Optical Coherence Tomography in Neuroretinitis: Epipapillary Infiltrates and Retinal Folds

Vertebrobasilar dolichoectasia (VBD) is characterized by significant dilation, elongation, and tortuosity of the vertebrobasilar system. We present a unique case of VBD, confirmed by neuroimaging studies, showing vascular compression of the right optic tract and lower cranial nerves leading to an incongruous left homonymous inferior quadrantanopia and glossopharyngeal neuralgia.

Photo Essay Section Editor: Timothy J. McCulley, MD Optical Coherence Tomography in Neuroretinitis: Epipapillary Infiltrates and Retinal Folds Luca Zatreanu, MD, Patrick A. Sibony, MD, Mark J. Kupersmith, MD FIG. 1. Neuroretinitis. Columns represent Patients 1-4. Patient 1, 35-year-old woman; right eye, Bartonella henselae. Patient 2, 31-year-old man; right eye, toxoplasmosis. Patient 3, 57-year-old man; right eye, idiopathic. Patient 4, 19-year-old woman; right eye, toxoplasmosis. Row A, Initial fundus appearance. Row B shows the 9-mm SD-OCT axial raster at baseline, displayed in the default Cirrus 5000 OCT (Zeiss Meditec) ·3 magnified format (3:2 aspect ratio). The labeled box scale in 2B applies to all raster images. Location of raster on the optic disc is shown with yellow line in Row A. Black arrows point to the EI. Arrowheads point to peripapillary retinal folds. The number in upper right corner of row B is the mean RNFL thickness in microns. In 3B, the mean RNFL thickness could not be obtained due to artifacts. Red arrowhead in 4B shows an example of a peripapillary outer retinal crease. Row C is the initial en face SD-OCT at the vitreoretinal interface showing concentric rings of peripapillary folds that extend out to macula (1C and 4C are montages of the optic disc and macula). Row D shows SD-OCT axial raster at the follow-up visit with complete resolution of the EI in Cases 1 and 3 (at 1 month and 3 weeks, respectively) and greatly improved in Case 2 (at 1 week) and Case 4 (at 2 weeks). Note that intraretinal edema has also improved in all cases. Row E is the follow-up en face SD-OCT at the vitreoretinal interface showing resolution of the peripapillary folds in Cases (2E-4E). Missing cells indicate that the image was of poor quality or not available. EI, epillary infiltrates; SD-OCT, spectral-domain optical coherence tomography. Department of Ophthalmology (LZ, PAS), State University of New York at Stony Brook, Stony Brook, New York; and Department of Neurology and Ophthalmology (MJK), Icahn School of Medicine at Mount Sinai, Mt. Sinai Roosevelt and the New York Eye and Ear Infirmary, New York, New York. Address correspondence to Patrick A. Sibony, MD, Department of Ophthalmology, State University of New York at Stony Brook, Health Sciences Center, L2, Rm 152, Stony Brook, New York 117948223; E-mail: [e-mail] 176 Abstract: Neuroretinitis is a form of papillitis associated with the delayed onset of an exudative macular star among other inflammatory ocular findings. We describe 4 patients with neuroretinitis who displayed several distinctive and novel findings on spectral-domain optical coherence tomography (OCT) that include "epipapillary infiltrates" and an atypical pattern of inner retinal folds. The recognition of these findings on OCT may help in the early diagnosis of neuroretinitis. Journal of Neuro-Ophthalmology 2017;37:176-178 doi: 10.1097/WNO.0000000000000501 © 2017 by North American Neuro-Ophthalmology Society Zatreanu et al: J Neuro-Ophthalmol 2017; 37: 176-178 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay F our patients (2 men and 2 women), 19-57 years of age, presented with rapid vision loss and ocular discomfort. Vision ranged from 20/30 to 20/400, and all 4 had optic disc edema. Within 2 weeks of onset, each of the patients developed a macular star, consistent with a clinical diagnosis of neuroretinitis (Figs. 1, 1A-4A). Serological testing revealed that Case 1 was due to Bartonella henselae, Cases 2 and 4 due to toxoplasmosis, and Case 3 was idiopathic. Spectral-domain optical coherence tomography (SDOCT) scan that included an optic nerve head (ONH) cube, axial raster, and en face imaging (Cirrus 5000; Zeiss Meditec, Dublin, CA) was performed on the day of presentation of each patient. All scans displayed an illdefined infiltration (Figs. 1, 1B, 2B, 4B) or a wellcircumscribed mass (Fig. 1, 3B) on the surface of the ONH. In 3 patients, the infiltrate preceded the appearance of the macular star. Small opacities, presumably vitreous cells, were scattered around the ONH. These epipapillary infiltrates (EI) resolved or greatly improved (Figs. 1, 1D- 4D) over 1 to 4 weeks of treatment with antibiotics and oral prednisone. All patients also displayed concentric rings of inner retinal folds that expanded outwardly from the ONH. In 2 of the cases, these folds extended into the macula (Figs. 1, 1C, 4C). These folds resolved over the same time interval as the infiltrates. Neuroretinitis is an inflammatory optic neuropathy associated with vitreous cells and an exudative macular star. The macular exudates, which usually develop 9-12 days after the disc edema, originate from the vasculature of the ONH. Other ocular findings include small chorioretinal lesions and venous sheathing (1). Although idiopathic in many cases, infectious causes for neuroretinitis have been reported, including B. henselae, toxoplasmosis, toxocariasis, and syphilis (1-3). Findings on SD-OCT have been described in a small number of patients (2,4,5). They include the presence of severe optic disc edema with thickening of the retinal nerve fiber layer (RNFL), subretinal and intraretinal edema, macular exudation, and late thinning of the RNFL. The infiltrates displayed with SD-OCT in these patients are, in some respects, similar to the "consolidated cellular debris" described in 1 case (2) or what others have ophthalmoscopically described as "optic disc granulomas" (1,5). We would propose calling these "epipapillary infiltrates", a term that obviates the histological specificity of a "granuloma" and does not precisely define the composition of the infiltrates. Although we have no pathological confirmation, we suspect that EI are inflammatory byproducts based on the rapid disappearance and other inflammatory signs (i.e., vitreous cells, edema) within weeks of onset and treatment. To our knowledge, EI have not been reported in other forms of optic disc edema. Moreover, we have not observed similar infiltrates among Zatreanu et al: J Neuro-Ophthalmol 2017; 37: 176-178 the axial rasters of 125 study eyes with papilledema (6) and 50 eyes with anterior ischemic optic neuropathy (AION) (unpublished data). Folds are the result of biomechanical forces applied to a surface layer. Their pattern is a product of the structural geometry of the layer and loading force conditions. The pattern of folds described in our patients with neuroretinitis differs from the patterns previously described in papilledema (6). This difference presumably reflects the distinctive loading forces that occur in each. We have demonstrated that there are 4 patterns of folds that occur in papilledema: peripapillary wrinkles (Paton folds), peripapillary outer retinal folds/creases ("high water marks"), inner retinal folds that spare the choroid, and choroidal folds. Each type has its own characteristics based on location, configuration, spatial wavelength, and loading forces. The development of folds in papilledema appears to be the product of a complex interaction between 2 principal mechanical determinants: volume expansion of the ONH from axonal distension and anterior displacement of the peripapillary retina secondary to elevated intracranial pressure at the scleral flange (6,7). The latter is notably absent in patients with neuroretinitis. While inner retinal folds of the posterior pole have been described in both papilledema and neuroretinitis, the pattern in papilledema is oriented radially or horizontally around the ONH (6), whereas the inner retinal folds in neuroretinitis consist of outwardly expanding concentric rings that can extend into the macula. We speculate that the concentric rings are due to the mechanical effects of intraretinal and subretinal edema characteristically seen in patients with neuroretinitis. In our 4 patients, the concentric folds resolved as the optic disc edema and subretinal fluid improved. The frequency and specificity of EI and the concentric ring pattern of inner retinal folds in neuroretinitis are unknown. We suspect that EI can occur in any papillitis associated with significant vitritis. EI are difficult to see ophthalmoscopically, but their recognition using OCT might expedite the early diagnosis of neuroretinitis before the macular star appears. If retinal edema is, as we hypothesize, responsible for the concentric ring pattern of retinal folds, then a similar pattern might occur in other forms of optic disc edema with peripapillary retinal edema, such as AION. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: Patrick A Sibony; b. Acquisition of data: Patrick A Sibony, Luca Zatreanu; c. Analysis and interpretation of data: Patrick A Sibony, Luca Zatreanu, Mark J. Kupersmith. Category 2: a. Drafting the manuscript: Luca Zatreanu; b. Revising it for intellectual content: Patrick A Sibony, Luca Zatreanu, Mark J. Kupersmith. Category 3: a. Final approval of the completed manuscript: Patrick A Sibony, Luca Zatreanu, Mark J. Kupersmith. 177 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay REFERENCES 1. Solley WA, Martin DF, Newman NJ, King R, Callanan DG, Zacchei T, Wallace RT, Parks DJ, Bridges W, Sternberg P. 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Zatreanu et al: J Neuro-Ophthalmol 2017; 37: 176-178 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.