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 Table of Contents  
Year : 2021  |  Volume : 11  |  Issue : 1  |  Page : 93-96

Optic disc edema due to peripapillary choroidal neovascularization

1 Lions International Eye Centre, Korle Bu Teaching Hospital, Accra, Ghana
2 Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
3 Department of Ophthalmology; Department of Neurology, Emory University School of Medicine, Atlanta, Georgia

Date of Submission31-Aug-2020
Date of Acceptance13-Oct-2020
Date of Web Publication20-Jan-2021

Correspondence Address:
Dr. Naa Naamuah Tagoe
Lions International Eye Centre, Korle Bu Teaching Hospital, Accra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjo.tjo_77_20

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A 35-year-old myopic woman developed right-eye optic disc edema with normal visual function. The presence of a subtle crescent-shaped peripapillary subretinal hemorrhage in addition to the disc edema raised concern for a peripapillary choroidal neovascular membrane, which was confirmed by enhanced depth optical coherence tomography.

Keywords: Choroidal neovascularization, myopia, optic nerve, optic nerve diseases, optical coherence tomography, papilledema

How to cite this article:
Tagoe NN, Sharma RA, Biousse V. Optic disc edema due to peripapillary choroidal neovascularization. Taiwan J Ophthalmol 2021;11:93-6

How to cite this URL:
Tagoe NN, Sharma RA, Biousse V. Optic disc edema due to peripapillary choroidal neovascularization. Taiwan J Ophthalmol [serial online] 2021 [cited 2022 Nov 26];11:93-6. Available from: https://www.e-tjo.org/text.asp?2021/11/1/93/307517

  Introduction Top

A peripapillary choroidal neovascular membrane (CNVM) is a classic cause of visual loss. Most CNVM occur in association with age-related macular degeneration (AMD) and other underlying inflammatory or degenerative chorioretinal disorders, but CNVM may also occur following ocular trauma or in association with high myopia.[1] We report a case of peripapillary CNVM causing optic disc edema in a young myopic woman.

  Case Report Top

A 35-year-old myopic woman was referred for assessment of right-eye optic disc edema. The patient had a past medical history of migraine which had been stable for many years; her past ocular history was significant only for anisometropic myopia (−3.50 right eye; −5.00 left eye). Several months prior, the patient was assessed by an outside eye care provider due to blurred vision in her right eye and was found to have right optic disc edema. Neuro-ophthalmology consultation was requested.

Her best-corrected Snellen visual acuity was 20/20 in both eyes. Color vision was full, as assessed by Ishihara pseudoisochromatic plates. There was no relative afferent pupillary defect. Her anterior segment examination was normal, with no findings of active or previous intraocular inflammation. Intraocular pressures were 22 mmHg in the right eye and 21 mmHg in the left eye. Dilated fundus examination showed elevation of the superonasal right optic nerve with mild swelling and a crescent-shaped peripapillary subretinal hemorrhage in the same area [Figure 1]a. The left optic nerve was tilted and elevated nasally without disc edema [Figure 1]b. The remainder of the ocular fundus examination in both eyes was normal.
Figure 1: Fundus photograph of the right eye (a) and the left eye (b)centered on the optic nerves show tilted optic discs with mild superonasal elevation in both eyes and mild swelling of the right optic nerve head. In the right eye, a peripapillary hemorrhage (black arrow) is seen along the superonasal margin of the disc. (c) Enhanced depth imaging-optical coherence tomography centered on the optic nerve shows a hyperreflective area (white outline) adjacent to the optic nerve head, suggesting a choroidal neovascular membrane. (d) Enhanced depth imaging-optical coherence tomography centered on the left optic nerve was normal.

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Optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL) confirmed increased peripapillary thickness of the right eye but did not demonstrate intra- or subretinal fluid near the optic nerve head. OCT-RNFL was normal in the left eye. OCT of the ganglion cell complex was normal in both eyes. B-scan ultrasonography showed elevation of the right optic nerve without hyperechoic signal to suggest optic disc drusen. Enhanced depth imaging-OCT showed a hyperreflective area in the peripapillary subretinal space [Figure 1]c, suggesting a peripapillary CNVM. Suggesting a peripapillary CNVM but normal in the left eye [Figure 1]d. Intravenous fluorescein angiography (IVFA) in the right eye showed subtle late leakage at the superonasal margin of the optic disc, consistent with disc edema [Figure 2]. No disc leakage was noted in the left eye.
Figure 2: Intravenous fluorescein angiography in the right eye in early (left image; 41 s) and late phases (right image; 5 min and 41 s), showing late leakage most apparent at the superonasal margin of the optic disc, indicating disc edema

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  Discussion Top

Choroidal neovascular membranes are new, abnormal blood vessels that originate from the choroidal circulation and enter the subretinal space via a break in Bruch's membrane; they may be associated with a number of underlying inflammatory or degenerative chorioretinal disorders or may occur following ocular trauma.[2]

Some CNVMs develop in the peripapillary area, within 1 disc diameter of the optic disc, as a result of disorders affecting the optic nerve head and adjacent chorioretinal areas.[2] Peripapillary CNVMs have a predilection for women[2],[3] and comprise 10% of all CNVMs.[2] They have been most commonly described in patients diagnosed with exudative or “wet” AMD[1],[4],[5] who are usually over 50 years of age.[1] Other ocular pathologies including optic disc drusen, optic pits, chronic papilledema, myopia, angioid streaks, ocular histoplasmosis syndrome, trauma, and uveitis may also cause peripapillary CNVMs.[1],[4] Idiopathic CNVM, or CNVM without an apparent underlying cause, represent up to 17% of cases in some series[4],[6],[7] and is more common in younger patients.[6] Because of their close relationship with the optic nerve head, peripapillary CNVM may present as unilateral optic disc edema, which may create diagnostic confusion and prompt unnecessary neurologic diagnostic investigations.

In our patient, the presence of a peripapillary crescent subretinal hemorrhage raised concern for a peripapillary CNVM, especially due to the patient's myopia. Sibony et al. described a case series of patients with peripapillary CNVM and subretinal hemorrhage and described average myopia of − 4.50 D in nine patients and as high as − 8.63 D.[3] Peripapillary CNVM is presumed to occur even more commonly in patients with pathological myopia (spherical correction of − 6.00 D or greater),[7],[8] presumably due to progressive elongation of the globe and the presence of abnormal choroidal vasculature and posterior staphyloma.[6] Our patient did not have a posterior staphyloma, but was noted to have tilting of her optic nerves, which was a finding also described by Sibony et al.[3]

As in our patient, peripapillary CNVM may cause mild optic disc edema. However, peripapillary CNVM may also be the result of severe chronic disc edema, particularly in patients with papilledema due to intracranial hypertension. Cases involving both of these clinical scenarios have been reported in the literature and are summarized in [Table 1]. The pathogenesis of peripapillary CNVM in the setting of chronic papilledema is presumed to be secondary to pressure deformity of the border of Bruch's membrane at the edge of the optic disc, resulting in discontinuity of the normal anatomic apposition of the chorioretinal layers. This anatomic dehiscence, hypoxia created by axonal swelling, as well as resultant vascular perfusion of tissues, are believed to promote angiogenesis with the resultant formation of subretinal neovascular membranes.[9]
Table 1: Reported cases of chronic disc edema causing a secondary peripapillary choroidal neovascular membrane and of peripapillary choroidal neovascular membrane causing secondary disc edema

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Recognition of peripapillary CNVM as a cause of mild disc edema is important.[18] Ancillary ophthalmic investigations including optic disc photographs, B-scan ultrasonography to screen for optic disc drusen,[18] OCT, and IVFA are helpful in making the diagnosis.[4],[18] Peripapillary CNVM are often observed when asymptomatic and, similar to other CNVM, may be treated with intravitreal anti-vascular endothelial growth factor if symptomatic or enlarging.[2],[12],[13],[14],[19] Retinal laser,[2],[7] photodynamic therapy,[2],[7]or vitreoretinal surgery are alternative treatment options but are rarely used.[19]

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Conflicts of interest

The authors declare that there are no conflicts of interests of this paper.

  References Top

Alharthi RA, Almalki AM, Alotibi FA. Case of idiopathic peripapillary subretinal neovascular membrane in an otherwise healthy young male: A case report. Egypt J Hosp Med 2018;70:1421-3.  Back to cited text no. 1
Jutley G, Jutley G, Tah V, Lindfield D, Menon G. Treating peripapillary choroidal neovascular membranes: A review of the evidence. Eye (Lond) 2011;25:675-81.  Back to cited text no. 2
Sibony P, Fourman S, Honkanen R, El Baba F. Asymptomatic peripapillary subretinal hemorrhage: A study of 10 cases. J Neuro Ophthalmol 2008;28:114-9.  Back to cited text no. 3
Browning DJ, Fraser CM. Ocular conditions associated with peripapillary subretinal neovascularization, their relative frequencies, and associated outcomes. Ophthalmology 2005;112:1054-61.  Back to cited text no. 4
Xiaoyan D, Mrinali P, Chan CC. Molecular pathology of AMD. Prog Retin Eye Res 2009;28:1-18.  Back to cited text no. 5
Cheung CM, Arnold JJ, Holz FG, Park KH, Lai TY, Larsen M, et al. Myopic choroidal neovascularization: Review, guidance, and consensus statement on management. Ophthalmology 2017;124:1690-711.  Back to cited text no. 6
Cohen SY, Laroche A, Leguen Y, Soubrane G, Coscas GJ. Etiology of choroidal neovascularization in young patients. Ophthalmology 1996;103:1241-4.  Back to cited text no. 7
Moriyama M, Ohno-Matsui K, Futagami S, Yoshida T, Hayashi K, Shimada N, et al. Morphology and long-term changes of choroidal vascular structure in highly myopic eyes with and without posterior staphyloma. Ophthalmology 2007;114:1755-63.  Back to cited text no. 8
Morse PH, Leveille AS, Antel JP, Burch JV. Bilateral juxtapapillary subretinal neovascularization associated with pseudotumor cerebri. Am J Ophthalmol 1981;91:312-7.  Back to cited text no. 9
Wendel L, Lee AG, Boldt HC, Kardon RH, Wall M. Subretinal neovascular membrane in idiopathic intracranial hypertension. Am J Ophthalmol 2006;141:573-4.  Back to cited text no. 10
Sathornsumetee B, Webb A, Hill DL, Newman NJ, Biousse V. Subretinal hemorrhage from a peripapillary choroidal neovascular membrane in papilledema caused by idiopathic intracranial hypertension. J Neuro Ophthalmol 2006;26:197-9.  Back to cited text no. 11
Jamerson SC, Arunagiri G, Ellis BD, Leys MJ. Intravitreal bevacizumab for the treatment of choroidal neovascularization secondary to pseudotumor cerebri. Int Ophthalmol 2009;29:183-5.  Back to cited text no. 12
Belliveau MJ, Xing L, Almeida DR, Gale JS, Ten Hove MW. Peripapillary choroidal neovascular membrane in a teenage boy: Presenting feature of idiopathic intracranial hypertension and resolution with intravitreal bevacizumab. J Neuro Ophthalmol 2013;33:48-50.  Back to cited text no. 13
Lee IJ, Maccheron LJ, Kwan AS. Intravitreal bevacizumab in the treatment of peripapillary choroidal neovascular membrane secondary to idiopathic intracranial hypertension. J Neuro Ophthalmol 2013;33:155-7.  Back to cited text no. 14
Kumar N, Tigari B, Dogra M, Singh R. Successful management of peripapillary choroidal neovascular membrane secondary to idiopathic intracranial hypertension with intravitreal ranibizumab. Indian J Ophthalmol 2018;66:1358-60.  Back to cited text no. 15
[PUBMED]  [Full text]  
Ozgonul C, Moinuddin O, Munie M, Lee MS, Bhatti MT, Landau K, et al. Management of peripapillary choroidal neovascular membrane in patients with idiopathic intracranial hypertension. J Neuro Ophthalmol 2019;39:451-7.  Back to cited text no. 16
Hawy E, Sharma RA, Peragallo JH, Dattilo M, Newman NJ, Biousse V. Isolated unilateral paucisymptomatic optic nerve head edema. J Neuro Ophthalmol. [In press].  Back to cited text no. 17
Rebolleda G, Kawasaki A, de Juan V, Oblanca N, Muñoz-Negrete FJ. Optical coherence tomography to differentiate papilledema from pseudopapilledema. Curr Neurol Neurosci Rep 2017;17:74.  Back to cited text no. 18
Gelisken F, Szurman P, Bartz-Schmidt KU, Aisenbrey S. Surgical treatment of peripapillary choroidal neovascularisation. Br J Ophthalmol 2007;91:1027-30.  Back to cited text no. 19


  [Figure 1], [Figure 2]

  [Table 1]

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