Optic neuritis (ON) is an inflammatory process involving the optic nerve. Optic neuritis may be associated with other neuro-inflammatory syndromes that cause systemic effects.
In children, most cases of optic neuritis are due to an immune-mediated process. Children younger than 10 years usually present with bilateral optic neuritis, while older children usually present with unilateral optic neuritis.[1] These cases of optic neuritis may be associated with a viral or other infection or with immunization. Less commonly, pediatric 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).[2] 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.
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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.
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. Patients with optic neuritis due to neuromyelitis optica spectrum disorder lack the aquaporin-4 antibody.[1]
The incidence of pediatric optic neuritis in Canadian children is estimated to be 0.2 per 100,000 (95% CI, 0.16-0.3).[3] 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.[4] In one combined series, children comprised 5% of cases. In addition, children may also present with optic neuritis in combination with other inflammatory conditions such as acute disseminated encephalomyelitis or neuromyelitis optica.[1]
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.[5]
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
The prognosis for visual recovery generally is considered good to excellent, regardless of medical treatment, except in cases of neuromyelitis optica (see Medical Care).[4, 7]
Approximately one third of children with optic neuritis present with recurrent symptoms.[1]
Multiple sclerosis
Older children and those with brain MRI abnormalities outside the visual system (viewed using T2-weighted and FLAIR sequences) at presentation are at greater risk for MS.[6] The increased risk of MS in older children may result from a difference in their immune system, hormonal change, or puberty; further research is required. In addition, the risk of evolving multiple sclerosis is probably less in children than in adults, but pediatric optic neuritis is more likely to be an initial manifestation of acute disseminated encephalomyelitis.[7]
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.[6] Fifty percent 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.
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 2 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.[6]
A smaller study by Wilejto et al found that 36% of children with optic neuritis developed MS.[8] All who did had abnormalities on the initial MRI, and bilateral cases were more likely to go on to develop MS.
Table 2. Life-Table Analysis of the Risk for Development of MS in Children With an Isolated Attack of Optic Neuritis[9]
View Table
See Table
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.
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.
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.
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]
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]
Table 3. Visual Acuity on Examination in Children with Optic Neuritis
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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.
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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.
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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 ne....
Optic nerve abnormalities include papillitis (acute stage) and optic nerve pallor (chronic stage).[1]
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]
Visual acuity, color vision, afferent pupillary defect (if visual loss is asymmetrical), and confrontation fields should be documented. Perimetry and optical coherence tomography (OCT) of the optic nerve should be performed whenever feasible. Visual evoked potentials may be helpful.
Exclude infectious, genetic, or neoplastic processes.
Systemic laboratory studies can be performed directed by features in the history and physical examination consistent with other non–immune-mediated causes of optic neuritis.
Neuromyelitis optica (NMO) antibody in serum (anti aquaporin-4 immunoglobulin) helps establish a diagnosis of neuromyelitis optica, although a spectrum of diseases may have this antibody. Testing for myelin oligodendrocyte glycoprotein may be helpful, as the disease course in patients with this spectrum of NMO may be relatively benign.
Lumbar puncture with measurement of opening pressure excludes papilledema secondary to intracranial hypertension.
Cerebrospinal fluid (CSF) studies may indicate the presence of a simultaneous meningitis or encephalitis, but a mild lymphocytic pleocytosis may be present with optic neuritis.
MRI of the brain and orbits with contrast should be performed. The MRI should exclude extrinsic compressive lesions.
Enhancement of the optic nerve in the orbit or the intracranial segment of the optic nerve or of the chiasm is helpful in confirming the diagnosis. Some enlargement of the optic nerve is present in optic neuritis, and a diagnosis of optic nerve glioma should not be made unless the clinical course dictates reconsideration of the diagnosis of optic neuritis.
Meningeal enhancement suggests some form of infectious or noninfectious meningitis and may merit additional workup and different therapy.
Changes in the CNS white matter may confirm other neurologic involvement found on physical examination, may affect the prognosis of MS in the future, or may indicate the presence of acute disseminated encephalomyelitis. One third of children with optic neuritis will have asymptomatic white matter lesions of the brain as compared to one half of adults with optic neuritis.
The images below depict characteristics relevant to a diagnosis of optic neuritis.
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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 ....
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T1 contrast enhanced coronal section of the MRI of the orbital optic nerve. The arrow points to the enhancing left optic nerve.
View Image
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 ....
View Image
T1 contrast enhanced coronal section of the MRI showing the optic nerves that both enhance. The arrow points to the left optic nerve.
View Image
T2 axial section of an MRI through the cerebral hemisphere of a boy with bilateral optic neuritis. Note high-signal abnormalities in the cerebral whit....
To either diagnose or exclude neuromyelitis optica (Devic disease), an MRI of the spinal cord with contrast is necessary if symptoms and signs consistent with a spinal cord process are present.
Optical coherence tomography (OCT) is often used to assess neuronal injury, including retinal nerve fiber layer (RNFL) thickness. Pediatric patients with MS have been reported to have a 10%-20% thinner RNFL layer.[1]
Optic neuritis may be a primary manifestation of an underlying disorder such as MS or a system autoimmune condition. Recurrence of ON occurs in approximately one third of patients.[1]
The prognosis for visual recovery is excellent in adults with or without medical therapy, as found in the Optic Neuritis Treatment Trial (ONTT).[12] The first pilot study of pediatric optic neuritis was launched in 2016, a 4-year study of children aged 3-15 years with acute optic neuritis in one or both eyes.[10] Data are not yet available from this trial.
Oral corticosteroids should not be used in treatment, as data from the Optic Neuritis Treatment Trial indicate that they increase the risk for optic neuritis relapse.[10]
The established treatment for children with optic neuritis is intravenous methylprednisolone 30 mg/kg per day (maximum 1 g daily) for 3-5 days. While steroids are not expected to change the medical outcome of an optic neuritis episode, they increase the speed of recovery to baseline.[1]
No prospective study of the prognosis for visual recovery in children is available; most, but not all, studies of children have found that visual recovery is good. Specifically, studies have indicated good recovery of high-contrast visual acuity in children.[1] In children with optic neuritis, pulse steroids may speed recovery from 7 weeks to 2 weeks. This may be valuable to avoid psychosocial challenges, including the need to make up schoolwork, or affect other tasks because of visual limitations.[1]
Treatment of the initial event in neuromyelitis optica, whether it is optic neuritis or myelitis, also uses high-dose intravenous steroids, but refractory cases are common and other therapies may be needed. Currently, plasma exchange is favored in those cases, but intravenous immune globulin (IVIg) has also been used.
The equivalent doses of 4 commonly used drugs to treat optic neuritis are provided in Table 4.
Table 4. Equivalent Doses of Commonly Used Corticosteroid Medications
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See related CME at Optic Neuritis: Diagnosis, Treatment, and Prognosis.
The management of a child with optic neuritis is a combined effort by the ophthalmologist and the neurologist. Ophthalmologic input is required to distinguish between optic neuritis and neuroretinitis and to monitor visual response. Neurologic input is required to evaluate possible generalized CNS involvement and to make appropriate decisions and recommendations based on the future risk of MS. An immunologist should also be consulted for children with underlying immunologic pathology.
Children taking etanercept or other tumor necrosis factor (TNF) inhibitors should be reevaluated by their rheumatologists.
Children who do develop MS may be more likely to develop disability at a younger age. Monitoring patients who had no associated neurologic signs or symptoms at the time of their optic neuritis will allow early diagnosis. Treatment of those patients for early onset MS with the disease modifying agents that are used in adults may be an option.
If optic neuritis is a suspected first manifestation of an underlying disorder, prophylactic treatment must be dictated by the causative disorder. For example, with rheumatologic disorders, steroids and long-term immunotherapy may prevent the recurrence of optic neuritis or other inflammatory CNS events.
Recurrence of optic neuritis during or shortly after the discontinuation of steroids indicates a steroid-dependent optic neuritis and requires reevaluation and a more prolonged taper of corticosteroids.
Outpatient follow-up care should include monitoring both visual recovery and recovery from neurologic or systemic disorders that were associated with optic neuritis.
A widely used group of drugs. They have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. GI tract absorption is approximately 85% of intravenously administered doses. Corticosteroids are well tolerated. A brief course of steroids is not expected to produce significant adrenal complications. Many physicians prescribe an H2 blocker to prevent GI tract distress.
What is childhood optic neuritis?What is the pathophysiology of childhood optic neuritis?What is the prevalence of childhood optic neuritis?What is the morbidity associated with childhood optic neuritis?What are the racial predilections of childhood optic neuritis?What are the sexual predilections of childhood optic neuritis?How do the clinical features of optic neuritis vary between adults and children?What is the prognosis of childhood optic neuritis?How does childhood optic neuritis affect the risk for development of multiple sclerosis?What is included in patient education about childhood optic neuritis?Which clinical history findings are characteristic of childhood optic neuritis?Which physical findings are characteristic of childhood optic neuritis?What causes childhood optic neuritis?What is the visual acuity on examination in childhood optic neuritis?Which fundus exam findings suggest childhood optic neuritis?What is the visual prognosis after childhood optic neuritis?Which conditions are included in the differential diagnoses of childhood optic neuritis?What are the differential diagnoses for Childhood Optic Neuritis?What is the workup approach for childhood optic neuritis?What is the role of lab testing in the workup of childhood optic neuritis?What is the role of imaging studies in the workup of childhood optic neuritis?What is the role of optical coherence tomography (OCT) in the workup of childhood optic neuritis?How is childhood optic neuritis treated?Which specialist consultations are beneficial to patients with childhood optic neuritis?What is included in the long-term monitoring of childhood optic neuritis?What is included in outpatient follow-up care for childhood optic neuritis?What are the goals of drug treatment for childhood optic neuritis?Which medications in the drug class Corticosteroids are used in the treatment of Childhood Optic Neuritis?
Varsha Sivagami Sathappan, Baylor College of Medicine
Disclosure: Nothing to disclose.
Coauthor(s)
Honey H H Herce, MD, Assistant Professor, Departments of Ophthalmology and Pediatrics, Assistant Fellowship Program Director for Pediatric Ophthalmology and Strabismus Fellowship, Baylor College of Medicine, Texas Children's Hospital
Disclosure: Nothing to disclose.
Specialty Editors
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
Edsel Ing, MD, MPH, FRCSC, Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Active Staff, Michael Garron Hospital (Toronto East Health Network); Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital, Canada
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
Disclosure: Nothing to disclose.
John E Carter, MD, Professor, Departments of Neurology and Ophthalmology, University of Texas Health Science Center at San Antonio
Disclosure: Nothing to disclose.
Martha P Schatz, MD, Chief, Pediatric Ophthalmology Service, Clinical Professor of Ophthalmology, University of Texas Health Science Center at San Antonio
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
Bonhomme GR, Liu GT, Balcer LJ. Isolated pediatric optic neuritis. Brain MRI abnormalities and risk of multiple sclerosis. Paper presented at: Annual Meeting of the North American Neuro-ophthalmology Society; 2005.
Optic disc swelling in the right eye and left eye in a child with bilateral optic neuritis.
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.
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.
Adult Optic Neuritis
Pediatric Optic Neuritis
Unilateral
Often bilateral in children < 10 years[6]
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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