Macular hole

Macular Hole
Presenter: Dr Nusrat Jahan Bukhari
Moderator: Dr Archis Shedbale

 Case Presentation
 Introduction
 Classification
 History
 Pathogenesis
 OCT classification
 Clinical Features
 Investigation
 Treatment
 Recent Advances

Case Presentation
 45 yr old male pt Mr ABC came in April 2012 with c/o
sudden DOV since few days, gave a h/o RE injury
(Blunt Trauma) with a Vn of 6/18, N12, diagnosed
Traumatic Maculopathy
 August 2014 Vn in RE dropped to FC 11/2metre, on
retinal examination diagnosed Traumatic Macular
Hole
 In Jan 2015 Patient underwent RE Vit+ ILM Peeling+
FAE+ C3F8 , Day 1 post op Vn improved to FC 2metre

Case Presentation:
 c/o DOV in RE since April 2012
 O/E RE LE
 Ant Seg: WNL WNL
 IOP: 20 mmHg 17 mm Hg
 Fundi: Traumatic 0.4:1
Macular Hole

Introduction
 A full-thickness depletion of the neural retinal tissue
in the center of the macula#
 Most commonly unilateral**
 Atraumatic “idiopathic” macular holes of the elderly
comprise the vast majority of these lesions*
* Colin A. McCannel et al. Population Based Incidence of Macular Holes.
Ophthalmology. 2009 Jul; 116(7): 1366–1369
** Chew E, Sperduto R, Hiller R, et al: Clinical course of macular holes. Arch Ophthalmol
117:242, 1999
# Chapter: Macular Hole, Yanoff & Duker Ophthalmology

 Sen P et al evaluated the prevalence of Macular Hole
in a study conducted in south India
 1.7 / thousand population
Sen P et al, Prevalence of idiopathic macular hole in adult rural and urban south
Indian population.Clin Experiment Ophthalmol 2008 Apr;36(3):257-60

Colin A. McCannel et al. Population Based Incidence of Macular Holes. Ophthalmology.
2009 Jul; 116(7): 1366–1369

Etiology
 Common causes:
 Idiopathic
 Trauma
 high myopia
 Other causes:
 cystoid macular edema
 proliferative diabetic retinopathy
 severe hypertensive retinopathy
 Choroidal neovasculatrisation
 Solar retinopathy

Classification
 Primary macular hole: is commonly an idiopathic
macular hole
 Caused by vitreous traction on the foveal from an
abnormal vitreous seperation
 Secondary Macular hole: caused by other pathologies
not associated with vitereomacular traction
 blunt trauma, high myopia, macular telangiectasia type2,
diff causes of macular oedema

History
 Macular hole first recognized approximately 100 years
ago
 First described by Knapp in late 1800s
 Later described by Noyes
 First histopathologic descriptions of full-thickness
macular holes were provided by Fuchs (1901)* and
Coats (1907)**
 Gass first described a series of stages of formation of
idiopathic macular hole in 1988
*Fuchs E. Zur Veranderung der Macula Lutea Nach Contusion. Ztschr Augenheilk 1901;6:181
**Coats G. The pathology of macular holes. Roy Lond Hosp Rep 1907; 17-69

Pathogenesis
 Traumatic Theory*
 associated with direct or indirect ocular trauma
 Trauma causes immediate macular hole formation from
mechanical energy created by vitreous fluid waves and
contrecoup macular necrosis or laceration
 More common in young boys
*Kopp CJ.Macular holes:a clinical contribution.Am ophthalmology 1908; 11:518-528

 Cystoid degeneration theory*:
 cystic degeneration of the central macula
 due to :hypertension, retinal vessel occlusion, trauma
 Cyst coalescence FTMH
*Coats G. The pathology of macular holes. Roy London Hospital Report 1907; 17:69-96

 Vascular theory:
 Age related changes of retinal vasculature
cystoid degeneration
macular hole formation

 Vitreous Theory:
 Antero posterior fibrous traction band
Macular traction
Macular cystoid degeneration
Macular hole

Current theory Posterior hyaloid applies
traction to the
foveola/umbo and
causes it to stretch
umbo dehisces because
it is the thinnest point in
the fovea
middle and inner retina
absorbs vitreous fluid at
the exposed edges of the
hole and begins to swell

hole enlarges because of
a lateral extension of
fluid into the outer
plexiform layer
inner retina is breached
due to the hydration of
the fovea and perifoveal
macula, the macular
hole progresses

 Concept of tangential traction*
 Spontaneous tangential traction of external part of the
perifoveolar cortical vitreous detaches foveolar retina
 Creates an intraretinal yellow spot approximately 100-
200μm in diameter
 Yellow color may result from intraretinal xanthophyll
pigment
* Avila MP, Jalkh AE, Murakami K, et al. Biomicroscopic study of the vitreous in macular
breaks. Ophthalmol 1983; 90:1277-83

Role of ILM in pathogenesis of
Macular Hole
scaffold for proliferation
of cellular components
Like myofibroblasts,
fibrocytes,RPE cells,
fibrous astrocyts
Causing tangential
traction around fovea
FTMH formation
May also contribute to
enlargement of MH

Revised Gass classification:
 Gass first described a series of stages of formation of
idiopathic macular hole *
*GASS JIM. Reappraisal of biomicroscopically 0f stages of Development of a macular Hole. Am
J Ophthalmolgy.1995; 119 :752-59

Stage 1 a
 Spontaneous tangential
traction of prefoveolar
cortical vitreous detaches
foveolar retina
creating an intraretinal
enhanced lipofuscin-
colored yellow spot 100-
200μm in diameter
Decreased/ absent foveal
depression
Foveolar detachment
Retinal Pigment
Epithelium
Neurosensory RetinaPosterior Hyaloid
Normal Fovea

Stage 1 b
 Further traction causes
foveal detachment
 yellow spot ˆ200-300μm in
diameter
Foveal detachment

Stage 2
 First biomicroscopically identifiable full thickness
retinal defect
 Less than 400μ
Early hole, central
Early hole, eccentric

Stage 3
 Vitreofoveal seperation
 Enlarges to greater
than 400μ
 Complete PVD is
absent
Stage 3 Hole

Stage 4
 Complete posterior vitreous detachment
(Weiss’ ring) occurs in 20% - 40% of eyes
Stage 4 Hole

vitreous adhesion to
central macula with no
demonstrable retinal
morphology changes
vitreous adhesion to
central macula , demonstrable
changes like tissue cavitation,
cystoid changes, loss of foveal
contour, elevation of fovea
Jay S et al. The International Vitreomacular Traction Study Group. Classification of vitreomacular
adhesion, traction & macular hole. The American Academy of Ophthalmolgy. 2013.2611-19.
OCT based anatomic classification of
FTMH

Small Hole ≥250μ, round or have a
f flap adherent to vitreous,
operculum ₊/-
Medium FTMH hole 250 - 400μ,
• round/ flap adherent to vitreous
Large FTMH hole >400μ,
• vitreous more likely to be fully seperated
• from macula
Jay S et al. The International Vitreomacular Traction Study Group. Classification of vitreomacular
adhesion, traction & macular hole. The American Academy of Ophthalmolgy. 2013.2611-19.
OCT based anatomic classification of
FTMH

Clinical features
 Visual acuity the first indicator but sometimes
misleading
 Mild loss of central vision (Stage 1a & 1b)
 Metamorphopsia

 FTMH is diagnosed on slitlamp biomicroscopy
 By off centering the beam we can study the contour
of hole and vitreous interface
differentiates FTMH from other lesions
Positive & Negative Watzke - Allen Sign
Watzke RC, Allen L. Subjective slit- beam sign for macular disease. Am J Ophthalmol
1969; 449 - 453

 In eyes with ERM a fibrotic appearance with
distortion of perifoveal vessels seen

 Amsler Grid:
 Small absolute scotomas can be detected in 30 -40 %
of patients*
 Charting used but not specific for macular hole
 Can be used in post operative period to evaluate
scotoma and metamorphopsia
*Smith RG et al. Visual Performance in idiopathic macular holes. Eye 1990; 4: 190 -
194

Investigations
 OCT
 FFA

 Optical coherence tomography (OCT):
 diagnosis of macular hole but also in staging
 helpful in prognosticating depending upon size of the
macular hole

 Flourescein Angiography:
 Usually not indicated in diagnosis of macular hole
 But generally demonstrates early hyperfluoresence
(window defect)

 Laser Aiming Beam Test:
 Place a 50μm laser photocoagulator aiming beam
within a lesion
 Patient with FTMH cannot detect the aiming beam
within lesion but is able to detect it in its surrounding
 Patients with ERM or Pseudomacular hole shall be
able to detect

 B- Scan Ultrasonography:
 Predictive of vitreomacular relationship and therefore
may be helpful in staging

Differential Diagnosis
 Epiretinal membrane with pseudomacular hole
 Lamellar macular hole
 Chronic cystoid macular edema

ERM with Pseudomacular hole
 have a median visual acuity of 20/30
 retinal vascular tortuosity
 not associated with a rim of subretinal fluid

Lamellar macular hole
 Sharply circumscribed
 Partial-thickness defects of the macula
 Represents either as an aborted full-thickness lesions
or a complication of chronic cystoid macular edema*
 Characterized by a flat, reddish hue-type lesion with
intact outer retinal tissue
 Careful evaluation will reveal retinal tissue in the base
of the lesion
 No evidence of subretinal fluid
 Do not progress to full-thickness lesions
* Patel B, Duvall J, Tullo AB. Lamellar macular hole associated with idiopathic
juxtafoveolar telangiectasia. Br J Ophthalmol 1988;72:550

Chronic cystoid macular edema
 Seen sometimes post cataract surgery
 In diabetic macular edema

Pre operative parameters
Hole form factor > 0.9 and Macular Hole
index > 0.5 also have a better prognosis
a = base diameter, b = minimum diameter
c = left arm length, d = right arm length

Correlation of hole form factor and best
corrected postoperative visual acuity
S. Ullrich et al. Macular hole size as a prognostic factor in macular hole surgery.
Br J Ophthalmol. 2002 Apr; 86(4): 390–393

Management
 Symptoms of impending holes : visual distortion,
decreased visual acuity, and changes observed with
home Amsler grid testing
 Macular holes can resolve spontaneously

 This most commonly occurs in stage 1 but has been
reported for stage 2 holes as well
 The resolution occurs when the posterior hyaloid
separates
 Hence, it is better to observe them for a few months
 If vision deteriorates or the hole progresses, vitreous
surgery is indicated
Management

Surgery
 Pars Plana Vitrectomy with internal limiting
membrane peeling with gas tamponade is performed
for stage 2-4 FTMH

Pars Plana surgical procedures
 Using three- port system
 After removing central vitreous the posterior cortical
vitreous is identified and seperated from retinal
surface

Chromovitrectomy
 Use of vital dyes to stain pre retinal tissues during
vitreoretinal surgery
 Allows visualization of the thin, transparent tissues in
vitreoretinal interface : ILM, epiretinal ERM, or the
vitreous posterior surface
 Indocyanine Green Dye(ICG): 0.25mg/ml

 Trypan Blue(0.15%): stains ERM, but not ILM
 Triamcinalone Acetonide(40mg/ml): stains residual
vitreous
 Brilliant Blue(0.025% & 0.05%): excellent stain for ilm,
relatively non toxic

 In idiopathic FTMH the rationale would be to remove
or relieve foveal traction from within the retinal
surface1
 Helps by ensuring complete removal of any epiretinal
tissue above the ILM that could cause foveal traction2
as well as by increased cytokine release
 enhancing glial proliferation
ILM Peeling
1- Fekrat S, Wendel RE, de la Cruz Z, Green WR: clinicopathologic correlation of an epiretinal
membrane associated with a recurrent macular hole. Retina 1995; 1:53-57
2- Yooh HS, Brooks HL Jr, Capone A Jr, et al. Ultra structural features of tissue removed during
idiopathic macular hole surgery. Am J Ophthalmol 1996;1:67-75

Types of closure
 On the basis of post operative OCT findings closed
macular holes are:
 Type 1 & type 2 closure
S W Kang et al. Types of macular hole closure and their clinical implications.
Br J Ophthalmol 2003; 87: 1015 - 1019

Type 1 Closure
 Indicates that macular hole is closed without foveal
defect of the neurosensory retina

Type 2 closure
 Indicates a foveal defect of neurosensory retina
persists postoperatively
 Although thewhole rim of macular hole is attached to
the underlying RPE with flattening of the cuff

Post operative parameters
 OCT parameters: Type 1 closure of MH without
neurosensory defect) has a better visual outcome
compared to Type 2 closure (with neurosensory
defect)
 Continuous IS/OS junction and external limiting membrane as well as
increased photoreceptor outer segment thickness predicts a better
functional outcome
1Kang ST, Ahn K, Ham DI. Types of macular hole closure and their clinical
implications. Br J Ophthalmol. 2003; 87:1015-19
2San M, Shimoda Y, Hashimoto H.Restored photoreceptor outer segment and visual
recovery after macular hole closure . Am J Ophthalmol 2009; 147:313-18

Recent Advances
Pharmacologic vitreolysis
 new nonsurgical option that can aid closure of
macular holes associated with VMT
 degrades the macromolecular vitreous attachment
complex
 relieves the tractional forces that cause the foveal
lesion

 In MIVI TRUST study patients with FTMHs less than
400 microns in width, the closure of holes occurred in
40.6% of ocriplasmin treated eyes and 10.6% of
placebo treated eyes*
 In patients with small hole the success rate was even
higher
 This occurred without face down position, surgery or
gas bubble
 Makes it an appealing option for appropriate patients
* Stalmans P, Benz MS, Gandorfer A, Kampik A.et al. MIVITRUSTal study group.
Enzymatic vitreolysis with Ocriplasmin for Vitreomacular Traction and Macular
holes.N Engl J Med 2012; 367: 606-15
Pharmacologic vitreolysis

Macular hole

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