Eye (2016), 1–16

© 2016 Macmillan Publishers Limited All rights reserved 0950-222X/16

www.nature.com/eye

Advances of optical DSC Ng, CYL Cheung, FO Luk, S Mohamed,

REVIEW
ME Brelen, JCS Yam, CW Tsang and TYY Lai
coherence tomography
in myopia and
pathologic myopia

Abstract
The natural course of high-axial myopia is Introduction
variable and the development of patho-logic
Myopia is a major growing public health problem
myopia is not fully understood. Advancements in
worldwide. Pathologic myopia is defined as myopia
optical coherence tomo-graphy (OCT) technology
with complications of the posterior segment
have revealed peculiar intraocular structures in
associated with progressive and excessive
highly
elongation of the globe. It is accompanied by
myopic eyes and unprecedented pathologies that
various degenerative changes in the posterior
cause visual impairment. New OCT findings include
segment structures including the sclera, optic disc,
posterior precortical vitreous pocket and precursor choroid, Bruch’s membrane, retinal pigment
stages of posterior vitreous detachment; peripapillary epithelium (RPE), and neural retina. Pathologic
intrachoroidal cavitation; morphological patterns of myopia has been found to be one of the leading
scleral inner curvature and dome-shaped macula. causes of visual impairment in many developed
Swept source OCT is capable of imaging deeper 1
countries. Asian populations are well known for
layers in the posterior pole for investigation of optic
nerve pits, stretched and thinned lamina cribrosa, having the highest rates
elon-gated dural attachment at posterior scleral of myopia in the world with increasing prevalence
2
canal, and enlargement of retrobulbar subarachnoid and severity rates from population-based studies.
spaces. This has therefore enabled further evaluation The cut-off value of high-axial myopia varied
of various visual field defects in high myopia and the (between refractive error of − 6 to − 8 diopters (D)
patho-genesis of glaucomatous optic neuropathy. or axial length of 26–26.5 mm) among population-
OCT has many potential clinical uses in managing based or histological studies and beyond these
visual impairing conditions in pathologic myopia. values, the prevalence of pathologic myopia
3
Understanding how ret-inal nerve fibers are increases exponentially.
redistributed in axial elongation will allow the Optical coherence tomography (OCT) has been
development of auto-segmentation software for widely used for assessing retina and optic nerve by Department of
diagnosis and monitoring progression of glaucoma. providing quantitative and qualitative assessment of Ophthalmology & Visual
OCT is indispensable in the diagnosis of various macula and retinal nerve fiber layer (RNFL) in the Sciences, The Chinese
conditions associated with myopic traction last decade. Before the use of OCT, the pathological University of Hong Kong,
changes of highly myopic eyes in human could only Hong Kong Eye Hospital,
maculopathy and monitoring of post-surgical
Hong Kong, China
outcomes. In addition, OCT is commonly used in the be studied in enucleated globes and ocular structures
multimodal imaging assessment of myopic choroidal were measured histomorphometrically. Jonas et al
Correspondence:
neovascu-larization. Biometry and topography of the recently reviewed the histological changes of high- DSC Ng, Department of
retinal layers and choroid will soon be validated for 4 Ophthalmology & Visual
axial myopia. In brief, there is profound thinning of
the classification of myopic maculopathy for Sciences, The Chinese
the choroid associated with the loss of University of Hong Kong,
utilization in epidemiolo-gical studies as well as choriocapillaris and RPE. The thinning of the sclera Hong Kong Eye Hospital,
clinical trials. starts at or behind the equator with maximal thinning 147K Argyle Street, Hong
at the posterior pole, and the elongated peripapillary Kong, China
scleral flange (defined as the canal between the optic Tel: +852 29465858;
Fax: +852 27159490.
nerve border and the point where dura mater merges E-mail: [email protected]
with the sclera) and lamina cribrosa is stretched thin
with subsequent decreased distance between the Received: 12 October
Eye advance online publication, 8 April 2016; 2015 Accepted in revised
doi:10.1038/eye.2016.47 form: 29 January 2016
 OCT in myopia
DSC Ng et al
2

retrobulbar cerebrospinal fluid space with the intra-ocular Spectral domain (SD) OCT is the second generation of OCT
pressure compartment. The weakened peripapillary scleral that takes light from the interferometer and passes in through a
flange may have a consequence for the biomechanical grating to separate out the component wavelengths. Using a
stability of the lamina cribosa and the nerve fibers passing Fourier transform it is possible to determine where, and how
through it. Although histological studies have provided strongly, different reflections in the sample arm originated
valuable information regarding the structure of highly from, simultaneously. SD OCT is, therefore, much more
myopic eyes, the examinations were performed after efficient (up to 100 times faster than TD OCT) in extracting
postmortem or after enucleation. Therefore, these results image of all tissue layers at any given light exposure.
might have been influenced by deformation during fixation
and sectioning. With the advent of OCT, in vivo studies of When the peak sensitivity of SD OCT is placed
the structure of highly myopic eyes became possible. posteriorly at the inner sclera, deeper structures such
as the choroid can be seen. Enhanced depth imaging (EDI)
The natural course of high myopia is variable, while some with SD OCT, by positioning the choroid–scleral interface
eyes maintain vision with relatively minor changes; others adjacent to the zero delay, can now provide a non-invasive
9
developed pathologic myopia due to a spectrum of visual way to increased visualization of the choroidal anatomy. It
5 can be performed in commercialized SD OCT instruments by
threatening conditions. OCT has revealed peculiar minute
and subtle intraocular deformities selecting the EDI function in the software.
in highly myopic eyes, which has not been clearly observed in
histological studies or with other in vivo imaging modalities Swept source (SS) OCT is the latest generation of
such as ultrasonography or magnetic resonance imaging OCT that uses a laser that sweeps across a range of
6 wavelengths in an orderly fashion. The interference of
(MRI). It allows the analysis of spatial relationship between
the light from the sample and reference arms
various layers at the posterior pole. OCT studies of altered
biometry and topography of retinal and choroidal layers produces a signal that can be read out in nearly real-time by a
10
enabled evaluation of their correlations with demographics, photodiode. This light source is inherently more
visual function, and fundoscopic observations in pathologic complicated than what is used by SD OCT and the detectors in
7 SS OCT are capable of operating at higher speeds. Lasers used
myopia. Furthermore, OCT is indispensable in the diagnosis
in SS OCT have longer wavelength, which has improved
ability to penetrate through tissue to a greater extent, in
and monitoring of the spectrum of visual impairing 11
particular tissue that contains melanin. SS OCT have
conditions, for example, glaucoma and diabetic macular
enabled high-resolution
edema. This review summarizes current findings on the
images of the vitreous and vitreo–retinal interface; fine
application of OCT as a tool to study pathologic myopia and
delineation of the choroid layer; better visibility of the entire
discuss the clinical implications of these findings as well as thickness of the sclera, lamina cribrosa, and the retrobulbar
future research directions. 11
subarachnoid space.

Principles of OCT Assessment of vitreous by OCT in myopia

Because light travels in high speed (3 × 108 m/s), it is Kishi used fluorescein stain in autopsy eyes to demonstrate
impossible to measure the time-of-flight delay within the that the liquefied lacuna anterior to the macular area was
eyeball with any external measurement system. physiologically found to be present in adults and named it the
However, it is possible to time how long light takes to travel 12
posterior precortical vitreous pocket (PPVP). The posterior
a given distance using its wave-like character. Coherency of wall of PPVP consists of a thin layer of vitreous cortex and its
light is a measure of how one wave of light is correlated anterior wall is vitreous gel. Further studies demonstrated the
with another and coherence length is the distance light presence of PPVPs during triamcinolone-assisted
would need to travel during the coherence time. Images with 13
vitrectomy.
micron-scale level of resolution could be achieved by The in vivo structure and physiological function of PPVP have
comparing the time of flight of the sample reflection with the eluded scientists and vitreoretinal surgeons in the past using
known delay of biomicroscopy and B-scan ultrasonography. TD OCT could
a reference reflection by using interference to find phase only depict the vitreous cortex when it was slightly detached
8 from the retina but failed to show the inner structure of the
differences in light waves. This is the basis for time-domain
(TD) OCT, in which a small portion of tissue is sampled at a vitreous. SD OCT allowed the first visualization of PPVP in
single time point. TD OCT is not efficient because longer vivo but has low sensitivity with limited scan length despite
14
duration of time is needed to acquire the image of the entire EDI OCT. With increased scan length of 12mm and
posterior pole. improved resolution,

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 OCT in myopia
DSC Ng et al
3

visualization of the vitreoretinal interface is enhanced by using paramacular area and progressed to a perifoveal PVD and
15 later vitreomacular separation (Figure 1a). This finding
SS OCT (Figure 1). Itakura et al was able to observe the
correlates well with the clinical observation that PVD occurs
boat-shaped configuration of PPVP and the connecting
in younger age in highly myopic eyes compared with non-
channel within PPVP, which suggested that it may not be an
19
isolated lacunae with possible route to the aqueous humor. myopic eyes.
However, even with extended imaging protocols and en face Residual vitreous cortex has been noted by surgeons in
imaging, SS OCT was still unable to detect the entire superior patients treated for vitreomacular diseases despite the apparent
border of the PPVP in vast majority of subjects and the 20
PVD with a Weiss ring and was called ‘vitreoschisis’
communication between PPVP with the anterior chamber is (Figure 1b). It was observed that vitreous cortex frequently
16–18
not yet fully understood. remained on the retina in highly myopic eyes despite a Weiss
21
ring. Using SS OCT in a case–control study, Itakura et al
SS OCT demonstrated that the size of PPVP increases with found the presence of residual vitreous cortex in 40.5% of
15 highly myopic eyes, compared with only 8.7% of controls.
increasing severity of myopia. Larger PPVP reflects earlier
vitreous liquefaction in high myopia, which may predispose Vitreoschisis may be related to the underlying multi-lamellar
partial and subsequently complete posterior vitreous structure of the posterior wall of PPVP, which can be split into
detachment (PVD). With SS OCT, the precursor stages of separate layers during anomalous PVD. The peculiar structure
PVD in high-myopia subjects were observed for the first time of
in vivo. Before the development of a complete PVD with a PPVP has a key role in various vitreomacular disorders and
Weiss ring, PVD began in the contradicts with the conventional concept that

Figure 1 Optical coherence tomography scans of abnormal vitreoretinal interface. (a) Swept source OCT (SS OCT) has high penetration that
allows enhanced imaging of the posterior vitreous and vitreoretinal interface (outlined by white arrows) in cross sections. The posterior wall of the
posterior precortical vitreous pocket is a thin vitreous cortex attached to the retina. The curvature of the inner sclera of this highly myopic eye is
asymmetrical around the fovea. There is posterior vitreous detachment in the paramacular region just above the steepest point of the curvature and
extending toward the perifovea. (b) SS OCT images of a highly myopic eye with the location of the cross sections indicated by the straight line
lying on the infrared fundus photos on the left side. Top: a full-thickness macular hole with parafoveal vitreous traction (white arrows) in myopic
traction maculopathy. Bottom: another cross-sectional OCT image of the same eye revealed vitreoschsis (white arrow).

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 OCT in myopia
DSC Ng et al
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vitreomacular traction was due to anteroposteriorly oriented when interpreting the macular thickness changes for
vitreous fiber exerting direct traction to the fovea. diagnosing and monitoring of diseases such as diabetic
macular edema or glaucoma in myopic patients.

Retina
Choroid
Contrary to histopathological findings of thinned sclera and
retina in myopic eyes, earlier generations of OCT did not The choroid is a primarily vascular structure responsible for
identify any association between mean macular thickness and delivery of blood and nutrients to the outer retina,
22–24 25 thermoregulation of the retina and secretion of growth factors.
axial length of the eye. Lam et al used TD OCT with
higher axial scanning resolution Given its unique position between the retina and sclera, the
and sampling density, and demonstrated that there were choroid may be a source of scleral growth regulators in
regional variations of retinal thickness within the macular response to such local visual stimuli, making it potentially
28
region that correlated with axial length. The outer ring macular important in emmetropization and axial elongation. The
thickness was found to be reduced with longer axial length, advent of high-resolution SD OCT enabled the evaluation of
while the inner ring macular thickness increased with axial choroidal biometry in vivo, elucidating important information
length. The absence of large blood vessels and optic fibers regarding the choroid in human myopia progression and
could render the peripheral retina less resistant to traction and susceptibility to pathologic myopia. In general, there was good
stretch, and the decrease inter-system reproducibility of choroidal thickness measure-
in peripheral retinal thickness may compensate for the ments between EDI OCT and SS OCT and also between three
stretching force over the entire retina to preserve the central different SD OCT devices: Cirrus HD OCT, Spectralis SD
retinal thickness. This finding is confirmed in population-based 29,30
OCT, and RTVue. Large population-based studies found
study in which retinal thinning with increasing axial length significant correlation between increased age and increased
occurs in the outer macular regions but not in the central 30–34
26
axial length with decreased choroidal thickness.
macular. Furthermore, the diminution
27
Recently, Liu et al used ultrahigh-resolution OCT to
analyze the intraretinal structure changes in myopic eyes and in choroidal thickness with age was approximately the same
developed automated layer segmentation algorithms to study in absolute quantities in highly myopic eyes as in eyes
the thickness of different intraretinal layers. 35
without high myopia. When highly myopic eyes grow with
In the central macular region, only the outer segment of the age, the choroid may become very thin and even completely
receptor layer (outer plexiform layer, myoid, and ellipsoid absent. Chorioretinal atrophy (CRA) in high-myopic eyes
zones) was thickened with increased axial appeared white in fundoscopy due to overlying RPE
length. In the pericentral and peripheral regions, all layers hypoplasia and the underlying sclera became readily visible.
except the ganglion cell and inner plexiform layer were found
to have thickness changes in high myopia. The total thickness Subfoveal choroidal thickness in high myopia was found to
of the peripheral region was significantly less compared with 35–37
be consistently correlated with visual function. Studies
emmetropic controls mainly due to thinner inner nuclear layer, evaluating the choroid in myopic eyes using ultrasonography
combined Henle fiber, and outer nuclear layer. Clinicians and indocyanine green angiography (ICGA) demonstrated that
should be aware of the pattern of regional and intraretinal the density of choroidal vasculature and circulation were
variations of macular thickness in myopia subjects when reduced.
38–40
It is hypothesized that in the process of globe
interpreting the significance of a particular macular thickness
elongation in axial myopia, the choroid may well be stretched
in aiding diagnosis and monitoring of diseases such as diabetic without development of additional vasculature. Progression of
macular edema or glaucoma in myopic patients. choroidal thinning continues with age until some point the
choroid would have difficulty in supplying enough oxygen and
Nevertheless, magnification as a result of change in other metabolites. The compromised choroidal circulation may
refractive power and axial length of the eye can affect the account, in part for the visual function loss that is seen in high
23 myopia.
OCT scanning radius. Magnification is not routinely
corrected in retinal thickness measurements in commercially
available OCT instruments, thus resulting in under or In emmetropic eyes as well as myopic eyes, the
overestimations in the measurement of macular thickness. choroidal thickness varied topographically within the
Furthermore, the current normative databases in commercially posterior pole. The choroidal thickness was noted to
available OCT systems have not taken into account the axial be thinner in the inferior and nasal macula as compared with
length of high-myopia subjects. Clinicians should be aware of 33,41–43
the superior and temporal macula. In high-myopic
the pattern of regional and intraretinal variations of macular eyes, the topographic difference was even more
thickness in subjects with high myopia 33,41
pronounced. The reason for such topographic

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 OCT in myopia
DSC Ng et al
5

variations in choroidal thickness is not fully understood and thickness decreases, curvatures change, emissary openings
this might be related to the regional differences in the widen, and the scleral canal can be enlarged, becomes tilted,
metabolic demands of the retina, pattern of choroidal and distorted. There is also local exacerbation of ocular
vasculature distribution, and position of choroidal watershed expansion manifested as regional out-pouching, which is
zones. Furthermore, EDI and SS OCT have revealed 49
known as staphyloma. Curtin studied the fundoscopy
intrachoroidal cavitation (ICC) that is typically located
immediately inferior to the optic nerve in highly myopic findings of staphyloma formation in myopes and classified
44,45 them into 10 different patterns. Nonetheless, the Curtin
eyes (Figure 2). It was also known as peripapillary
classification is not exhaustive of all types of staphyloma.
46,47
detachment or choroidal schisis. One study reported that More recently, Moriyama
47 50
ICC was found in 4.9% of high-myopic eyes. The overlying et al used high-resolution MRI and three-dimensional (3D)
retina, RPE and Bruch’s membrane complex remained intact rendering to identify additional configurations of staphyloma.
over the region of ICC. This cavitation was created by 3D MRI has the ability to image the entire width of the
expansion of the distance between the inner wall of the sclera staphyloma, which may not fit into the maximum length of an
and the posterior surface of Bruch’s membrane. The process of OCT scan. Sometimes, the steepened curve of the posterior
deformation of sclera during staphylomatous expansion at the sclera caused by axial elongation may be confused with a
inferotemporal portion of the disc in axial myopia may have 51–54
45 staphyloma in some of the OCT studies. Nevertheless,
resulted in the cavitation in the choroid. ICC has been
images obtained from T2-weighted MRI represented the fluid-
reported in the macular region of high myopes and fluid may filled chamber in the eye, which was not exactly the contour of
dissect through the defect underneath the retina resulting in the outer shell of the eye. OCT can be useful in studying the
48
localized retinal detachment (RD) (Figure 2b). biometrics of the sclera, detecting more minute and subtle
deformities within the staphyloma, and allows the analysis of
the spatial relationship between morphology of the retinal and
55
choroidal layers with the protruded sclera.
Sclera and staphyloma
In the process of axial myopia development in humans, there In OCT, the sclera appears as a relatively uniform,
is expansion of the volume of vitreous cavity as well as the hyperreflective structure exterior to the choroid. Age, axial
surface area of the posterior sclera. The scleral wall length, presence of staphyloma, central retinal

Figure 2 Optical coherence tomography (OCT) images of intrachoroidal cavation (ICC). (a) Swept source OCT slice scanned along the line lying
on the infrared fundus photo (left) shows ICC below the optic nerve. Hyporeflective space (white arrow) suggesting an existence of fluid is
observed within the ICC. (b) Enhance depth imaging spectral domain OCT revealed a macular retinal detachment associated with ICC. During
enlargement of the ICC in a highly myopic eye, the overlying retinal tissue develops a full-thickness defect, allowing the vitreous cavity to
communicate directly with the cavity of the ICC. Macular retinal detachment occurs when the communication extends into the subretinal space.

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 OCT in myopia
DSC Ng et al
6

thickness, and choroidal thickness were associated with the retrobulbar blood vessels, which was confirmed by ICGA
55,56 55
visibility of the scleral layer by OCT. A head-to-head findings. Nevertheless, deep vessels and structures were
comparative study reported that the detection rates of posterior only visible in eyes when the amount of CRA was severe.
border of the sclera were 67% using EDI OCT and 78% using Future longitudinal studies are necessary for the understanding
SS OCT, but in eyes with myopia the detection rates dropped of how alterations of intrascleral vascular structures are related
to 31% with EDI OCT and 53% with SS OCT. Therefore, SS to the development of chorioretinal complications or optic
OCT is preferred over EDI OCT for imaging of the sclera. nerve damages in patients with pathologic myopia.

Subfoveal scleral thickness in highly myopic eyes using

OCT has only slight variation between EDI OCT, SS OCT,
55,57 55 Dome-shaped macula
and histological studies. Ohno-Matsui et al studied 488
highly myopic eyes with both 3D MRI and SS OCT and they 61
In 2008, Gaucher et al first described dome-shaped macula
found that the most protruded part of the sclera existed along as inward bulge of the macula within the concavity of a
the visual axis in 78% and inferior to the central axis in 22%. posterior staphyloma in highly myopic eyes based on OCT
The sclera in the lower half of the eye is the area where the observations. Subsequently, with the use of EDI OCT,
embryonic ocular fissure closes, and it may be possible that 62
Imamura et al reported that dome-shaped macula was the
this part of the sclera is structurally weaker and more
result of a localized variation in thickness of the sclera in the
susceptible to elongation. Four patterns of curvatures of the 63
inner sclera were observed: curvatures that sloped toward the macular area. Caillaux et al further used SD OCT and 3D
optic nerve, symmetrical and centered on the fovea, reconstructions to classify the morphology of dome-shaped
asymmetrical around the central fovea (Figure 1a), and macula include round-shape, horizontal oval-shape, and
55 vertical oval-shape. The authors recommended both vertical
irregular (without a circular arc). Irregular curvature was
and horizontal OCT scans to be performed in order to avoid
found to be associated with older age and visual field defects overlooking a dome-shaped macula.
with higher incidence of macular pathology including myopic
traction maculopathy (MTM), myopic choroidal
There are still many uncertainties regarding dome-shaped
55
neovascularization (mCNV), and CRA. When analyses of macula. Postulations for the exact mechanism for its
both 3D MRI images and SS OCT were combined, eyes with development include: resistance to deformation of scleral
temporally dislocated staphyloma might represent a condition staphyloma, scleral infolding through the collapse of the
in which the sclera was extremely thin and became the posterior portion of the eye wall, and tangential vitreoretinal
58
irregular curvature shown by SS OCT. This finding was also traction. There is no population-based study that reported its
correlated with the steeply curved staphyloma observed by prevalence, and it is unclear whether various macular
55 complications reported to occur in eyes with dome-shaped
stereoscopic fundus photography. It is hypothesized that in
highly myopic eyes, the thinned sclera may no longer maintain macula are unique to it or are common complications of highly
the integrity of the globe and results in irregular curvature, myopic eyes regardless of the presence of dome-shaped
which may potentially cause abnormal stresses on RNFL and macula. Visually threatening macular complications have been
vitreomacular interface predisposing to visual threatening suggested to be more frequent in eyes with dome-shaped
55 macula than eyes with low or no myopia, which include serous
conditions.
RD, mCNV, macular holes (MHs), and foveal and extrafoveal
64 65
Emissary blood vessels ordinarily penetrate the eye, often at schisis. Liang et al reported the largest cohort of 1118
oblique angles, and course through the sclera toward the highly myopic eyes and found 225 (20.1%) eyes had a dome-
choroid. With elongation of the eye and stretching of the shaped macula. Younger age and longer axial length were
sclera, these passageways may become much shorter and their positively associated with the presence of dome-shaped
openings also become stretched. The drainage of choroidal macula. The most common morphology was the vertically
veins is mainly though the vortex system that exits the sclera oriented subtype (77.3%), followed
at the equatorial region of the globe. Previous ICGA study
suggested that choroidal venous blood is also drained around by the bidirectional (20.1%) and horizontal subtype (2.6%)
the macula in highly myopic eyes but was unable to be (Figure 3). The rates of foveal and extrafoveal retinoschisis
confirmed by using computer tomographic angiography and serous RD were significantly associated with the presence
59 of a dome-shaped macula. Nevertheless, the rate of serous RD
because of its relatively low resolution. Using SS OCT, the
entire course of a macular vortex vein running through the was highly variable among different studies, ranging from 1.7
60 65
choroid until its exit from the sclera could be observed. SS to 52.1% due to possible selection bias. mCNV had been
OCT was also able to image the lateral long posterior ciliary reported to be another frequent complication of dome-shaped
65
artery, short posterior ciliary arteries, and some macula, with range of 12.2–47.8%. Nevertheless, Liang et
65
al

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DSC Ng et al
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Tilting of the optic nerve is more common among high

66
myopes in large population-based studies. The
terminology used for describing a tilted disc is a
misnomer, as it refers to the optic disc being rotated in the
transverse plane from a two-dimensional fundoscopic view. In
the process of globe elongation, there are
three possible axes of rotation for the optic nerve head:
horizontal axis, vertical axis, and torsional axis. Usually, the
temporal portion of the nerve is more posterior than the nasal
portion. In the past, there was an attempt to measure the angle
of optic disc tilting by measuring the ratio between the
minimum and maximum diameter of the optic nerve in fundus
photo. However, this index was not comparable among
patients because the optic disc dimensions measured in fundus
photography may not be the true dimensions of the optic
67
discs. The true angle of tilt can now be measured using
68
OCT.
Although the size of optic discs in normal eyes showed
significant variation, its relationship with axial length and
myopia is still a matter of contention. Two groups of
investigators using SD OCT reported an inverse correlation
between optic discs size with either refractive error or axial
length, but the authors did not perform image size corrections
69,70
for magnification variations. In a population-based study,
71
Jonas and colleagues divided those with large optic discs
into primary and secondary subtypes. The primary macrodiscs
had no relationship with refractive error while the secondary
type, generally observed in refractive error of − 8D or greater,
had increasing size with increasing myopia. Enlargement of
the optic nerve head in high myopes occurred due to
expansion and stretching of the scleral canal and the lamina
cribrosa. OCT was able to observe the acquired pits of the
Figure 3 Cross section enhanced depth imagining spectral domain
OCT images with their orientation indicated by the thick straight lines
optic nerve, dehiscence of the lamina cribrosa, expansion of
lying on the left side fundi images. Top: vertically oriented dome- the dural attachment posteriorly with enlargement of the
shaped maculopathy. Second from top: horizon-tally oriented dome- subarachnoid spaces immediately behind the eye and
shaped maculopathy. Third from top and bottom: a bidirectional type expansion of the circle of Zinn-Haller, with potential
dome-shaped maculopathy. A small juxtafoveal pigmented epithelial compromised circulation into the prelaminar portion of the
detachment is shown in the vertical orientation. optic nerve (Figure 4).

OCT was able to image the dilated subarachnoid space

performed multivariate analyses and showed that the overall
around the exit of the optic nerve in highly myopic eyes in
rate of mCNV was significantly associated with age but not
vivo. The subarachnoid space was triangular, with the base
with the presence of dome-shaped macula. toward the eye surrounding the optic nerve in the region of the
scleral flange. Hypothetically, the expanded area of exposure
Optic nerve head to cerebrospinal fluid pressure along with thinning of the
posterior eye wall may influence staphyloma formation and the
The anatomic changes in the optic nerve head and pathogenesis of glaucoma.
surrounding structures in high myopia are becoming more Similar to the abnormal expansion in size of the scleral
readily evident by OCT imaging. There is altered mechanical emissaries, analogous abnormalities have been observed in the
stress on the nerve fibers and compromised prelaminar region centered around the optic nerve. Using SS OCT, Ohno-
perfusion in myopic eyes. However, the functional changes 55
Matsui et al observed pit-like changes in the region of the
induced by the structural changes and the possible conus similar to those in the macular region in 16.2% of
pathophysiologic mechanisms are still being researched. highly myopic eyes. There was absence of RNFL tissue
overlying the pits, and this discontinuity

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DSC Ng et al
8

cause a shift of RNFL entering the optic nerve head. In these

eyes, the automatic segmentation protocols used in
commercially available OCT machines could not accurately
measure the thickness of the RNFL, and therefore highly
myopic patients were among the most
7,72,73
difficult patients to evaluate for glaucoma (Figure 5).
The pattern of RNFL distribution was altered in high myopes
with thinner average, superior, nasal, and inferior but thicker
temporal nerve fiber layer thickness and a temporal shift in the
73
superior and inferior peak locations. Torsion of the optic
disc occurs when it is rotated along the coronal plane, and the
direction of rotation is more commonly counterclockwise such
that the superior aspect of the long axis is rotated temporally
when viewing the right eye. The RNF from the temporal
periphery courses around the central macula, converging on
the optic canal either superiorly or inferiorly. Using Cirrus SD
7
OCT, Leung et al measured the angle between the
superotemporal and inferotemporal RNFLf bundles, and
reported decreasing magnitude of the angle with increasing
axial length. As the normative database of the Cirrus OCT
largely comprises data collected from normal eyes with no or
low myopia, interpreting RNF layer thickness deviation maps
in highly myopic patients was likely to be inaccurate. An
alternative could be to measure the cell bodies instead of the
axons. The ganglion cell complex can be visualized,
segmented, and measured in eyes by SD OCT, but further
longitudinal, large-scale studies are necessary to validate its
74–76
translation into clinical practice.

Myopic traction maculopathy

77
The term MTM was proposed by Panozzo and Mercanti in
2004 to unify all of the abnormal OCT features generated by
Figure 4 Enlargement of the optic nerve head in highly myopic eyes traction in pathologic myopia. Although some of the clinical
occurs due to stretching of the scleral canal and lamina cribrosa. The manifestations of MTM might be observed on
lamina is torn from the peripapillary sclera and eventually the ophthalmoscopic exam, OCT is necessary for its diagnosis.
overylying nerve fiber is disrupted, and this stage is observed as optic Eyes with MTM include those with vitreomacular traction
disc pits, especially at the superior and inferior poles of the optic disc. (Figure 1a), epiretinal membrane, macular retinoschisis,
Top: optical coherence tomography (OCT) shows a hyporeflective gap lamellar MH, and MH (Figure 1b) with or without RD. Using
indicating the acquired pit of the optic nerve (white arrow). Middle: SD OCT, macular retinoschisis could be observed as the
OCT shows the subarachnoid spaces as hyporeflective triangular
splitting of the inner retina from the outer retinal layers with
spaces along both the upper and lower borders of the optic nerve
(white arrows). The elongated dural attachment at posterior scleral
multiple columnar structures connecting the split retinal
78
canal in highly myopic eye leads to widening of retrobulbar layers. The splitting of the outer retina appeared to occur
subarachnoid spaces comparing with that observed in an ametropic between the outer plexiform layer and the outer nuclear
eye (Bottom). 78
layer. The columnar structures corresponded to the retention
may account for visual field defects observed in highly of the Henle’s nerve fiber layer. Retinoschisis may also be
myopic eyes. present at the level of the inner plexiform layer or an inner
78
The peripapillary region of large optic discs secondary to limiting membrane (ILM) detachment. Macular RD may
high myopia invariably has prominent parapapillary atrophy sometimes coexist with macular retinoschisis, which may also
that involves the choroid, RPE, and outer retina. Furthermore, 79
be accompanied by an outer lamellar MH.
rotations of the optic nerve are expected to

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DSC Ng et al
9

Figure 5 Retinal nerve fiber layer (RNFL) analysis by Cirrus optical coherence tomography (OCT) shows abnormally reduced thickness in inferior
sector of the right eye and superotemporal and inferotemporal sectors in the left eye of a high myopia patient. In glaucoma eyes, there is
predilection of inferior and superior RNFL loss. However, the normative database of Cirrus OCT only comprises data collected from normal eyes
with no or low myopia. The interpretation of RNFL thickness deviation may need to account for its altered topographical distribution in highly
myopic eyes.

The occurrence of MTM was associated with increased with patchy CRA, and the sclera immediately underneath the
axial length and macular CRA. Recent studies using SS OCT ICC was bowed posteriorly. Sclera with an irregular inner
have evaluated the relationship between scleral curvature curvature more commonly had macular retino-schisis. These
48 observations suggested that the scleral contour affects the
alterations and the pathogenesis of MTM. Ohno-Matsui et al
demonstrated that eyes with macular retinoschisis more development of MTM. Furthermore, OCT showed
frequently had ICC in the macular area paravascular abnormalities including

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DSC Ng et al
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paravascular lamellar holes, vascular microfolds, and 79

Shimada et al recommended surgery in macular
80
paravascular retinal cysts in eyes with MTM (Figure 6). retinoschisis eyes between stage 3 and 4, because delayed PPV
Glial cells that exist abundantly around the retinal vessels can (in stage 4) has the increased risk of developing full-thickness
migrate and proliferate through the paravascular lamellar holes MH postoperatively. The need for ILM peeling is also
to produce collagen and facilitate the proliferative and controversial. ILM peeling is indicated when apparent ILM
contractile response of ILM. traction is recognized on OCT to prevent postoperative
81 83
Shimada et al observed MTM eyes longitudinally with recurrence of MTM. However, ILM peeling may increase
OCT and proposed that the progression from macular the risk of postoperative full-thickness MH, because peeling
retinoschisis to RD passed through four stages: (1) a focal may induce breaks within the already thinned and weakened
irregularity of the thickness of external retina, (2) an outer 84
central foveal tissue. A fovea-sparing ILM peeling
lamellar MH development within the thickened area and technique has been proposed, which enabled release of
subsequent development of small RD, (3) horizontal macular traction, reduced risk of surgical trauma to the central
separation of the column-like structures overlying the outer fovea, and centripetal contraction of the remaining ILM to
lamellar hole and vertical enlargement of outer lamellar hole, 85
prevent postop MH formation. Posterior scleral
and (4) elevation of the upper edge of
reinforcement surgery, intraocular expansible gas, and prone
the external retina and the attachment to the upper part of
posturing has been reported to treat macular retinoschisis in
retinoschisis layer accompanying with further enlargement 86
81 MTM. OCT is also an indispensable tool for monitoring of
of RD. Shimada et al also analyzed the largest cohort of
postoperative MTM patients (Figure 7).
MTM eyes to-date with follow-up of more than 24 months
and reported their variable natural courses. Of the 207
eyes, 11.6% experienced progression of MTM, and eyes
with more extensive macular retinoschsis were at Other types of MH and RDs in pathologic myopia
significantly higher risk
for progression. Around 4% of eyes showed a decrease or MH has been observed at the border between an old CNV and
complete resolution of the macular retinoschisis, which may the surrounding CRA in which RD developed in 89% of eyes
occur after spontaneous release of traction on the retina. In with complete PVD, and therefore, the mechanism of MH may
82 87
another study on the natural history of MTM, Tanaka et al not be completely due to vitreomacular traction. Macular
reported that the presence of a lamellar MH might be a RD has been observed in highly myopic eyes with
relatively stable condition in MTM eyes. 87
peripapillary intrachoroidal cavity. OCT revealed the
The indication and the optimal timing for pars plana communication between the ICC with vitreous cavity through
vitrectomy (PPV) in MTM eyes without RD is unknown. a full-thickness tissue defect in the

Figure 6 Optical coherence tomography (OCT) scans of myopic traction maculopathy (MTM). (a) A patient with MTM developed full-thickness
macular hole (white arrow) in swept source OCT. (b) A horizontal OCT slice of the same eye revealed a paravascular retinal cyst (white arrow)
associated with MTM.

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DSC Ng et al
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Figure 7 Left: fundus photo of the posterior staphyloma and chorioretinal atrophy. Second from left: spectral domain optical coherence
tomography (SD OCT) reveals retinoschisis with MH. Note that the sclera is strongly bowed posteriorly and the curve is symmetrical around the
fovea. The patient underwent posterior vitrectomy with internal limiting-membrane peeling and gas tamponade. Third from left: SD OCT 3
months post operation shows that the macular gradually flattened. Right: SD OCT at 4 years after operation. The MH remains closed.

overlying retina into the subretinal space, which may be 93

care of managing mCNV. High myopes frequently have
associated with macular RD. small areas of altered pigmentation in the posterior pole. Small
As for macular retinoschisis, there is no standard technique hyperpigmented spots are usually flat and do not have a
for treatment of MHRD. In general, the surgery proposed for surrounding area of atrophy. True CNV causes an elevation at
MHRD is vitrectomy, removal of adherent vitreous cortex, the level of the RPE. Multifocal choroiditis and panuveitis
removal of ERM, fluid–gas exchange, and intraocular gas or cause grayish-white inflammatory lesions at the level of the
silicone oil tamponade. Kuriyama et al reported the inverted RPE that can have associated subretinal fluid during the acute,
ILM flap technique in which the ILM was not removed 94
active phase. OCT shows the inflammatory lesions to be
completely from the retina during vitrectomy but was left 94
conical elevations of the sub-RPE. Clues that the eye
80
attached to the edge of the MH. The ILM was then harbors multifocal choroiditis are multiple lesions in the
massaged gently over the MH so that the MH was covered fundus with clinically evident inflammatory cells.
with the inverted ILM flap.
Intravitreal injection of anti-vascular endothelial growth
factor (anti-VEGF) is the current treatment of choice for
Myopic choroidal neovascularization
mCNV patients and OCT was indicated in the RADIANCE
In patients with mCNV, OCT can show exudative features and MYRROR studies, both multicenter randomized
including intra- or subretinal fluid, and the hyperreflective controlled Phase III trials for the monitoring of disease activity
lesion located beneath the neurosensory retina representing the 95,96
after treatment (Figure 8). When a treated lesion shows
mCNV. The mCNV can cause the contour of the RPE cessation of activity, the lesion becomes more compact, the
monolayer to elevate. Small hemorrhages are usually not easy internal reflectivity is often less than the surface, the boundary
to visualize with OCT, which may also be associated with between the lesion and retina is sharp, and there is no
lacquer crack instead of the CNV. Because of the typically associated intra- or subretinal fluid. When the lesion becomes
minimal amount of exudation associated with myopic CNV, active, any
the presence of subretinal or intraretinal fluid in OCT may not
of these parameters may change. Besides baseline visual
be a sensitive and reliable imaging marker for new onset or
acuity, size of the CNV and the presence of CRA, the
88–91 90
recurrent myopic CNV. Leveziel et al compared the subfoveal choroidal thickness have been shown to be
use of FA with OCT in a cohort of 90 eyes for the detection of prognostic factor of mCNV recurrence. It is possible that
new onset mCNV and reported that FA leakage was observed reduced choroidal blood flow in the subfoveal area predispose
in 82% of cases, while exudative sign on SD OCT was only 97
to the development of mCNV. Recently, subretinal
observed in 48.6% of cases. These findings suggest that FA
hyperreflective exudation imaged by OCT
may be more sensitive in the diagnosis of active mCNV and in mCNV patients has been suggested in monitoring response
the confirmation of active myopic CNV still largely relies on
to anti-VEGF agents by both qualitative (regression) and
90,91
dye leakage detected in FA. The discordance between FA quantitative (thickness) assessments. The typical type 2 CNV
and SD OCT findings has not been clearly reported in CNV in pathologic myopia grows under the RPE and penetrates the
92 Bruch membrane to extend into the subretinal space, which
associated with age-related macular degeneration.
may facilitate the deposition of these hyperreflective lesions
Nonetheless, SD OCT are useful in detecting various
98
differential diagnoses of mCNV which include MH, small into the subretinal space. The presence of a subretinal
focal areas of CRA or scarring, and inflammatory conditions hyperreflective exudation on SD OCT could help in assisting
such as multifocal choroiditis and panuveitis and should be the decision on whether to perform FA or not, and making
included in the standard of decision on retreatment.

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DSC Ng et al
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99
myopia study group in 2015. The new system adopted the
fundus photo classification based on long-term observational
studies that is reliable and convenient. It has five categories of
myopic maculopathy including: no myopic retinal
degenerative lesion, tessellated fundus, diffuse CRA, patchy
CRA, and macular atrophy. Three additional ‘plus’ features
were lacquer cracks, mCNV, and Fuchs spot. A posterior
staphyloma may or may not be limited to the macular area and
its grading is considered in a separate category.

Spaide analyzed the features of tessellated fundus with EDI

100
OCT and reported reduction of mean choroidal thickness.
Attenuation of choroid might occur with RPE hypoplasia that
allows the underlying choroidal vessels to be more visible,
thereby causing the appearance of tessellated fundus. The
attenuation is one of the earliest visible signs in eyes with high
myopia, which begins to develop around the optic disc and the
area between the optic disc and the central fovea. Lacquer
cracks are fine, irregular yellow lines, often branching, and
crisscrossing. OCT may detect the discontinuity of Bruch’s
101
membrane and the underlying increased penetrance.
Nonetheless, it is seldom visualized by OCT because lacquer
cracks are narrow. Diffuse CRA is an ill-defined yellowish-
white atrophy on fundus photography. OCT shows marked
thinning or even absence of choroid in the area of diffuse
CRA. Large choroidal vessels may be observed to protrude
toward the retina. It is interesting to observe that even in area
where most of the choroidal layer is absent, the outer retinal
Figure 8 Optical coherence tomography (OCT) scans of myopic layer may still be present. This might explain the relatively
choroidal neovascularization (CNV). Top: SD OCT reveals intra-and 100
preserved vision in some eyes with diffuse myopic CRA.
subretinal fluid associated with an active myopic CNV. Bottom: after When diffuse CRA is only present around the optic disc, OCT
treatment with intravitreal anti-VEGF injection, there is resolution of
is useful in differentiating CRA from peripapillary ICC
both intra- and subretinal fluid. The lesion becomes more compact and
45
the boundary between the lesion and retina becomes apparent. because of their similar ophthalmoscopic appearance.

Nevertheless, multimodal imaging is necessary to Patchy CRA is represented by a grayish-white well-defined

distinguish it from other causes of hyperreflective atrophy on fundus photo. Based on long-term natural course
subretinal lesions such as hemorrhage and fibrosis. study of eyes with pathologic myopia, three types of patchy
The subretinal hyperreflective exudation correlates to the atrophy were observed: patchy atrophy that develops from
fundus photography observation of a subtle yellowish material lacquer cracks, patchy atrophy that develops within the area
deposit and autofluorescence imaging showing it as an of an advanced diffuse CRA, and patchy atrophy that can be
isoautofluorescent lesion. 102
seen along the border of the posterior staphyloma. For the
first two types, OCT images show a loss of photoreceptors,
RPE, and inner choroid in the area of patchy atrophy in the
Myopic maculopathy and CRA
macular area, while the inner retina appeared to be attached
Myopic maculopathy was characterized by ophthalmoscopic 48
directly to the sclera. Hence, despite the loss of outer retinal
findings of various retinal and choroidal lesions associated tissue, the inner retinal layer is maintained in the area of
with excessive axial elongation of the globe. Owing to a lack patchy atrophy. A recent OCT study that evaluated patchy
of common classification scheme, a direct comparison of the atrophy along the edge of
incidence or prevalence and the burden of myopic
maculopathy in epidemiological studies has not been possible. posterior staphyloma has revealed severe thinning of both
Hence, an international classification and grading system for inner and outer retinal layers, which was not observed in the
myopic maculo-pathy was published by the meta-analysis for 103
two other types of patch atrophy. It could be possible that
pathologic the entire retina is bent and thinned along

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DSC Ng et al
13

the steep edge of the staphyloma, which predisposes the Conflict of interest
development of patchy CRA and subsequently the inner retina The authors declare no conflict of interest.
may be disrupted mechanically. There is also visual field
defect corresponding to the course of the RNF layer across the
area of patchy atrophy. Nevertheless, it is not known whether Acknowledgements
such defect is due to genuine glaucoma, or due to the The work has not been previously presented.
disruption of the nerve fibers overlying the patching atrophy,
103
or caused by refractive scotoma.
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