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IMI Pathologic Myopia Special Issue IMI Pathologic Myopia Kyoko Ohno-Matsui,1 Pei-Chang Wu,2 Kenji Yamashiro,3,4 Kritchai Vutipongsatorn,5 Yuxin Fang,1 Chui Ming Gemmy Cheung,6 Timothy Y. Y. Lai,7 Yasushi Ikuno,8–10 Salomon Yves Cohen,11,12 Alain Gaudric,11,13 and Jost B. Jonas14 1Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo, Japan 2Department of Ophthalmology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan 3Department of Ophthalmology and Visual Sciences, University Graduate School of Medicine, Kyoto, Japan 4Department of Ophthalmology, Otsu Red-Cross Hospital, Otsu, Japan 5School of Medicine, Imperial College London, London, United Kingdom 6Singapore Eye Research Institute, Singapore National Eye Center, Singapore 7Department of Ophthalmology & Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, Hong Kong 8Ikuno Eye Center, 2-9-10-3F Juso-Higashi, Yodogawa-Ku, Osaka 532-0023, Japan 9Department of Ophthalmology, Osaka University Graduate School of Medicine, Osaka, Japan 10Department of Ophthalmology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan 11Centre Ophtalmologique d’Imagerie et de Laser, Paris, France 12Department of Ophthalmology and University Paris Est, Creteil, France 13Department of Ophthalmology, APHP, Hôpital Lariboisière and Université de Paris, Paris, France 14Department of Ophthalmology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany Correspondence: Kyoko Pathologic myopia is a major cause of visual impairment worldwide. Pathologic myopia is Ohno-Matsui, Department of distinctly different from high myopia. High myopia is a high degree of myopic refractive Ophthalmology and Visual Science, error, whereas pathologic myopia is defined by a presence of typical complications in Tokyo Medical and Dental the fundus (posterior staphyloma or myopic maculopathy equal to or more serious than University, Tokyo, Japan; diffuse choroidal atrophy). Pathologic myopia often occurs in eyes with high myopia, [email protected]. however its complications especially posterior staphyloma can also occur in eyes without Received: January 6, 2021 high myopia. Accepted: January 8, 2021 Published: April 28, 2021 Owing to a recent advance in ocular imaging, an objective and accurate diagnosis of pathologic myopia has become possible. Especially, optical coherence tomography has Citation: Ohno-Matsui K, Wu P-C, revealed novel lesions like dome-shaped macula and myopic traction maculopathy. Wide- Yamashiro K, et al. IMI Pathologic myopia. Invest Ophthalmol Vis field optical coherence tomography has succeeded in visualizing the entire extent oflarge Sci. 2021;62(5):5. staphylomas. The effectiveness of new therapies for complications have been shown, such https://doi.org/10.1167/iovs.62.5.5 as anti-VEGF therapies for myopic macular neovascularization and vitreoretinal surgery for myopic traction maculopathy. Myopia, especially childhood myopia, has been increasing rapidly in the world. In parallel with an increase in myopia, the prevalence of high myopia has also been increas- ing. However, it remains unclear whether or not pathologic myopia will increase in parallel with an increase of myopia itself. In addition, it has remained unclear whether genes responsible for pathologic myopia are the same as those for myopia in general, or whether pathologic myopia is genetically different from other myopia. Keywords: pathologic myopia, myopic maculopathy, optical coherence tomography, gene analyses, myopic macular neovascularization, myopic foveoschisis, myopic traction macu- lopathy, dome-shaped macula DEFINITION OF PATHOLOGIC MYOPIA entities are distinctly different; high myopia is defined as a high degree of myopic refractive error, whereas “pathologic ccording to the IMI,1 pathologic myopia is an exces- myopia” is characterized by the presence of typical myopic A sive axial elongation associated with myopia that leads lesions in the posterior fundus. Duke-Elder2 defined patho- to structural changes in the posterior segment of the eye logic myopia as “that type of myopia which is accompanied (including posterior staphyloma, myopic maculopathy, and by degenerative changes occurring especially in the poste- high myopia-associated optic neuropathy) and that can lead rior pole of the globe.” to loss of best-corrected visual acuity. The term “pathologic Characteristic myopic lesions in the posterior fundus myopia” is often confused with “high myopia.” These two include myopic maculopathy equal to or more serious than Copyright 2021 The Authors iovs.arvojournals.org | ISSN: 1552-5783 1 This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. Downloaded from iovs.arvojournals.org on 04/29/2021 IMI Pathologic Myopia IOVS | Special Issue | Vol. 62 | No. 5 | Article 5 | 2 diffuse chorioretinal atrophy (equal to category 2 in the The prevalence of high myopia is linked to the over- META-analysis for Pathologic Myopia [META-PM] classifica- all prevalence of myopia. In Taiwan, the prevalence of tion3) and/or the presence of a posterior staphyloma.4 The myopia is as high as 84% in 18-year-old individuals and cut-off values of the myopic refractive error and axial length the prevalence of high myopia is about 21%.22 Approxi- were not set for the definition of pathologic myopia because mately 22.9% of the world population were diagnosed with a posterior staphyloma has been reported to occur in eyes myopia in 2000, and 11.6% had high myopia among the with normal axial length5 or in eyes with an axial length of myopia population. It is estimated that by 2050, approxi- less than 26.5 mm,6 although long axial length is one of the mately one-half of the world’s population will have myopia risk factors for fundus complications. and up to one-fifth of the myopia population will be Because progressive choroidal thinning and formation highly myopic.23 Thus, areas with high prevalence rates of of Bruch’s membrane defects in the macular region are myopia would have increased rates of high myopia and key phenomena associated with myopic maculopathy, the pathologic myopia. Pathologic myopia will be associated lesions of myopic maculopathy are better classified by their with a high number of individuals suffering from vision appearance in the optical coherence tomography (OCT) impairment and will have a significant negative impact on images than by the morphology as detected on fundus society. photographs.7 The degree of myopia is associated with the risk of patho- logic myopia. The prevalence of pathologic myopia is only 1% to 19% in the low-to-moderate myopia (–3 D) popula- tion, but its prevalence is as high as 50% to 70% in the high BASIC ASPECTS OF PATHOLOGIC MYOPIA myopia population.17,18,24 A 1-D increase in myopia is asso- Epidemiology of Pathologic Myopia ciated with a 67% increase in the prevalence of pathologic myopia.25 A linear trend is observed for increasing myopia Prevalence and Its Impact on Vision. It has been diopters until −7.00 D, followed by an exponential trend in reported that pathologic myopia affects up to 3% of the the prevalence rates of pathologic myopia.26 world’s population, with racial differences regarding the Age. In addition to the degree of myopia, age is an prevalence of the disease.8 Approximately 1% to 3% of important factor in the development of pathologic myopia. Asians and 1% of Caucasians have pathologic myopia. The prevalence of myopic maculopathy increased and expo- However, the definition of pathologic myopia used in earlier nentially with increasing spherical equivalent and age in studies was not consistent and pathologic myopia was Singapore.19 The prevalence of pathologic myopia is low confused with high myopia. Thus, an accurate prevalence in children and adolescents, but increases with advanc- needs to be determined based on the new standard defini- ing age. In individuals with high myopia aged 40 years or tion of pathologic myopia. older, a progressive increase was noted in the prevalence Pathologic myopia causes vision impairment or blindness and severity of maculopathy. Pathologic myopia changes in 0.2% to 1.5% of Asians and 0.1% to 0.5% of Caucasians.8 with chorioretinal atrophy were found in 52.9% and 19.3% Particularly, it is one of the major causes of low vision in of the Chinese and Singaporean adult groups with high working-aged populations, as well as in the elderly popula- myopia, respectively.27,28 In the Taiwanese elderly popu- tion. In Asia, pathologic myopia is the leading cause of irre- lation, myopic maculopathy increased from 2.2% in indi- versible blindness in Taiwan, Japan, and China. In Taiwan, viduals aged 65 years to 14.8% in relatively older adults. pathologic myopia is the second leading cause of vision Myopic maculopathy is rare in children with high myopia.29 impairment in individuals aged 65 years or older.9 In Japan, However, a long-term follow-up study showed that 83% of pathologic myopia is the third leading cause of bilateral low adults with pathologic myopia and myopic maculopathy had vision and the leading cause of monocular blindness in indi- already had diffuse choroidal atrophy around the optic disc viduals aged 40 years or older.10 In China, pathologic myopia in their childhood.30 This finding suggested the possibil- is the leading cause of blindness and low vision in indi- ity that children who eventually develop pathologic myopia viduals aged 40 to 49 years.11 In Western countries, patho- may be different, even at an early age. logic myopia is the third cause of blindness according to the Genetic or Acquired. There are two types of Rotterdam Study, the Copenhagen City Eye Study, and the myopia—congenital myopia or infantile myopia and Los Angeles Latino Eye Study.12–14 acquired myopia or school myopia. Congenital myopia has High Myopia and Myopia. Recently, owing to family aggregation and is strongly influenced by genetic changes in environmental and lifestyle factors, the preva- factors. However, the prevalence of congenital myopia is lence of myopia and high myopia has increased rapidly.
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