Childhood Optic Neuritis

Updated: Dec 06, 2018
  • Author: Martha P Schatz, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

Background

Optic neuritis implies an inflammatory process involving the optic nerve.

In children, most cases of optic neuritis are due to an immune-mediated process. These cases of optic neuritis may be associated with a viral or other infection or with immunization. Less commonly, optic neuritis may be the first manifestation of multiple sclerosis (MS) or part of a more diffuse demyelinating disorder, including acute disseminated encephalomyelitis or neuromyelitis optica (Devic disease). [1] Optic neuritis may be related to specific infections, diseases of the adjacent sinuses or orbital structures, and infectious and infiltrative diseases of the brain or meninges that involve the optic nerves. The image below depicts optic disc swelling in a child with bilateral optic neuritis.

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.

The following definitions aid in further understanding optic neuritis:

  • Papillitis - Optic neuritis involving the optic disc with disc edema

  • Retrobulbar optic neuritis - Optic neuritis involving the optic nerve behind the globe. The optic disc appearance should be normal in first-time episodes of retrobulbar optic neuritis.

  • Bilateral simultaneous optic neuritis - Optic neuritis in both eyes occurring within 3 weeks of each other

  • Bilateral sequential optic neuritis - Optic neuritis occurring in both optic nerves but separated by a period of more than 3 weeks

  • Neuroretinitis - Inflammatory process involving the optic discs with exudative changes in the nerve fiber layer of the retina producing a partial or complete macular star. In the past, this condition was called Leber idiopathic stellate neuroretinitis, but now a number of underlying causes for this condition are known. Because this condition is not associated with demyelinating disease and does not imply a future risk of MS, the distinction is important.

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Pathophysiology

Possible mechanisms of inflammation in immune-mediated optic neuritis are the cross-reaction of viral epitopes and host epitopes and the persistence of a virus in central nervous system (CNS) glial cells.

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Epidemiology

Frequency

United States

Optic neuritis is much less common in children than in adults but is not rare and accounts for approximately a quarter of pediatric acute demyelinating syndromes. [2] In one combined series, children comprised 5% of cases.

Mortality/Morbidity

Patients with optic neuritis have a good prognosis, but a minority of patients experience persistent visual loss. Patients with neuromyelitis optica generally have a poorer recovery. When optic neuritis is associated with other CNS diseases, the morbidity and mortality of those disorders contribute substantially to the final outcome.

Race

Optic neuritis is more common in whites than in other races. [3]

Sex

In both children and adults, a female predominance exists. Females comprise 60-75% of patients.

Age

Optic neuritis may occur at any age, including in infants younger than 1 year.

A comparison of adult optic neuritis and childhood optic neuritis is presented in Table 1. These features are generally true but are not absolute, and they do overlap.

Table 1. Comparison of Features of Optic Neuritis in Adults and Children (Open Table in a new window)

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

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Prognosis

The prognosis for visual recovery generally is considered excellent (see Medical Care). [2]

Multiple sclerosis

Adults with isolated optic neuritis have a substantial risk of developing MS. Approximately 15% of patients with normal MRIs at the time of their optic neuritis develop MS in the next 4 years. However, MRI findings are strongly correlated with risk of recurrence of demyelinating events, and 50% of patients whose MRIs demonstrate white matter lesions characteristic of MS at the time of their optic neuritis develop clinically definite MS in the next 4 years.

Children with optic neuritis are less likely than adults to develop MS, but the risk is still present. A large study from the Mayo Clinic with a mean follow-up of 20 years produced a life-table analysis showing 13% of children with optic neuritis had progressed to clinically or laboratory-supported definite MS at 10 years (see Table 3 below). As in adult studies, those patients converting to MS were more likely to do so early; however, the longer the follow-up interval, the more patients there were who developed MS. [4]

A smaller study by Wilejto et al found that 36% of children with optic neuritis developed MS. [5]  All who did had abnormalities on the initial MRI, and bilateral cases were more likely to go on to develop MS.

Table 3. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis [6] (Open Table in a new window)

Age

Risk for Development of MS

10 years

13%

20 years

19%

30 years

22%

40 years

26%

MRI abnormalities in children are associated with the likelihood of developing MS.

Both adults and children with more severe optic disc swelling are less likely to develop MS, and those with both severe optic disc swelling and retinal exudates rarely develop MS.

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Patient Education

With Uhthoff symptom, patients who have had demyelinating lesions with recovery often have symptoms return with exercise, exposure to heat (eg, hot baths), or febrile illnesses; a rapid return to baseline occurs when body temperature returns to normal. Warning patients about Uhthoff symptom is important so that they do not think they are having a recurrence.

For excellent patient education resources, see eMedicineHealth's patient education article, Multiple Sclerosis.

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