Treatment of Pediatric Optic Neuritis

Abstract

Pediatric optic neuritis is a rare disorder causing a subacute loss of vision (often bilateral) in children, often of a severe degree. Common clinical findings include dense visual field loss, afferent pupillary defect (APD) in unilateral cases, and papillitis that is often bilateral. Contrary to optic neuritis in the adult population, the differential diagnosis for this disorder in pediatric patients is quite extensive, so neuroimaging and serologic evaluation are necessary to exclude an infectious or neoplastic process. Pediatric optic neuritis may occur as a clinically isolated syndrome, or in association with diffuse neurologic dysfunction, such as acute disseminated encephalomyelitis (ADEM). Optic neuritis may also be the first episode heralding the onset of pediatric multiple sclerosis or neuromyelitis optica (NMO or Devic's disease). Therefore, formal neurologic evaluation and follow-up are indicated. Although the management of this disorder in adults is well described, there is a paucity of evidence-based, prospective clinical data on its management and treatment in a pediatric population. The current treatment of pediatric optic neuritis consists of 3 to 5 days of intravenous methylprednisolone (4-30 mg/kg per day), followed by a prolonged oral corticosteroid taper. A prolonged course of oral steroid (2-4 weeks) is recommended to avoid recurrence, which is common in this age group. Some controversy persists concerning the exposure of children to high-dose parenteral corticosteroids to treat an entity that is usually self-limited, but given the severity of vision loss in one or both eyes in this population, this intervention is standard in neuro-ophthalmologic practice.

Pediatric optic neuritis

“…Papillitis has been noted in 46%-69% 6,8 of children with ON compared to one-third of those in the adult Optic Neuritis Treatment Trial (ONTT). 9 Although MRI is not required to diagnose ON in children, it may show focal abnormalities of the anterior visual pathway. Typical MRI findings in dedicated orbital MRI studies consist of thickening of the optic nerves on T1-weighted imaging, bright T2 signal along the optic nerve or chiasm, and postgadolinium enhancement.…”

Early Experience with Diode Laser Combined with Systemic Steroid Therapy for Severe Stages of Retinopathy of Prematurity

“…It might not be precluded that post-steroids complications could be associated with sulfite preservatives contained in the drug [36]. Relating to the data from available literature, it is worth mentioning that there are no clinical observations in connection to steroids application due to retinopathy of prematurity, although their use is commonly known in different retinal or retino-vitreous diseases even in early childhood, so it appears to be impossible to rely on an opinion of other authors in this field [37][38][39][40][41][42][43][44]. Three-year follow-up of combined laser-steroid course indicate beneficial effect of such therapy in the form of retina attachment which has been confirmed in 88.8 % selected cases.…”

“…Some authors present a lot of evidence that Dexamethasone provides a protective action mechanism against the development of severe retinopathy and extraretinal neovascularization [8,11,14,30,41]. There exists a similarity to inhibitory effects of dexamethasone on TNF-α expression in the retina, as in endotoxin-induced uveitis with the use of local delivery steroid system in experimental model [11][12][13][14], what has been applied in many ophthalmic diseases in children and adults [38,[40][41][42][43]. In the mentioned processes rapid nongenomic effects on cell metabolism and cell membrane functions such as altering intracellular signal transduction pathways, limiting tissular inflammatory reactions by inhibiting migration and aggregation of macrophages with enzyme release and growth factors, fibroblastic activity suppression, reducing vascular permeability and also regulating endothelial nitric oxide synthase by non-nuclear effects are used [9,11-13, 29,36].…”

Should patients with optic neuritis be treated with steroids?

“…A greater proportion of patients with severe and/or bilateral vision loss is also characteristic of pediatric optic neuritis (26,27). Children with optic neuritis tend to recover faster than adults and a higher percentage involve secondary causes than in adults (28).…”

“…Despite the lack of prospective clinical data in children to support its use, high-dose intravenous methylprednisolone is considered first-line treatment for pediatric optic neuritis by extension from the ONTT, as affected children often present with impairment of visual acuity severe enough to justify its use. Following IVCS, a prolonged oral corticosteroid taper over 2-6 weeks is common, as some experts believe there is an increased rate of relapse with early cessation of corticosteroids (27,29,30). One recent small retrospective study of 26 consecutive pediatric patients with optic neuritis treated for 3 days with intravenous methylprednisolone compared those treated with an additional two weeks of oral corticosteroids to those treated for more than two additional weeks.…”