A 42-year-old man presented with fever, chills, a syncopal episode, and recent visual loss in both eyes. He acknowledged homosexual encounters and had lost 80 pounds during the past year.
Best-corrected visual acuities were 20/400 OD and finger counting OS. Pupils measured 5 mm in dim illumination and constricted adequately to light OU without afferent pupillary defect. Visual field examination revealed cecocentral scotomas OU. Ophthalmoscopic examination disclosed rare vitreous cells, moderate disc swelling, telangiectatic surface vessels, and small nerve fiber layer hemorrhages OU. Significant retinal thickening extended into the maculae, but the retina otherwise appeared normal (Fig. 1).
Fluorescein angiography showed hyperfluorescent discs OU with late sub-macular accumulation of dye but no retinal vascular leakage (Fig. 1). Optical coherence tomography (OCT) showed significant outer plexiform layer retinal edema extending from the discs to the fovea and subfoveal detachments (Fig. 1).
Pertinent positive laboratory studies included CD4+ of 86/μL (401-1532), Bartonella henselae IgG of 1:128 (<1:128), cytomegalovirus IgG of 309.5 Au/mL (0-14), cytomegalovirus pp65 of 8/400,000 cells, a positive HIV by Western blot assay, and HIV-1 RNA of >100,000 copies/mL (50-100,000). Brain magnetic resonance imaging and cerebrospinal analysis were negative.
With serologic evidence of HIV and cytomegalovirus infections, treatment with lamivudine/zidovudine, efavirenz, valganciclovir, ciprofloxacin, and azithromycin was begun. Hard exudates appeared in both macula 11 days after presentation (Fig. 2). During the next 5.5 months, serial OCT studies showed gradual resolution of the outer plexiform edema and subfoveal edema OU, leaving only minimal residual findings in the OS (Fig. 3). Segmental temporal disc pallor developed OU. Visual acuities measured 20/30 OD and 20/400 OS.
Neuroretinitis represents an inflammatory optic neuropathy characterized by increased permeability of deep optic nerve blood vessels, leading to macular edema and lipid exudates in a star pattern. The disease usually runs a 6- to 12-week course, typically with good visual recovery (1). Although neuroretinitis has served as an AIDS-defining infection in HIV patients (2), we are unaware of its being a presenting feature.
Peripapillary neurosensory retinal detachments are believed to result from fluid that escapes from deep optic disc vessels and seeps directly into the subretinal space (3). The fluorescein angiographic and OCT findings in our case are consonant with subfoveal detachments. The fluorescein angiogram showed a 1950μ circular area of dye accumulation beneath the right fovea; the OCT electronic calipers measured a 2005μ diameter neurosensory detachment, in excellent agreement with the fluorescein angiogram. The OCT showed extensive fluid within the outer plexiform layer but no evidence of communication between the neurosensory detachment and the optic disc margin. These findings resemble those of Puliafito (4), who noted substantial cystic outer plexiform layer edema, and a neurosensory detachment that did not communicate with the disc in a patient with an optic pit. Similar exudative neurosensory retinal detachments secondary to macular edema can occur in retinal vascular diseases, suggesting that limited subfoveal fluid accumulation may occur more commonly than previously believed (5).
Because the fluorescein angiogram showed no evidence of retinal vascular or retinal pigment epithelial leakage, the exact mechanism of the neurosensory detachments remains uncertain in our case. Subfoveal neurosensory detachment secondary to macular edema can occur because of protein and fluid movement across the outer limiting membrane (6). We postulate that retinal edema in neuroretinitis occurs primarily by fluid movement from the optic disc into the outer plexiform layer, with secondary transit through the outer limiting membrane, resulting in neurosensory subfoveal detachments.
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© 2005 Lippincott Williams & Wilkins, Inc.
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