(F) Fundus Autofluorescence now Shows an Area of Stippled Hyperfluorescence in an Area of Hypofluorescence (arrowheads). (B) Corresponding Optical Coherence Tomography (OCT) Shows Hyperreflectivity of the Inner Retina (asterisk) and Outer Retinal Edema (arrows), Whereas (C) Fundus Autofluorescence Shows Subtle Hypofluorescence (arrows). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). Following therapy, OCT showed an overall reduction in retinal thickness; clinically, large atrophic areas of retina were observed. The progressive outer retinal necrosis syndrome. The progressive outer retinal necrosis syndrome. It is possible that these fundus autofluorescence changes occur subsequent to photoreceptor death and outer retinal injury and inflammation by varicella zoster virus. Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). Primary treatment includes antiviral agents both intravitreally and intravenously. Less than approximately 40% of patients have an anterior chamber reaction and less than 20% have vitreous cell. These lesions can rapidly progress to full thickness as well as confluence without consistent directionality of spread. The role of CMV as an etiologic agent in PORN remains controversial, however, as often evidence of CMV-retinitis is an exclusion criteria in a number of studies. Get full access to the content of this case. Retention of ambulatory vision has been demonstrated in some studies in up to 70% of patients. In 1971, Akira Urayama described acute retinal necrosis (ARN) as consisting of a clinical syndrome of an acute unilateral panuveitis associated with retinal periarteritis and necrotizing retinitis. Fundus autofluorescence revealed stippled hyperfluorescence within extensive zones of hypofluorescence, which progressed during follow-up. Foscarnet has recently been found to have increased resistance, and also can cause nephrotoxicity. At this stage, the inner retina appeared hyperreflective and the outer retina showed mild decreased reflectivity and disorganization indicative of early inflammation and tissue damage. [3] Fluorescein angiography (FA) findings can be variable. In a study, 73% patients had a history of cutaneous zoster and 73% patients had suspected viral involvement of the central nervous system (Ormerod et al.). PATIENTS AND METHODS: A retrospective review was performed on six consecutive patients who were diagnosed with PORN and were treated with various … Atrophy of the RPE and all layers of the neurosensory retina in areas of prior necrosis may also be evident. 1998 Jan;26(1):34-45; discussion 46-7. In 1971, Akira Urayama described acute retinal necrosis (ARN) as consisting of a clinical syndrome of an acute unilateral panuveitis associated with retinal periarteritis and necrotizing retinitis. Clin Infect Dis. Progressive outer retinal necrosis may involve any part of the retina at presentation (including macula) with little or no inflammation in anterior chamber or vitreous. Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). Progressive outer retinal necrosis, or PORN, is a destructive, rapidly progressing necrotizing herpetic retinopathy with features distinct from those of ARN. Atypon Eur J Ophthalmol. 2005 Jun;243(6):607-9. doi: 10.1007/s00417-004-0998-4. Atypon Purchase this case now for $29. Following pars plana vitrectomy, retinectomy, and silicone oil tamponade, retinal attachment was achieved. Of patients with history of cutaneous involvement, 41% involved the V1 distribution and 14% had concurrence of skin and intraocular manifestations. COVID-19 is an emerging, rapidly evolving situation. Rapidly progressive herpetic retinal necrosis: a blinding disease characteristic of advanced AIDS. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. And denied any history of cutaneous involvement, 41 % involved the V1 distribution and 14 % concurrence. Necrosis demonstrate late staining, while inactive, atrophic regions display window.. 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