MACULAR HOLE

Nawat Watanachai

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Ramathibodi Hospital

INTRODUCTION

- Macular Hole (MH) is a full-thickness depletion of the neural tissue in the center of the macula that result in visual loss

History

1869 : Herman Knapp : initial published description of MH

EPIDEMIOLOGY

In USA , MH affect about 100,000 people1.9 % of visual impaired eyes (20/40-

20/200)Female > male (2:1)age 60 – 80 years ( mean 65 years )VA 20/20 - 20/400Incidence of MH in fellow eye : 5-10% not associated with medical dis, refractive

error

Natural Hx

EDCCS : vision/ progression45% loss > 2 snellen lines in 4.5 yrs28% loss > 3 snellen lines in 4.5 yrs30% increased in size in 4.5 yrs8% spont. resolution/regression after 6 yrsonly 3% spont. improve vision

Natural Hx

EDCCS : MH in opposite eye5% at 3 yrs7% at >6 yrs0+ % in pre-existing PVD eyes

rarely associated with RRD, higher incidence in high myopia with posterior staphyloma

Causes

- most common cause is idiopathic- the others

- non-surgical trauma

- surgical trauma

- pathologic myopia

- vascular disease

PATHOGENESIS

idiopathic MH begins with contraction ofprefoveolar vitreous cortex that is adherentto ILM of Mueller cell cone.Foveal pseudocyst formation Dehiscence of pseudocyst and Mueller

cellFull – thickness MH formation (FTMH) +/- avulsion of operculum (Muller cell cone.

ILM, Henle’s layer, cone nuclei)

Pathogenesis

Pathogenesis

Unknown in traumatic MHtangential vit

tractionretinal necrosis?

Estrogen?Elevated serum

fibrinogen levels (EDCCS)

Abnormal traction forces of the vitreous on the macula?

Observed withCL examinationU/SOCTLaser biomicroscopy

CLASSIFICATION

Gass and Johnson classification stage 1 - pre – macular hole lesion

1a yellow spot

1b yellow ring

stage 2 - eccentric or concertric FTMH < 400

stage 3 - FTMH > 400

stage 4 - FTMH with PVD

Stage 1

- localized shrinkage of prefoveal cortical vitreous formed the traction shallow detachment of foveola- loss of normal foveola depression and light reflex - 1a small yellow spot ( 250 -300 ) - 1b yellow ring ( halo form of foveal detachment )

-+/-pseudooperculum- VA < 20/40 , metamorphopsia- 50 % had spontaneous PVD

Stage 2

- eccentric or oval full thickness- defect diameter < 400 micron- VA 20/50 - 20/80- 74 % progress to stage 3

Stage 3

- hole > 400 micron, may be fovealedema & surrounding cuff of subretinal fluid or operculum- VA 20/100 – 20/400- no PVD

Stage 4

- FTMH with complete PVD - may be associated to ERM

- FFA in stage 3 , 4 - mottle hyperfluorescene from RPE thining, RPEdepigment, loss of xanthophyl

MH stage IV

HISTOPATHOLOGY

- MH is the full thickening circular retinal defect at fovea

- size 100 – 800 micron

- operculum composed of ILM, Mueller

cell cone, superficial inner cone fiber and

cone nuclei.

CLINICAL AND DIAGNOSIS

VA loss 20/80 – 20/400 mean 20/200

Central scotoma / Amsler grid metamorphopsia

• Watzke – Allen test ( WAT )

• laser aiming beam test ( LAM )

CLINICAL AND DIAGNOSIS

CLINICAL AND DIAGNOSIS

FFA - transmission defect at hole or partial blockage at surrounding subretinal fluid

OCT and SLOMacular perimetry - absolute

scotoma surrounding with relative scotoma

MH WITH SPECIFIC CAUSE

1. TRAUMA

- trauma with cystic macular degeneration

associated with MH formation

- concussion effect & residual macular

traction after incomplete PVD

traumatic MH

traumatic MH

2.PATHOLOGIC MYOPIA

- progressive thining and strechingof posterior pole, loss of choriocapillarislead to cystic formation and macularatrophy

- FFA - abnormal slow choroidaland retinal blood flow

3. LASER

- LASER eg. Argon, dye laser, Xenon,

Krypton, YAG - Thermal pigment absorption

LASER

LIGHTNING

4.ELECTRIC CURRENT

- electric current can cause of cataract by current pass to the eye

- study ; 2 from 159 electric burn

patient develop MH

5.ORTHERS

- pilocarpine

- Best’s disease

- intravitreous ceftazidime

- Alport

- Von Hippel

NATURAL COURSE

Different between stage 1 – 4stage 1 - MH s PVD progress to FTMH 33-52 %

- MH c PVD not turn to FTMHstage 2 - most turn to stage 3, 4stage 3,4 – almost always stable or slowly

progress

Chance of FTMH in fellow eye

1. if no PVD in both eyes - high risk 2. if PVD in FTMH eye but no PVD in fellow eye - intermediate risk 3. if PVD in fellow eye - no/very low risk

DIFFERENTIAL DIAGNOSIS

1. PSEUDOHOLE- may be retinal excavation without

tissue loss, RPE atrophy, granular pigmentchange around normal foveal depression

- may because of dehiscence of gliotic preretinal membrane on the macular

- associated with ERM, vitreomaculartraction syndrome, PDR, RRD, inflammation

- VA normal or slightly reduce or

distortion

- FFA normal

- good prognosis

- no evidence of leser therapy

- vitrectomy surgery or membrane

peeling when VA < 20/80 or distortion

pseudohole

2.MACULAR CYST - intact inner and outer retinal

layer, Intraretinal fluid cystic maculardegeneration with loss of nerve fiberlayer, ganglion cell, IPL , inner aspect ofInner nuclear layer

- large cyst in chronic or severeCystoid macular edema looklike MH

- Watzke – Allen test normal- VA 20/20 – 20/100

- FFA - pooling in cystic space inlate venous phase

- CME associated with intraocularGas, trauma, inflammation, exudate macular degeneration, DM

- fluid accumulated between innernuclear and outer plexiform layer

- prognosis depend on underlyingCause, size, chronicity of cytoid edema

- no treatment

3.PARTIAL THICKNESS HOLE

- outer lamella hole (OLH ) - inner lamella hole ( ILH )

• outer lamella hole ( OLH )

- collapse of outer wall of Cyst follow break down of outer BRB at RPE

- associated with Berlin’ s edema,macular schisis, LASER

- slightly irregular deep round or ovalExcavation with intact inner retinal tissue

- VA 20/20 – 20/400- FFA - window defect

• Inner lamella hole ( ILH )

- common, may be intermediate stageto develop FTMH

- rarely develop from chronic CME, spontaneous rupture at inner wall of cyst result in round oval excavation in retinasize < 500 micron

- may develop from radiation, gasC3F8, telangiectasia

- VA 20/20 – 20/80

- FFA - no transmitted fluorescene,minimal window defect or accumulated inperifoveal cystoid space

Treatment

Kelly and Wendel 1991

conceptsrelease traction forcere-position the

displaced neural tissue

TREATMENTBasic step of MH surgeryPPV Remove of post. HyaloidERM dissectionCheck peripheral retinal breakFAXInject adjunctive agent ( if use )Air – gas/SO exchange prone position

1. PPV and delamination of vitreous cortex- remove AP, tangential,circumferential force

- fish – strike or diving rod sign

2. Delamination of ERM- peeling of visible ERM and / or

ILM- prevalence of ERM

- 80% in Pseudophakic eye- 63 % in phakic eye

3.Delamination of ILM

- fibroblast like cell- +/- 0.2 -0.4 cc. Of 0.5% ICG stain- complication s

- trauma to retina - Light toxicity – 15 min - ICG RPE toxicity

4.Adjuvant

- growth factor B2, collagen plug,thrombin – activated fibrinogen, thrombinautologous platlet concentration, \autologous serum

- endolaser

5.Temponade of MH

- gas or silicone oil- postop. 12 -16 % C3F8 facedown

1-3 wks then 6 hr. per day until no gas( 4 – 6 wks )

6.Elimination or reducing duration position- short acting gas ( 4 days )

- SO 6 – 12 wks

7.Orther

- macular scleral buckle may beUsed in high myopia, post. StaphylomaOr MH with extensive subretinal fluid

8.Repaired reopened MH- repeat vitrectomy or FGX, FGX

With laser photocoagulation

Other alternatives

macular buckleminimal vitrectomy (Rick Spaide)pharmacological vitreolysis eg plasmin,

hyaluronidase, TPA, urokinase, plasminogen

RPE laser treatment

RESULT OF SURGERY

Stage 1 lesion

- no benefit to PPV

- stage 1

- VA 20/40 30% turn FTMH

- VA 20/50 – 20/80 % turn FTMH

Stage 2 – 4

position of hole - elevate or flat

edges of hole - open or closed

Anatomical outcome

1. elevate/oper - failed surgical2. flat/open - VA rarely better than

20/503. flat/close - VA > 20/30

COMPLICATIONS

cataract 70% in 2 yrsRD 2 – 11%ERMPeriphery iatrogenic retinal break 5.5 %VF defect ( temporal wedge)Increase IOPRPE changeEndophthalmitis < 1 : 1000Ulnar neuropathy

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