis are associated with a recent immunization or an infection, usually a viral infection.

    • Optic neuritis can be associated with a preceding nonviral infection, such as pertussis, infectious mononucleosis, toxoplasmosis, or brucella.

    • Common history: Flu-like illness preceding the onset of vision loss by a week

  • Multiple sclerosis 

  • Neuromyelitis optica, or Devic disease (inflammation of the optic nerve and spinal cord)

  • Myelin Oligodendrocyte Glycoprotein (MOG-IgG+) Seropositive Demyelinating Disease

  • Acute disseminated encephalomyelitis: Monophasic illness with multifocal involvement of the CNS, which could include the optic nerve

  • Idiopathic

  • Lyme disease in endemic areas

  • Specific meningeal infections and infiltrations involving the optic nerves, including cryptococcus, tuberculosis, and sarcoidosis

  • Vasculitis, such as systemic lupus erythematosus

  • Syphilis

  • Leukemia

  • Associated with bee and wasp stings

  • Several cases of optic neuritis have been seen in patients on anti-tumor necrosis factor (anti-TNF) drugs.

  • Causes of neuroretinitis

    • Following a viral syndrome

    • Cat scratch disease

    • Toxoplasmosis

    • Toxocariasis and helminths (The finding of a discrete white inflammatory mass overlying the optic disc is suggestive of toxocariasis and helminths.)

    • Lyme disease, usually stage 2

    • Syphilis, especially secondary syphilis as part of a meningitis

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Physical Examination

Visual Acuity

Table 3. Visual Acuity on Examination in Children with Optic Neuritis (Open Table in a new window)

Percentage (%) Visual Acuity
20 20/40 or better at presentation
20 20/50 - 20/190
60 20/200 or worse

Fundus examination

In children, most cases (60%-70%) of optic neuritis involve the optic disc with disc edema (as shown below), as compared to 35% in adults. In addition, most children present with optic nerve pallor.

Optic disc swelling in the right eye and left eye Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.

Most pediatric optic neuritis cases result from anterior optic neuritis, in contrast with retrobulbar optic neuritis, which is common in adults. In retrobulbar optic neuritis, the optic disc should be normal. Atrophy of the disc implies a prior episode of optic neuritis or another more chronic process, such as an optic nerve glioma, a craniopharyngioma, or other compressive process.

If macular edema or a macular star (as shown below) is associated, a diagnosis of neuroretinitis rather than optic neuritis should be made.

Neuroretinitis in the right eye of an adolescent w Neuroretinitis in the right eye of an adolescent with cat scratch disease. The optic nerve is swollen, and a deposition of yellowish exudate in the nerve fiber layer of the macula produces a macular star.

Optic nerve abnormalities include papillitis (acute stage) and optic nerve pallor (chronic stage). [1]

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Complications

The reported rates of visual recovery after optic neuritis in children vary in the literature depending on follow-up time and criteria for good vision. Most authors have found good visual recovery in 70%-85% of children following optic neuritis. [4, 8, 11]  

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