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Myopic Maculopathy

Introduction

High myopia is characterised by a refractive error of at least -6.00D, or an axial length of over 26.5mm
- Pathological myopia is the excessive axial elongation associated with myopia, which leads to structural changes in the posterior eye that can lead to loss of BCVA.
- Posterior staphyloma is a key feature that contributes to pathological myopia.
- The exact pathogenesis is unclear, but it is proposed that the stretching of the sclera leads to mechanical stress.

Dead Giveaways

Posterior Staphyloma:

  • Is a deformity or outpouching of posterior globe.

  • Is a hallmark of pathological myopia, typically leading to a worse prognosis

  • This predisposes the retina and optic nerve to mechanical damage.

  • There are 5 main types of staphyloma, with an additional 5 types (6-10) being combinations of the first 5.

    • Type 1: Wide staphyloma

    • Type 2: Papillomacular staphyloma

    • Type 3: Peripapillary staphyloma

    • Type 4: Nasal

    • Type 5: Inferior

  • The staphylomas often have such excessive bulging that it shows diffuse choroidal atrophy with visible vessels.

    Example of staphyloma
    Example of staphyloma
Staphyloma OCT
Staphyloma OCT

Atrophic Maculopathy:

Progressive chorioretinal degeneration at posterior pole which coalesce to become geographic. This can be thought of as the myopic equivalent of atrophic AMD. Describes the progression of atrophy in 5 separate stages.

A0:

  • No myopic retinal lesions

A1:

  • Tessellated fundus only, secondary to thinning of the IP layer, leading to the increased visibility of the choroidal vessels

  • Low progression chance (15%)

    Tessellated Fundus.
    Tessellated Fundus.
    OCT.
    OCT.

A2:

  • Diffuse chorioretinal atrophy. Can spread to affect entire staphyloma

  • Yellow-white appearance of posterior fundus

  • 50% progression chance

    Chorioretinal atrophy (diffuse) fundus.
    Chorioretinal atrophy (diffuse) fundus.
    OCT. Shows thin choroid and obvious underlying sclera
    OCT. Shows thin choroid and obvious underlying sclera

A3:

  • Patchy chorioretinal atrophy

  • Well-defined white grey areas of atrophy. Loss of RPE and choriocapillaris increases signal transmission

  • 70% progression chance

    Myopic maculopathy causing patchy atrophy.
    Myopic maculopathy causing patchy atrophy.
    OCT breakage, as well as increased transmission through to the choroid
    OCT breakage, as well as increased transmission through to the choroid

A4:

  • Complete macular atrophy. Complete loss of layers of the macular which can lead to significant loss of vision. Either due to:

    • Patchy atrophy coalescence and enlargement

    • CNV related as a secondary cause

  • Complete macular atrophy.
    Complete macular atrophy.
    Can go through an active phase, scarring phase and finally atrophic.
    Can go through an active phase, scarring phase and finally atrophic.

Tractional Maculopathy:

Traction in myopic maculopathy can be cause retinal, preretinal and subretinal tractions.

  • Retinal:

    • ILM rigidity - poor ILM is inability to adapt to changes in elongation.

    • Choroidal atrophy - poor blood supply to retina and subsequent adhesion between layers.

    • RPE atrophy

  • Preretinal:

    • Anomalous PVD

    • VMT, ERM

  • Subretinal:

    • Traction from staphyloma

T0:

  • No macular schisis

T1:

  • Inner or outer foveoschisis

  • Inner = affecting IPL, GCL, or RNFL

  • Outer = affecting OPL or ONL

T2:

  • Inner AND outer foveoschisis

T3:

  • Foveal detachment (neurosensory detachment from RPE)

T4:

  • Full thickness macular hole (associated with foveoschisis)

T5:

  • Macular hole and retinal detachment. IMMEDIATELY REFER


Maculoschisis Grading:

S0-3 have a 3-13% of progression, whereas S4 has a 42.9% of progression
S0-3 have a 3-13% of progression, whereas S4 has a 42.9% of progression

  • S0 = absent

  • S1 = extrafoveal

  • S2 = foveal only

  • S3 = fovea but not entire macula

  • S4 = entire macula


Tractional Features:

  1. Vascular Microfolds (20-44.6%)

    1. Retinal vessel inflexibility causes vitreous traction on retina

    2. Inward displacement of tissue around the vessels, thought to be due to the retinal inflexibility

      Appears on the surface of OCT, surrounding the vessels
      Appears on the surface of OCT, surrounding the vessels

  2. Paravascular retinal cysts (up to 50% of myopic eyes)

    1. Small hollow spaces mainly around large retinal vessels

      Appears like cystic spaces
      Appears like cystic spaces

  3. Paravascular lamellar holes (26.8%)

    1. May be due to detachment of the posterior hyaloid or rupture of the paravascular retinal cyst (cyst unroofing)

      A form of a pseudohole
      A form of a pseudohole

  4. ILM detachment (2.4-6%)

    1. Rigid ILM prevents retina from stretching to follow staphyloma

      Shows schisis and ILM detachment
      Shows schisis and ILM detachment

Neovascular Maculopathy:

Myopic CNV commonly arise from lacquer cracks (29%), patchy chorioretinal atrophy (20%) and diffuse chorioretinal atrophy (4%), and can reoccur at a different location especially with lacquer crack progression.


N0: [Coin Haemorrhage]

  • Myopic maculopathy can present without CNV, but still present with haemorrhages.

  • This is often due to the rupture. Is often associated with lacquer cracks

    Liu B et al. 2018. Arrow indicates location of cion haemorrhage. Faintly underneath is the lacquer crack
    Liu B et al. 2018. Arrow indicates location of cion haemorrhage. Faintly underneath is the lacquer crack

N1: [Lacquer Cracks]

  • The lacquer cracks are yellow-white lines at the macula. They represent ruptures in the Bruch's membrane caused by stretching and elongation. This breakage allows vessels to easily enter the choroid

  • Prevalence of 5-15% in pathological myopia

  • Associated with CNV or subretinal haemorrhage

    Lacquer Crack appearance
    Lacquer Crack appearance
    The cracks appear as a hyper-reflective line/column that extends into the choroid and sclera. This may come with a defect in Bruch's membrane
    The cracks appear as a hyper-reflective line/column that extends into the choroid and sclera. This may come with a defect in Bruch's membrane

N2a: [Active Phase]

  • Is characterised by the presence of active growth, identifiable on both OCT-A and OCT

  • Mixed homogenous and heterogenous reflectivity indicate potential neovascular material.
    Mixed homogenous and heterogenous reflectivity indicate potential neovascular material.
    From the eye-wiki (AOA). Shows the presence of neovascular and messy vessels in both the avascular zone, and massive proliferative growth in the choriocapillaris.
    From the eye-wiki (AOA). Shows the presence of neovascular and messy vessels in both the avascular zone, and massive proliferative growth in the choriocapillaris.
    A big giveaway is haemorrhaging over the macula. The macula being avascular should not have blood vessels which can cause haemorrhages. This thus reveals myopic maculopathy N2a
    A big giveaway is haemorrhaging over the macula. The macula being avascular should not have blood vessels which can cause haemorrhages. This thus reveals myopic maculopathy N2a

N2s: [Scarring Phase]

  • Characterised by the regression of the blood vessels on OCT, but also the Forster Fuch's spot, which is a pigmented patch.

  • The consequence leads to atrophic changes, especially A4

    Qiuying Chen et al. 2019. Shows the Fuch's spot and the loss of RPE and Bruch's membrane. The large red arrow pointing to the messy heterogenous mix of hyper/hypo reflectivity shows the scarred and regressed CNV. The loss of RPE and Bruch's membrane around it shows the atrophic consequence of CNV (A4)
    Qiuying Chen et al. 2019. Shows the Fuch's spot and the loss of RPE and Bruch's membrane. The large red arrow pointing to the messy heterogenous mix of hyper/hypo reflectivity shows the scarred and regressed CNV. The loss of RPE and Bruch's membrane around it shows the atrophic consequence of CNV (A4)

Dome Shaped Maculopathy:

  • A convex curved shape elevated profile that is within the concavity of a posterior staphyloma.

  • Has a prevalence of 10-20%, and is strongly associated with increasing myopia and axial length

  • Has 3 main types:

    • Horizontal Oval: Identified on vertical OCT scan, but not horizontal

    • Vertical Oval: Identified on horizontal OCT scan, but not vertical

    • Round: Identified on BOTH horizontal OCT scan and vertical

  • In a horizontally oriented oval, the horizontal line scan will depict a normal retina. But on the vertical OCT, both concave staphyloma and convex dome can be visualised
    In a horizontally oriented oval, the horizontal line scan will depict a normal retina. But on the vertical OCT, both concave staphyloma and convex dome can be visualised
    Garcia-Ben A, et al. 2017. Displays what the horizontally oriented dome would look like in 3D.
    Garcia-Ben A, et al. 2017. Displays what the horizontally oriented dome would look like in 3D.
    An opposite effect is seen in the vertically oriented dome, in which case the vertical line scan will depict a normal retina, but on horizontal OCT, both concave staphyloma and convex dome can be visualised. There is also serous detachment.
    An opposite effect is seen in the vertically oriented dome, in which case the vertical line scan will depict a normal retina, but on horizontal OCT, both concave staphyloma and convex dome can be visualised. There is also serous detachment.
    Garcia-Ben A, et al. 2017. Displays what the vertically oriented dome would look like in 3D.
    Garcia-Ben A, et al. 2017. Displays what the vertically oriented dome would look like in 3D.

diagnostic features

Atrophy Myopic Maculopathy:

  • The natural history progresses as: Tessellated fundus --> diffuse atrophy --> patchy atrophy --> macular atrophy

  • Common pattern of progression:

    • Patchy atrophy enlargement and fusion

    • Diffuse atrophy enlargement

    • Diffuse --> patchy atrophy

    • Tessellated fundus --> diffuse atrophy

  • Risk of Progression

    • A1 Tessellated fundus --> 15:85 chance

    • A2 Diffuse atrophy --> 50:50 chance

    • A3 Patchy atrophy --> 70:30 chance

    • Age, female gender, degree of myopia, axial length, presence of posterior staphyloma, lacquer cracks at baseline, worse BCVA baseline.


Tractional Myopic Maculopathy:

  • The natural history typically is stable. In fact, improvements can usually occur. This is usually due to a complete PVD or spontaneous traction release.

  • Progression is not associated with age or axial length

  • Those with severe macular involvement had a higher progression rate. (Some may progress to foveal detachments or FTMH)

  • Continued monitoring is mandatory.


Neovascular Myopic Maculopathy:

  • Typically the long term prognosis is very poor, and VA in over 95% can drop to 6/60 within 10 years of CNV onset.

  • Chorioretinal atrophy is the main cause of the poor prognosis

  • Furthermore, CNV can develop in fellow eye (35%) within 8 years.

  • If it presents without CNV (N0), it is typically associated with better prognosis.


Dome Shaped Myopic Maculopathy:

  • Complications include myopic CNV, serous detachment and macular schisis

2025, made by Eric Qin. UNSW. SOVS

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