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Myopic Choroidal Neovascularisation: Current Concepts and Update On BJO Online First, published on July 1, 2014 as 10.1136/bjophthalmol-2014-305131 Review Br J Ophthalmol: first published as 10.1136/bjophthalmol-2014-305131 on 1 July 2014. Downloaded from Myopic choroidal neovascularisation: current concepts and update on clinical management Tien Y Wong,1 Kyoko Ohno-Matsui,2 Nicolas Leveziel,3 Frank G Holz,4 Timothy Y Lai,5 Hyeong Gon Yu,6 Paolo Lanzetta,7 Youxin Chen,8 Adnan Tufail9 For numbered affiliations see ABSTRACT PATHOGENESIS OF MYOPIC CNV end of article. Choroidal neovascularisation (CNV) is a common vision- Several theories have been proposed to explain the threatening complication of myopia and pathological development of myopic CNV, reviewed in detail Correspondence to 4 Dr Tien Y Wong, Singapore Eye myopia. Despite significant advances in understanding elsewhere. The mechanical theory is based on the Research Institute, Singapore the epidemiology, pathogenesis and natural history of assumption that the progressive and excessive National Eye Centre, National myopic CNV, there is no standard definition of myopic elongation of the anteroposterior axis causes a University of Singapore, 11 CNV and its relationship to axial length and other mechanical stress on the retina, leading to an imbal- Third Hospital Avenue, Singapore 168751, Singapore; myopic degenerative changes. Several treatments are ance between pro-angiogenic and anti-angiogenic 7 [email protected] available to ophthalmologists, but with the advent of factors, resulting in myopic CNV. In support, the new therapies there is a need for further consensus and presence of lacquer cracks has been shown to be a Received 7 March 2014 clinical management recommendations. Verteporfin predisposing factor for the development of myopic Revised 7 May 2014 89 Accepted 3 June 2014 photodynamic therapy has been an established CNV. treatment for subfoveal myopic CNV for many years, but The heredodegenerative theory states that this treatment does not restore visual acuity and is myopic refractive errors are genetically predeter- associated with long-term chorioretinal atrophy. More mined.4510In support, studies have shown that recently, clinical trials investigating the efficacy and single nucleotide polymorphisms in several genes safety of anti-vascular endothelial growth factor agents (eg, pigment epithelium-derived factor) are asso- in patients with myopic CNV have demonstrated ciated with the development and progression of substantial visual acuity gains and quality of life myopic CNV.51112 increases compared with photodynamic therapy. These The haemodynamic theory for the development enhanced outcomes provide updated evidence-based of myopic CNV relates to perfusion changes in the clinical management guidelines of myopic CNV, and choroidal circulation of the myopic eye, such as increase the need for a generally accepted definition for choroidal filling delay and diffuse thinning of the myopic CNV. This review critically summarises the latest choroid.413However, evidence has shown that myopic CNV literature in the context of clinical myopic CNV can develop in eyes with shallow sta- experience and recommends a myopic CNV treatment phyloma and preserved choroidal circulation, sug- algorithm. gesting that haemodynamic factors may not have a strong role in the development of myopic CNV.14 http://bjo.bmj.com/ DIAGNOSIS OF MYOPIC CNV Myopic CNV is typically seen as a small, flat, greyish membrane on slit-lamp biomicroscopy that INTRODUCTION may have a hyper-pigmented border if chronic or – Myopic choroidal neovascularisation (CNV) is a recurrent.1 4 Symptoms of myopic CNV include a common vision-threatening complication of decrease in vision, central scotoma and/or on September 26, 2021 by guest. Protected copyright. – myopia and pathological myopia (PM).1 4 The clin- metamorphopsia.15 16 ical definition and terminology surrounding myopic The standard tests for diagnosing myopic CNV CNV varies, with myopic CNV also commonly are fundus biomicroscopy, fluorescein angiography being referred to as subretinal neovascularisation in (FA) and optical coherence tomography (OCT). FA PM, Fuchs’ spot or Forster–Fuchs’ retinal spot in and OCT are generally recommended baseline diag- PM, and disciform degeneration in PM. While nostic tests for myopic CNV in conjunction with myopic CNV is historically thought to only occur colour photos and clinical examination. FA demon- in eyes with PM, it is now recognised that myopic strates the presence, type, area and activity of CNV can occur at any degree of myopia and in myopic CNV,and helps exclude other disorders.417 eyes without typical myopic degenerative fundus The majority of myopic CNV presents as a ‘classic’ changes.56Therefore, in clinical practice, CNV can pattern on FA,18 with well defined hyperfluores- be attributed to be ‘myopic’ in aetiology by the cence in the early phases and leakage of fluorescein refractive status of the eye and the exclusion of dye during the late phases.41517OCT is usually other disorders associated with CNV. mandatory for the identification of the fovea, To cite: Wong TY, Ohno- There are now effective therapeutic options for assessment of retinal thickness and presence of Matsui K, Leveziel N, et al. myopic CNV, in particular anti-vascular endothelial extracellular fluid, and for establishing a baseline to Br J Ophthalmol Published 15 Online First: [please include growth factor (VEGF) therapy. This review sum- judge future treatment response. On OCT, Day Month Year] marises current concepts in pathogenesis, epidemi- myopic CNV presents as a highly reflective area doi:10.1136/bjophthalmol- ology, natural history, and management options for contiguous above the retinal pigment epithelium 2014-305131 myopic CNV. (sometimes referred to as ‘type 2 CNV’) with WongCopyright TY, et al. Br JArticle Ophthalmol author2014;0:1 –(or8. doi:10.1136/bjophthalmol-2014-305131 their employer) 2014. Produced by BMJ Publishing Group Ltd under licence. 1 Review Br J Ophthalmol: first published as 10.1136/bjophthalmol-2014-305131 on 1 July 2014. Downloaded from with myopia and vision loss (table 1). Other complications of Table 1 Coexisting pathologies and differential diagnosis for PM should be identified with OCT/FA, such as myopic traction myopic CNV maculopathy, epiretinal membrane, vitreomacular traction and Other co-existing degenerative myopic full-thickness or lamellar macular hole, as these require changes associated with myopia Differential diagnosis for CNV different treatments from myopic CNV. In particular, retinal Myopic traction maculopathy (foveoschisis) Neovascular AMD haemorrhage due to new lacquer crack formation and macular Macular hole Myopic macular haemorrhage due exudative changes associated with a dome-shaped macula or a to lacquer cracks staphyloma should be identified and excluded on OCT/FA 4 Retinal tear/detachment Punctate inner choroidopathy (figure 1). In case of significant haemorrhage, indocyanine (usually coexists with myopia) green angiography (ICGA) can identify the presence of lacquer Dome-shaped macula Multifocal choroiditis cracks and/or CNV. It should be noted that OCT alone cannot Staphyloma Idiopathic CNV* differentiate myopic CNV from subretinal bleeding due to new Atrophic changes (patchy atrophy, lacquer crack formation, which could lead to unnecessary treat- tesselated changes and diffuse atrophy) ment by anti-VEGF therapy for subretinal bleeding without *Idiopathic CNV in a myope is myopic CNV. CNV. In addition, myopic CNV should be differentiated from AMD, age-related macular degeneration; CNV, choroidal neovascularisation. other causes of CNV (eg, multifocal choroiditis or punctate inner choroidopathy or age-related macular degeneration – (AMD)).19 21 Importantly, myopic CNV has different lesion minimal subretinal fluid.4 Fundus autofluorescence, which characteristics to AMD-CNV, especially in younger indivi- allows the visualisation of accumulated lipofuscin within the duals,422but is a predominantly ‘classic’, ‘type 2’ CNV; that is, retinal pigment epithelium, may be included as part of any basic smaller than that of AMD, with minimal subretinal fluid and an diagnosis and follow-up examination, as it may aid in the assess- absence of drusen at the typical age of onset.4 ment of myopic CNV progression (and associated geographic atrophy).4 EPIDEMIOLOGY OF MYOPIC CNV There are several differential diagnoses and pathologies that A recent systematic review has indicated that the prevalence of must be excluded from myopic CNV when examining a patient PM is 1–3% in adults, and that 5–11% of patients with PM http://bjo.bmj.com/ on September 26, 2021 by guest. Protected copyright. Figure 1 Differential diagnosis for myopic choroidal neovascularisation (CNV): (A and B) haemorrhage due to lacquer cracks; (C) dome-shaped macula with serous retinal detachment; and (D and E) macular fluid due to staphyloma. 2 Wong TY, et al. Br J Ophthalmol 2014;0:1–8. doi:10.1136/bjophthalmol-2014-305131 Review Br J Ophthalmol: first published as 10.1136/bjophthalmol-2014-305131 on 1 July 2014. Downloaded from develop CNV.23 While these data provide some insight into the 35% of patients within 8 years.8 There appears to be three main epidemiology of myopic CNV, the results should be interpreted stages of myopic CNV, all of which are associated with vision with caution, since the definitions of myopia, PM and myopic loss (figure 2).4 The initial phase results in direct damage to CNV between studies were not uniform.23 Furthermore, the photoreceptors, causing central visual loss.32526Then, as the
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