Presentation on macular hole and epiretinal membrane

1. MACULAR HOLE
&
EPIRETINAL MEMBRANE
KUMAR VAIBHAV (2ND YEAR RESIDENT MD OPHTHALMOLOGY)
BPKLCOS, IOM

2. LAYOUT
• Brief
• Vitreous
• Macula
• OCT
• Macular Hole
• Epiretinal Membrane

3. VITREOUS
• Transparent gel composed of water,
collagen, and hyaluronic acid
• Occupies 80% of the volume of the
eye
• Vitreous body is divided into
central/core, and peripheral/cortical
• Collagen fibers are dense in vitreous
base

4. VITREOUS
• Inserts into a
ringlike area that
extends 2 mm
anterior and 3–4
mm posterior to
the ora serrata.
• Firmly attached to
the lens capsule,
retinal vessels,
optic nerve, and
macula

5. POSTERIOR VITREOUS DETACHMENT
The posterior vitreous is detached and
the prepapillary hole in the posterior
vitreous cortex is anterior to the optic
disc

6. MACULA
• The central area measures 5.5 mm diameter and is centered
between the disc and the temporal vascular arcades.
• Macula is different from periphery by ganglion cell layer as here
ganglion cell layer is several cells thick, totaling half of all ganglion
cells in retina

8. OCT
• Optical coherence tomography is a non-invasive imaging technique
that allows for examination of ocular structures
• Infrared light at approximately 830 nm is scanned across the tissue
and focused with an internal lens
• A second beam internal to OCT unit is used as a reference and a
signal is formed by measuring the amount the reference beam is
altered to match the reflected beam from the retina
• Technology platforms
• Time Domain OCT
• Spectral Domain OCT
• Multifunctional OCT
• Swept source OCT
• High speed Ultra High Resolution OCT
• Adaptive Optics OCT (AO-OCT)

9. OCT

10. OCT

11. MACULAR HOLE
• Full thickness neural retinal defect in retinal center
• Round full thickness opening in macular center

12. HISTORY
• 1869: Knapp, described MH in a traumatic case
• 1871: Noyes, gave a detailed ophthalmoscopic description of a
traumatic case
• 1900: Ogilvie gave the term “hole at the macula.”
• 1900 and 1907:Kuhnt & Coats suggested that the origin of MH was
degenerative
• 1912 and 1924: Zeeman and Lister attributed it to a vitreoretinal
tractional mechanism.
• 1988: J D Gass on biomicroscopic observations proposed a staging
system ranging from impending to full-thickness MH
• Kelly and Wendel: performed the first successful surgery of MH
• Hee and Puliafito: were the first to describe the stages of MH on OCT
scans.

13. EPIDEMIOLOGY
• Prevalence
• 3.3 per 1000 (Baltimore Eye Study USA)
• 0.2 per 1000 (Blue Mountains Study Australia)
• 0.9 per 1000 ( Beijing study China)
• 1.7 per 1000 in a study in Southern India
• In the Beaver Dam study, the prevalence was 2.9 per 1000
• Macular holes are rare conditions occurring in approximately 1 in
5000 patients.

14. RISK FACTORS
• Age 65 and above
• Female
• PVD
• The risk of MH in fellow eye is estimated to be ~10–15%
• MH have been reported in association with:
• Trauma
• Nd-YAG Laser treatment
• Retinal vascular disease
• Retinal detachment repair
• Lightening and electrocution

15. PATHOGENESIS THEORIES
• Vitreomacular Traction: Grignolo 1952 provided histologic evidence
of strong vitreomacular adherence to the fovea,
• Anteroposterior traction of vitreous fibers on the fovea play a
role in the formation of MH

16. PATHOGENESIS THEORIES
• Foveal Cyst: 1982, McDonnell et al. analyzed fellow eyes and eyes
with MH & concluded that idiopathic macular cysts and holes are
part of the same disorder and described the role of the vitreous
body in their formation.
• 1998, Folk et al. used laser slit-lamp photography, to obtain a
direct image of the foveal cysts which they considered to be a
pre-hole condition
• Hee and Puliafito used OCT, which showed, for the first time, the
vitreous cortex attached on the roof of a foveal cyst

17. PATHOGENESIS THEORIES
• Contraction of the premacular vitreous cortex: 1988, Gass
postulated that tangential contraction of prefoveal “posterior
hyaloid membrane” resulted in detachment of central
photoreceptors and opening of the fovea.

18. OCULAR MANIFESTATIONS
• Variable decreased vision
• Metamorphosia
• Central Scotoma

19. SIGNS
• Visual acuity: Variable
• Bio microscopy: Round, red spot in the center of the macula, of
1/3rd to 2/3rd of disc diameter, may be surrounded by a gray halo
or cuff of sub retinal fluid.

20. SIGNS
• Positive Watzke Allen Test
• Amsler Grid Testing: Non specific distortion

21. STAGING PROPOSED BY GASS
• Stage 1: An impending hole, yellow spot, or ring in fovea.
• Stage 2: Small full-thickness hole.
• Stage 3: Full-thickness hole with cuff of SRF, no PVD.
• Stage 4: Full-thickness hole with cuff of SRF, with complete PVD.

22. STAGE 0 OF MH
• Early changes in foveal tissue
• Partial perifoveal vitreous detachment with no foveal cysts
• Foveal elevation of Cone Outer Segment Tips (COST) and inner
segment/ outer segment lines
• Asymptomatic eye with good vision

23. STAGE 1A OF MH : IMPENDING MH
• Central yellow spot & loss of the foveal depression associated with
no vitreofoveal separation.
• OCT shows an inner foveal cyst. The foveal floor is elevated by the
traction exerted by the posterior hyaloid which is detached around
the foveola but still attached at its center.

24. STAGE 1B OF MH: OCCULT MH
• Gass: Yellow ring in fovea + absence of vitreoretinal separation +
loss of foveal depression
• OCT shows posterior hyaloid is detached from macular surface
except at the foveal center, and that the inner foveal cyst
characterizing Stage 1A is completed by disruption of the outer
retina up to the retinal pigment epithelium (RPE)
• Stage 1B impending MH is a true occult MH. The yellow ring is due
to the edematous border of the disrupted outer retina.

25. STAGE 2 OF MH: FULL THICKNESS MH
• Eccentric oval, crescent or horseshoe-shaped retinal defect inside
the edge of the yellow ring
• Pre-foveolar vitreous tissue bridging the round retinal hole, with no
loss of foveolar retina
• OCT shows incompletely detached operculum is pulled in an oblique
direction by the incompletely detached posterior hyaloid

26. STAGE 3 OF MH: FULL THICKNESS MH
• Gass: Central round retinal defect more than 400 μm in diameter,
with a rim of elevated retina, with or without pre-foveolar pseudo-
operculum and without a Weiss’s ring.
• The posterior hyaloid (PH) is detached from the macular surface
and contains the operculum, edge of the hole has been thickened by
cystic spaces and the photoreceptors are elevated

27. STAGE 4 OF MH: FULL THICKNESS MH
• Complete PVD with a Weiss’s ring.

28. DIFFERENTIAL DIAGNOSIS
• Epiretinal membrane and pseudohole
• Lamellar macular hole
• Chronic cystic macular edema
• Central serous chorioretinopathy
• Solar retinopathy
• Central drusen in ARMD

29. NON IDIOPATHIC OR SECONDARY MACULAR
HOLE
• Orbital trauma
• High myopia
• Rare causes
• Drusen
• Electrical shock injuruy
• Fungal endophthalmitis
• Nd:YAG laser injury
• Retinitis pigmentosa
• Vitrectomy

30. TREATMENT
• Stage 1
• Observation as such holes have a spontaneous closure in 50%
cases surgery to be considered only if it is hampering vision
• Stage 2
• Vitrectomy has a good prognosis in such cases
• Ocriplasmin:Vitreopharmacolysis, FDA approved
recombinant protease
• Stage 3 & 4
• Vitrectomy ± ILM Peeling ± ERM Peeling + FAX + SF6/ C3F8

31. RECENT MODIFICATIONS
• INVERTED INTERNAL LIMITING MEMBRANE FLAP
TECHNIQUE
• INTERNAL LIMITING MEMBRANE FREE-FLAP TECHNIQUE

32. RECENT MODIFICATIONS
• ARCUATE RETINOTOMY
• RADIAL INCISIONS

33. COMPLICATIONS OF SURGERY
• Cataract: 80% patients within a year
• Retinal detachment: 1-5% patients
• Visual field defects: Upto 20% patients with mostly temporal visual
field defects
• Reopening of macular hole
• Endophthalmitis: 0.05% cases

34. EPIRETINAL MEMBRANE
• An avascular, fibrocellular membrane that proliferates on the inner
surface of the retina and produces various degrees of macular
dysfunction.
• Premacular fibroplasia, macular pucker, cellophane maculopathy,
and premacular gliosis have been used to describe this condition

35. PREVALENCE
• Beaver Dam Eye Study and Blue Mountains Eye Study
• Prevalence: 7–11.8%
• Idiopathic ERMs were bilateral in 19.5–31%, with a 13.5% 5-year
incidence of second eye involvement.
• ERM uncommon before 60 (1.9%) and peak between the ages of
70-79 (11.6%)
• ERM were more common in women in the Beaver Dam Eye Study

36. RISK FACTORS
• After retinal detachment surgery: older age, preoperative vitreous
hemorrhage, macular detachment, large retinal breaks,
intraoperative use of cryotherapy, and multiple operations
• Mild epiretinal membrane formation: Blunt or penetrating ocular
trauma, vitreous inflammatory conditions, long-standing vitreous
hemorrhage

37. PATHOGENESIS
• The pathogenesis of epiretinal membranes is not completely
understood.
• ERM formation represents a reactive gliosis in response to retinal
injury or disease involving inflammatory and glial cells.
• ERMs have two main components:
• Extracellular matrix (consisting of collagen, laminin, tenascin,
fibronectin, vitronectin, etc.)
• Cells of retinal and extra retinal origin (such as glial cells,
neurites, retinal pigment epithelium, immune cells and
fibrocytes)

38. PATHOGENESIS
• Idiopathic ERMs are composed predominantly of glial elements and
a clinically-detectable PVD is present in approximately 90% of eyes
and proliferation, fibrous metaplasia, and contraction of hyalocytes
left behind on the inner retinal surface after PVD (vitreoschisis)

39. PATHOGENESIS
• ERMs due to proliferative vitreoretinopathy (in retinal breaks) are
more heavily pigmented due to a high RPE content and retinal
pigment epithelial cells that are liberated into the vitreous cavity
and proliferate, along with other cellular constituents to form
contractile membranes on the retinal surface.

40. PATHOGENESIS
• ERMs that develop due to retinal ischemia and neovascular
proliferation have a larger vascular component and Cellular
proliferation stimulated by vitreous inflammation or breakdown of
the blood-retinal barrier

41. Harada C, Mitamura Y, Harada T. The role of cytokines and
trophic factors in epiretinal membranes: involvement of signal transduction in
glial cells. Prog Retin Eye Res 2006;25:149–64.)

42. ETIOLOGICAL CLASSIFICATION
• IDIOPATHIC
• SECONDARY
• Retinal vascular disease (BRVO, CRVO, Diabetic retinopathy)
• Intraocular inflammation
• Trauma
• Retinal detachment and retinal tears
• Intraocular tumors (Retinal angiomas, Hamartomas)
• Retinal dystrophies (Retinitis pigmentosa)
• IATROGENIC
• Postoperative (Cataract, Retinal detachment Silicon oil)
• Retinopexy (Laser or cryotherapy)

43. GRADING & CLINICAL FEATURES
• Grade 0/Cellophane maculopathy: Translucent membrane with no
underlying retinal distortion is observed, asymptomatic, incidental
finding

44. GRADING & CLINICAL FEATURES
• Grade 1: Irregular wrinkling of the inner retina involves the fovea,
patients often complain of distorted or blurred vision, loss of
binocularity, central photopsia, and macropsia. Eccentric grade 1
ERMs not involving fovea may be asymptomatic

45. GRADING & CLINICAL FEATURES
• Grade 2 ERM/ Macular Pucker: Opaque membrane causing
obscuration of underlying vessels and marked full thickness retinal
distortion, may be associated with cotton-wool spots, exudates, blot
hemorrhages and microaneurysms. 80% of patients with Grade 2
ERM have blurred vision or metamorphopsia

46. DIFFERENTIAL DIAGNOSIS
• VMT: incomplete separation of the posterior vitreous with
persistent macular adhesion and traction
• Increased retinal thickness, cystoid macular edema and
lamellar or full thickness macular holes
• ERMs may coexist with VMT in 26–83%
• VMT with ERM the vitreous in the mid-periphery is detached,
whereas in ERM without PVD the vitreous is attached

47. DIFFERENTIAL DIAGNOSIS
• Cystoid Macular Edema: multiple cyst-like areas of fluid appear in
the macula that cause retinal swelling or edema
• No distortion of the microvasculature
• Always centered on the fovea
• FFA: ‘Star Pattern’ in late venous phase
• May also coexist with ERM (in vein occlusions)

48. INVESTIGATIONS
• OCT: Epiretinal membrane is seen as a hyperreflective layer on the
surface of the retina associated with blunting of the foveal contour,
increased retinal thickness and intraretinal cysts.
• Idiopathic ERMs are adherent globally to the underlying retina,
secondary ERMs are likely to be characterized by focal retinal
adhesion
• Tool to monitor the clinical course of ERMs both prior to and
following surgery - OCT
OCT with ERM demonstrates significant surface retinal
distortion and inner retinal edema
OCT with highly reflective ERM, multiple points of retinal
attachment, loss of foveal depression, increased retinal
thickness.

49. INVESTIGATIONS
• FFA: Highlights extent of retinal wrinkling, degree of retinal vascular
tortuosity and presence of macular edema
Fluorescein angiogram showing retinal vascular
tortuosity as a result of an overlying epiretinal
membrane
Vascular leakage seen in late venous phase due
to traction from the overlying epiretinal
membrane

50. TREATMENT
• Observation: most patients will remain stable if observed
• Surgery: significant or progressive vision loss, debilitating
metamorphopsia or diplopia
• PPV + ERM peeling ± ILM peeling + FAX + Tamponade

51. PROGNOSTIC INDICATORS OF SURGERY
• Preoperative visual acuity
• Duration of diminished acuity before surgery
• Presence of preoperative cystoid macular edema
• Age of the patient
• Thickness of the epiretinal membrane
• Idiopathic versus nonidiopathic epiretinal membranes
• Presence of RPE window defects on fluorescein angiography

52. COMPLICATIONS OF SURGERY
• Intraoperative
• Petechial hemorrhages
• Preretinal hemorrhages
• Peripheral retinal breaks
• Lens may touch
• Postoperative
• Cataract
• Retinal Detachment
• Recurrence
• Endophthalmitis

53. VITAL DYES
• Chromovitrectomy: Using dyes to stain the ERM and ILM, has made
the peeling of the retinal surface more precise, more complete and
less traumatic
• Indocyanine green: (0.025% or 0.05%) and selectively stains ILM
but retinal pigment epithelium toxicity have been reported
• Trypan blue: stains ERM well but ILM less effectively, used after
fluid–gas exchange, non toxic to RPE*
• Brilliant Blue: (0.025% or 0.05%) Selective affinity for the ILM
• Reteblue

54. REFERENCES

55. THANK YOU