MACULAR HOLEDr samarth mishra

INTRODUCTION

• Defect of foveal retina involving its full thickness from the ILM to the outer segment of the photoreceptor layer

• Knapp (1869) – 1st described • Oglive (1900) – 1st coined• 1970- 80% idiopathic

- 10% trauma

DEMOGRAPHIC

• Prev.-worldwide 3.3 / 1000 (>55 yrs) - India 1.7 / 1000 (>67 yrs)

• F>M• Fellow eye (12%)• Risk – CVD, HTN, H/O Hysterectomy

ETIOLOGY• Idiopathic • Vitreous synersis• Post. Vitreous separation• CME• Trauma– contusion injury (6% ), accidental laser injury , lightening

• Progressive high myopia (foveal schisis)– foveal schisis and/or lamellar holes (31%)

• Preceding rhegmatogenous retinal detachment- successfully repaired rhegmatogenous retinal detachment (<1%)

PATHOGENESIS

• Lister (1924) –vitreous as pathogenesis• Gass (1988)– Viteomacular traction theory

Focal shrinkage of foveal vitreous cortex↓

Intraretinal foveolar cyst formation ↓

Unroofing of the cyst

• Tornambe et al (2003)Hydration theoryPost hyaloid traction of fovea↓Tear in inner fovea↓Seepage of fluid vitreous into spongy layers macula↓Cavity in inner retina ↓Enlargement of hole↓Spread to outer retina↓Swollen retina remains elevated & retracted

• Retinal / choroidal ischaemia theory– RPE dysfunction & possible intraretinal fluid

accumulation in the fovea• Involutional retinal thinning

GASS STAGING Stage 1a: ‘Impending’ macular hole

a Signs: yellow spot b Pathology: Müller cell cone detach from the underlying photoreceptor layer, with the formation of a schisis cavity (pseudocyst)

Stage 1b: Occult macular hole a Signs: a yellow ring (donut-shaped)b Pathology: photoreceptor layer undergo centrifugal displacement

Stage 2: Small full-thickness hole a Signs: < 400 µm , central, slightly eccentric

or crescent-shaped. b Pathology: dehiscence seen in the roof of the schitic cavity, pseudo-operculum

Stage 3: Full-size macular hole a Signs: - > 400 µm- red base with yellow-white dots- surrounding grey cuff of subretinal fluid

b Pathology: avulsion of the roof of the cyst with an operculum and persistent parafoveal attachment of the vitreous cortex.

Stage 4: Full-size macular hole with complete PVD

a Signs: as above b Pathology:PVD is complete (Weiss ring)

CLINICAL FEATURES

• VA– Depends according to the size, location, and the

stage of the macular hole– Stage I – metamorphosia ,6/9 to 6/12– Stage II – small & eccentric 6/9 to 6/12 or central – Stage III & IV – 6/24 to 2/60

DIAGNOSTIC TECHNIQUES

• Direct ophthalmoscopy – well-defined round or oval lesion in the macula

with yellow-white deposits at the base– lipofuscin-laden macrophages or nodular

proliferations of the underlying pigment epithelium with associated eosinophilic material

• Biomicroscopic (slit lamp) examination– A round excavation with well-defined borders interrupting

the beam of the slit lamp can be observed.– An overlying semitranslucent tissue (pseudo-operculum)– surrrounding cuff of subretinal fluid – Cystic changes of the retina at the margins of the hole– Fine crinkling of the inner retinal surface (ERM)

• Watzke-Allen test– slit lamp using a macular lens and placing a narrow

vertical slit beam through the fovea– positive test detect a break in the bar of light

• Laser aiming beam test– a small 50-µm spot size laser aiming beam– positive test ( fails to detect )

• Ocular coherence tomography (OCT)– detect the presence of a macular hole as well as changes in the surrounding

retina.– distinguish lamellar holes and cystic lesions of the macula from

macular holes.– status of the vitreomacular interface can be evaluated– evaluate the earliest of the stages & association of surrounding cuff of

subretinal fluid.

• Fluorescein angiography (FA)– differentiating macular holes from CME and CNV– Full-thickness stage 3 holes- granular hyperfluorescent window

associated with the overlying pigment layer changes

• B Scan USGStage I – retinal suface irregularity

- perifoveal PVD - VMT - pseudooperculum

Stage II – I + partial foveal PVDStage III – double hump irregularity

- echodense operculm - partial PVD attached to OD

Stage IV – double hump - echodense operculum - complete PVD with weiss ring

• Amsler grid – small central scotomas– bowing of the lines and micropsia

• Microperimetry and multifocal ERG– loss of retinal function corresponding to the

macular hole with subsequent recovery of function following surgical repair of the hole.

DIFFERENTIAL DIAGNOSIS

• Pseudohole• Foveal RPE atrophy• CME• Idiopathic CSR• Foveal drusen• RPE detachment• CNV• Lamellar macular lesions

MANAGEMENT• NO MEDICAL t/t• Autologous plasmin – Idiopathic and traumatic macular holes– Intravitreal injection of plasmin – October 2012, ocriplasmin (Jetrea) was approved by the

USFDA for the treatment of vitreomacular adhesion– Recombinant proteolytic enzyme– MIVI-TRUST study group– Activity against fibronectin and laminin– Randomized, double-blind study, 652 eyes with vitreomacular

adhesion were treated with an intravitreal injection of ocriplasmin

– 40.6% of treated eyes compared to 10.6% in the placebo group ]

SURGICAL CARE

• Indications– stage 2 or higher full-thickness macular hole– Stage 1 holes and lamellar holes are managed

conservatively • Contraindications – Coexisting choroidal rupture– Traumatic RPE rupture– Chronic cystoid macular edema– Optic nerve disorders

• Gonver & Machemer (1982)- 1st recommended surg. Procedure

• Kelly & Wendel (1991)– 1st demonstrated– 58% ASR & 42% VI of 2 lines– Mechanism is relief of traction, stimulation of

fibroglial proliferation• Time of surgery– Best <1yr– Chronic holes (1-5yrs) esp if fellow eye has

progressive macular / ON pathology

PROCEDURE

1. PPV / Delamination of cortical vitreous– standard 3-port (ie, 25 gauge, 23 gauge, 20 gauge)– Anterior and middle vitreous is removed– Relieved either by removing perimacular

vitreous / combining it with complete PVD– Soft tipped silicon cannula / vitrectomy cutter– Fish –strike sign / bending of silicon cannula

2. Delamination of ILM & ERM– Stained by DYE (ICG, Tryphan blue, Brilliant blue G)ICG – stains good

- possibility of renal toxicity - safety measure reduces toxicity (0.5 mg/ml dose, fast surg., slow injection, use of 20G, VINCE brush)

Triamcinolone acetonide – facilitate peeling of ILM

3. Adjuvant – Bovine TGF-b, recombinant TGFb, autologous

serum, plasmin– “chemovitrectomy”

4. Tamponade of hole– nonexpansile concentration of a long-acting gas is

exchanged for air– perfluoropropane or sulfur hexafluoride– Silicone oil has also been used as an internal

tamponade for patients with difficulty positioning or altitude restrictions

– Interfacial tension is the mechanism

5. Face-down positioning• Strict face-down positioning had been

recommended for patients for up to 4 weeks

COMPLICATIONS

• Cataract (75%)• Late macular hole reopening(2-10%)• RD (3%)• Retinal breaks (5.5%)• Raised IOP (1stwk)• Endophthalmitis(<1%)• Ulnar neuropathy• VF defects

PROGNOSIS

• Depends on:– Preop VA & duration– Preop minimum hole diameter & base diameter– Larger size & longer duration outcome is adversly

affected

PROGNOSIS

• Visual prognosis depends on the type of closure– U-pattern : normal foveal contour– V-pattern : steep foveal contour– W-pattern : foveal defect of neurosensory retina U>V>W

REOPERATION• Failed primary surg.

– 1-15% cases -↓use of adjuvents, ILM peel

• Late reopening– 2-10% cases– ↑axial length, pseudophakia, ERM

• Procedure –– Rpt PPV– PPV+ILM peel– PPV+ILM peel + DYE– Operate FAE with laser to RPE– Use longer acting gas – Stress on face down position– Silicon oil tamponade

CURRENT T/T STATAGIES• Almost all macular holes can achieve success• ILM peel improves hole closure (85-95%)• ILM peel slows visual recovery• Use of dye staining facilitates complete peel• Prone positioning duration decreased with ILM peel• 1Wk gas adequate in most eyes• Long term (5 yrs) results excellent 60% with6/12• Morizane et al ( 10 patients with refractory macular holes )

with autologous transplantation of ILM• PHACOVITRECTOMY

CONCLUSION

• Significant cause of loss in central VA• Becoming more common• Increased surgical closure rate (58% -90%)• Decreased complication rate• VA & VF improve in majority of pts.