HSV-1--induced acute retinal necrosis syndrome presenting with severe inflammatory orbitopathy, proptosis, and optic nerve involvement

OBJECTIVE: To present a unique case in which orbital inflammation, proptosis, and optic neuritis were the initial symptoms of acute retinal necrosis (ARN). The clinical presentation of ARN, as well as the currently recommended diagnostic procedures and guidelines for medical treatment of ARN, are summarized. DESIGN: Interventional case report. TESTING: Polymerase chain reaction (PCR) techniques were made on the vitreous for cytomegalovirus, Epstein-Barr virus, herpes simplex virus (HSV), varicella zoster virus, and toxoplasmosis. A full laboratory evaluation was made together with HLA-typing and serologic tests measuring convalescent titers for HSV and other micro-organisms. Magnetic resonance imaging scan, computed tomography (CT) scan, and fluorescein angiographic examination were performed. The patient was treated with acyclovir and oral prednisone. MAIN OUTCOME MEASURES: The patient was evaluated for initial and final visual acuity and for degree of proptosis, periocular edema, and vitreitis. RESULTS: The first symptoms and signs of ARN were eye pain, headache, proptosis, and a swollen optic nerve on CT scan. Other than increased C-reactive protein, all blood samples were normal. PCR was positive for HSV-type I in two separate vitreous biopsies. The patient had the strongly ARN-related specificity HLA-DQ7. CONCLUSIONS: This is the first report of HSV-induced ARN presenting with inflammatory orbitopathy and optic neuritis. Polymerase chain reaction for HSV-1 was positive more than 4 weeks after debut of symptoms, which is a new finding. The combination of severe vitreitis and retinal whitening, with or without proptosis, should alert the clinician to the possibility of herpes infection and treatment with intravenous acyclovir started promptly.