Macular Hole

Retina
2
Priyank Garg MS, FNB, 1Arindam Chakravarti, 1,3Sundaram Natarajan

T he term Macular Hole implies that a part of macula is missing.

These are an important cause of vision loss in elderly.
It represents an excavation of the inner layers of retina
characterized by recognizable retinal tissue at its base.
These can be: - Causes: -
Partial-Thickness. Chronic CME.
Full-Thickness. Radiation retinopathy.

Partial-Thickness Macular Hole Idiopathic perifoveal telangiectasia.

Two types of partial-thickness holes occur: - Clinical Features: -

Outer lamellar hole (OLH). Visual acuity ranges from 20/20 (6/6) to 20/80 (6/24).

Inner lamellar hole (ILH). Distortion may be noted without scotoma on Amsler grid
testing.
Outer Lamellar Hole(OLH): -
Borders may not appear as sharp as in FTMHs because of
Causes: - sloping margins & thin, irregular edges.
Collapse of outer wall of large cyst. There is usually no cuff of surrounding subretinal fluid & no
Trauma- there is a breakdown outer blood-retinal barrier at RPE alteration.
the RPE. Can be differentiated from FTMH by slit-beam test.
Optic pits associated with macular schisis. F/A Either no transmitted fluorescence or minimal window
Solar retinopathy. defect if the ILH is nearly a FTMH.

Clinical Features: - Progression to a FTMH with subsequent fall in vision is

possible, but rare, if the ILH has been present for many
Vision varies from 20/20 (6/6) to 20/400 (3/60), depending months.
on the size & degree of photoreceptor destruction of the &
on the cause (e.g., traumatic, solar). There is no known treatment.

On biomicroscopic examination, a slightly irregular deep, Full-Thickness Macular Hole

round, or oval excavation is seen with intact inner retinal An FTMH is a round break that involves all the layers of the retina,
tissue. from the internal limiting membrane through the outer segments
F/A- demonstrates a variable degree of window defect of photoreceptor layer.
resulting from the RPE atrophy & may fortuitously show dye
Etiology
in a retinal capillary direct overlying part of OLH.
Most Macular Holes occur in the absence of antecedent injury
OLHs may increase in size over time, with subsequent
& are referred to as idiopathic.
decrease in vision.
Trauma (4-5%).
They may also transform into FTMH if the intact inner retina
atrophies or is avulsed by vitreal traction. Pathological Myopia.
There is no known treatment. Laser.
Inner Lamellar Hole(ILH): - Electric Current.
An ILH is much more common than an OLH & may be an Misc. causes
intermediate stage in the development of some FTMHs.
Idiopathic Macular Hole
1. Aditya Jyot Eye Hospital Pvt. Ltd., Introduction: (ICD-9 #362.54)
Mumbai, Maharashtra, India.
2. Dept. of Ophthalmology, The term Macular Hole implies that a part of macula is
LLRM Medical College, Meerut, UP.
3. TNMGR University, missing.
Chennai, Tamilnadu, India.

www.dosonline.org 65
 This is an important cause of vision loss in elderly. Hypertension.

Epidemiology Diabetes mellitus.

Prevalence rate of macular hole in India is a reported 0.17%,
with a mean age of 67 years.1
Idiopathic macular holes develop predominantly in older
patients at an incidence ranging from 0.03% to 0.05%.2
The prevalence is three times greater among women than
men.2
Macular Holes are bilateral in upto 29% of patients.3
Peak incidence is seen in seventh decade of life.
In a case-control study, 72% of the idiopathic macular holes
occurred in women and more than 50% of the macular holes
occurred in patients 65 to 74 years old. Only 3% were found
to occur in patients under age 55 years.4
Risk Factors
Largely unknown.
A few epidemiologic risk factors of uncertain importance
have been reported:
Coronary artery disease.
Previous cerebrovascular accident.
Elevated plasma fibrinogen levels.
Pathogenesis
Vitreous traction, anteroposterior as well as tangential, plays an
important role in the pathogenesis of macular holes. Posterior
vitreous detachment decreases the risk for future macular hole
formation. Fellow eyes with vitreomacular separation have less
than a 5% risk for macular hole18,19,20 versus as high as a 29% risk
for macular hole in eyes with no posterior vitreous detachment20.
As described above, tangential traction caused by the condensed
perifoveal cortical vitreous was supposed to be the main factor
in the initiation and progression of macular hole formation17.
Evaluation of the early stages of hole formation with OCT by
Gaudric et al21 led to the postulation of a new mechanism for
macular hole formation, which is based on anterior-posterior
tractional forces. These investigators have documented that
posterior hyaloid detachment begins in the posterior pole around
the macula and gradually spreads with focal attachment to the

Historical Timeline of Macular Hole Theories

Year Author Description

1869 Knapp First case description of macular hole (traumatic)5

1871 Noyes First detailed clinical description of macular hole (traumatic)6
1900 Ogilvie Published case series & proposed terminology including macular hole7
1900 Kuhnt Atraumatic theories of cystic retinal degeneration leading to macular hole8
1901 Fuchs Early histopathologic descriptions of macular hole including cystic retinal
changes9,10
1907 Coats
1912 Zeeman Histopathologic recognition of premacular vitreous condensation219
1924 Lister Vitreous forces and vitreous traction bands (anteroposterior) may cause
macular holes11
1967 Reese et al Vitreous separation critical to macular hole formation12
1982 McDonnell et al Possible female hormonal influence on vitreous separation and macular hole
formation2
1983 Avila et al Vitreous separation not necessary in formation of a macular hole13
1986 Morgan & Schatz Involutional macular thinning is a premacular hole condition14
1988 Gass & Johnson Tangential vitreous traction and Gass biomicroscopic classification of Johnson
premacular hole and macular hole lesions15,16
1995 Gass Centrifugal displacement of retinal receptors with umbo dehiscence
Reappraisal of biomicroscopic classification of premacular hole and macular
hole lesions17

66 DOS Times - Vol. 16, No. 10, April, 2011

foveal center. This focal attachment creates retinal convexity
consistent with anterior-posterior vitreoretinal traction.
More recently, Gass has emphasised the importance of the foveal
Muller cell cone,22 originally described by Yamada23 and Hogan
et al24 in histological studies of the normal human foveola. These
studies showed the foveola to be composed of an inverted cone
of Muller glia with a truncated apex up to the external limiting
membrane (ELM). Between the apex and the ELM were radially
oriented inner cone segments radiating towards the beginning
of the outer nuclear layer of cone nuclei. The base of the cone
formed the umbo and extended into the clivus in the perifoveolar
region. The internal limiting membrane (ILM) lining the base
of the cone was extremely thin (1020 nm) compared with the
peripheral fovea. The sides of the cone were apposed to radiating
inner segments centrally and cone nuclei in the outer nuclear layer
more peripherally towards the perifoveolar region.
Staging & Clinical features: -
Based on clinical observations, Gass in 1988 proposed a four-stage
classification for macular holes15,16 & modified it in 199517.
Stage 1:
Further sub-divided into 2 stages: -
Stage 1a (Impending Macular Hole):
Rarely seen clinically.
Usually detected in a patient with a full thickness macular
hole in other eye.
Loss of the foveal depression and a yellowish foveal spot
(100 to 200 microns in diameter)
Localized shallow detachment of the perifoveal vitreous
cortex with persistent adherence to the foveola initially
Vitreofoveolar traction may cause a split of the retina at
the fovea (pseudocyst) that corresponds to the yellow
spot
Epiretinal membranes are uncommon
Visual acuity 20/25 to 20/80
Stage 1b (Occult Macular Hole):
Yellow ring 200 to 350 microns in diameter
Posterior extension of the pseudocyst with disruption
of the outer retinal layer
Roof remains intact with persistent adherence of the
posterior hyaloid to the retina
Epiretinal membranes are uncommon
Visual acuity 20/25 to 20/80
Results from centrifugal displacement of the foveolar
retina & xanthophyll.
Characterized by a yellow ring with bridging interface
of vitreous cortex.
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May be associated with recent mild decrease in visual
acuity or metamorphosia.
F/A: -Faint hyperfluorescence or no abnormalities.
Visual acuity may be only mildly reduced & Amsler grid
abnormalities are detected only 50% or more of the time.
About 50% of holes resolve following spontaneous vitero-
foveolar separation.
Stage 2 (Early FTMH): -
Small full thickness (less than 300 microns in diameter) retinal
defect in an eye with a stage 1 macular hole
Epiretinal membranes are uncommon
Visual symptoms include metamorphopsia and decreased
vision
Visual acuity 20/25 to 20/80
At this stage, the posterior cortical vitreous is still attached at
the fovea and, in many cases, radial tractional striae may be
visible around the edges of the hole at the level of the inner
retina and internal limiting membrane.
In addition, intraretinal cystoid spaces appear around the
edges of the hole.
Two congurations may occur at stage 2:
Centric: The full thickness tear begins at the centre of the
fovea (umbo dehiscence) and expands in a symmetric
fashion.
Pericentric: A full thickness tear arises at an eccentric
position in the fovea and extends in a can opener
fashion to form a crescentic hole progressing to a
horseshoe shaped hole and eventually, when the can
opener is complete, to a round hole with a fully detached
operculum of tissue suspended on the posterior vitreous
cortex in the prefoveal plane. This conguration is typical
in the majority of stage 2 FTMH (8090%).
Stage 3 (Established FTMH): -
Full thickness hole 300 to 400 microns in diameter
Posterior hyaloid is completely separated over the macula but
may be attached to the optic disc and more peripherally
Operculum or a flap on the posterior hyaloid over the hole
usually evident on optical coherence tomography (Figure 4)
and may be apparent clinically
Cuff of subretinal fluid, intraretinal edema, and cysts
Drusen-like deposits (may represent macrophages at the level
of the retinal pigment epithelium and may indicate chronicity
of disease) often in the base of the hole.
Rim of retinal pigment epithelium hyper/hypopigmentation
often present at the junction between edematous or detached
retina and normal-appearing, attached retina in long-
standing cases.
67
 Figure 1: Colour Photo of Full Thickness Figure 2: Red Free Photo of Full Thickness
Macular Hole Macular Hole

Figure 3: SD-OCT of Full Thickness Figure 4: SD-OCT of Full Thickness Macular

Macular Hole Hole with operculum

Epiretinal membranes may be present Some patients achieve better visual acuity by employing
eccentric fixation.
Visual acuity 20/100 to 20/400.
Diagnostic Tests
Characterized by a round retinal defect greater than 400
with an attached posterior vitreous face with or without an Watzke-Allen Test: -
overlying pseudo-operculum.
Performed by projecting a narrow slit beam over the centre
No Weiss ring present. of the hole both vertically & horizontally with a 90D or 78D
lens.
Rim of elevated retina with or without prefoveolar opacity.
Pt. With macular hole will report that the beam is broken or
Stage 4: -
thinned.
Characterized by enlargement of the round defect which is
Laser aiming beam test: -
now surrounded by cuff of sub-retinal fluid & exhibits tiny
yellowish deposits at the base of the crater (Figure 1,2 & 3). Performed by projecting a 50 spot of a laser aiming beam
(e.g. He-Ne) at the centre of the hole.
The posterior vitreous is completely detached, as evidenced
by a Weiss ring. A patient with macular hole will report that the spot has
disappeared.
Visual acuity tends to deteriorate progressively, stabilizing at
6/60 or worse as the hole reaches its maximum diameter.

68 DOS Times - Vol. 16, No. 10, April, 2011

 Figure 6: SD-OCT of Full Thickness Macular
Hole with Retinal Detachment

Figure 5: Full Thickness Macular Hole

with Retinal Detachment

Fluorescein Angiography:-
Shows a corresponding area of hyperfluorescence.
This result from unmasking of background choroidal
fluorescence caused by a window defect in xanthophyll d/t
centrifugal displacement.
Optical coherence tomography:-
Figure 7: SD-OCT showing Type 1 closure of
Provides high resolution optical sections of the retina & Macular Hole
affords measurement of retinal thickness.
Its useful in diagnosis & staging of macular holes.
It also measures the volume of full-thickness macular hole.
Scanning Laser ophthalmoscopy:-
It is used to evaluate the characteristics of macular holes &
outcomes of surgery.
Retinal Thickness Analyzer: -
This has shown promise in detecting & accurately measuring
macular holes.
Fundus Autofluorescence:-
Has been used as indication of the level of lipofuscin in the Figure 8: SD-OCT showing Type 2 Closure
retinal pigment epithelium. of Macular Hole

Normally, autouorescence of the retinal pigment epithelium

underlying the fovea is decreased because the overlying
macular yellow pigments absorb the exciting light. Stage 1 macular holes without a PVD were found to progress
to an FTMH in 33% of patients but no stage 1 lesions with a
Without the overlying absorptive pigments, a macular hole
PVD progressed to an FTMH.
creates a window defect so that autouorescence is increased.
Most stage 2 FTMHs progress to stage 3 or 4 (especially
Thus, this change in visible autouorescence can be used as
centric stage 2 holes with pericentral hyperfluorescence &
an indication of a full-thickness macular hole or of failure of
eccentric stage 2 holes).
surgical repair.
Stage 2 FTMHs that are 400 microns or smaller without a
Natural History: -
PVD may have a greater risk of enlarging with a larger rim
The natural outcomes of stages 1, 2, 3 & 4 FTMHs is different. of sub-retinal fluid & decreased visual acuity.

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 Stage Management Follow up

1A Observation Prompt return if new symptoms.

1B Every 4 to 6 months in the absence of
symptoms.
2 PPV + BBG assisted ILM peeling + C3F8 + 1 to 2 days postop, then 1 to 2 weeks
Prone position for 2 weeks If no surgery, every 4 to 8 months
3 1 to 2 days postop, then 1 to 2 weeks
4

Stage 2 holes progressed 84% of the time, stage 3 holes

Hole form factor (HFF) = c + d/a
a = base diameter
b = minimum diameter
c = left arm length
d = right arm length
Differential Diagnosis: -
Pseudoholes: -
Macular Hole Index (MHI) = b/a
Visual acuity: -
The vitrectomy for Prevention of Macular Hole Study Group
found that:-
the risk of progression to an FTMH was much higher in
eyes with stage 1 macular hole who had best-corrected
visual acuity 20/50 or worse.
progressed 55% of the time & stage 4 holes enlarged 16%.
Visual acuity decreased two or more lines in the majority
of stage 2 holes (68%) & less significantly for the other
stages (stage 1 - 30%; stage 3 - 29%; stage 4 13%).
In another study
96% of stage 2 holes progressed to stage 3 or 4, with 71%
having a two or more line reduction in visual acuity.
The authors concluded that even though vitreomacular
separation may improve the prognosis of a macular hole,
stage 2 lesions usually will develop an enlarged hole and
decreased visual acuity.
The incidence of disappearance of idiopathic FTMHs is low.
Most pseudoholes are often undiagnosed & are the result
of a dehiscence of a portion of gliotic preretinal membrane
overlying the macula, producing a lesion with surprisingly
sharp but often irregular borders.
Seen most often in association with: -
Idiopathic epiretinal membrane formation
Vitreomacular traction syndromes
Proliferative diabetic retinopathy
Rhegmatogenous retinal detachment
Intraocular inflammation
Venous occlusive disease
Trauma.
White dot fovea
May be normal or slightly reduced.
Contraction of associated fibrous proliferation may produce
macular pucker, which may effect vision by virtue of distortion
more than acuity decline, since the retina underneath is intact.

70 DOS Times - Vol. 16, No. 10, April, 2011

 A circular dehiscence in a prefoveal membrane may allow for
surprisingly good visual acuity.
Extensive preretinal membranes may be associated with
perifoveal axoplasmic debris accumulation, which may
influence the visual status.
Fluorescein angiography: -
No abnormal fluorescence except for traction-induced retinal
vascular leakage.
However, faint central fluorescence coincident with choroidal
filling that later fades occurs in pseudoholes with epiretinal
membranes.
Red-free photography is helpful in outlining the extent of the
preretinal membrane bordering the defect.
Prognosis: -
Usually good but depends on the underlying cause of the
preretinal membrane.
Progressive contraction may increase perifoveal intraretinal
vascular leakage(macular edema).
Treatment: -
There is no evidence that laser treatment, even in eyes with
associated angiographic leakage, is of value.
Indeed, photocoagulation may worsen contraction of the
membrane on the surface of retina because of thermal energy
absorption.
Surgery is successful in vast majority of cases.
If the vision is 20/80 or less & the patients vision is disturbed
by distortion then vitrectomy surgery to peel the epiretinal
membrane is justified, this relieves surface traction.
This management has yet to be evaluated since the return Prognosis:-
of vision is usually incomplete & the risks of vitrectomy &
progression of nuclear sclerosis always exist.
Macular Cysts: -
It has an intact inner & outer retinal layer with fluid present
intraretinally.
Cystic macular degeneration involves extensive inner retinal
ischemic atrophy with loss of nerve fiber layer, ganglion cells,
inner plexiform & inner aspects of inner nuclear layers.
Cystoid spaces in the macula develop from degeneration of
muller cells & eventually progress to irreversible liquefaction
necrosis of these cells & neuronal degeneration.
Collapse of smaller cystic cavities, creating a large confluent
cyst, in chronic or severe CME creates a lesion that may
appear similar to macular hole on direct ophthalmoscopy.
CME may be associated with : -
Use of intraocular gas. Case Selection: -
Choroidal tumors.
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Retinal vascular occlusive disease.
Trauma.
Ocular inflammation.
Exudative macular degeneration.
Diabetes mellitus.
Macular pucker.
Retinitis pigmentosa.
Macular telangiectasias.
Visual acuity : -
Usually ranges from 20/20 to 20/100.
Fluorescein Angiography:-
Reveals pooling of the dye in the cystoid spaces in the late
venous phase.
An RPE detachment can be distinguished by slow & complete
subpigment epithelial leakage & neurosensory detachment
will be associated with a focal RPE leak.
Traction neurosensory retinal detachment will show no
abnormal fluorescence.
Diagnosis: -
Watzke-Allen test differentiates a hole from a cyst.
If a complete interruption or an interruption greater than one
half the width of the slit then FTMH.
No interruption or slight narrowing or distortion in the slit
correlates pseudohole, cyst or lamellar hole.
The Amsler grid although sensitive is not specific.
Depends on the underlying cause.
The size & chronicity of the cystoid edema, associated vitreal
traction & disruption in the anatomic architecture of the
perifoveal tissue may lead to inner lameller or FTMH.
Treatment : -
No treatment is indicated except in patients with partial PVD
with ongoing traction to a cystic macula, esp. in a patient with
poor vision in the fellow eye.
Pars plana vitrectomy is used to separate partially detached
posterior hyaloid face from underlying cystic retina.
Treatment of FTMH: -
Indications of Surgery: -
Stage 2 MH with VA < 6/12
Stage 3 or 4 MH
Careful patient selection is critical to a successful outcome.
71
 Ideal candidate would be a patient with bilateral holes of
recent onset, with vision in his better eye inferior or equal to
6/36.
Patients with symptomatic unilateral holes with recently
reduced vision to 6/24 or worse are also good candidates.
Since stage 1 macular holes have a high rate of spontaneous
resolution & reported studies have failed to demonstrate any
benefit from vitrectomy, surgery is not usually recommended
for this early stage.
Prognostic Factors
i) Stage of hole earlier the better
ii) Pre-op VA the good the better
iii) Pre-op Lens Status the clearer the better
iv) Duration of hole longer the duration, grim surgical results.
v) Hole Form Factor (HFF) indirect indicator of the size of
hole. The higher the value of HFF (>0.9) the better are the
results. Calculated as follows: -
vi) Macular hole Index (MHI) is basically the ratio of hole height
with base diameter. Higher MHI (0.5) correlates with better
visual outcomes.
vii) No. of surgeries - Patients who have had two failed surgeries
for a macular hole generally derive little or no visual benefit
even if a third surgery closes the hole.
viii) Scanning Laser Microperimetry maximum para hole
sensitivity showed a positive correlation with post-op VA.
Stimuli are positioned on the retina around the macular hole.
The first stimuli are presented at 1520 dB. If the patient
identified all stimuli around the hole or if none of the stimuli
are seen, the intensity was adjusted until some stimuli are
seen. The intensity is then decreased gradually until the
patient did not see any stimuli around the hole, which is
defined as the maximum para hole sensitivity.
ix) Shorter axial length
Results: -
Two types of hole closure has been defined: -
Type 1 closure indicates that the macular hole is closed
without foveal defect of the neurosensory retina (Figure 7).
Type 2 closure indicates that a foveal defect of the
neurosensory retina persists postoperatively although the
whole rim of the macular hole is attached to the underlying
RPE with flattening of the cuff (Figure 8).
Visual improvement is achieved in 55% to 85% of eyes with
a final visual acuity of 6/12 or better in upto 65%.
Stage 2 holes randomized to surgery had a significantly lower
incidence of hole enlargement.
Bilateral visual function improves after successful FTMH
surgery, especially in patients with subnormal (<20/40) visual
acuity in fellow eye.
72
Chemical vitrectomy/ Enzymatic Vitrectomy: -
An in office procedure
Enzymatic manipulation of posterior hyaloids & vitreous
cortex is carried out using 0.4 IU autologous plasmin & the
solution is lavaged followed by 14% C3F8.
Adjunctive Agents: -
Thrombin:-
A known stimulator of RPE & glial cells.
Failed to show marked increase in the success rate of macular
hole closure.
Increased post-op inflammation.
Gelatin plugs: -
Preliminary results of vitrectomy followed by placement of
an absorbable cross-linked gelatin plug in stages 3 & 4 have
been encouraging.
Recommended use in high myopes with posterior staphyloma
or recurrent macular hole.
TGF -2: -
It appears to promote wound healing by stimulating collagen
fibril growth, glycoproteins & proteoglycans.
Studies have suggested a potential benefit using human
recombinant TGF -2 for persistent FTMHs following failed
primary surgery & for traumatic holes.
Autologous platelet concentrate: -
It induces a localized fibrocellular response that seals the
retinectomy edge, involving a mixed population of glial, RPE
& fibroblastic cells.
It has a beneficial effect in primary FTMH surgery & also
increased the surgical success rate in reoperations combined
with rigorous epiretinal membranectomy.
References
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traumen auf dem augapfel. Arch Augenklinik 1:629, 1869
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First Author
Priyank Garg MS, FNB

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