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 Ophthalmol117:242, 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 xanthophyllpigment

* 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 formationMay 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 prefoveolarcortical vitreous detaches foveolar retina

creating an intraretinalenhanced lipofuscin-colored yellow spot 100-200μm in diameter

Decreased/ absent fovealdepression

Foveolar detachment

Retinal Pigment Epithelium

Neurosensory RetinaPosterior Hyaloid

Normal Fovea

Stage 1 b

Further traction causes foveal detachment

yellow spot 2̂00-300μm 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 fovealcontour, elevation of fovea

Jay S et al. The International Vitreomacular Traction Study Group. Classification of vitreomacularadhesion, 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 vitreomacularadhesion, 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 lesionsPositive & Negative Watzke - Allen Sign

Watzke RC, Allen L. Subjective slit- beam sign for macular disease. Am J Ophthalmol1969; 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

Lamellar Macular Hole

Chronic cystoid macular edema

Seen sometimes post cataract surgery

In diabetic macular edema

Pre operative parameters

Hole form factor > 0.9 and Macular Holeindex > 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

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 hyaloidseparates

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 epiretinaltissue 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 epiretinalmembrane 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 fovealdefect of the neurosensory retina

V/A: 6/36, N 10

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 neurosensorydefect)

Continuous IS/OS junction and external limiting membrane as well as increased photoreceptor outer segment thickness predicts a better

functional outcome1Kang 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 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 foveallesion

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