Myopic Maculopathy: What Is It and How Is It Treated?

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June 3, 2024

By Zeinab Fakih, BOptom, MPH 

Avoiding higher myopia and longer axial lengths is the best way to prevent development of pathological myopia. Efforts to target myopia awareness and management of myopia progression in childhood are imperative. 

High myopia increases the risk of complications including retinal detachment, cataract, glaucoma, and myopic maculopathy.¹ These complications pose a particularly significant challenge to health practitioners and systems in the context of rising myopia prevalence. Myopic maculopathy is one of the most serious, vision-threatening consequences of high myopia. It is estimated that by 2050, 55.7 million people will have visual impairment from myopic maculopathy, of whom 18.5 million people will be legally blind.²

Myopic maculopathy encompasses macular atrophy, maculoschisis, myopic choroidal neovascularization, and macular hole. The prevalence of myopic maculopathy has been reported to range from 0.2% to 4.0% in myopic patients,³ with higher degrees of myopia associated with higher prevalence of myopic maculopathy (13.3% to 65.4%) compared to low myopia (0.1-7%).^4-6

Classifying Myopic Maculopathy 
An international classification and grading system for myopic maculopathy was proposed in 2015.⁷ The classification system applies clinical observations to evaluate and assess the severity of myopic maculopathy. It represents myopic maculopathy as a progressive disease with “plus lesions” seen as important indicators for future progression.  

Classification of Myopic Maculopathy as described by Ohno-Matsui and the META-PM study group:⁷

Progressing Myopic Maculopathy 
Following the META-PM study group international classification, an eye is considered to have pathological myopia when categories 2, 3, 4, or any plus lesions are observed. A recently published systematic review on progression of myopic maculopathy found that the risk of progression was greater in eyes with pathological myopia at baseline and greater in eyes with severe myopic maculopathy compared to mild macula maculopathy.^11,12

Other factors that have been associated with progression of myopic maculopathy include choroidal thickness,¹³ spherical equivalent values, and axial length.^13-15 It has been proposed that these factors may contribute to progression of myopic maculopathy through degeneration and ischemia of the outer retina.¹² Bullimore et al. estimated that each 1.00D increase in myopia is associated with a 67% increase of myopic maculopathy, and that slowing myopia progression by 1.00D should reduce the likelihood of myopic maculopathy by 40%.¹⁶

A recently published observational study, has highlighted that progression of myopic maculopathy is not limited to adults.¹⁷ Twelve percent of pediatric high myopes studied showed progression of their myopic maculopathy over a four-year follow-up period. It is therefore critical for practitioners, patients, and families to understand that all people with high myopia, including children, are at increased risk of progressive myopic maculopathy and need to be closely reviewed. 

Myopic Choroidal Neovascularization and Maculoschisis
Myopic choroidal neovascularization (CNV) is considered one of the most serious vision-threatening complications of pathological myopia, and when untreated can result in sudden vision loss. It has been estimated to affect 5-11% of patients with high myopia.¹⁸ Patients with myopic CNV in one eye have a 35% chance over an eight-year period of developing CNV in the fellow eye.¹⁹ Anti-VEGF agents are the current standard of care for myopic CNV. However, studies have questioned the long-term benefit of anti-VEGF in myopic CNV.^20-21 It has been postulated that in highly myopic eyes with very thin choroids, anti-VEGF treatments may exacerbate secondary chorio-retinal atrophy, developing as a result of the CNV. A number of studies, with follow-up periods of five or more years, have failed to show long-term improvement in best corrected visual acuity.^22-23 

Myopic maculopathy may also present clinically with tractional changes such as maculoschisis, retinal/foveal detachment, lamellar macular hole, or full thickness macular hole with/without retinal detachment. These variations of myopic tractional maculopathy are best determined using optical coherence tomography.²⁴ Treatment of myopic tractional maculopathy depends on the stage of maculopathy and degree of vision impairment. Options include pars-planar vitrectomy, macular buckle, or a combination of both.⁹ 

In summary, options are available for the management of myopic choroidal neovascularization and myopic tractional maculopathy, so monitoring and correct management at all life stages is critical. However, treatment remains challenging. So, future research to evaluate and advance management options for manifest pathological myopia is warranted.

Perhaps more importantly, prevention is preferable: avoiding higher myopia and longer axial lengths is the best way to prevent development of pathological myopia. Efforts to target myopia awareness and management of myopia progression in childhood are imperative. 

 

Zeinab Fakih is the manager of pediatric and rehabilitative services at the Australian College of Optometry (ACO). She leads the ACO public myopia clinic and myopia teaching clinics. Zeinab graduated from Optometry at the University of Melbourne and went on to complete a Graduate Certificate in University Teaching from The University of Melbourne, a master’s degree of Public Health from University of Sydney, and a Certificate of Children’s Vision through the Australian College of Optometry. She has a keen interest in teaching and is passionate about the provision of pediatric eye care to disadvantaged populations.

 

References

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