Bilateral acute retinal necrosis caused by two separate vira
Now open: Certificate Course in Management of Covid-19 by Govt. Of Gujarat and PlexusMDKnow more...Now open: Certificate Course in Management of Covid-19 by Govt. Of Gujarat and PlexusMDKnow more...
Acute retinal necrosis (ARN) is an infectious retinitis and retinal vasculitis characterized by prominent vitreous inflammation and progressive retinal necrosis. Viruses implicated in causing ARN include varicella zoster (VZV), which accounts for most cases, as well as herpes simplex viruses (HSV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV). Late complications can include retinal detachment and macular ischemia, and visual prognosis is generally guarded.

A 67-year-old man with history of lupus nephritis presented for retinal evaluation with a 7-day history of floaters and blurred vision in the left eye. His medications included hydroxychloroquine 200mg daily, mycophenolate mofetil (Cellcept) 1000 mg twice daily, and prednisone 10 mg daily. He had been hospitalized 1 month earlier for treatment of community-acquired pneumonia, at which time he was also noted to have a dermatomal vesicular rash along his left groin. He was diagnosed with varicella zoster infection and treated with 7 days of intravenous acyclovir followed by a 3-day course of valacyclovir 1 g by mouth twice daily.

At the presentation, his best-corrected visual acuity was 20/25 in the right eye and 20/100 in the left eye. Slit-lamp examination was notable for trace conjunctival injection and 2+ cell in the anterior chamber of the left eye. Fundus examination of the left eye revealed 1+ vitritis, few mid-peripheral intraretinal hemorrhages, and peripheral scalloped hypopigmented lesions with pigmented posterior borders in all quadrants of the peripheral retina . An anterior chamber paracentesis of the left eye was performed, and the aqueous sample was positive for VZV by polymerase chain reaction (PCR) and negative for HSV and CMV. The patient was treated with valacyclovir 1 g three times per day and three intravitreal injections of foscarnet. Over the next several weeks, the vitritis improved, the retinal lesions healed with atrophic changes and mottled pigmentation, and visual acuity improved to 20/50. Six weeks later, the patient developed a retinal detachment in the left eye that was treated with pars plana vitrectomy, scleral buckle, laser, and silicone oil tamponade. The patient was maintained on valacyclovir 1 g three times per day postoperatively with sustained reattachment and quiescence .

Four months after the initial presentation, the patient presented urgently for pain, redness, and vision loss in the right eye. There were 2+ vitritis, and the fundus exam was notable for a bullous temporal retinal detachment without breaks or tears. The patient was taken to the operating room for repair of the retinal detachment the following day and underwent pars plana vitrectomy, lensectomy, scleral buckle placement, laser, and silicone oil fill. The retina appeared white, consistent with necrotizing retinitis. Indicating that he had a serous retinal detachment. An anterior retinotomy was made superiorly and subretinal fluid was aspirated after perfluoro-N-octane (PFO) was injected. The subretinal fluid was proteinaceous and milky. The vitrectomy specimen was submitted for culture and molecular diagnostics, and the PCR was positive for EBV and negative for VZV, HSV, and CMV. Of note, during induction of anesthesia, the patient was noted to have a diffuse, white plaque over his tongue that was vertically corrugated and thickly furrowed . An infectious disease consultation confirmed suspicion for oral hairy leukoplakia, a clinical sign associated with active EBV infection in immunocompromised patients. One month later, the patient presented again with right eye pain and hand-motions acuity with an intraocular pressure of 46 mmHg. The increased intraocular pressure was associated with anterior chamber flattening from a worsening serous retinal detachment. He underwent cyclophotocoagulation of the right eye and remains comfortable with no light perception (NLP).

In summary, this is a rare case of bilateral ARN with independent infection of each eye by different viruses; VZV affecting the left eye and, four months later, EBV the right eye. In addition to PCR evidence, the patient demonstrated clinical signs of systemic viral infection, with dermatomal vesicular rash preceding VZV-ARN and oral hairy leukoplakia at the time of presentation with EBV-ARN. Immunocompromised patients are vulnerable to ARN from any of its inciting viral causes, and intraocular fluid should be obtained for diagnostic confirmation at the time of second eye presentation in cases of bilateral ARN.