Macular Hole

Presenter: Dr Nusrat Jahan Bukhari

Moderator: Dr Archis Shedbale

Case PresentationIntroductionClassificationHistoryPathogenesisOCT classificationClinical FeaturesInvestigationTreatmentRecent Advances

Case Presentation45 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 2012O/E RE LE Ant Seg: WNL WNLIOP: 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

EtiologyCommon causes:

IdiopathicTrauma high myopia

Other causes:cystoid macular edemaproliferative diabetic retinopathysevere hypertensive retinopathyChoroidal neovasculatrisationSolar retinopathy

Classification Primary macular hole: is commonly an

idiopathic macular holeCaused by vitreous traction on the foveal from an

abnormal vitreous seperationSecondary Macular hole: caused by other

pathologies not associated with vitereomacular traction blunt trauma, high myopia, macular

telangiectasia type2, diff causes of macular oedema

HistoryMacular hole first recognized approximately

100 years agoFirst described by Knapp in late 1800sLater described by NoyesFirst 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

PathogenesisTraumatic 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 theoryPosterior 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 aSpontaneous

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 200-�300μm in diameter

Foveal detachment

Stage 2First biomicroscopically identifiable full

thickness retinal defectLess than 400μ

Early hole, central

Early hole, eccentric

Stage 3Vitreofoveal

seperationEnlarges to

greater than 400μ Complete PVD is

absent

Stage 3 Hole

Stage 4Complete 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 featuresVisual acuity the first indicator but

sometimes misleadingMild 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

holeCan 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

InvestigationsOCTFFA

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

holeBut generally demonstrates early

hyperfluoresence (window defect)

Laser Aiming Beam Test: Place a 50μm laser photocoagulator aiming

beam within a lesionPatient 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 DiagnosisEpiretinal membrane with pseudomacular

holeLamellar macular holeChronic cystoid macular edema

ERM with Pseudomacular holehave a median visual acuity of 20/30retinal vascular tortuositynot associated with a rim of subretinal fluid

Lamellar macular holeSharply circumscribedPartial-thickness defects of the maculaRepresents 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

Lamellar Macular Hole

Chronic cystoid macular edemaSeen sometimes post cataract surgeryIn 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 diameterc = 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

ManagementSymptoms 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

SurgeryPars Plana Vitrectomy with internal limiting

membrane peeling with gas tamponade is performed for stage 2-4 FTMH

Pars Plana surgical proceduresUsing three- port systemAfter 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 ILMTriamcinalone 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 closureOn 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 ClosureIndicates that macular hole is closed without

foveal defect of the neurosensory retina

V/A: 6/36, N 10

Type 2 closureIndicates 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 parametersOCT 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 AdvancesPharmacologic vitreolysisnew nonsurgical option that can aid closure

of macular holes associated with VMTdegrades the macromolecular vitreous

attachment complexrelieves 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 bubbleMakes 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