Multimodal Imaging in a Case of Peripapillary Choroidal Neovascular Membrane Associated With Idiopathic Intracranial Hypertension

Optical coherence tomography angiography is one of the latest noninvasive imaging modalities for visualizing the vasculature of retina and choroid. We describe its application in the diagnosis, treatment, and monitoring of a patient with peripapillary choroidal neovascular membrane in the setting of idiopathic intracranial hypertension, who responded well to a course of ranibizumab intravitreal injections.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Multimodal Imaging in a Case of Peripapillary Choroidal Neovascular Membrane Associated With Idiopathic Intracranial Hypertension Chinedu N. Igwe, MBBS, Paul Nderitu, MBChB, Stefanos Eframidis, MD, Haralabos Eleftheriadis, MD, Eoin O’Sullivan, MD Downloaded from http://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 05/04/2022 FIG. 1. There is a peripapillary superficial and deep hemorrhage in the right eye (A). In the late phase of fluorescein angiogram (B), there is leakage from peripapillary choroidal neovascular membrane and optic disc as well as areas of hypofluorescence due to masking effect of subretinal and intraretinal haemorrhages. Optical coherence tomography shows hyperreflective subretinal lesion associated with subretinal fluid (C). Abstract: Optical coherence tomography angiography is one of the latest noninvasive imaging modalities for visualizing the vasculature of retina and choroid. We describe its application in the diagnosis, treatment, and monitoring of a patient with peripapillary choroidal neovascular membrane in the setting of idiopathic intracranial hypertension, who responded well to a course of ranibizumab intravitreal injections. Journal of Neuro-Ophthalmology 2021;41:e116–118 doi: 10.1097/WNO.0000000000000948 © 2020 by North American Neuro-Ophthalmology Society A n 18-year-old white woman presented with 3-week history of worsening headache and bilateral tran- Department of Ophthalmology, Kings College Hospital, London, United Kingdom. The authors report no conflicts of interest. Address correspondence to Chinedu Igwe, MBBS, Kings College Hospital, Denmark Hill, Brixton, London SE5 9RS, United Kingdom; E-mail: [e-mail]. e116 sient visual loss provoked by bending down. Visual acuity measured 6/6 in the right eye and 6/9 in the left eye. No afferent pupillary defect or color vision deficit was demonstrated on examining. Bilateral optic disc swelling was detected on fundoscopy. Subsequent computerized tomography (CT) of the head showed features of idiopathic intracranial hypertension (IIH); however, CT venography was normal. Lumbar puncture opening pressure was 34 cmH2o then reduced to 22 cmH2o resulting in therapeutic relief of the headaches. Cerebrospinal fluid composition was normal. A diagnosis of IIH was made. After this acute episode, she was seen in clinic 6 months later. She reported painless rapidly progressive deterioration in vision of the right eye over a month. The headaches were less frequent with no associated episodes of visual obscuration. She had lost 13 kg of weight on advice of her physician and body mass index fell to 40 kmg/m2. Her visual acuity was 6/24-1 and 6/5-1 in the right and left eyes, respectively. Right eye red Igwe et al: J Neuro-Ophthalmol 2021; 41: e116-e118 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Optical coherence tomography angiography of the right eye showing choroidal neovascular membrane within deep retina (A) and a feeder vessel from the right optic disc’s margin connecting to choroidal neovascular membrane (B). desaturation was noted. Humphrey’s visual 24-2 field showed mild central loss in the right eye; however, the left eye had no field loss. Fundoscopy revealed bilateral chronic mild disc swelling; however, in the right eye, a deep juxtapapillary subretinal and intraretinal hemorrhage temporal to the optic disc, extending into macula, was observed (Fig. 1A). Fundus autofluorescence and ultrasonography ruled out the presence of optic nerve head drusen. A diagnosis of peripapillary choroidal neovascular membrane (PCNVM) was made after the emergence of its characteristic findings on fundus fluorescein angiography (FFA) (Fig. 1B) and spectral domain optical coherence tomography (SD-OCT) (Heidelberg, Germany) (Fig. 1C). OCT angiography (OCTA) identified a deep feeder vessel at disc margin anastomosing with PCNVM (Fig. 2A, B). In the presence of symptomatic PCNVM, a course of ranibizumab intravitreal injections was commenced. At 2-month visit after 4 monthly intravitreal injections of ranibizumab, the right vision was 6/ 9, with no signs of leakage on SD-OCT and involution of PCNVM but persistence of the connecting feeder vessel on OCTA (Fig. 3A–E). PCNVM is a rare manifestation of IIH and is an infrequent cause of sight loss in such patients, with a reported incidence of 0.53% (1,2). It is postulated that the pressure effect of disc swelling disrupts and deforms adjacent chorioretinal layers causing a breach in Bruch’s membrane. This accompanied by hypoxia induced by disruption of axoplasmic flow secondary to disc swelling Igwe et al: J Neuro-Ophthalmol 2021; 41: e116-e118 promotes angiogenesis and subsequent ingrowth of vessels from choriocapillaris resulting in PCNVM (3,4). The findings from our case lend support to this presumed pathogenesis because OCTA revealed a feeder vessel of the PCNVM emanating from the choroidal circulation. This feeder vessel is delineated clearly on OCTA but not on FFA because its presence is obscured by the diffuse leakage of fluorescein in the area of the PCNVM and the choroid. Thus, this finding would have been missed without the adjunct use of OCTA. Although there is no evidence-based guidance for treatment of PCNVMs secondary to IIH, treatments to lower intracranial pressure and local therapies including intravitreal injections of anti–vascular endothelial growth factor agents, focal laser, photodynamic therapy, and submacular surgery have been described (1,5–7). Advances in OCT technology has led to expansion of its use in neuro-ophthalmology particularly the status of retinal nerve fiber layer thickness in determining papilloedema. The role of OCTA in neuro-ophthalmology is slowly developing; however, to the best of our knowledge (8), OCTA identification of PCNVM in IIH patients has not been described. We have demonstrated the clinical application of OCTA not only in the diagnosis but also in the follow-up and the decision-to-treat PCNVM in IIH patients because it provides high-resolution, noninvasive visualization of such membranes in the setting of optic disc swelling. In our case, OCTA has also revealed a very significant reduction in the e117 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 3. This series of optical coherence tomography angiography (A–D) of the right eye shows the resolution of choroidal neovascular membrane after 4 injections of ranibizumab. Optical coherence tomography demonstrates improved foveal contour and resorption of subretinal fluid with minimum and stable intraretinal cysts overlying an area of subretinal fibrosis (E). capillary density of IIH-associated PCNVM with 4 ranibizumab injections (Fig. 3A–D). STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: C. N. Igwe, E. O’Sullivan, and H. Eleftheriadis; b. Acquisition of data: C. N. Igwe, P. Nderitu, and S. Eframidis; c. Analysis and interpretation of data: C. N. Igwe, P. Nderitu, H. Eleftheriadis, and E. O’Sullivan. Category 2: a. Drafting the manuscript: C. N. Igwe, P. Nderitu, H. Eleftheriadis, and E. O’Sullivan; b. Revising it for intellectual content: C. N. Igwe, H. Eleftheriadis, and E. O’Sullivan. Category 3: a. Final approval of the completed manuscript: C. N. Igwe, H. 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