Peripheral myopic degenerations and myopic choroidal neovascularisation

Referral priority: Low or urgent – depending on symptoms and clinical signs

If a patient develops a myopic choroidal neovascularisation (CNV), they must be urgently referred to an ophthalmologist following local guidelines.

Patients with peripheral myopic degeneration and no signs of Posterior Vitreous Detachment (PVD) should be routinely followed up.

Click on one of the cards below to read more about the specific eye condition.

Written by
Marko Lukic
Edited by
Svein Tindlund and Jon Gjelle
Published
June 2023

Sections
01
Introduction

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02
Symptoms

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03
Clinical signs

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04
Diagnostic procedures

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05
Management and treatment

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06
References

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01

Introduction

Pathologic myopia is the leading cause of visual impairment and low vision worldwide.(1-3) The condition refers to degenerative changes in the sclera, choroid, and retinal pigment epithelium (RPE) induced by abnormal axial length elongation in eyes with high myopia. It represents a subgroup of myopia and affects up to 3% of the population.(4) High myopia is defined as a refractive error of at least -6.00D or an axial length of 26.5mm or more.(4) The prevalence of pathologic myopia-related visual impairment has been reported as 0.1%-0.5% in European studies and 0.2% to 1.4% in Asian studies.(5)

Myopic choroidal neovascularisation
There are various clinical manifestations of myopic maculopathy (macular changes due to myopia): diffuse chorioretinal atrophy, patchy chorioretinal atrophy, myopic CNV, lacquer cracks, and atrophy secondary to CNV. Myopic CNV is one of the most severe complications of myopia, regardless of the axial length. It has been reported in 5-11% of patients with pathologic myopia. Most patients develop CNV before the age of 50.(6) Approximately, there is a risk of 35% of developing CNV in the fellow eye.(7) It is presumed that excessive elongation of the globe and posterior pole causes mechanical stress and retinal damage.(8) Another theory presupposes that CNV results from choroidal hypoperfusion secondary to choroidal thinning in myopic patients.(9)

Retinal peripheral degenerations
Retinal degenerations are common lesions involving the peripheral retina, and most of them are clinically insignificant.(10) Those lesions are classified according to several criteria:

  • Location: equatorial, peripheral, or combined
  • Pathomorphology: trophic, atrophic, tractional, or combined
  • Depth of changes: vitreoretinal, intraretinal, chorioretinal
  • The risk for retinal detachment
  • Prognosis: stationary or progressive.(11)

For clinical purposes, the risk for retinal detachment is the most important criterion when we consider peripheral retinal degenerations. Lattice degeneration, degenerative retinoschisis, and cystic retinal tufts can result in a rhegmatogenous retinal detachment.(12) Peripheral retinal changes are often present in myopic eyes.(12)

According to depth, we classify the peripheral retinal degenerations as below:

  • Vitreoretinal degenerations: lattice degeneration, snail-track degeneration, retinal tufts, and peripheral retinal breaks.
  • Intraretinal degenerations: senile retinoschisis, white-without-pressure, dark-without-pressure, peripheral cystoid degeneration, snowflake degeneration, and pearl degeneration.
  • Chorioretinal degenerations: paving stone degeneration and peripheral retinal drusen.
02

Symptoms

Patients with myopic CNV report sudden scotoma in central or paracentral visual fields and consequent drop in vision. Likewise, patients often report symptoms of distorted vision (metamorphopsia).

Retinal peripheral degenerations do not cause visual disturbances. However, as some of them bring an increased risk of retinal detachment, patients with peripheral retinal degeneration may report symptoms related to retinal tear/retinal detachment development (new onset of floaters and flashing lights).

03

Clinical signs

The myopic CNV is almost always a type 2 or “classic” type of CNV membrane. It is relatively small (compared to CNV in age-related macular degeneration (AMD) cases) and usually smaller than 1000 microns. The presence of intraretinal fluid is uncommon. A subretinal haemorrhage often is present along with the membrane.(13) Bear in mind that subretinal haemorrhages in myopic patients may be secondary to lacquer cracks.(14) Those changes represent ruptures of the Bruch’s membrane and may be precursors of the CNV. In any case, subretinal haemorrhage needs to be reviewed by an experienced clinician. The inactive or old CNV becomes pigmented and is noted as a pigmented spot in the posterior pole (called Fuchs spot). The resolution of untreated CNV can end up with the atrophy of the respective retinal tissue.

Lattice degenerations represent localised retinal lesions with thinning of the neurosensory retina and adherent vitreous at the edge of the lesions. Typically, they look like white crossing lines. However, they may be present in radial patterns adjacent to retinal vessels. The lattice degenerations may have pigment, yellow deposits, and small oval atrophic holes. It is important to remember that retinal tears can be near the lattice degenerations and that retinal detachment is found in 14-35% of cases.(10,11)

Snail-track degeneration represents glistening white dots. Some experts consider them the early stage of lattice degeneration. The snail tracks as lattice degeneration represent thinning of the neurosensory retina with adherent vitreous and traction at the edges of the lesion. It is most common in myopic eyes, and retinal tears may be seen in 54% of patients with this type of peripheral degeneration.(10,11)

Peripheral retinal tufts are small, peripheral, focal areas of elevated glial hyperplasia associated with vitreous or zonular attachment and traction. They can be cystic (5% of adults), non-cystic (72% of adults), and zonular (15% of adults). Both cystic and zonular tufts increase the risk of retinal tears.(15)

Senile or degenerative retinoschisis represents the splitting of retinal layers, while retinal detachment is the complete detachment of the neurosensory retina from the retinal pigment epithelium (RPE). It is present in 2-7% of the population, mainly in people who are older than 40 years and hyperopic. It is almost always present in temporal quadrants, and it is asymptomatic. It causes absolute visual field scotoma, while retinal detachment causes relative scotoma. Furthermore, the retinoschisis is not mobile, while the retinal detachment is.(16) Bear in mind that retinoschisis may cause retinal detachment in 0.05-2.5% of cases. In addition, peripheral cystoid degeneration, which represents peripheral microcysts, may lead to retinoschisis.

White-without-pressure and dark-without-pressure are peripheral retinal changes that are benign. They give a white or brown appearance to the peripheral retina. Dark-without-pressure lesions are more posterior than white-without-pressure lesions.

04

Diagnostic procedures

Slit lamp examination – you can easily recognise the myopic CNV son a slit lamp. In most cases, it is represented by central or paracentral subretinal haemorrhage. The peripheral retinal lesions are also easily seen on a slit lamp. For examination of the peripheral retina, you should use a 90D non-contact lens or cornea-contact lens with a wide field.

Colour fundus photography is a great tool to catch most peripheral retinal changes, including the macula. Keep in mind that some cameras have limitations, and when you check the peripheral retina in myopic patients, ask for montage imaging or use ultra-widefield cameras.

Image 1. Colour fundus photo of inferotemporal retinoschisis (at the white arrow). Note that non-corrugated appearance with no signs of retinal tears. Drusen can be seen in the macula.
Image 2. Colour fundus photo of lattice degeneration in the far temporal retina (see small white arrow) and a retinal tear posterior to the lattice degeneration (see red arrow). Within the posterior pole, you can see the presence of lipid exudates (non-related to lattice or retinal tear).
Clinical feature  Rhegmatogenous retinal detachment (RRD Retinoschisis 
Surface  Corrugated  Smooth 
Haemorrhage or pigment  Present  Absent 
Scotoma  Relative  Absolute 

Table 1. The table displays how to recognise rhegmatogenous retinal detachment (RRD) from retinoschisis.

Optical coherence tomography is crucial in diagnosing myopic CNV. The active membrane represents a “fluffy” subretinal hyperreflective material with no fluid. Likewise, this imaging modality becomes more helpful in identifying peripheral lesions (i.e., differentiating retinoschisis from retinal detachment).

Image 3. OCT scan of active myopic CNV. Note a “fluffy” appearance with no presence of intraretinal fluid.
Image 4. OCT scan of the inactive CNV. Note hyperreflective, a well-defined band that indicates inactive myopic CNV.
05

Management and treatment

Patients with active myopic CNV need to be treated urgently. The golden treatment standard is intravitreal injections of anti-VEGF. Several studies (RADIANCE, REPAIR, and MYRROR) have proven the benefits and safety of intravitreal ranibizumab and aflibercept in the treatment of myopic CNV.(3,17,18)

There is controversy in the prophylactic treatment of lattice degenerations or other peripheral lesions that increase the risk of retinal detachment. According to some reports that suggest laser treatment, there is no strong evidence of efficacy in preventing retinal tear formation or developing retinal detachment.(19)

It is important to familiarise yourself with the differentiation between retinal detachment and retinoschisis. Retinoschisis does not require any surgical procedure. Yet, you should regularly monitor it, as it has a risk of developing retinal detachment.

06

References

1 Spaide RF. Staphyloma: part 1. InPathologic myopia 2014 (pp. 167-176). Springer, New York, NY.

2 Ohno-Matsui K. Myopic chorioretinal atrophy. InPathologic myopia 2014 (pp. 187-209). Springer, New York, NY.

3 Ikuno Y, Ohno-Matsui K, Wong TY, Korobelnik JF, Vitti R, Li T, Stemper B, Asmus F, Zeitz O, Ishibashi T, Myrror Investigators. Intravitreal

aflibercept injection in patients with myopic choroidal neovascularization: the MYRROR study. Ophthalmology. 2015 Jun 1;122(6):1220-7.

4 Ohno-Matsui K, Ishibashi T. Pathologic myopia. In: Schachat AP, Wilkinson CP, Hinton DR, Sadda SR, Wiedemann P, eds. Ryan’s Retina. 6th ed. Philadelphia. Elsevier/Saunders; 2018:chap71.

5 Wong TY, Ferreira A, Hughes R, Carter G, Mitchell P. Epidemiology and disease burden of pathologic myopia and myopic choroidal neovascularization: an evidence-based systematic review. American journal of ophthalmology. 2014 Jan 1;157(1):9-25.

6 Silva R. Myopic maculopathy: a review. Ophthalmologica. 2012;228(4):197-213.

7 Ohno-Matsui K, Yoshida T, Futagami S, Yasuzumi K, Shimada N, Kojima A, Tokoro T, Mochizuki M. Patchy atrophy and lacquer cracks predispose to the development of choroidal neovascularisation in pathological myopia. British Journal of Ophthalmology. 2003 May 1;87(5):570-3.

8 Tokoro T. Atlas of posterior fundus changes in pathologic myopia. Types of fundus changes in the posterior pole.

9 Cheung CM, Arnold JJ, Holz FG, Park KH, Lai TY, Larsen M, Mitchell P, Ohno-Matsui K, Chen SJ, Wolf S, Wong TY. Myopic choroidal neovascularization: review, guidance, and consensus statement on management. Ophthalmology. 2017 Nov 1;124(11):1690-711.

10 Lewis H. Peripheral retinal degenerations and the risk of retinal detachment. American journal of ophthalmology. 2003 Jul 1;136(1):155-60.

11https://eyewiki.aao.org/Peripheral_Retinal_Degenerations

12 Bansal AS, Hubbard III GB. Peripheral retinal findings in highly myopic children≤ 10 years of age. Retina (Philadelphia, Pa.). 2010 Apr;30(4 Suppl):S15.

13 Tokoro T. Types of fundus changes in the posterior pole. In Atlas of posterior fundus changes in pathologic myopia 1998 (pp. 5-22). Springer, Tokyo.

14 Ohno-Matsui K, Akiba M, Modegi T, Tomita M, Ishibashi T, Tokoro T, Moriyama M. Association between shape of sclera and myopic retinochoroidal lesions in patients with pathologic myopia. Investigative ophthalmology & visual science. 2012 Sep 1;53(10):6046-61.

15 Byer NE. Cystic retinal tufts and their relationship to retinal detachment. Archives of Ophthalmology. 1981 Oct 1;99(10):1788-90.

16 Reed D, Garg AJ. Degenerative retinoschisis. In: Schachat AP, Wilkinson CP, Hinton DR, Sadda SR, Wiedemann P, eds. Ryan’s Retina. 6th ed. Philadelphia. Elsevier/Saunders; 2018: chap100.

17 Wolf S, Balciuniene VJ, Laganovska G, Menchini U, Ohno-Matsui K, Sharma T, Wong TY, Silva R, Pilz S, Gekkieva M, RADIANCE Study Group. RADIANCE: a randomized controlled study of ranibizumab in patients with choroidal neovascularization secondary to pathologic myopia. Ophthalmology. 2014 Mar 1;121(3):682-92.

18 Tufail A, Narendran N, Patel PJ, Sivaprasad S, Amoaku W, Browning AC, Osoba O, Gale R, George S, Lotery AJ, Majid M. Ranibizumab in myopic choroidal neovascularization: the 12-month results from the REPAIR study. Ophthalmology. 2013 Sep 1;120(9):1944-5.

19 Wilkinson CP. Interventions for asymptomatic retinal breaks and lattice degeneration for preventing retinal detachment. Cochrane Database of Systematic Reviews. 2014(9).