Pathologic Myopia: Kyoko Ohno-Matsui
Pathologic Myopia: Kyoko Ohno-Matsui
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Pathologic myopia
Kyoko Ohno-Matsui
Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo, Japan
Correspondence to: Kyoko Ohno-Matsui, MD, PhD. Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo,
Japan. Email: [email protected].
Abstract: Pathologic myopia is the major cause of the loss of the best-corrected visual acuity (BCVA)
worldwide, especially in East Asian countries. The loss of BCVA is caused by the development of
myopic macula patchy, myopic traction macula patchy, and myopic optic neuropathy (or glaucoma).
The development of such vision-threatening complications is caused by eye deformity, characterized by
a formation of posterior staphyloma. The recent advance in ocular imaging has greatly facilitated the
clarification of pathologies and pathogenesis of pathological myopia and myopia-related complications.
These technologies include ultra-wide field fundus imaging, swept-source optical coherence tomography,
and 3D MRI. In addition, the new treatments such as anti-VEGF therapies for myopic choroid all
neovascularization have improved the outcome of the patients. Swept-source OCT showed that some of
the lesions of myopic maculopathy were not simply chorioretinal atrophy but were Bruch’s membrane holes.
Features of myopic traction maculopathy have been analyzed extensively by using OCT. The understanding
the pathophysiology of complications of pathologic myopia is considered useful for better management of
this blinding eye disease.
Keywords: Pathologic myopia (PM); posterior staphyloma; myopic maculopathy; myopic traction maculopathy (MTM)
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Category 1 Tessellated fundus Well-defined choroidal vessels can be observed clearly around the fovea and
arcade vessels
Category 2 Diffuse chorioretinal atrophy The posterior pole appears yellowish white, extent of which is variable
Category 3 Patchy chorioretinal atrophy Well-defined, grayish white lesions, size variable between 1 and several
choroidal lobules
Category 4 Macular atrophy Well-defined, round chorioretinal atrophic lesion that is grayish white or whitish
around a regressed fibrovascular membrane that enlarges with time. Generally
macular atrophy is centered on the central fovea and has a round shape
Fuchs spot Pigmented spot representing the dry fibrovascular scar of myopic CNV
CNV, choroidal neovascularization.
study classification, PM was defined as the eyes having (META-PM classification; Table 1), myopic maculopathy
chorioretinal atrophy equal to or more severe than diffuse lesions are categorized into 5 categories from “no myopic
atrophy or as the eyes having posterior staphyloma (15,16). retinal lesions” (Category 0), “tessellated fundus only”
(Category 1), “diffuse chorioretinal atrophy” (Category
2; Figure 1A), “patchy chorioretinal atrophy” (category
Complications of PM
3; Figure 1B), to “macular atrophy” (Category 4). Three
PM maculopathy additional features were added to these categories and were
included as “plus signs”: (I) lacquer cracks (Figure 1C); (II)
Curtin and Karlin first proposed a definition of myopic myopic CNV (Figure 1D); and (III) Fuchs spot. The reason
maculopathy that included the features of chorioretinal for separately defining these “plus signs” is that these 3
atrophy, central pigment spot, lacquer cracks, posterior lesions have been shown to be strongly associated with
staphyloma and optic disc changes in 1970 (12). Later, central vision loss, but they do not fit into any particular
Tokoro (17) updated the classification of myopic macular category and may develop from, or coexist, in eyes with
lesions into four categories: (I) tessellated fundus; (II) any of the myopic maculopathy categories described above.
diffuse chorioretinal atrophy; (III) patchy chorioretinal Based on this new classification, PM is defined as myopic
atrophy; and (IV) macular hemorrhage. Subsequently, maculopathy category 2 or above, or presence of “plus”
Avila et al. (18) developed a classification which included sign, or the presence of posterior staphyloma (15,19).
myopic retinopathy according to severity, from M0- Currently, the updated modification of the META-PM
normal-appearing posterior pole; M1-choroidal pallor and classification is going on based on our recent longer follow-
tessellation; M2-M1 changes with posterior staphyloma; up study (>10 years). A longer follow-up data showed that
M3-M2 changes with lacquer cracks; M4-M3 changes with the progression from C3 to C4 is uncommon, and most of
focal areas of deep choroidal atrophy; to the most severe the C4 is myopic CNV-related. In addition, Fuchs spot is
grade M5-M4 changes with large geographic areas of deep pigmented scar of myopic CNV. Not all of scar phase of
chorioretinal atrophy and bare sclera. myopic CNV shows an increased pigmentation. Thus, it
Recently an international panel of researchers in myopia might be better not to separately identify Fuchs spot and
reviewed previous published studies and classifications and better to have active phase and scar phase together under
proposed a simplified, uniform classification system for the category of myopic CNV. Finally, the lesions from C2
PM for use in future studies (14). In this simplified system through C4 are specific to PM, whereas C1 is seen even
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A B
C D
Figure 1 Maculopathy due to pathologic myopia. (A) Diffuse atrophy is observed as yellowish, ill-defined lesion in the posterior fundus.
(B) Patchy atrophy. Areas of patchy atrophy are observed as whitish, well-defined lesions lower to the macula. (C) Myopic choroidal
neovascularization (myopic CNV). Myopic CNV in this case is observed as grayish fibrovascular membrane (arrow). Subretinal bleeding is
observed around the CNV. (D) Lacquer cracks are observed as yellowish, linear lesions (arrows). CNV, choroidal neovascularization.
in mild myopia and C0 is just normal fundus. Thus, new The main feature of diffuse atrophy is a marked thinning
classification is expected to include only the lesions specific (almost absence) of choroid. Optical coherence tomography
to PM (thus, C2, C3, C4, lacquer cracks, myopic CNV) (OCT) shows a marked thinning of the choroid in the
and it is better to call “PM maculopathy”. The updated area of diffuse atrophy (Figure 3). In most of the cases, the
classification based on a longer follow-up study is expected choroid is almost absent except sporadically-present large
to be published soon. choroidal vessels. A presence of outer retina and RPE
even in the area where most of the choroid is gone might
explain a relatively preserved vision in eyes with diffuse
Features of each lesion of PM maculopathy atrophy. Okisaka reported that the choroidal changes in
Diffuse atrophy PM began from the obliteration of precapillary arterioles
or postcapillary venules, then followed by an occlusion
Diffuse chorioretinal atrophy is observed as ill-defined of choriocapillaris. Finally, large choroidal vessels are
yellowish lesion in the posterior fundus of highly myopic also obliterated and the choroid seems to be absent. In
eyes (Figure 1). This lesion begins to appear around the parallel to the vascular changes in the choroid, choroidal
optic disc (peripapillary diffuse chorioretinal atrophy; melanocytes disappear as well. Although a choroid becomes
PDCA) and enlarges with age and finally covers the entire thinned in eyes with tessellated fundus, the degree of
area within the staphyloma (Figure 2). choroidal thinning is much more serious in eyes with diffuse
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A B
Figure 2 Progression from peripapillary diffuse chorioretinal atrophy (PDCA) to macular diffuse atrophy [cited from (20)]. (A) At age 12,
the diffuse chorioretinal atrophy (arrows) is observed temporal to the optic disc. Refractive error is −13.0 diopters (D) and axial length is
28.4 mm. (B) At age 41, the diffuse atrophy has enlarged beyond the macular area (especially in the inferior fundus). Refractive error is −20.0
D and axial length is 30.6 mm.
A B
Figure 3 Extreme thinning of choroid in eyes with diffuse atrophy shown by optical coherence tomography (OCT). (A) OCT image of
emmetropic eye shows normal thickness of choroid; (B) OCT image of eyes with diffuse atrophy shows almost absence of choroid between
retina and sclera.
atrophy. And such disproportionate thinning of choroid show white color. Large choroidal vessels and intrascleral
compared to the surrounding tissue (RPE, outer retina, and vessels seem to course within the area of patchy atrophy. In
sclera) might be a key phenomenon in diffuse atrophy. some cases, retrobulbar blood vessels are observed through
Recently, Yokoi et al. (20) reported that in 83% of the the patchy atrophy with moving according to the gaze
adults with PM had had PDCA in their childhood in a shift. Fluorescein angiogram (FA) as well as indocyanine
long-term follow-up study >20 years since childhood. green angiogram (ICGA) shows a choroidal filling defect in
OCT showed that PDCA was sudden and focal loss of the area of patchy atrophy, suggesting that this lesion is a
peripapillary choroid (21). complete closure of choriocapillaris (17).
By using a recent imaging technology; swept-source
OCT, Ohno-Matsui et al. (22) recently reported that patchy
Patchy atrophy (Figure 3; color, AF, OCT); macular
atrophy was not simply a chorioretinal atrophy but it was a
Bruch’s membrane (BM) defects
hole of BM (Figure 4). In the area of patchy atrophy without
Patchy chorioretinal atrophy is observed as a grayish-white, BM, most of the thickness of choroid, RPE, and outer
well-defined atrophy (Figure 1) (17). Due to an absence of retina are lost and the inner retina directly sits on the sclera.
RPE and most of the choroid, the sclera can be observed This is contrary to the fact that the RPE and outer retina
through transparent retinal tissue, which is considered to are preserved in most of the eyes with diffuse atrophy,
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A B
Figure 4 Defects of Bruch’s membrane (BM) in the area of patchy atrophy (22). (A) Fundus of a right eye shows three areas of patchy
atrophy (wide arrows). Long narrow black arrows show scanned lines examined by optical coherence tomography (OCT). (B,C) OCT
sections across the area of patchy atrophy shows the end of retinal pigment epithelium (black arrows) in a horizontal scan (B) as well as in a
vertical scan (C). An increased light penetrance is observed in the area defective of RPE. The remnants of BM are observed near the edge of
the BM defect (black arrowheads).
although it is not certain if the remaining photoreceptors enlarged in all directions (25). However, it is uncommon for
and RPE function normally in those eyes. extrafoveal patchy atrophy later involves the central fovea.
Patchy atrophy is subclassified into three types; patchy This means that it is rare for patchy atrophy causes the
atrophy which develops from lacquer cracks; P(Lc), patchy central vision loss although this lesion causes a paracentral
atrophy which develops within the area of an advanced absolute scotoma due to a loss of photoreceptors within the
diffuse chorioretinal atrophy; P(D), and patchy atrophy atrophic area.
which can be seen along the border of the posterior
staphyloma; P(St) (23). P(Lc) is considered an enlarged
Lacquer cracks
break of BM at the site of Lc. P(St) is considered a
mechanical break of BM at the staphyloma edge. P(D) is Lacquer cracks are fine, irregular, yellow lines, often
newly-developed BM holes in the area of extremely thin branching and crisscrossing, seen in the posterior fundus
choroid. of highly myopic eyes (Figure 1). Curtin and Karlin (12)
Jonas et al. (24) recently reported that macular BM reported that lacquer cracks were found in 4.3% of highly
defects were found in 30.8% of highly myopic eyes whose myopic eyes. Histologically, the lacquer cracks represent
axial length was ≥26.5 mm histologically. A lack of BM healed mechanical fissures in the RPE-BM-choriocapillaris
defects might be related to the development of macular complex (26).
ICC. Due to a lack of tensile BM in addition to a lack of Lacquer cracks can develop at a relatively early age in
choroid on the thin sclera, the area of patchy atrophy is highly myopic patients (e.g., in the 30’s). The greatest
very fragile against the inner pressure load. The sclera can incidence of lacquer cracks was noted in the age groups
be bowed posteriorly in the area of the patchy atrophy, of 20 to 39 years. Klein and Curtin (27) reported that the
which resembles the intrachoroidal cavitation that mean age of the patients with lacquer cracks was 32 years
develops adjacent to a myopic conus in highly myopic eyes with a range of 14 to 52 years. Tokoro (17) reported that the
(peripapillary intrachoroidal cavitations; peripapillary ICC). frequency of lacquer cracks was low in the patients younger
Ito-Ohara et al. (25) examined the direction of than age 20 and in the elderly, but increases around ages 40
enlargement of patchy atrophy, and found that the patchy and 60 years. The frequency distribution of lacquer cracks
atrophy in marginal lesions of a staphyloma enlarged showed two peaks in the age between 35–39 years and the
toward the macula, and the patchy atrophy in the macula age between 55–59 years.
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A B C
Figure 7 Three phases of myopic choroidal neovascularization (CNV). (A) active phase. CNV surrounded by subretinal bleeding is seen. (B)
Scar phase. Scarred CNV with some pigmentation is seen. (C) Atrophic phase also known as CNV-related macular atrophy.
detachment, lamellar or full-thickness macular hole and/or Thus, most of the studies examining the prevalence of
macular detachment. glaucoma have excluded highly myopic eyes. The papillary
Several mechanisms have been proposed for the and peripapillary regions of highly myopic eyes are distorted
development of MTM, including vitreomacular traction by the mechanical stretching of the globe. The stretching
from partial posterior vitreous detachment (42,43), results in the formation of various kinds of deformities of
epiretinal membrane, intrinsic internal limiting membrane the optic discs (Figure 8) including tilted optic discs (49),
noncompliance (44), and retinal arteriolar stiffness (45). acquired megalodiscs (50-52), and small discs. Nagaoka
Shimada et al. (46) have classified MTM according et al. (50) showed that HM eyes with megalodiscs had a 3.2
to its location and extent from S0 through S4: S0: no times higher risk of having glaucomatous optic neuropathy
retinoschisis; S1: extrafoveal; S2: foveal; S3: both foveal and than HM eyes with normal sized or smaller optic discs.
extrafoveal but not the entire macula; and S4: entire macula. In addition to the mechanical deformity of the optic
Due to the possible mechanisms involved in the disc itself, other factors have been reported to be related
pathogenesis of MTM, vitrectomy is the most common to VF defects in eyes with PM. By using swept-source
treatment to release all retinal tractions, which include OCT, Ohno-Matsui et al. (53) reported that the optic
cortical vitreous and epiretinal membrane. Special surgical subarachnoid space (SAS) appeared to be dilated in the eyes
technique; foveolar ILM (inner limiting membrane) sparing with PM. The dilated area exposed to the cerebrospinal
technique was used in an attempt to reduce the development fluid pressure along with thinning of the posterior eye wall
of post-vitrectomy macula hole, which was a severe may influence the formation of staphylomas and the way in
complication and resulted in poor visual recovery (47,48). which certain diseases, such as glaucoma, are manifested.
In some cases, they also reported a direct communication
between intraocular cavity and the SAS space (53).
Glaucoma and visual field (VF) defects in PM
Pit-like clefts at the outer border of the optic disc or
The results of earlier studies indicated that the high within the adjacent scleral crescent have been observed in
prevalence of glaucoma in highly myopic eyes was a great 16% of PM eyes but none in emmetropic eyes (54). The
concern because the diagnosis of glaucoma was difficult in nerve fiber tissue overlying the pits was discontinuous at
highly myopic eyes and thus it tended to be overlooked. the site of the pits, and this might explain the cause of VF
The VF findings are difficult to interpret in eyes with PM. defects in highly myopic eyes in some cases. In addition,
PM eyes usually have a large conus together with various the loss of the nerve fiber tissue overlying peripapillary
extents of maculopathy, which make the analyses and intrachoroidal cavitation (ICC) (55) was also observed in
interpretations of automated VF examinations difficult. PM eyes, and it is considered as a cause of VF defects (56).
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A B C
Figure 8 The deformity of optic nerve head in pathologic myopia. Due to severe mechanical load onto the optic nerve, severe deformity is
observed and causes visual field defects. (A,B) Acquired megalodisc; (C) extreme tilting of the optic disc.
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Figure 10 Imaging of posterior staphyloma. In wide-field fundus image, the edge of wide and deep staphyloma is seen. In 3D MRI images
from nasally and from the back of the eye, an outpouching of posterior segment of the eye is clearly observed.
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Cite this article as: Ohno-Matsui K. Pathologic myopia. Ann
Eye Sci 2018;3:8.
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