Acute Retinal Necrosis: Signs, Treatment, Complications and Outcome

Abstract

Background: The Acute Retinal Necrosis (ARN) is an inflammatory, rapidly progressive necrotizing retinitis and vasculitis, most frequently caused by Varicella-Zoster-Virus (VZV), followed by Herpes-Simplex-Virus (HSV), Cytomegalovirus (CMV) and Epstein-Barr-Virus (EBV). The diagnosis is based on clinical signs that were first defined by the American Uveitis Society in 1994 that include one or more foci of retinal necrosis, rapid progression without treatment, circumferential progression, occlusive vasculopathy, and inflammatory signs of the vitreous and anterior chamber Methods: In this retrospective analysis, we included 16 eyes of 10 patients, six patients with simultaneous or delayed bilateral affection, treated for ARN. Status of disease, corrected distance visual acuity (CDVA, decimal), intraocular pressure (IOP), pathogen proof, therapy, and complications were evaluated at diagnosis and 3 months later.

Results: In nine patients, the pathogen was identified (six VZV, two HSV, one CMV, one EBV). All patients were treated with systemic and intravitreal virustatic agents. In nine eyes with a CDVA of 0.2 ± 0.2 at hospital admission, vitrectomy was performed, and in seven eyes with CDVA of 0.5 ± 0.3, no vitrectomy was performed (p = 0.04). After 3 months, CDVA of the vitrectomized eyes decreased to 0.1 ± 0.1 vs. 0.4 ± 0.3 (p = 0.01) without vitrectomy. CDVA of fellow eyes affected was 0.6 ± 0.2 at initial presentation vs. 0.2 ± 0.2 for eyes affected first and 0.4 ± 0.3 vs. 0.1 ± 0.1 after 3 months. We observed several complications including retinal detachment, recurrence of the disease, and bulbar hypotony.

Conclusion: For fellows eyes affected, diagnosis could be confirmed earlier, leading to a more successful treatment. The success of vitrectomy is difficult to evaluate because vitrectomy is most frequently performed just in the advanced stages of the disease. Early treatment with an appropriate approach is essential to avoid loss of vision.

Keywords: Varicella-Zoster-Virus; acute retinal necrosis; uveitis.

Figure 1

Findings in acute retinal necrosis with retinitis and perivascular infiltrations (arrowheads) as well as perivascular hemorrhage, whitish fundus, and retinal edema (a). (b) Parapapillary occlusive vasculitis, pale optic nerve disc, and peripheral retinal inflammation with secondary necrosis.

Figure 2

59-year-old patient with acute retinal necrosis (ARN) in the left eye: first presentation with suspected retinal branch artery occlusion and iritis treated with topical prednisolone for about 10 days and “non improving vision”. (a) Fundus photography of the left eye. Visual acuity 0.5; conjunctiva injected, anterior chamber cells +, Arlt’s triangle with pattern of mutton-fat keratic precipitates, retina with circular whitish foci and hemorrhages, especially localized temporally. (b) The right eye was not affected. Treatment including vitrectomy, intravitreal ganciclovir, and air (asterisk) (c) followed by detection of varizella zoster virus (VZV) using polymerase chain reaction (PCR) and parenteral aciclovir treatment (10 mg/kg of body weight every 8 h + prednisolone and two ganciclovir intravitreal injections. Ten days later, retinal detachment occurred and was treated with vitrectomy, ganciclovir injection, and silicone oil tamponade. The retina was stable postoperatively, and the visual acuity was 0.2 (d). Four weeks after the onset of the ARN in the left eye, the right eye achieved a visual acuity of 1.0, but showed a temporal fresh necrotic focus (e, arrowhead). Immediate start with intravitreal Ganciclovir. Follow-up 10 days later with a clearly demarcated infiltrate (f) followed by weekly checkups and long-term treatment with 800 mg Aciclovir five times a day. For further details see Table 1, Patient 4.

Figure 3

Bilateral onset of acute retinal necrosis (ARN). Right eye findings on first presentation: (a) Funduscopy with peripheral retinal infiltrations, (b) ischemic inner retinal layers (arrows) and edema in optical coherence tomography (OCT), (c) vascular leakage in fluorescein angiography (arrowheads). (d–h) follow-up with resolving infiltrates, development of bleedings (d), and retinal necrosis (g), arrowhead. For further details see Table 1, patient 8.

Figure 4

Corresponding left eye of the patient in Figure 3 on first presentation: (a) funduscopy with peripheral retinal infiltrations, (b) ischemic inner retinal layers (arrows), and edema in optical coherence tomography (OCT), (c) vascular leakage in fluorescein angiography (arrowheads). (d–h) follow-up with resolving infiltrates, development of bleedings (d), and retinal necrosis (g), arrowhead. For further details see Table 1, patient 8.