Acute Retinal Necrosis: Clinical Features, Early Vitrectomy, and Outcomes Jost Hillenkamp, MD, 1 Bernhard Nölle, MD, 1 Claudia Bruns, MD, 1 Peter Rautenberg, MD, 2 Helmut Fickenscher, MD, 2 Johann Roider, MD 1 Objective: To determine the viral diagnosis and the outcome of eyes with acute retinal necrosis (ARN) treated with intravenous acyclovir and oral prednisolone alone or combined with early vitrectomy and intravitreal acyclovir lavage. Design: Nonrandomized, retrospective, interventional, comparative, consecutive series. Participants: A cohort of 27 human immunodeficiency virus–negative patients with ARN comprising 24 unilateral and 3 bilateral cases. Intervention: Vitreous biopsy for viral diagnosis. Twenty eyes were treated with intravenous acyclovir in combination with oral prednisolone (group A). Ten eyes were treated additionally with early vitrectomy, intravitreal acyclovir lavage, laser demarcation of necrotic retinal areas when feasible—with or without scleral buckling, and gas or silicone oil tamponade (group B). Vitrectomy was performed in all cases of secondary rhegmatogenous retinal detachment (RD). Main Outcome Measures: Results of vitreous biopsy, rate of RD, rate of phthisis bulbi, and course of best-corrected visual acuity (BCVA). Results: Varicella zoster virus (VZV) was detected in 26 eyes, followed by herpes simplex virus (5 eyes), and Epstein-Barr virus (2 eyes, in conjunction with VZV). An RD developed in more eyes in group A (18 of 20 eyes) than in group B (4 of 10 eyes; P = 0.007). In 2 of 20 eyes in group A and in 0 of 10 eyes in group B, phthisis bulbi developed without a significant difference between groups A and B. Mean BCVA (logarithm of the minimum angle of resolution) at first visit was 1.09 (standard deviation [SD], 0.83), and mean final BCVA was 1.46 (SD, 0.88) without significant difference between groups A and B. Conclusions: Varicella zoster virus is the leading cause of ARN. Visual prognosis is guarded. Early vitrec- tomy with intravitreal acyclovir lavage was associated with a lower incidence of secondary RD; however, it did not improve mean final visual acuity. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. Ophthalmology 2009;116:1971–1975 © 2009 by the American Academy of Ophthalmology. The syndrome of peripheral necrotizing retinitis with retinal arteritis was described first by Urayama et al in 1971. 1 Young and Bird 2 introduced the term acute retinal necrosis (ARN) for this uncommon, but devastating, potentially blinding syndrome that occurs most commonly in immune competent individuals of either gender at any age. Varicella zoster virus (VZV), herpes simplex virus (HSV), and Epstein- Barr virus (EBV) have been shown to be implicated in the pathogenesis of ARN. 3–9 The clinical diagnosis is based on standard diagnostic criteria proposed by the American Uve- itis Society. 10 The disease is characterized by anterior uveitis, vitreitis, and patchy or confluent areas of white- or cream- colored areas of necrotizing retinitis that rapidly extend poste- riorly from the peripheral retina. Retinal atrophy resulting from necrosis often leads to secondary rhegmatogenous retinal de- tachment (RD). Occlusive vasculitis involves the retinal and choroidal vasculature. The functional outcome often is poor. Given the low incidence of ARN, available data mostly derive from small case series. A number of issues pertaining to ARN management thus remain uncertain. Current management is empirical and varies between centers. 11 The most common therapeutic approach includes diagnostic confirmation by vit- reous biopsy for antiviral antibodies or polymerase chain re- action (PCR) viral DNA analysis and prompt treatment with intravenous acyclovir and systemic corticosteroids. 11 How- ever, successful treatment with intravitreal injections of ganci- clovir or foscarnet 12,13 or oral famciclovir or valacyclovir combined with oral corticosteroids 14,15 also has been reported. Vitrectomy usually is performed only for dense vitreous opac- ities, for vitreous hemorrhage secondary to retinal neovascu- larization, or when secondary RD develops. Given the often rapid progression of retinal necrosis with frequent secondary RD leading to a poor final functional outcome, the authors hypothesized that an intensified therapeutic approach including early vitrectomy with intravitreal acyclovir lavage, and, when feasible, laser demarcation of necrotic retinal areas, scleral buckling, and gas or silicone oil tamponade may help to halt the progression of the disease, to prevent secondary RD and phthisis bulbi, and to help to preserve visual function. To the authors’ knowledge, early vitrectomy and intravitreal applica- 1971 © 2009 by the American Academy of Ophthalmology ISSN 0161-6420/09/$–see front matter Published by Elsevier Inc. doi:10.1016/j.ophtha.2009.03.029