The Open Ophthalmology Journal




ISSN: 1874-3641 ― Volume 13, 2019
CASE REPORT

Polypoidal Choroidal Vasculopathy in Highly Myopic Eyes with Elongated Axial Length



Gregg T. Kokame1, 5, *, Elysse S. Tom5, Jessica G. Shantha2, 4, 6, Kyle N. Kaneko2, 4
1 Division of Ophthalmology, Department of Surgery, University of Hawaii School of Medicine, 651 IIalo St. Honolulu, HI, 96813, USA
2 The Retina Center at Pali Momi, 98-1079 Moanalua Road, Suite 470, Aiea, HI 96701, USA
3 Retina Consultants of Hawaii, 98-1079 Moanalua Road, Suite 470, Aiea, HI 96701, USA
4 Hawaii Macula and Retina Institute, 98-1079 Moanalua Road, Suite 410, Aiea, HI 96701, USA
5 John A. Burns School of Medicine, University of Hawaii School of Medicine, 651 Ilalo St, Honolulu, HI 96813, USA
6 Department of Ophthalmology, Emory University, Emory Eye Center, Atlanta, Georgia

Abstract

Purpose:

To retrospectively review the prevalence of myopia and elongated axial length in eyes with polypoidal choroidal vasculopathy (PCV) and to evaluate treatment response of PCV in highly myopic eyes. PCV has rarely been reported in myopic eyes.

Methods:

A retrospective review of all eyes diagnosed with PCV at the clinics of Retina Consultants of Hawaii and the Hawaii Macula and Retina Institute was performed between February of 2007 and April of 2017 to evaluate for eyes with significant myopia and elongated axial length.

Results:

A total of 282 eyes were diagnosed with PCV by ICG angiography. There were 144 males (59%) and 99 females (41%). 204 patients had unilateral PCV and 39 patients had bilateral PCV. A total of 3 patients with PCV had significant myopia less than -6 diopters or confirmed elongated axial length. One of these patients had bilateral PCV so there were 4 eyes noted with significant myopia and elongated axial length out of 282 eyes with PCV (1.4%). All 3 patients were Asian and presented with active leakage or bleeding related to PCV diagnosed on indocyanine green angiography and optical coherence tomography. Treatments typically used to treat PCV including intravitreal antiangiogenic medications and photodynamic therapy were utilized.

Conclusion and Importance:

High myopia is rare in eyes diagnosed with PCV, even though choroidal neovascularization is a common cause of vision loss in myopic macular degeneration. However, even in highly myopic eyes, PCV may show signs of resistance to antiangiogenic medications.

Keywords: Polypoidal choroidal vasculopathy, High Myopia, Exudative macular degeneration, Photodynamic Therapy, Indocyanine Green Angiography, Bevacizumab, Aflibercept, Ranibizumab.


Article Information


Identifiers and Pagination:

Year: 2017
Volume: 11
First Page: 326
Last Page: 333
Publisher Id: TOOPHTJ-11-326
DOI: 10.2174/1874364101711010326

Article History:

Received Date: 21/07/2017
Revision Received Date: 19/10/2017
Acceptance Date: 02/11/2017
Electronic publication date: 21/11/2017
Collection year: 2017

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© 2017 Kokame et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


* Address correspondence to this author at the Hawaii Macula and Retina Institute, 98-1079 Moanalua Road, Suite 470, Aiea, Hawaii, 96701, USA; Tel: 1-808-4878928; Fax: 1-808-4873699; E-mail retinahi@aol.com




1. INTRODUCTION

Polypoidal Choroidal Vasculopathy (PCV) is a variant of neovascular age related macular degeneration. The subretinal neovascularization is type I according to the classification system of Gass [1Gass JD. Stereoscopic atlas of macular diseases: Diagnosis and treatment 4th ed. 1997; 26-30.]. Type I subretinal neovascularization, including PCV, is due to abnormal blood vessels growing below the retinal pigment epithelium and above Bruch’s membrane [2Kokame GT. Prospective evaluation of subretinal vessel location in polypoidal choroidal vasculopathy (PCV) and response of hemorrhagic and exudative PCV to high-dose antiangiogenic therapy (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc 2014; 112: 74-93.]. The subretinal neovascularization is made up of polypoidal vascular dilations with or without a Branching Vascular Network (BVN) [2Kokame GT. Prospective evaluation of subretinal vessel location in polypoidal choroidal vasculopathy (PCV) and response of hemorrhagic and exudative PCV to high-dose antiangiogenic therapy (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc 2014; 112: 74-93.].

Choroidal Neovascularization (CNV) in highly myopic eyes is not uncommon, but is usually related to myopic macular degeneration [3El Matri L, Chebil A, Kort F. Current and emerging treatment options for myopic choroidal neovascularization. Clin Ophthalmol 2015; 9: 733-44.
[http://dx.doi.org/10.2147/OPTH.S49437] [PMID: 25987831]
]. In fact PCV has been shown to be an extremely rare cause of choroidal neovascularization in myopic eyes. In a large study of CNV in myopic eyes looking specifically with indocyanine green angiography (ICG) for PCV, Kang and Koh did not find even one case of PCV in a series of 297 eyes with treatment-naïve myopic choroidal neovascularization [4Kang HM, Koh HJ. Lack of polypoidal lesions in patients with myopic choroidal neovascularization as evaluated by indocyanine green angiography. Am J Ophthalmol 2014; 157(2): 378-383.e1.
[http://dx.doi.org/10.1016/j.ajo.2013.09.018] [PMID: 24315295]
]. Although high myopia is a significant cause of visual impairment worldwide, it lacks a standard definition [5Ohno-Matsui K. What is the fundamental nature of pathologic myopia? Retina 2017; 37(6): 1043-8.
[http://dx.doi.org/10.1097/IAE.0000000000001348] [PMID: 27755375]
]. Common previous markers have included refractive error < -6.0 D or -8.0 D, or elongated axial length [5Ohno-Matsui K. What is the fundamental nature of pathologic myopia? Retina 2017; 37(6): 1043-8.
[http://dx.doi.org/10.1097/IAE.0000000000001348] [PMID: 27755375]
]. Elongated axial length is an endophenotype of myopia and the primary determinant of myopia [6Meng W, Butterworth J, Malecaze F, Calvas P. Axial length of myopia: A review of current research. Ophthalmologica 2011; 225(3): 127-34.
[http://dx.doi.org/10.1159/000317072] [PMID: 20948239]
]. In a recent study of highly myopic eyes defined as -6 D or greater, axial length was noted to be on average 26.56 mm and to range from 24.95 mm to 38.86 mm as compared to an average of 23.71 mm in normal controls [7Wang S, Wang Y, Gao X, Qian N, Zhuo Y. Choroidal thickness and high myopia: A cross-sectional study and meta-analysis. BMC Ophthalmol 2015; 15: 70.
[http://dx.doi.org/10.1186/s12886-015-0059-2] [PMID: 26138613]
]. In this retrospective study of PCV, herein we report on the clinical characteristics of PCV noted on both Optical Coherence Tomography (OCT) and Indocyanine Green Angiography (ICGA) in highly myopic eyes based on a refractive error of -6 diopters or greater with corresponding elongated axial length.

2. METHODS

This study involved a retrospective review of all patients seen with a diagnosis of Polypoidal Choroidal Vasculopathy (PCV) diagnosed by ICG angiography between February of 2007 and April of 2017 at the clinics of Retina Consultants of Hawaii and the Hawaii Macula and Retina Institute. Since PCV has been rarely reported in eyes with significant myopia [3El Matri L, Chebil A, Kort F. Current and emerging treatment options for myopic choroidal neovascularization. Clin Ophthalmol 2015; 9: 733-44.
[http://dx.doi.org/10.2147/OPTH.S49437] [PMID: 25987831]
], and because high myopia lacks a standard universally agreed upon definition, eyes were included in this study if myopia was < -6.0 diopters in phakic eyes or had a confirmed elongated axial length of 24.95 mm or more based on the study by Wang and colleagues [7Wang S, Wang Y, Gao X, Qian N, Zhuo Y. Choroidal thickness and high myopia: A cross-sectional study and meta-analysis. BMC Ophthalmol 2015; 15: 70.
[http://dx.doi.org/10.1186/s12886-015-0059-2] [PMID: 26138613]
], which correlated to findings in eyes with < -6.0 D. All eyes included in this high myopic category had typical funduscopic features of high myopia including peripapillary atrophy or myopic crescent. This retrospective study was deemed exempt from Institutional Review Board (IRB) approval by the Western Institutional Review Board (#1-1019457-1), and the study was conducted in accordance with the declarations of Helsinki.

Patient data was evaluated to include the age and sex of patients, the refractive error, the funduscopic findings, the optical coherence tomography findings, the Fluorescein Angiogram (FA) findings and the indocyanine green angiography used to confirm the diagnosis of PCV. Axial length data were obtained retrospectively in all myopic eyes to confirm an elongated axial length usually associated with eyes with high myopia of < -6 diopters or more.

3. RESULTS AND PATIENTS

Between February of 2007 and April of 2017 at the clinics of Retina Consultants of Hawaii and the Hawaii Macula and Retina Institute there were 282 eyes diagnosed with PCV by ICGA on retrospective review of clinic records. 204 patients had unilateral PCV, and 39 patients had bilateral PCV, so in total there were 282 eyes of 243 patients with a diagnosis of PCV. The average age was 77.6 years with a range in age from 47 to 102 years. There were 144 males (59%) and 99 females (41%). Elongated axial length and high myopia was documented in 4 of the 282 eyes (1.4%) with a diagnosis of PCV by ICGA. Two of the myopic patients had unilateral PCV and one myopic patient had bilateral PCV. Herein are the three patients described with PCV in highly myopic eyes.

3.1. Patient 1

A 73-year-old Chinese female presented with vision loss in her right eye with thin subretinal hemorrhage. The anterior segment exam of the right eye showed a well centered posterior chamber implant. Funduscopic examination showed a thin subretinal hemorrhage in the macula with superior edema with cystic changes (Fig. 1A). Fluorescein angiography showed a blocking defect from the blood and occult subretinal leakage centrally without any evidence of retinal vascular leakage (Figs. 1B-D). Her axial length was 25.21 mm by ultrasound biometry confirming an elongated myopic eye. Her past medical history included non-insulin dependent diabetes mellitus and quiescent proliferative diabetic retinopathy with previous macular laser and panretinal laser photocoagulation (PRP). Her vision in the right eye was as good as 20/25 prior to a vitreous hemorrhage, which required vitrectomy, removal of epiretinal membranes and endolaser PRP in 2005. She presented with subretinal hemorrhage in the right eye five years following the vitrectomy. ICG angiography showed a central hyperfluorescent branching vascular network with multiple hyperfluorescent polyps classic for PCV (Fig. 1E).

Fig. (1)
(A) Fundus photograph of the right eye of patient 1. Note the small subretinal hemorrhage within the macula and peripheral panretinal laser photocoagulation for proliferative diabetic retinopathy. (B) Fluorescein angiography shows leakage in the inferotemporal fovea. (C, D) ICG angiogram showing PCV complex with nasal polyp and horizontal line showing ICG location for optical coherence tomography line scan. Note the characteristic inverted U-shaped polyp with a height of 171 um, the adjacent branching vascular network (shallow elevation of the RPE), and diffuse intraretinal edema with serous detachment. (E) ICG angiography shows multiple hyperfluorescent polyps with associated branching vascular network.


On OCT there were multiple inverted U-shaped polyps with diffuse intraretinal edema (Fig. 1D). Her subfoveal choroidal thickness was 170 um, which is normal and not thick as often seen in PCV. Initial treatment for the subretinal neovascularization was performed with four intravitreal bevacizumab injections and two ranibizumab injections with a poor response with persistent macular edema. Two photodynamic therapy treatments combined with injections of intravitreal bevacizumab 1.25 mg and dexamethasone 400 ug were performed with resolution of leakage and macular edema with a final visual acuity of 1/200. Polypoidal choroidal vasculopathy was not present in the left eye on ICG angiography.

3.2. Patient 2

A 66-year-old Japanese female presented with blurred vision in the left eye for six months and a visual acuity of 20/80. Her past ocular history in the left eye included prior vitrectomy with membrane peeling, and cataract extraction. Anterior segment examination was significant for a well-centered posterior chamber IOL with central capsulotomy. Funduscopic exam showed nasal RPE thickening and edema without subretinal hemorrhage (Fig. 2A). FA showed nasal focal leakage in the area of edema (Fig. 2B). ICG angiography showed an inferonasal hyperfluorescent polyp with a surrounding hypofluorescent ring (Figs. 2C, E). Optical coherence tomography showed macular cystic changes overlying a nasal inverted U-shaped elevation of the retinal pigment epithelium typical for a polyp (Fig. 2D). The choroidal thickness was thin typical of highly myopic eyes (subfoveal choroidal thickness of 80um) (Fig. 2F), and similar to the fellow eye. Her axial length was 27.95 mm by ultrasound biometry consistent with an elongated highly myopic eye. She received seven intravitreal bevacizumab injections with improvement in vision to 20/25 and resolution of edema, which was then maintained without treatment for eight months. There was no PCV in the right eye on ICG angiography.

Fig. (2)
(A) Fundus photograph of the left eye of patient 2 shows nasal retinal thickening. (B) Fluorescein angiography shows leakage in the area of nasal retinal thickening (C, D) ICG angiogram showing central hyperfluorescent lesion with hypofluorescent border and a horizontal line showing ICG location for optical coherence tomography line scan. Note the characteristic inverted U-shaped polyp overlying macular edema and cystic change. (E) Indocyanine green angiography shows a hyperfluorescent polyp with a surrounding hypofluorescent halo. (F) Choroidal thickness measurement on OCT confirming thin choroid (80 microns) underneath the fovea.


Fig. (3)
(A) Fundus photograph of the right eye of patient 3. Note the myopic fundus with peripapillary RPE atrophy and laquer cracks and serous macular detachment. (B, C) ICG angiography showing PCV complex with polypoidal dilations and branching vascular network. Note the inverted U-shaped polyp and adjacent branching vascular network with shallow elevation of the RPE. There is overlying serous detachment. (D) ICG angiogram showing magnified view of PCV complex with branching vascular network and terminal polyps. (E, F) ICG angiogram of PCV complex with horizontal line scan through polyps inferiorly outside the area of leakage. Note the multiple inverted U-shaped polyps and loss of the photoreceptor layer above the polyps.


3.3. Patient 3

A 75-year old Chinese male presented with blurred vision in the right eye for 6 months and a visual acuity of 20/50. Visual acuity in the left eye was 20/20. Refraction was OD: -9.50+0.75 x 005 OS: -9.75+0.75 x 173. Anterior segment examination was significant for a moderate nuclear sclerotic cataract in both eyes. Funduscopic examination showed a myopic fundus with peripapillary RPE atrophy and laquer cracks in both eyes. There was subretinal fluid in the right eye (Fig. 3A). Fluorescein angiography showed occult leakage temporally in the right eye, no leakage in the left eye, and hyperfluorescent laquer cracks in both eyes. ICG angiography showed inferotemporal polyps with a branching vascular network in the right eye (Figs. 3B, D, E) and a PCV complex in the left eye. OCT showed subretinal fluid centrally (Fig. 3C) and typical inverted U-shaped polyps (Figs. 3C, F). The choroidal thickness on OCT was 310 um in the right eye and 282 um in the left eye. The axial length was 28.95 mm in the right eye and 28.38 mm in the left eye by ultrasound biometry. He initially received 4 sequential intravitreal bevacizumab injections in the right eye, but had persistent subretinal fluid, and was treated with intravitreal aflibercept. Subretinal fluid decreased but persisted, and a repeat ICG angiogram showed persistent polyps and branching vascular network. After 28 treatments with intravitreal aflibercept there was persistent shallow subretinal fluid, but visual acuity remained 20/50. The PCV complex in the fellow left eye was not actively leaking and did not require treatment.

4. DISCUSSION

Polypoidal choroidal vasculopathy is rare in eyes with high myopia, but in this retrospective study there were definite cases of PCV in eyes with elongated axial length and high myopia making up 1.4% of the cases diagnosed with PCV by ICGA over a 10-year period. These myopic eyes with PCV received the usual treatments provided for exudative macular degeneration, including initial treatment with antiangiogenic drugs. For patient 2 the leakage responded well to intravitreal bevacizumab, and the treatments were eventually discontinued without recurrence. This patient also had a very thin choroid with a subfoveal choroidal thickness of just 80 um (Fig. 2F). High myopic eyes tend to have thinner choroid, which increases with elongated axial length [7Wang S, Wang Y, Gao X, Qian N, Zhuo Y. Choroidal thickness and high myopia: A cross-sectional study and meta-analysis. BMC Ophthalmol 2015; 15: 70.
[http://dx.doi.org/10.1186/s12886-015-0059-2] [PMID: 26138613]
, 8Flores-Moreno I, Lugo F, Duker JS, Ruiz-Moreno JM. The relationship between axial length and choroidal thickness in eyes with high myopia. Am J Ophthalmol 2013; 155(2): 314-319.e1. [Choroidal thickness decreased with elongated axial length.].
[http://dx.doi.org/10.1016/j.ajo.2012.07.015] [PMID: 23036569]
]. The normal mean subfoveal choroidal thickness is 272 um with a range of 191 um to 353 um [9Manjunath V, Taha M, Fujimoto JG, Duker JS. Choroidal thickness in normal eyes measured using Cirrus HD optical coherence tomography. Am J Ophthalmol 2010; 150(3): 325-329.e1.
[http://dx.doi.org/10.1016/j.ajo.2010.04.018] [PMID: 20591395]
], so the choroidal thickness in patient 2 was significantly thin. As can be seen with exudative myopic macular degeneration, which is often associated with a thin choroid, antiangiogenic medications can sometimes be tapered off without recurrence of leakage or bleeding.

PCV has a higher risk of resistance to antiangiogenic drugs [10Cho M, Barbazetto IA, Freund KB. Refractory neovascular age-related macular degeneration secondary to polypoidal choroidal vasculopathy. Am J Ophthalmol 2009; 148(1): 70-8.e1.
[http://dx.doi.org/10.1016/j.ajo.2009.02.012] [PMID: 19403115]
, 11Stangos AN, Gandhi JS, Nair-Sahni J, Heimann H, Pournaras CJ, Harding SP. Polypoidal choroidal vasculopathy masquerading as neovascular age-related macular degeneration refractory to ranibizumab. Am J Ophthalmol 2010; 150(5): 666-73.
[http://dx.doi.org/10.1016/j.ajo.2010.05.035] [PMID: 20719300]
], but it is uncertain whether or not this resistance is a potential problem in PCV in highly myopic eyes, which have a thinner choroid and an elongated axial length. In patient 1 there was a poor anatomic response to treatments with intravitreal bevacizumab and ranibizumab, so photodynamic therapy was required to resolve the macular edema and active leakage. For Patient 3 there was persistent subretinal fluid even after 4 injections of intravitreal bevacizumab and 28 injections of intravitreal aflibercept at frequent and consistent intervals, indicating resistance to antiangiogenic medications with a poor anatomic response, although vision has been maintained. These cases demonstrate that there may be an increased risk of resistance to antiangiogenic drugs in PCV even in eyes with high myopia and elongated axial length. Because of the decreased response to therapy noted in eyes with PCV, diagnostic testing to rule out the diagnosis of PCV, including the gold standard of ICG angiography [12Kokame GT. Polypoidal choroidal vasculopathy an important diagnosis to make with therapeutic implications. Retina 2012; 32(8): 1446-8.
[http://dx.doi.org/10.1097/IAE.0b013e3182695bf8] [PMID: 22922845]
], should be considered in exudative macular degeneration in myopic eyes, especially when there is persistent disease activity after treatment.

Axial length is the main determinant of refractive error and high myopia. Elongated axial length is an endophenotype of myopia, and all eyes in this series had elongated axial lengths ranging from 25.21 to 28.95 mm (average= 27.6 mm), which are above the lower range (24.95 mm) noted in eyes with high myopia of less than -6 diopters [7Wang S, Wang Y, Gao X, Qian N, Zhuo Y. Choroidal thickness and high myopia: A cross-sectional study and meta-analysis. BMC Ophthalmol 2015; 15: 70.
[http://dx.doi.org/10.1186/s12886-015-0059-2] [PMID: 26138613]
]. The polypoidal choroidal vasculopathy was typical with the subretinal neovascularization occurring between the retinal pigment epithelium and Bruch’s membrane on OCT [2Kokame GT. Prospective evaluation of subretinal vessel location in polypoidal choroidal vasculopathy (PCV) and response of hemorrhagic and exudative PCV to high-dose antiangiogenic therapy (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc 2014; 112: 74-93.] and visible as hyperfluorescent polypoidal dilations with a hypofluorescent halo on ICG angiography. In a retrospective cross-sectional study in South Korea, evaluating myopic CNV in 297 eyes with ICG angiography, none of the patients had polypoidal lesions [4Kang HM, Koh HJ. Lack of polypoidal lesions in patients with myopic choroidal neovascularization as evaluated by indocyanine green angiography. Am J Ophthalmol 2014; 157(2): 378-383.e1.
[http://dx.doi.org/10.1016/j.ajo.2013.09.018] [PMID: 24315295]
]. These studies support that PCV does not develop commonly in highly myopic eyes. Even though PCV is more common in Asian populations [13Ciardella AP, Donsoff IM, Huang SJ, Costa DL, Yannuzzi LA. Polypoidal choroidal vasculopathy. Surv Ophthalmol 2004; 49(1): 25-37.
[http://dx.doi.org/10.1016/j.survophthal.2003.10.007] [PMID: 14711438]
], and all of the PCV cases in these myopic eyes were in East Asian patients, and even though high myopia is more prevalent in Asia [14Wong TY, Foster PJ, Hee J, et al. Prevalence and risk factors for refractive errors in adult Chinese in Singapore. Invest Ophthalmol Vis Sci 2000; 41(9): 2486-94.
[PMID: 10937558]
], PCV is only rarely seen in highly myopic eyes. Most eyes with PCV have a thick choroid [15Sasahara M, Tsujikawa A, Musashi K, et al. Polypoidal choroidal vasculopathy with choroidal vascular hyperpermeability. Am J Ophthalmol 2006; 142(4): 601-7.
[http://dx.doi.org/10.1016/j.ajo.2006.05.051] [PMID: 17011852]
]. In Patients 1 and 2, as well as in other highly myopic eyes, the choroid is very thin [7Wang S, Wang Y, Gao X, Qian N, Zhuo Y. Choroidal thickness and high myopia: A cross-sectional study and meta-analysis. BMC Ophthalmol 2015; 15: 70.
[http://dx.doi.org/10.1186/s12886-015-0059-2] [PMID: 26138613]
, 8Flores-Moreno I, Lugo F, Duker JS, Ruiz-Moreno JM. The relationship between axial length and choroidal thickness in eyes with high myopia. Am J Ophthalmol 2013; 155(2): 314-319.e1. [Choroidal thickness decreased with elongated axial length.].
[http://dx.doi.org/10.1016/j.ajo.2012.07.015] [PMID: 23036569]
], although patient 3 had choroidal thickness well within the normal range. We speculate that the thin choroid in most myopic eyes could be a factor in the low prevalence of PCV in highly myopic eyes.

In a previous multicenter study by Mauget-Faysse et al. [16Mauget-Faÿsse M, Cornut PL, Quaranta El-Maftouhi M, Leys A. Polypoidal choroidal vasculopathy in tilted disk syndrome and high myopia with staphyloma. Am J Ophthalmol 2006; 142(6): 970-5.
[http://dx.doi.org/10.1016/j.ajo.2006.06.063] [PMID: 17046703]
] PCV was noted to be associated with the tilted disc syndrome or high myopia with a posterior staphyloma in 8 eyes. In 5 of the 8 eyes PCV was documented on ICG angiography in highly myopic eyes, but none of these cases had a documented elongated axial length. The authors previously postulated that PCV developed at the border of hypoplastic and normal choroid at the edge of a posterior staphyloma, but this was not noted in our cases, which had high myopia with an elongated axial length without a posterior staphyloma. To the best of our knowledge our cases are the first reports of PCV in highly myopic eyes without a posterior staphyloma. Although Mauget-Faysse thought PCV was a complication associated with tilted disc syndrome and posterior staphyloma (16), we feel that the development of PCV is rare in myopic eyes, and that the thin choroid could potentially be a protective factor against the development of PCV.

Treatment in Patient 2 was performed with antiangiogenic therapy alone and was successful at resolving the edema and improving vision to 20/25 from 20/80 without laser or photodynamic therapy. In the series by Mauget-Faysse treatment was only performed with laser or photodynamic therapy in their cases [16Mauget-Faÿsse M, Cornut PL, Quaranta El-Maftouhi M, Leys A. Polypoidal choroidal vasculopathy in tilted disk syndrome and high myopia with staphyloma. Am J Ophthalmol 2006; 142(6): 970-5.
[http://dx.doi.org/10.1016/j.ajo.2006.06.063] [PMID: 17046703]
]. This successful treatment reported herein with antiangiogenic therapy in patient 2 does indicate that antiangiogenic therapy for PCV in myopic eyes can be successful, and since myopic eyes can have spontaneous resolution [3El Matri L, Chebil A, Kort F. Current and emerging treatment options for myopic choroidal neovascularization. Clin Ophthalmol 2015; 9: 733-44.
[http://dx.doi.org/10.2147/OPTH.S49437] [PMID: 25987831]
], it is reasonable to follow these eyes after regression of the active lesions without treatment on an as needed basis for treatment. A thin choroid as noted in patient 2 may be a possible positive predictive factor for following patients as needed after a good anatomic response to antiangiogenic drugs. However, patients 1 and 3 both showed a poor response to antiangiogenic therapy, and highlight the potential need to consider other treatment options including photodynamic therapy or combination photodynamic therapy with antiangiogenic therapy with or without steroids. The use of combination therapy with photodynamic therapy and antiangiogenic therapy for myopic choroidal neovascularization is supported by Rishi and colleagues, which showed better visual outcomes with combination therapy than with photodynamic monotherapy or antiangiogenic monotherapy [17Rishi P, Rishi E, Bhende M. Comparison of photodynamic therapy, ranibizumab/bevacizumab or combination in the treatment of myopic choroidal neovascularization. Br J Ophthalmol 2016; 100: 1337-40.
[http://dx.doi.org/10.1136/bjophthalmol-2015-307802] [PMID: 26792945]
].

CONCLUSION

PCV is rare in highly myopic eyes occurring in 1.4% of eyes diagnosed with PCV. However, PCV with typical features on ICG angiography of polypoidal dilations and a branching vascular network (Figs. 3B, D, E), as well as typical OCT findings of the double line sign showing the branching vascular network and the inverted U-shaped elevations of the retinal pigment epithelium typical of polyps (Figs. 3C, F) can occur in highly myopic eyes with elongated axial length. PCV lesions in highly myopic eyes can show resistance to antiangiogenic therapy, as noted in PCV lesions in general, which may require alternative treatment options. Appropriate diagnostic testing including ICG angiography to make the diagnosis of PCV is important, especially when there has been a poor response to antiangiogenic therapy.

ETHICS APPROVAL AND CONSENT TO PARTICIPATE

This retrospective study was deemed exempt from institutional review board approval by the Western Institutional Review Board (#1-1019457-1), and the study was conducted in accordance with the declarations of Helsinki.

HUMAN AND ANIMAL RIGHTS

No Animals were used in this research. All human research procedures followed were in accordance with the ethical standards of the committee responsible for human experimentation (institutional and national), and with the Helsinki Declaration of 1975, as revised in 2008.

CONSENT FOR PUBLICATION

The patients provided written consent for publication of non-identifying information including medical record details and photographs.

CONFLICT OF INTEREST

The authors do not have any proprietary interest in the materials described.

The authors have the following financial disclosures:

GK:

1. Commercial Interest: Regeneron Role: Speaking/Consultant

2. Commercial Interest: Bayer Role: Speaking/Consultant

3. Commercial Interest: Zeiss Role: Consultant

4. Commercial Interest: Bausch and Lomb Role: Consultant

5. Commercial Interest: Allergan Role: Consultant

6. Commercial Interest: Santen Role: Consultant

JS:

1. Commercial Interest: Santen Role: Consultant

The following authors have no financial disclosures: ET, KK

ACKNOWLEDGEMENTS

Maria Won and Sheila Chamian, Research Coordinators at Hawaii Macula and Retina Institute

REFERENCES

[1] Gass JD. Stereoscopic atlas of macular diseases: Diagnosis and treatment 4th ed. 1997; 26-30.
[2] Kokame GT. Prospective evaluation of subretinal vessel location in polypoidal choroidal vasculopathy (PCV) and response of hemorrhagic and exudative PCV to high-dose antiangiogenic therapy (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc 2014; 112: 74-93.
[3] El Matri L, Chebil A, Kort F. Current and emerging treatment options for myopic choroidal neovascularization. Clin Ophthalmol 2015; 9: 733-44.
[http://dx.doi.org/10.2147/OPTH.S49437] [PMID: 25987831]
[4] Kang HM, Koh HJ. Lack of polypoidal lesions in patients with myopic choroidal neovascularization as evaluated by indocyanine green angiography. Am J Ophthalmol 2014; 157(2): 378-383.e1.
[http://dx.doi.org/10.1016/j.ajo.2013.09.018] [PMID: 24315295]
[5] Ohno-Matsui K. What is the fundamental nature of pathologic myopia? Retina 2017; 37(6): 1043-8.
[http://dx.doi.org/10.1097/IAE.0000000000001348] [PMID: 27755375]
[6] Meng W, Butterworth J, Malecaze F, Calvas P. Axial length of myopia: A review of current research. Ophthalmologica 2011; 225(3): 127-34.
[http://dx.doi.org/10.1159/000317072] [PMID: 20948239]
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Endorsements



"Open access will revolutionize 21st century knowledge work and accelerate the diffusion of ideas and evidence that support just in time learning and the evolution of thinking in a number of disciplines."


Daniel Pesut
(Indiana University School of Nursing, USA)

"It is important that students and researchers from all over the world can have easy access to relevant, high-standard and timely scientific information. This is exactly what Open Access Journals provide and this is the reason why I support this endeavor."


Jacques Descotes
(Centre Antipoison-Centre de Pharmacovigilance, France)

"Publishing research articles is the key for future scientific progress. Open Access publishing is therefore of utmost importance for wider dissemination of information, and will help serving the best interest of the scientific community."


Patrice Talaga
(UCB S.A., Belgium)

"Open access journals are a novel concept in the medical literature. They offer accessible information to a wide variety of individuals, including physicians, medical students, clinical investigators, and the general public. They are an outstanding source of medical and scientific information."


Jeffrey M. Weinberg
(St. Luke's-Roosevelt Hospital Center, USA)

"Open access journals are extremely useful for graduate students, investigators and all other interested persons to read important scientific articles and subscribe scientific journals. Indeed, the research articles span a wide range of area and of high quality. This is specially a must for researchers belonging to institutions with limited library facility and funding to subscribe scientific journals."


Debomoy K. Lahiri
(Indiana University School of Medicine, USA)

"Open access journals represent a major break-through in publishing. They provide easy access to the latest research on a wide variety of issues. Relevant and timely articles are made available in a fraction of the time taken by more conventional publishers. Articles are of uniformly high quality and written by the world's leading authorities."


Robert Looney
(Naval Postgraduate School, USA)

"Open access journals have transformed the way scientific data is published and disseminated: particularly, whilst ensuring a high quality standard and transparency in the editorial process, they have increased the access to the scientific literature by those researchers that have limited library support or that are working on small budgets."


Richard Reithinger
(Westat, USA)

"Not only do open access journals greatly improve the access to high quality information for scientists in the developing world, it also provides extra exposure for our papers."


J. Ferwerda
(University of Oxford, UK)

"Open Access 'Chemistry' Journals allow the dissemination of knowledge at your finger tips without paying for the scientific content."


Sean L. Kitson
(Almac Sciences, Northern Ireland)

"In principle, all scientific journals should have open access, as should be science itself. Open access journals are very helpful for students, researchers and the general public including people from institutions which do not have library or cannot afford to subscribe scientific journals. The articles are high standard and cover a wide area."


Hubert Wolterbeek
(Delft University of Technology, The Netherlands)

"The widest possible diffusion of information is critical for the advancement of science. In this perspective, open access journals are instrumental in fostering researches and achievements."


Alessandro Laviano
(Sapienza - University of Rome, Italy)

"Open access journals are very useful for all scientists as they can have quick information in the different fields of science."


Philippe Hernigou
(Paris University, France)

"There are many scientists who can not afford the rather expensive subscriptions to scientific journals. Open access journals offer a good alternative for free access to good quality scientific information."


Fidel Toldrá
(Instituto de Agroquimica y Tecnologia de Alimentos, Spain)

"Open access journals have become a fundamental tool for students, researchers, patients and the general public. Many people from institutions which do not have library or cannot afford to subscribe scientific journals benefit of them on a daily basis. The articles are among the best and cover most scientific areas."


M. Bendandi
(University Clinic of Navarre, Spain)

"These journals provide researchers with a platform for rapid, open access scientific communication. The articles are of high quality and broad scope."


Peter Chiba
(University of Vienna, Austria)

"Open access journals are probably one of the most important contributions to promote and diffuse science worldwide."


Jaime Sampaio
(University of Trás-os-Montes e Alto Douro, Portugal)

"Open access journals make up a new and rather revolutionary way to scientific publication. This option opens several quite interesting possibilities to disseminate openly and freely new knowledge and even to facilitate interpersonal communication among scientists."


Eduardo A. Castro
(INIFTA, Argentina)

"Open access journals are freely available online throughout the world, for you to read, download, copy, distribute, and use. The articles published in the open access journals are high quality and cover a wide range of fields."


Kenji Hashimoto
(Chiba University, Japan)

"Open Access journals offer an innovative and efficient way of publication for academics and professionals in a wide range of disciplines. The papers published are of high quality after rigorous peer review and they are Indexed in: major international databases. I read Open Access journals to keep abreast of the recent development in my field of study."


Daniel Shek
(Chinese University of Hong Kong, Hong Kong)

"It is a modern trend for publishers to establish open access journals. Researchers, faculty members, and students will be greatly benefited by the new journals of Bentham Science Publishers Ltd. in this category."


Jih Ru Hwu
(National Central University, Taiwan)


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