Macular hole

•A macular hole (MH) is a retinal break commonly involving the fovea
•A macular hole is a full thickness defect, or hole, in the neurosensory
retina located within, or just eccentric to the center of the fovea.
•An impending macular hole, also known as a stage 1 macular hole, is
considered the precursor to a full thickness idiopathic macular hole.
Impending macular holes have a splitting of the inner retina with the
clinical appearance of a foveolar cyst or pseudocyst.
•In some cases, this inner splitting can be associated with a defect in
the underlying outer retina (Lee, Kang et al. 2011).

•An impending macular hole with a defect in the outer retina can have a
very thin intact inner retina referred to as the roof of the impending
macular hole. These impending macular holes can progress to become
full thickness macular hole once a break in the inner retina or roof
occurs.
•Most macular holes, unless otherwise specified, refer to idiopathic
macular holes. Idiopathic macular holes occur from tractional forces on
the foveola at the vitreoretinal interface not associated with other
causes.
• Other types of macular holes include those associated with trauma,
high myopia, retinal detachments, lasers accidents, lightning strikes,
diabetic retinopathy, and epiretinal membranes

Etiology and Risk Factors
Idiopathic macular hole is the most common presentation. Risk factors
include age, female gender, myopia, trauma, or ocular inflammation.
Pathophysiology
It has been hypothesized that MHs are caused by tangential traction
as well as anterior posterior vitreoretinal traction of the posterior
hyaloid on the parafovea. MHs are noted to be a complication of a
posterior vitreous detachment (PVD) at its earliest stages

Diagnosis
•This is a clinical diagnosis based on history and clinical exam,
including slit lamp and dilated fundus examination.
In some cases, optical coherence tomography (OCT) is useful in the
diagnosis and management of this condition. It is important to
distinguish between a full-thickness macular hole versus a lamellar
hole (irregular foveal contour with defect in the inner fovea) or
pseudohole (an irregular foveal contour with steep edges without
true absence of retinal tissue often associated with an epiretinal
membrane).

History
Patients with MHs typically present over the age of 60 and females are more
frequently affected. Idiopathic MH occur with an estimated incidence of 8.69 eyes
per 100,000 population per year in one study. A careful history should be obtained
to investigate for any of the risk factors mentioned above.
Physical examination
Slit lamp examination with special attention to the macula is important in the
evaluation of this disorder . The Watzke-Allen sign can be used as a clinical test in
cases of a suspected full thickness macular hole by shining a thin beam of light
over the area of interest. The patient would perceive a “break” in the slit beam in
cases of a positive test.
Careful examination of the fellow eye is also recommended given that MHs are
bilateral in up to 30% of patients.
Special attention should be paid to the vitreoretinal interface, involutional macular
thinning, and retinal pigment epithelial window defects because these are risk
factors for MH development in the fellow eye.
Patients without a PVD in the fellow eye have an intermediate risk (up to 28%) of
developing a MH whereas patients with a PVD are at low risk for developing a MH.

Signs
Depending on the stage of the MH, a subfoveal lipofuscin-color
spot or ring can be noted. In more advanced cases, a partial or full-
thickness macular break is observed.
Symptoms
Metamorphopsia (distortion of the central vision), central visual
loss, or central scotoma can be reported.

Clinical diagnosis
There are two main classification schemes for macular holes. Gass first
described his clinical observations on the evolution of a macular hole:
Stage 1 MH, or impending MH, demonstrates a loss of the foveal
depression. A stage 1A is a foveolar detachment characterized a loss of
the foveal contour and a lipofuscin-colored spot. A stage 1B is a foveal
detachment characterized by a lipofuscin-colored ring.
Stage 2 MH is defined by a full thickness break < 400µm in size. It might
be eccentric with an inner layer “roof.” This can occur weeks to months
following Stage 1 MHs. A further decline in visual acuity is also noted. In
most cases, the posterior hyaloid has been confirmed to be still attached
to the fovea on OCT analysis.
Stage 3 MH is further progression to a hole ≥400 µm in size. Nearly
100% of stage 2 MHs progress to Stage 3 and the vision further declines.
A grayish macular rim often denotes a cuff of subretinal fluid. The
posterior hyaloid is noted to be detached over the macula with or
without an overlying operculum.
Stage 4 MH is characterized by a stage 3 MH with a complete posterior

Figure 1: Clinical photo demonstrating a full thickness macular hole with a grayish macular rim
suggestive of subretinal fluid. Note the retinal pigment epithelial changes at the base of the hole

Full-thickness macular hole showing a surrounding cuff of subretinal fluid

Fundus photograph of a stage 1a macular hole with characteristic yellow spot at the
center of the fovea.

Gass Biomicroscopic Classification
Stage 1a Seen as a yellow spot. This is not specific for macular hole -
can be associated with central serous chorioretinopathy, cystoid
macular oedema, and solar maculopathy.
Stage 1b Occult hole: doughnut-shaped yellow ring (approximately
200-300 μm) centred on the foveola. Approximately 50% of holes
progress to stage 2.
Stage 2 Full thickness macular hole (<400 μm). Prefoveolar cortex
usually separates eccentrically creating a semi-transparent opacity,
often larger than the hole, and the yellow ring disappears. These
generally progress to stage 3.
Stage 3 Holes >400 μm associated with partial vitreomacular
separation.
Stage 4 Complete vitreous separation from the entire macula and
optic disc

Figure 3: This figure demonstrates the
stages of macular holes based on the
1995 paper by J. Donald M. Gass.(9) This
figure shows the range of pathology
between cystic changes (Stage 1) to full
thickness defects with a complete
posterior vitreous detachment (Stage 4)
Gass Stages:
Stage 1 - cystic foveal change
Stage 2 - 100-300 micron full-thickness
retinal defect with pseudo-operculum
Stage 3 - 250-600 micron full-thickness
retinal defect with a persistent hyaloid
attachment
Stage 4 - stage 3 with complete PVD

Figure 2. Stages of macular hole as seen by
optical coherence tomography. A) Stage 1B
occult hole, vision 20/40. B) As the
pseudo-operculum lifts, the hole goes to
stage 2 and becomes apparent on clinical
examination, vision 20/70. C) The pseudo-
operculum is now separated from the
retina in this stage 3 macular hole, vision
20/160. D) The macular hole is stage 4
when the posterior vitreous detaches,
vision 20/200. E) Two months after surgery
with vitrectomy, fluid-gas exchange and
face- down positioning, the hole is closed
and the vision has improved to 20/30.

Stage 0 Minimal changes in the foveal contour
with detachment of the perifoveal
vitreous cortex without traction.
VMA
Stage 1A: Imminent MH Foveal cysts and sensory foveolar
detachment associated with perifoveal
detachment with traction of the
posterior vitreous on the foveal internal
limiting membrane.
VMT
Stage 1B Cyst in the outer retina causing rupture
of the cones layer. Perifoveal
detachment of posterior vitreous.
VMT
Stage 2: Small MH Full-thickness MH of small diameter,
with partial rupture of the internal wall
of the cyst. Partial detachment of the
posterior vitreous, which remains
adhered to the operculum.
FTMH small / medium with VMT
Stage 3: Large MH MH of a larger size. Total detachment of
the posterior vitreous at the level of
the macular area, which persists
adhered to the papillae. Occasionally, a
free operculum adhered to the
posterior vitreous can be seen.
FTMH medium / large with VMT
Stage 4: Full-thick MH with PVD Total detachment of the posterior
vitreous. In some cases, the vitreous is
not observed on OCT scans. Larger
diameter of the hole with halo of outer
retinal detachment in many occasions.
FTMH small / medium / large without
VMT
Table 1. Comparison of Gass and IVTS classification from Garcia-Lavana et al 20152 (FTMH: full-thickness macular hole; MH:
macular hole; PVD: posterior vitreous detachment; VMA: vitreomacular adhesion; VMT: vitreomacular traction2)

figure 1 showing a stage 1B macular hole. There is a defect in the outer layers of the retina.

OCT of a stage 2 macular hole with a break in the roof and cystoid hydration.

Full thickness stage 3 macular hole with overlying operculum. This macular hole would be
classified as stage 4 if the posterior vitreous completely detached from the macula and optic
nerve

More recently, the The International Vitreomacular Traction Study (IVTS)
Group also formed a classification scheme of vitreomacular traction and
macular holes based on OCT findings[5]:
Vitreomacular adhesion (VMA): No distortion of the foveal contour;
size of attachment area between hyaloid and retina defined as focal if
</= 1500 microns and broad if >1500 microns
Vitreomacular traction (VMT): Distortion of foveal contour present or
intraretinal structural changes in the absence of a full-thickness macular
hole; size of attachment area between hyaloid and retina defined as
focal if </= 1500 microns and broad if >1500 microns
Full-thickness macular hole (FTMH): Full-thickness defect from the
internal limiting membrane to the retinal pigment epithelium. Described
3 factors: 1) Size -- horizontal diameter at narrowest point: small (≤ 250
μm), medium (250-400 μm), large (> 400 μm); 2) Cause -- primary or
secondary; 3) Presence of absence of VMT
Other features to note on exam include yellow deposits at the base of
the hole, retinal pigment epithelial changes at the base of the hole, and
epiretinal membrane adjacent to hole.

Figure 2: Optical coherence tomography image of a macular hole with an overlying operculum. The
classic “anvil-shaped” deformity of the edges of the retina is noted due to intraretinal edema.

igure 3: A small full thickness macular hole on optical coherence tomography with intraretinal
and subretinal fluid.

Diagnostic procedures
Fluorescein angiography demonstrates a hyperfluorescence pattern
consistent with a transmission defect due to loss of xanthophyll at base
of the MH. It typically shows a window defect early in the angiogram
that does not expand with time, and there is no leakage or
accumulation of dye.There may be Amsler grid abnormalities. However,
plotting small central scotomas is often difficult.
However, OCT is the gold standard in the diagnosis and management of
this disorder. This high-resolution image can allow evaluation of the
macula in cross-section and three-dimensionally. OCT can be helpful
detecting subtle MHs as well as staging obvious ones.
OCT can also help guide management. OCT can assist in the
determination of whether there is an associated epiretinal membrane
or if the posterior hyaloid is still attached or not, which can be critical in
deciding on the surgical approach. It can also be used to aid in gauging
the prognosis of the fellow eye.

The Amsler Grid is a simple test that will help you determine if your vision is distorted in this
way
Left: An Amsler Grid with straight lines as
seen by a normal-sighted person
Right: A person with macular problems may
notice distortion of the grid pattern such as
bent lines and irregular box shapes or a grey
shaded area

Differential diagnosis
The clinical appearance of a MH is fairly distinctive. However, epiretinal
membrane with a pseudohole, lamellar hole, and vitreomacular traction
must also be considered. Cystoid macular edema, subfoveal drusen,
central serous chorioretinopathy, or adult vitelliform macular dystrophy
are also in the differential diagnosis of a stage 1 MH.

SD-OCT demonstrating lamellar hole features described by Witkin et al.8 The OCT
demonstrates an irregular foveal contour, a break in the inner fovea, an intraretinal split with
bridging columns and the absence of a full thickness foveal defect. An ERM is also present.

SD-OCT demonstrating partial PVD with vitreo-foveal traction and concurrent ERM.

SD-OCT of foveal detachment. This has variously been classified as stage 1b macular hole by
Altaweel and Ip2 and also type 1A impending hole by Yeh et al.10 and thought to be the
precursor of FTMH.

Stratus OCT of vitreofoveal traction associated with inner retinal pseudocyst formation (type
1B impending hole), which is suggested by Yeh et al.10 to be a precursor of LMH.

Stratus OCT demonstrating foveal detachment. The presence of a FTMH cannot be ruled out
because of the presence of artifact that may mask the presence of a combined inner and outer
retinal break (arrowhead).

Flowchart of evolution of macular interface pathology and reported mechanisms of
pathogenesis.

SD-OCT demonstrating MPH (ERM and steepened foveal contour), but with reduced foveal
retinal thickness more commonly associated with LMH

SD-OCT of FTMH with LMH features of intraretinal split and ERM.

Common features of lamellar macular hole are depicted, including an irregular foveal contour, a
defect of the inner fovea (between bars), a separation between the inner and outer retina, and
an intact outer retina (lack of full-thickness hole). There is also an associated ERM present,

Additional typical examples of lamellar macular hole,

In a lamellar macular hole, the defect between the inner and outer retina often conforms to an
anvil shape. This area also may have schisis-like clefts.

Depending on the exact cross section imaged, the defect between the inner and outer retina in
a lamellar macular hole may be asymmetric.

Macular hole. (A) Stage 1
macular hole in a 63-year-old
woman with a 3-month history
of decreased visual acuity
(20/60). An outer retinal defect
can be observed in the B-scan
(arrow). (B) A full-thickness
retinal defect developed after 2
months of follow-up with
worsening in the visual acuity
(20/80). The posterior vitreous
remains adhered to the edge of
the macular hole. (C) One
month after surgery, the
macular hole was closed and
the visual acuity improved to
20/50, but a persistent foveal
outer defect could be observed
(arrowhead).

Preoperative OCT of an eccentric stage 2 macular hole with vitreous adhesion to the roof of the
macular hole

Fundus photo of the traumatic macular hole with associated subretinal hemorrhage and
choroidal rupture

Medical therapy
In general, most Stage 1 MH can be followed conservatively given
approximately 50% chance of spontaneous closure[. However, if the patient
has symptomatic VMT or even a full-thickness macular hole with associated
VMT, some may consider one of the following treatment options:
Intravitreal ocriplasmin. Ocriplasmin is a 27 kilodalton serine protease that
essentially performs pharmacolytic vitreolysis, separating the hyaloid from the
underlying retina. In the registration MIVI-TRUST clinical trials. At the day 28
post injection time-point, eyes receiving ocriplasmin (injected intravitreally)
exhibited greater release of the vitreoretinal attachment (primary endpoint,
26.5% vs. 10.1% in controls who received an intravitreal injection of drug
vehicle, p < 0.001) and closure of macular hole (40.6% vs. 10.6%, p < 0.001).
There have been some rare adverse effects reported in association with use of
this drug including electroretinography changes, lens subluxation, and
dyschromatopsia.
Intravitreal gas or air. More recently, some have found success in treating
patients with a small bolus of injected intravitreal gas or air. Research studies
are ongoing, but some have reported success rates as high as 83% for the
closure of FTMH

Macular hole

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Macular hole