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2014 ; 5
(2
): 138-43
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Acute retinal necrosis in childhood
#MMPMID24932179
Pikkel YY
; Pikkel J
Case Rep Ophthalmol
2014[May]; 5
(2
): 138-43
PMID24932179
show ga
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.