INTRODUCTION Defect of foveal retina involving its full thickness from the ILM to the outer segment of the photoreceptor layer Knapp (1869) – 1 st described Oglive (1900) – 1 st coined 1970- 80% idiopathic - 10% trauma
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 theory Post 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 USG Stage I – retinal suface irregularity - perifoveal PVD - VMT - pseudooperculum Stage II – I + partial foveal PVD Stage 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.
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)- 1 st recommended surg. Procedure Kelly & Wendel (1991) 1 st 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
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
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.