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Childhood Optic Neuritis Clinical Presentation

  • Author: Martha P Schatz, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Nov 30, 2015
 

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.

Optic neuritis produces a subacute loss of vision, reaching its maximum deficit in a few days to 2-3 weeks. In many cases, recovery is already underway at 2-3 weeks.

Headache is common in children with optic neuritis. Periorbital pain, especially if it worsens with eye movements, supports a diagnosis of optic neuritis.

Visual symptoms reflect the expected deficit observed in any optic neuropathy, including loss of visual acuity, change in color perception, change in brightness sense, and loss of portions of the visual field.

In reviewing neurologic symptoms, prior resolved neurologic symptoms imply a recurrent process, such as MS, whereas ongoing neurologic symptoms may indicate MS, acute disseminated encephalomyelitis, or neuromyelitis optica.

A review of systemic symptoms should be aimed at detecting recent vaccinations, infections, or vasculitis.

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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.

Decreased color acuity: 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.

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.

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.

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.

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.
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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.
  • Multiple sclerosis
  • Neuromyelitis optica (Devic disease)
  • 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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Contributor Information and Disclosures
Author

Martha P Schatz, MD Chief, Pediatric Ophthalmology Service, Clinical Professor of Ophthalmology, University of Texas Health Science Center at San Antonio

Martha P Schatz, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, North American Neuro-Ophthalmology Society, Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Ophthalmological Society

Disclosure: Nothing to disclose.

John E Carter, MD Professor, Departments of Neurology and Ophthalmology, University of Texas Health Science Center at San Antonio

John E Carter, MD is a member of the following medical societies: American Academy of Neurology, North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Acknowledgements

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

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Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.
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.
T1 contrast enhanced axial section of an MRI of the orbital optic nerve of a child with optic neuritis on the left side. The arrows point to the left optic nerve that enhances along its entire orbital course.
T1 contrast enhanced coronal section of the MRI of the orbital optic nerve. The arrow points to the enhancing left optic nerve.
T1 contrast enhanced axial section of an MRI of the intracranial optic nerves. Enhancement of both optic nerves is seen. The arrow indicates the left optic nerve.
T1 contrast enhanced coronal section of the MRI showing the optic nerves that both enhance. The arrow points to the left optic nerve.
T2 axial section of an MRI through the cerebral hemisphere of a boy with bilateral optic neuritis. Note high-signal abnormalities in the cerebral white matter that are most prominent in the posterior hemispheres. This is suspicious for mild acute disseminated leukoencephalitis.
T2 axial sections of MRI of a man with optic neuritis showing scattered white matter lesions. The arrows point to 2 examples of the numerous lesions.
Table 1. Comparison of Features of Optic Neuritis in Adults and Children
Adult Optic Neuritis Pediatric Optic Neuritis
Unilateral Bilateral
Retrobulbar optic neuritis Papillitis
Commonly associated with pain on eye movements Commonly associated with headache
Most often idiopathic Most often postinfectious or postimmunization
High probability of recurrent inflammatory demyelinating events in the CNS and a diagnosis of MS Low probability of recurrent demyelinating events and a diagnosis of MS
Table 2. Equivalent Doses of Commonly Used Corticosteroid Medications
Corticosteroid Drug Approximate Equivalent Dose
Prednisone 5 mg
Prednisolone 5 mg
Methylprednisolone 4 mg
Dexamethasone 0.75 mg
Table 3. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis [7]
Age Risk for Development of MS
10 years 13%
20 years 19%
30 years 22%
40 years 26%
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