Juxtapapillary Choroidal Neovascular Membrane as a Complication of Optic Disc Drusen: Multimodal Imaging With Swept Source-Optical Coherence Tomography and Optical Coherence Tomography Angiography

A 55-year-old Caucasian man presented to the neuro-ophthalmology department for follow-up evaluation due to long-standing bilateral optic nerve head drusen (ONHD). On examination, the BCVA was 20/20-2 in both eyes. Dilated fundus examination revealed extensive ONHD in both eyes, retinal hemorrhages, exudates inferonasal to the macula, and macular edema inferotemporal to the disc margin. Automated visual field testing revealed generalized depression in both eyes. Late phase leakage was observed on fluorescein angiography (FA). Optical coherence tomography angiography identified a small juxtapapillary choroidal neovascular membrane inferonasal to the macula in the right eye correlating with the area of retinal hemorrhage and exudates.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Juxtapapillary Choroidal Neovascular Membrane as a Complication of Optic Disc Drusen: Multimodal Imaging With Swept Source-Optical Coherence Tomography and Optical Coherence Tomography Angiography Vivian Paraskevi Douglas, MD, DVM, MBA, Konstantinos A. A. Douglas, MD, DVM, MBA, John B. Miller, MD, Dean M. Cestari, MD FIG. 1. Fundus photographs, autofluorescence, automated visual fields, and ganglion cell layer analysis; (A, B) optic disc drusen are striking and extensive on both the fundus photographs and autofluorescence with cup obscuration (left eye not shown). Arteriovenous nicking was evident on the right eye; (C, D) automated visual field test showed generalized depression on both eyes (gray scale and pattern deviation); and (E) ganglion cell complex thickness map with marked ganglion cell layer loss in both eyes. e430 Douglas et al: J Neuro-Ophthalmol 2022; 42: e430-e433 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay Abstract: A 55-year-old Caucasian man presented to the neuroophthalmology department for follow-up evaluation due to longstanding bilateral optic nerve head drusen (ONHD). On examination, the BCVA was 20/20-2 in both eyes. Dilated fundus examination revealed extensive ONHD in both eyes, retinal hemorrhages, exudates inferonasal to the macula, and macular edema inferotemporal to the disc margin. Automated visual field testing revealed generalized depression in both eyes. Late phase leakage was observed on fluorescein angiography (FA). Optical coherence tomography angiography identified a small juxtapapillary choroidal neovascular membrane inferonasal to the macula in the right eye correlating with the area of retinal hemorrhage and exudates. Journal of Neuro-Ophthalmology 2022;42:e430–e433 doi: 10.1097/WNO.0000000000001227 © 2021 by North American Neuro-Ophthalmology Society A 55-year-old Caucasian man presented to the neuroophthalmology department for routine follow-up for long-standing (.40 years) visual field loss, with central vision sparing, secondary to bilateral optic nerve head drusen (ONHD) since childhood. A remote head computed tomography (CT) had confirmed isolated ONHD that had greater clinical involvement in the right eye. Within 5 years after initial diagnosis, he demonstrated significant visual field loss that has been relatively stable. His medical history was significant for hypertension and T2DM and positive family history of ONHD. On examination, the BCVA was 20/20-2 in both eyes. Pupils were equal and reactive to light with trace relative afferent pupillary defect in the right eye that has been present in all follow-up visits. Ocular motility showed full versions and ductions. Intraocular pressure was within normal limits in both eyes. Biomicroscopy of the anterior segment was unremarkable, color perception was intact, and no metamorphopsias were documented. Dilated fundus examination revealed extensive ONHD in both eyes, retinal hemorrhages, exudates inferonasal to the macula, and macular edema inferotemporal to the disc margin in the right eye. The vessels were mildly engorged and arteriovenous nicking was detected in the right eye. The ONHD are striking on both the fundus photographs (Fig. 1A) and the autofluorescence (Fig. 1B). Automated visual Department of Ophthalmology (VPD, KAAD, JBM), Retina Service, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts; Harvard Retinal Imaging Lab (VPD, KAAD, JBM), Harvard Medical School, Boston, Massachusetts; and Department of Neuro-Ophthalmology (VPD, KAAD, DMC), Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts. The authors report no conflicts of interest. Address correspondence to Vivian Paraskevi Douglas, MD, DVM, MBA, Department of Ophthalmology, Retina Service, Massachusetts Eye and Ear, 243 Charles Street, Boston, MA 02114; E-mail: [e-mail] Douglas et al: J Neuro-Ophthalmol 2022; 42: e430-e433 field testing revealed significantly worsened generalized depression in both eyes but more pronounced in the right eye (Fig. 1C, D). Ganglion cell layer analysis was characterized by bilateral loss of ganglion cell layer that was also correlated with the visual field defects (Fig. 1E). Fluorescein angiography showed late leakage on inferonasal macula consistent with a juxtapapillary choroidal neovascular membrane (CNVM) in the right eye (Fig. 2A, B). Swept Source OCT (Triton, Topcon) also illustrated both the juxtapapillary subretinal fluid inferotemporal to the disc and optic disc drusen (Fig. 2C). ONHD were noted in the left eye. Optical coherence tomography angiography (OCTA) identified a small juxtapapillary CNVM inferonasal to the macula correlating with the area of retinal hemorrhage and exudates (Fig. 2D). Given the presence of intraretinal fluid in the right eye along with the long-standing significant visual field loss, observation was not considered and monthly intravitreal injections of bevacizumab in the right eye were offered for the next 3 months. The patient was on close observation, and a year later and after receiving 3 injections, BCVA remained stable at 20/20 in both eyes. Subretinal fluid was resolved and only trace inferonasal thickening was observed on OCT. Blood sugar and blood pressure control were emphasized to the patient in every visit. Optic nerve head drusen are usually benign deposits that arise from the prelaminar portion of the optic nerve. Generally, ONHD are a common cause of pseudopapilledema and progressive visual field defects and can be rarely associated with visual-threatening complications, such as neovascular glaucoma, central retinal artery or vein occlusion, retinal hemorrhages, and development of CNVM (1–4). It has been suggested that ONHD can cause peripapillary circulation impairment and result to optic nerve ischemia and Bruch membrane rupture that could further trigger CNVM formation (5). Fluorescein angiography has been a useful diagnostic method for differentiating pseudopapilledema from pathologic ONH edema but also for the detection of the abovementioned complications. In the case of CNVM, leakage can be noted in early or late phase or in both phases especially in superficial ONHD when compared with buried ones (6). Indocyanine green angiography (ICGA) has been a useful tool for detecting classic or occult CNV with several advantages over FA including infrared fluorescence for better penetration through pigment and fluid (7). We do not perform ICGA routinely unless FA or OCTA are not sufficient to clarify the absence or presence of CNVM. As such, its evidence on both the FA and OCTA diminished the need for additional invasive testing. e431 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay FIG. 2. Fluorescein, OCT, and OCTA; (A, B) FA showed mid/late phase leakage and juxtapapillary choroidal neovascularization in the right eye; (C) optical coherence tomography (OCT) with intraretinal fluid inferonasal to the macula in the right eye and normal foveal contour in both eyes; and (D) optical coherence tomography angiography (OCTA) identified a small juxtapapillary choroidal neovascular membrane (CNVM) inferonasal to the macula in the right eye correlating with the area of retinal hemorrhage and exudates. Optical coherence tomography angiography is a dyeless imaging technique that allows for precise in vivo localization of the retinal and choroidal vasculatures, including the peripapillary network (8). When compared with FA, OCTA offers greater resolution of the microvasculature and depth specific information not possible with traditional angiography. Although FA can identify leakage (9), it provides no vertical location for the source of the leakage. Although observation has been considered a viable option for asymptomatic cases or when the macula is not involved, individualized treatment should be offered. Laser photocoagulation, photodynamic therapy with verteporfin, and surgery have been successfully used for treating CVNM secondary to ONHD (10–12). e432 Antivascular endothelial growth factor (anti-VEGF) agents have been used to treat CNVM that develop secondary to other causes such as wet age-related macular degeneration (AMD), myopic degeneration, and presumed ocular histoplasmosis syndrome (POHS) (13–15). Studies in children with ONHD and CNVM have supported the efficacy of anti-VEFG agents (16– 18). To the best of our knowledge, this is the first case where an anti-VEGF agent was successfully used in a middle-aged patient. Regarding prognosis, visual outcomes are highly variable and depend on the extent of macular involvement. Timely follow-up visits are important for early detection and management of complications. This report adds to the growing literature that OCTA can be a helpful noninvasive Douglas et al: J Neuro-Ophthalmol 2022; 42: e430-e433 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay tool for the clinical assessment of wide variety of neuroophthalmic lesions. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: V. P. Douglas, K. A. A. Douglas, J. B. Miller, and D. M. Cestari; b. Acquisition of data: V. P. Douglas and K. A. A. Douglas; c. Analysis and interpretation of data: V. P. Douglas, K. A. A. Douglas, J. B. Miller, and D. M. Cestari. Category 2: a. Drafting the manuscript: V. P. Douglas and K. A. A. Douglas; b. Revising it for intellectual content: V. P. Douglas, K. A. A. Douglas, J. B. Miller, and D. M. Cestari. Category 3: a. Final approval of the completed manuscript: V. P. Douglas, K. A. A. Douglas, J. B. Miller, and D. M. Cestari. REFERENCES 1. 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