The progressive outer retinal necrosis (PORN) syndrome is a clinical variant of necrotizing herpetic retinopathy in profoundly immunocompromised patients with a large percentage of those infected with advance AIDS (< 50 cells CD4). It is characterized by multifocal, patchy, retinal opacification that starts in the posterior pole and spreads rapidly to involve the entire retina. Patients with PORN usually present rapid progression with minimal anterior chamber inflammation, with no vitritis or retinal vasculitis allowing this entity to be distinguished from acute retinal necrosis. Optic nerve involvement can also occur, presenting as a papillitis or neuroretinitis.
Despite aggressive therapy with antiviral drugs, prognosis is poor. The disease progression and/or recurrence are common and the majority of patients develop no light perception vision. Total retinal detachments are common.
Clinical symptoms include unilateral or sometimes bilateral decreased vision, floaters, and constriction of visual fields. There are no complaints of pain.
Differential Diagnoses:
- Cytomegalovirus retinopathy
- Acute retinal necrosis (ARN)
