Clinical Features of Neuroretinitis

A 13-year-old girl was seen for assessment of blurred vision and optic disc edema in her right eye. Her examination showed: best-corrected visual acuity of hand motion OD and 20/25 OS; pupils: no relative afferent pupil defect; color vision: 0/14 plates OD and 14/14 plates OS; humphrey visual fields: no response OD and full OS. Her fundus examination showed an edematous right optic disc, macular subretinal fluid and yellow macular exudate in the configuration of a "macular star". Also noted was a small, elevated white granuloma along the superior vascular arcade, with vascular sheathing in the same area. The left fundus was normal. Based on the results of her examination and ophthalmic investigations, the patient was diagnosed with neuroretinitis of the right eye. A serological work-up was positive for bartonella henselae.

Clinical Features of Neuroretinitis Rahul A. Sharma, MD, MPH Jason H. Peragallo, MD Valérie Biousse, MD A 13-year-old girl was seen for assessment of blurred vision and optic disc edema in her right eye. Her examination showed: • • • • Best-corrected visual acuity: hand motion OD and 20/25 OS Pupils: no relative afferent pupil defect Color vision: 0/14 plates OD and 14/14 plates OS Humphrey visual fields: no response OD and full OS Figure 1A Right eye (OD) Left eye (OS) Color photographs of the posterior poles showed an edematous right optic disc, macular subretinal fluid and yellow macular exudate in the configuration of a "macular star". Also noted was a small, elevated white granuloma along the superior vascular arcade, with vascular sheathing in the same area. The left fundus was normal. Figure 1B Right eye (OD) Figure 1B Small (elevated, white) retinal granuloma Retinal arterial sheathing along superior arcade (blue dotted line) "Macular star": yellow macular exudates Elevated, hyperemic optic disc with blurred disc margins Retinal hemorrhages Extensive subretinal fluid in posterior pole (dotted white line) Right eye (OD) Figure 2 Optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL) showed increased thickness of the RNFL in the right eye (red boxes), consistent with optic disc edema OD. Figure 3 OD OS Optical coherence tomography (OCT) of the right macula showed subretinal fluid with visible exudates in the outer plexiform layer/"Henle" layer. OS was normal. Figure 3 OD OS Optical coherence tomography (OCT) of the right macula showed subretinal fluid in right eye (red arrow) with visible exudates in the outer plexiform/"Henle" layer (green outline). OS was normal. Figure 4A Wide-field intravenous fluorescein angiography (IVFA) of the right eye showed an expanding area of hyperfluorescence around the right optic disc, consistent with disc leakage due to optic disc edema. Also noted were areas of leakage in the area of the granuloma and along the superior retinal arcade, suggesting retinal vasculitis. IVFA OS was normal. Early OS (0:27) Early OD (0:20) Late OS (5:32) Late OD (2:27) Figure 4B Early OD (0:27) Late OD (5:32) Wide-field intravenous fluorescein angiography (IVFA) of the right eye showed an expanding area of hyperfluorescence around the right optic disc, consistent with disc leakage due to optic disc edema (red arrow). Also noted were areas of leakage in the area of the granuloma (green arrow) and along the superior retinal arcade (blue circle), suggesting retinal vasculitis. Based on the results of her examination and ophthalmic investigations, the patient was diagnosed with neuroretinitis of the right eye. Figure 5: Neuroretinitis refers to an inflammatory disorder affecting both the anterior optic nerve and the retina. Selected causes Neuroretinitis • • • • • HIV Toxoplasmosis EBV Hepatitis B and C Tuberculosis Infectious Inflammatory • • • • • Bartonella Syphilis Lyme disease Histoplasmosis Brucellosis Non-Infectious Inflammatory •• •• • Sarcoidosis Sarcoidosis Polyarteritis nodosa nodosa (PAN) Polyarteritis Idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) Vogt-Koyanagi-Harada syndrome (VKH) Behcet's disease (Bacterial, viral, fungal, parasitic) • • "Idiopathic" (No diagnosis after a work-up; ½ of all cases) • Masquerades Pathologic entities that mimic neuroretinitis • • • • Non-arteritic anterior ischemic optic neuropathy (NAION) / diabetic papillopathy Hypertensive retinopathy Disc and juxtapapillary tumors Posterior vitreous traction Toxic retinopathies A serological work-up in our patient was positive for bartonella henselae. Summary • Neuroretinitis is a clinical diagnosis: • Typically unilateral • Optic disc edema • Resolves over weeks but may cause optic atrophy • Retinal findings (typically appear after days-weeks): • Subretinal fluid in peripapillary retina and/or macula • Lipid-rich fluid in outer plexiform layer (macular star) • Retinal vasculitis may be seen • In certain infections, retinal granulomas may occur Optic nerve granuloma in a separate patient with Bartonella neuroretinitis Summary • Optical coherence tomography of the macula may show retinal thickening, subretinal fluid, and fluid or exudates in the outer plexiform layer • Fluorescein angiography may show disc edema and leakage, blockage of fluorescence in areas of hard exudates and retinal vasculitis Late OD (5:32)