Acute Retinal Necrosis in Childhood

Abstract

Background: Acute retinal necrosis (ARN) is a viral syndrome consisting of uveitis/vitritis, occlusive vasculitis and peripheral necrosis. Few incidents are reported in children. The etiology is reactivated herpes simplex virus (HSV) or varicella-zoster virus (VZV). Treatment with acyclovir is often used. The administration of oral glucocorticosteroids is of unproven benefit. Prognosis is variable but poor. Methods: Three weeks after contracting mild chickenpox, a healthy 4-year-old girl developed blurred vision in her right eye. Severely reduced visual acuity was noted, together with anterior uveitis, ‘mutton-fat' precipitates and vitral flare. Retinal vasculitis with necrosis was present. Serology for toxoplasma, cytomegalovirus and HIV was negative, while HSV and VZV IgG antibodies were positive. She was treated with 30 mg/kg of intravenous methylprednisolone (3 days), 30 mg of oral prednisone (3 days), and tapering for 8 weeks. Intravenous acyclovir was given for 10 days, followed by oral acyclovir for 4 months. Aspirin (100 mg/day) was given for 4 months. Results: At 12 months, the girl felt good. Her right eye acuity was 6/9, with an intraocular pressure of 17 mm Hg. The peripheral retina showed scarring but no detachment. Conclusions: This is the first report of a once-daily high-dose methylprednisolone pulse therapy in one of the youngest known ARN cases. Pulsed steroid therapy was based on its known effectiveness in vasculitis, which is the main pathophysiology in ARN. There was no evidence of steroid-related viral over-replication. Our case achieved an excellent clinical and ophthalmic recovery in spite of the poor prognosis. The positive result of this case report provides a basis for further evaluation of high-dose steroid pulse therapy in ARN.

Case Report: Herpes Simplex Virus Type 2 Acute Retinal Necrosis With Viral Encephalitis in Children

“…ARN syndrome generally has a poor prognosis ( 9 ) and can quickly affect the other eye if left untreated ( 30 ). Early diagnosis and therapy are key to maintaining vision and preventing the involvement of the other eye ( 16 ).…”

“…ARN syndrome is a serious viral infectious disease, and its main pathogenic viruses are varicella zoster virus (VZV), herpes simplex virus types 1 or 2, CMV, and the Epstein-Barr virus (EBV) (6)(7)(8). ARN can occur in both adults and children; however, it occurs more commonly in adults, with an average age of onset reported to be between 20 and 60 years (9). Several studies have reported that adult ARN is mainly due to VZV and HSV-1 (10).…”

Acute retinal necrosis results in low vision in a young patient with a history of herpes simplex virus encephalitis

“…The use of loaners and training with lower magnification may contribute to greater success for the patient and the provider. Providing handouts, follow‐up visits with demonstrations and peer group advice can be helpful for the new low vision patient …”