Acute retinal necrosis in childhood

Area of Science:

• Ophthalmology
• Pediatric Medicine
• Infectious Diseases

Background:

• Acute retinal necrosis (ARN) is a severe viral syndrome characterized by uveitis, vasculitis, and retinal necrosis, rarely reported in children.
• Common etiologies include reactivated herpes simplex virus (HSV) or varicella-zoster virus (VZV), with a generally poor prognosis.
• Standard treatment involves acyclovir, but the benefit of oral glucocorticosteroids remains unproven.

Observation:

• A 4-year-old girl presented with blurred vision, severe visual acuity reduction, anterior uveitis, and retinal vasculitis with necrosis following mild chickenpox.
• Ocular findings included 'mutton-fat' precipitates and vitreal flare, with positive serology for HSV and VZV.
• Initial investigations ruled out other infectious causes like toxoplasma, cytomegalovirus, and HIV.

Findings:

• The patient received a novel treatment regimen including high-dose intravenous methylprednisolone pulse therapy, oral prednisone, and prolonged acyclovir.
• Aspirin was also administered for its anti-inflammatory and anti-vasculitic properties.
• At 12 months post-treatment, the patient exhibited excellent visual acuity (6/9) and no retinal detachment, despite peripheral retinal scarring.

Implications:

• This report is the first to describe high-dose methylprednisolone pulse therapy in a pediatric ARN case, demonstrating its potential efficacy.
• The treatment, based on the known effectiveness of steroids in vasculitis, showed no evidence of steroid-related viral over-replication.
• The excellent clinical outcome suggests a potential basis for further investigation into high-dose steroid pulse therapy for ARN in children.