The MYOPIA ISSUE

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Pathological Implications of High Myopia

We are facing a global epidemic of myopia, and high myopia in particular. To manage its impact and prevent sightthreatening complications, all eye health care providers have a moral responsibility to ensure patients with myopia are screened and cared for.

The public misconception that myopia is a ‘benign’ disease should be (and is) a key point of focus for re-education of wider health organisational structures.

In this article, ophthalmologists Dr Tanya Trinh and Dr Chris Qureshi describe the prevalence and impact of high myopia on vision impairment and blindness. Additionally, they discuss surgical solutions for high myopia currently available.

WRITERS Dr Tanya Trinh and Dr Chris Qureshi

LEARNING OBJECTIVES

On completion of this CPD activity, participants should be able to:
1. Understand the global prevalence and importance of high myopia,
2. Be aware of the pathological implications of high myopia, and
3. Appreciate the surgical management options for high myopia and its complications.

Although there is no global consensus on the definition of high myopia, most centres will use an approximate cut-off of -5.0 dioptres (D) or more of myopia as the division between myopia and high myopia. This was endorsed by the World Health Organization (WHO) in 2015.

In 2010, it was estimated that 27% (1,893 million) and 2.8% (170 million) of the global population were affected by myopia and high myopia respectively. Published studies highlight a higher prevalence in East Asian countries like China, Japan, Republic of Korea, and Singapore, where approximately 50% of the population is affected, as compared to Australia, Europe, and the Americas. By 2050, projections indicate that 52% (4,949 million) and 10% (925 million) of the world’s population will be afflicted by myopia and high myopia, respectively. These numbers are further supported by WHO’S Global Burden of Disease program, which demonstrated that the prevalence of myopia is likely to exceed 50% in 57% of the world’s countries – even in countries currently reporting low prevalence.1

When it comes to high myopia, this disease subset in particular, is projected to rise to 24% across all Global Burden of Disease regions and affluent Asia-Pacific nations by 2050. Notably, pathologic myopia more substantially impacts vision among Asians (0.2–1.4%) compared with Caucasians (0.1–0.5%), with an incidence of 5–10 per 100,000 annually in Asian populations.2

This anticipated surge underscores the concern as uncorrected distance refractive error, primarily caused by myopia, is the second leading cause of blindness and the principal cause of moderate and severe vision impairment, accounting for 53% of such cases.

The global burden of myopia and high myopia is not insignificant. The financial impact is staggering, with a direct and indirect loss to world productivity estimated at 269 billion international dollars.3

Addressing this issue is anticipated to cost approximately 28 billion dollars over five years.3 Efforts to halve the rate of myopia progression could potentially lead to a 90% reduction in the prevalence of high myopia, presenting a substantial global public health and economic benefit.3 The insights gained from these projections and regional analyses are paramount for shaping policies and strategies to mitigate the impact of myopia and high myopia worldwide.

HOW IS HIGH MYOPIA DEFINED?

Establishing a universally accepted operational definition on the thresholds for myopia or high myopia is essential for conducting cohesive, cross-country population-based studies. These studies aim to understand the prevalence of high myopia and related vision impairment or blindness. Additionally, they aim to explore the progression patterns of high myopia to pathological myopia across diverse countries, ethnic groups, and socio-economic environments. A standardised definition will facilitate longitudinal studies on high and pathologic myopia, allowing a more precise quantification of the risk for sight-threatening conditions attributed to high myopia.

The definition of high myopia is currently inconsistent, with various studies assigning different dioptre values ranging from < -5.00D to ≤ -8.00D, and others using an axial length greater than 26mm as a criterion. However, relying on axial length can lead to inaccuracies as it can naturally vary, even in normal eyes. The overall eye power, determined by the lens, cornea, and axial length, further complicates this, with some long or short axial length eyes showing no refractive error. The inconsistencies extend to measurements using various instruments, which can yield different results.

There is a collective agreement among participants that classifying high myopia as ≥ -5.00D is the most reliable definition, as individuals with -5.00D uncorrected myopia exhibit a visual acuity of 6/172, significantly below the blindness threshold. Additionally, pathological myopia is also ill-defined, with varying descriptions and criteria across studies concerning vision-threatening retinal changes, posterior staphyloma presence, axial length, and spherical equivalent refractive error. Reports define pathological myopia as high myopia accompanied by myopia-related fundus abnormalities like myopic macular degeneration (MMD) and glaucoma.

IMPACT OF HIGH MYOPIA ON VISION

Patients with myopia alone have a higher risk of sight-threatening complications, which can be grouped into four main categories:

• Glaucoma (open angle),

• Cataract (nuclear, cortical, and posterior subcapsular),

• Retinal tear and detachment, and

• Myopic macular degeneration and choroidal neovascular membrane formation.

When it comes to high myopia, the incidence of all four categories is significantly higher.

Glaucoma

Individuals with moderate-to-high myopia exhibit an approximately 50% elevated risk of developing glaucoma compared to their counterparts with low myopia, as indicated by an odds ratios (OR) of 2.5 and 1.7, respectively. This emphasises the need for heightened vigilance and targeted glaucoma screening within this patient demographic.4

Cataract

The prevailing evidence indicates a 17% increased likelihood for these individuals to require cataract surgery compared with those with moderate myopia, as evidenced by the odds ratios of 3.4 and 2.9, respectively.5

Retinal Tear and Detachment

Patients with high myopia face a five- to six-fold increased risk of retinal detachment compared with those with low myopia (OR >20 versus OR <4). This heightened risk is attributed to the axial elongation inherent in highly myopic eyes, leading to a ‘stretched’ retina that is more susceptible to peripheral retinal tears. The propensity for vitreous degeneration in myopic eyes further compounds this risk, enhancing the likelihood of vitreous collapse and separation from the retina, thereby augmenting the potential for retinal tears. High myopia may also induce central retinal degenerative changes, such as posterior staphyloma, lacquer cracks, and chorioretinal atrophy; metrics used in the grading of myopic maculopathy.6,7

Myopic Macular Degeneration

MMD stands as a significant contributor to visual impairment within the context of high myopia. A variety of terms encompass this condition, such as myopic maculopathy, myopic retinopathy, and myopic choroidal neovascularisation (CNV). Myopic macular degeneration afflicts 10% of individuals with pathological myopia, exhibiting bilateral symptoms in 30% of these cases. The probability of macular degeneration attributable to myopia escalates substantially with advancing age and progressing myopia. The advanced stage of myopic maculopathy results in central vision loss, with no existing treatment for its atrophic type.


“ The definition of high myopia is currently inconsistent, with various studies assigning different dioptre values… and others using an axial length greater than 26mm as a criterion ”


Clinically, MMD manifests as diffuse or patchy chorioretinal atrophy, lacquer cracks, choroidal neovascularisation, and related macular atrophy alongside high myopia. The progression of MMD in high myopia follows an observable pattern: initial early retinal alterations lead to a tessellated fundus, which may evolve into diffuse or patchy atrophy, or present as lacquer cracks, and CNV, culminating in MMD. Despite the observable progression, no universally accepted clinical grading system for MMD exists at present.

Managing CNV in MMD currently involves the use of anti-vascular endothelial growth factor agents. However, this approach leaves several unresolved questions concerning optimal treatment protocols, monitoring strategies, follow-up schedules, and long-term outcomes, pointing to the critical need for ongoing study and innovation in this area.

The burgeoning incidence of myopia augments the likelihood of visual impairment from this condition, underscoring a continual upward trend.6,7

SURGICAL SOLUTIONS FOR HIGH MYOPIA

Optometrist involvement during a patient’s developmental years is essential to help prevent their progression to high myopia. Early screening and detection, with a team-based, comanagement approach between optometrists trained in myopia management and paediatric ophthalmologists to help manage severe myopic pathologies, is paramount.

Highly myopic patients should also be managed conjointly between optometrist and ophthalmologist; not only to detect and manage the sight-threatening complications associated with higher myopia, but also to offer patients the full range of treatment options so that they may make a fully informed choice, empowering them to improve their quality of life.

Highly myopic patients, without optical correction in some form, are highly visually disabled. Spectacles and contact lenses are an excellent starting point as a conservative and non-surgical form of treatment.

However the ongoing cost and permanent dependence on prosthetics, inconvenience of broken or lost spectacles, and risk of serious contact lens infection and keratitis (up to one out of every 500 contact lens users per year) is not insignificant.8

Permanent visual correction in the form of surgery is a serious decision however, and should always be undertaken with the utmost care.

The surgical options for management of high myopia in patients 18 years of age and above are discussed here. Management of high myopia in the paediatric population will be discussed in an article to be published in mivision in February 2024.

Implantable Collamer Lenses

Implantable collamer lenses are rapidly becoming the preferred surgical choice for providing the best quality of vision overall in the management of younger highly myopic patients.

Since being approved by the United States Food and Drug Administration in 2005, the implantable collamer lens (ICL, STAAR Surgical, Nidau, Switzerland), a posterior chamber phakic intraocular lens, has proven to be a safe and effective way to correct high myopia in the peer reviewed literature. This small, thin lens, implanted in front of the patient’s crystalline lens in a 10-minute procedure, is the only refractive surgical procedure that is reversible. Since 2005, more than two million ICLs have been implanted into eyes around the world.

Advantages of ICL implantation include faster visual recovery, more stable refraction, and better visual quality than corneal refractive surgery. Recent developments in the ICL to improve its safety profile include the new CentraFlow technology of the Visian ICL (ICL V4c) where a 360 micron central port promotes natural aqueous humour flow around the crystalline lens. This reduces the risk of anterior subcapsular cataract formation, high intraocular pressure (IOP), and endothelial cell loss post-ICL implantation.

Additionally, once inserted, enhancements can be performed on the cornea with minimal impact on the corneal integrity and less impact on ectasia risk (given the reduced depth of laser treatments and thicker residual stromal thickness remaining). The superior quality of vision correction in ICL implantation for high myopia compared to cornea-based treatments for high myopia is supported by several studies reported in the peer reviewed literature.9,10

Benefits to the patient include no ongoing cost compared with frequent replacement of glasses and contact lenses, elimination of daily hassle with contact lens application, no risk of contact lens related infection, ocular surface intolerance/irritation or limbal stem cell failure, and a minimisation of dry eye impact. Most significantly, patients are freed from ongoing intervention.

Furthermore, once patients develop cataracts with age, cataract surgery can be performed as routine without the need for intraocular lens formulaic adjustment. This means the biometric accuracy of virgin eyes can be expected. The same cannot be said for post corneal refractive surgical eyes, where the lens calculation formulas are still yet to approach the same kind of accuracy enjoyed with virgin eyes. A further benefit is that the ICL is simply removed at the same surgery as the removal of cataract, eliminating the need for two separate surgeries.

To be eligible for the ICL, the phakic patient must be aged between 21 and 45 years, have an anterior chamber depth of 3mm from the endothelium, with an endothelial cell count above 3000 cells/mm and without narrow angles (Grade III cut off ). In several parts of South and Southeast Asia, the eligibility criteria is extended to 18 years of age, reflecting the sheer burden of high myopia in these areas of the world. Off-label use around the world is not uncommon, with proper informed consent and a weight of risk versus benefit discussed in detail between surgeon and patient.

The procedure is elective involving a day stay. Generally, both eyes are performed in the same day under topical anaesthesia with sedation, with the vast majority of expected recovery of vision within the first 24 hours. Postoperative visual acuity, IOP, chamber depth, and lens position check is performed day one, week one and month one. Topical steroids and antibiotics are used for one month in a similar regime to cataractous patients. Ideally patients should be annually monitored by their eye care provider.

Postoperative complications may include toric lens rotation, endophthalmitis, corneal decompensation, uveitis, elevated IOP and cataract formation, though collectively these are rare.11-15

LASIK, LALEX and PRK for High Myopia

Laser-in-situ keratomileusis (LASIK), laser assisted lenticule extraction (LALEX) and photorefractive keratectomy (PRK) do still play an important role in the management of high myopia where the anterior chamber depth or other ocular considerations (low endothelial count, narrow angle) may preclude the use of an ICL. Benefits include no intraocular risks, such as endophthalmitis, endothelial cell loss or cataract formation.


“ Advantages of ICL implantation include faster visual recovery, more stable refraction, and better visual quality than corneal refractive surgery ”


They are, however, limited in the amount of correction possible (where the maximal amount of corneal ablation may limit the amount of prescription able to be corrected). Additionally, the induction of higher order aberrations, the potential for lower residual stromal thickness (higher ectasia risk), and the higher risk of haze (particularly with PRK) need to be taken into account. Patient preoperative expectations must be carefully counselled.

The literature demonstrates relative equivalence between LASIK and LALEX for high myopia, where both procedures are safe, effective, and predictable. Large metaanalyses do reveal a tendency towards undercorrection in the SMILE (small incision lenticular extraction) groups for astigmatism correction, and further development is ongoing in this area. LASIK has a slightly greater probability of causing post-operative spherical aberration. For PRK, the higher the prescription treated, the higher the risk of corneal haze formation, and as such, the application of mitomycin C after performing PRK is the most common adjuvant therapy employed to reduce this risk.16,17

Surgery to Halt or Slow Progression Generally speaking, LASIK, LALEX or PRK for treating myopia are globally advocated for individuals aged over 20 years, who have demonstrated refractive stability for a minimum of two years. However, it should be understood that this treatment paradigm is based on a relative paucity of evidence of safety and efficacy rather than specific evidence against the use of refractive surgery in younger age groups or groups with refractive instability. This gap in the literature is largely owing to the ethical challenges of performing large, blinded, controlled studies in this patient population and the challenges of not having obedient conscious fixation in this patient group required for more corneabased procedures.

LASIK and PRK are already not infrequently conducted in paediatric patients suffering from persistent unilateral anisometropic amblyopia, and have been reported to be especially useful in patients with sensory processing challenges where the wearing of spectacles or application of contact lenses with good success has proved challenging or impossible. It is also occasionally performed in late adolescents (17–20 years old) around the world for professional considerations (like those applying to government security forces), even in the absence of established refractive stability. Thus, it is a burgeoning area of research as the eye care profession investigates all modalities in the attempt to slow myopic progression.

Hecht et al. reported on the short-term outcomes (mean follow-up of 7.1 months) of 607 myopic adolescents (mean age 16.9 years) undergoing myopic PRK or LASIK and demonstrated equivalent refractive and visual outcomes compared with young adults (aged 20–40 years). They even observed significantly better values of postoperative uncorrected distance visual acuity in the adolescent population. Alio et al. then reported on the long-term outcomes of LASIK (over three years of follow-up) in the late adolescent population (18–19 years of age at the time of surgery) that received LASIK for myopia treatment (mean spherical equivalent −4.04 ± 1.92), with or without myopic astigmatism, and found that myopic-LASIK in late adolescence is safe and effective, with only a mild myopic progression occurring (in the SE of less than −0.5 D).18,19

This ongoing area of research is based on a similar hypothesis to contact lenses inducing peripheral myopic defocus – that the positive spherical aberration induced by the myopic laser ablation may also play a role in halting myopia progression in this population group. More studies are needed to confirm the efficacy and safety of this approach.

In Australia, the preference is still to wait for older age and refractive stability prior to performing refractive surgery. Until further evidence in the peer reviewed literature exists regarding the safety and efficacy of these procedures in the younger patient population, they can continue to enjoy visual correction in their spectacles and contact lenses.

Refractive Lens Exchange or Cataract Surgery

The decision to undertake refractive lens exchange or cataract surgery in highly myopic patients needs careful consideration.

The aim in these patients is to surgically improve their visual function from their preexisting baseline. The refractive discussion around visual targets needs to be discussed and documented in detail. The state of the retina and macula in context of the patient’s age also needs to be taken into consideration. The presence or absence of a posterior vitreous detachment also needs to be taken into consideration.

Patients with existing retinal or myopic degeneration may not be candidates for multifocal intraocular lenses or at least need to be counselled about the fact that lens replacement surgery will not prevent future MMD and the future quality of their vision may be impacted by their lens choice. Extended depth of focus lenses may afford a more forgiving method of achieving greater range of vision with less visual compromise. Monovision, if tolerated, is also an excellent way to preserve as much visual quality as possible in anticipation of future degeneration.

The logistics of lens replacement surgery (for the pre-cataractous or cataractous lens) need to be cautiously approached by the surgeon. Biometric measurements can be challenging and optically based biometric methods are generally more accurate in accounting for the impact of staphylomatous changes in the retina and macula than traditional A-scan measurements. High myopes with astigmatism have a higher chance of toric intraocular lens rotation post-surgery due to the enlarged capsular bag integrity. A capsular tension ring placement may be prudent to prevent rotation as well as counselling the patient on the chances of requiring a small enhancement procedure (lens rotation, LASIK or PRK) post-operatively. Intraoperatively, the depth and stability of the anterior chamber are highly dynamic, necessitating the use of heavier dispersive viscoelastic material, with risks of posterior capsular tear and vitreous loss being higher.

The risks of retinal detachment in the process of lens replacement surgery in high myopes are closely related to the age and gender of the patient, total refractive correction required, presence/absence of a posterior vitreous detachment, presence of lattice degeneration, and the axial length of the involved eye.

The retinal detachment risk calculator is a helpful tool to aid discussion between surgeon, optometrist, and patient. The surgeon inputs the patient’s risk factors to generate an individualised risk profile for retinal detachment in:

• the patient’s natural state when left untouched,

• after successful lens replacement surgery, and

• after lens replacement surgery complicated by vitreous loss and posterior capsular tear.

Refractive lens exchange should be delayed in young, highly myopic eyes in the presence of:

• Advanced peripheral lattice degenerations,

• No posterior vitreous detachment,

• Lacquer cracks in high myopia or myopic CNV in the fellow eye, and /or

• Presbyopic eyes with macular degeneration beginning in the fellow eye.

In these instances, consideration should be given to the implantable collamer lens.


“ lens replacement surgery will not prevent future MMD and the future quality of their vision may be impacted by their lens choice ”


MYOPIC CHOROID NEOVASCULARISATION

One of the leading causes of choroidal neovascularisation is pathologic myopia, which accounts for 62% of cases in people under the age of 50.20

Although there is still debate on the pathophysiology of myopic CNV, an indocyanine green angiographic study found that lacquer cracks and choroidal filling delay co-occurred in most patients.21

One hypothesis is that CNV might enter the subretinal region through a lacquer crack, or a crack in Bruch’s membrane. Additionally, according to this study, lacquer cracks are essential for developing CNV.22

Significant choroidal thinning has been observed in situations with myopic choroidal neovascularisation, according to recent optical coherence tomography studies.23

The first-line treatment of myopic CNV involves intravitreal injections of anti-vascular endothelial growth factor, and two substantial prospective randomised studies found that vision significantly improved 12 months following treatment.24,25

In Australia, ranibizumab is approved by the Therapeutic Goods Administration for the treatment of pathologic myopia.

RETINAL TEAR AND DETACHMENT TREATMENT

Degenerations of the peripheral retina, such as lattice degeneration, degenerative retinoschisis, peripheral retinal tears, and cystic retinal tufts, may contribute to developing rhegmatogenous retinal detachment (RD).

Axial length influences peripheral retinal degeneration, and the prevalence of RDs rises with increasing myopia. Retinal detachment carries a lifetime risk of more than 20 times greater than emmetropia.26

According to increased vitreous body liquefaction, posterior vitreous detachment happens earlier in severe high myopia. This is a contributing factor in its rising prevalence.27

Management of RD involves a range of surgical techniques, such as scleral buckling, pneumatic retinopexy, and pars plana vitrectomy. The ideal situation is to establish retinal reattachment as quickly as feasible, without retinal displacement, outer retinal folds, discontinuity of the external limiting membrane, ellipsoid zone and interdigitation zone, and with an intact foveal bulge.28

Scleral buckling or pneumatic retinopexy is preferred in pre-presbyopic patients to avoid post-vitrectomy cataract formation.

LASER PROPHYLAXIS FOR LATTICE

The evidence to date supports the prophylactic treatment of all symptomatic tractional tears. It is suggestive for the treatment of large, symptomatic operculated tears, high-risk fellow eyes with nontraumatic giant retinal breaks, retinal breaks with subclinical retinal detachments threatening progression, and retinal breaks prior to cataract surgery.

At best, there is ambiguous evidence to support the preventative treatment of asymptomatic retinal breaks in aphakic and pseudophakic eyes with or without an intact posterior capsule. In phakic eyes with lattice degeneration, severe myopia, and concomitant eye detachments, asymptomatic retinal breaks do not significantly benefit from prophylaxis and should be monitored without treatment.29,30

For predisposed degenerative retinal abnormalities, prophylactic barrier laser photocoagulation must be performed at least two weeks before refractive or intraocular surgery. A retinal examination should be performed regularly to monitor the longterm development or progression of posterior vitreous detachment, retinal tears, retinal detachment, and macular diseases.31-33

To earn your CPD hours from this article, visit mieducation.com/pathological-implications-ofhigh-myopia.

The authors acknowledge the review of, and contributions to this article by Heidi Hunter, the practice owner of Custom Eyecare in Newcastle.

References
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Dr Tanya Trinh BAppSc MBBS FRANZCO FWCRSVS is an Australian ophthalmologist specialising in complex cornea, cataract and refractive surgery. She is a fellow of the Royal Australian and New Zealand College of Ophthalmology and in 2023 became the first Australian female to be awarded a fellowship of the World College of Refractive Surgery and Visual Sciences. She co-directs Australia’s only Keratoprosthesis Service and teaches at the University of Sydney, University of Toronto in Canada, and the University of Queensland.

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Dr Chris Qureshi BSc(Med) MBBS MMed(OphthSc) FRANZCO is a specialist vitreoretinal surgeon with over 13 years’ experience in the field of ophthalmology. He has completed two vitreoretinal fellowships at Westmead Hospital, Sydney and at Oxford University and Oxford Eye Hospital in the UK. Dr Qureshi is a senior staff specialist at Westmead Hospital and participates in research and training of registrars, residents, optometrists, and medical and optometry students. His special interests include treatment of the retina and macula such as retinal detachment, macular hole, epiretinal membrane, age-related macular degeneration, diabetic retinopathy, retinal vein occlusion, and vitreous floaters.