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Macular hole

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Macular hole

  2. 2. •A macular hole (MH) is a retinal break commonly involving the fovea •A macular hole is a full thickness defect, or hole, in the neurosensory retina located within, or just eccentric to the center of the fovea. •An impending macular hole, also known as a stage 1 macular hole, is considered the precursor to a full thickness idiopathic macular hole. Impending macular holes have a splitting of the inner retina with the clinical appearance of a foveolar cyst or pseudocyst. •In some cases, this inner splitting can be associated with a defect in the underlying outer retina (Lee, Kang et al. 2011).
  3. 3. •An impending macular hole with a defect in the outer retina can have a very thin intact inner retina referred to as the roof of the impending macular hole. These impending macular holes can progress to become full thickness macular hole once a break in the inner retina or roof occurs. •Most macular holes, unless otherwise specified, refer to idiopathic macular holes. Idiopathic macular holes occur from tractional forces on the foveola at the vitreoretinal interface not associated with other causes. • Other types of macular holes include those associated with trauma, high myopia, retinal detachments, lasers accidents, lightning strikes, diabetic retinopathy, and epiretinal membranes
  4. 4. Etiology and Risk Factors Idiopathic macular hole is the most common presentation. Risk factors include age, female gender, myopia, trauma, or ocular inflammation. Pathophysiology It has been hypothesized that MHs are caused by tangential traction as well as anterior posterior vitreoretinal traction of the posterior hyaloid on the parafovea. MHs are noted to be a complication of a posterior vitreous detachment (PVD) at its earliest stages
  5. 5. Diagnosis •This is a clinical diagnosis based on history and clinical exam, including slit lamp and dilated fundus examination. In some cases, optical coherence tomography (OCT) is useful in the diagnosis and management of this condition. It is important to distinguish between a full-thickness macular hole versus a lamellar hole (irregular foveal contour with defect in the inner fovea) or pseudohole (an irregular foveal contour with steep edges without true absence of retinal tissue often associated with an epiretinal membrane).
  6. 6. History Patients with MHs typically present over the age of 60 and females are more frequently affected. Idiopathic MH occur with an estimated incidence of 8.69 eyes per 100,000 population per year in one study. A careful history should be obtained to investigate for any of the risk factors mentioned above. Physical examination Slit lamp examination with special attention to the macula is important in the evaluation of this disorder . The Watzke-Allen sign can be used as a clinical test in cases of a suspected full thickness macular hole by shining a thin beam of light over the area of interest. The patient would perceive a “break” in the slit beam in cases of a positive test. Careful examination of the fellow eye is also recommended given that MHs are bilateral in up to 30% of patients. Special attention should be paid to the vitreoretinal interface, involutional macular thinning, and retinal pigment epithelial window defects because these are risk factors for MH development in the fellow eye. Patients without a PVD in the fellow eye have an intermediate risk (up to 28%) of developing a MH whereas patients with a PVD are at low risk for developing a MH.
  7. 7. Signs Depending on the stage of the MH, a subfoveal lipofuscin-color spot or ring can be noted. In more advanced cases, a partial or full- thickness macular break is observed. Symptoms Metamorphopsia (distortion of the central vision), central visual loss, or central scotoma can be reported.
  8. 8. Clinical diagnosis There are two main classification schemes for macular holes. Gass first described his clinical observations on the evolution of a macular hole: Stage 1 MH, or impending MH, demonstrates a loss of the foveal depression. A stage 1A is a foveolar detachment characterized a loss of the foveal contour and a lipofuscin-colored spot. A stage 1B is a foveal detachment characterized by a lipofuscin-colored ring. Stage 2 MH is defined by a full thickness break < 400µm in size. It might be eccentric with an inner layer “roof.” This can occur weeks to months following Stage 1 MHs. A further decline in visual acuity is also noted. In most cases, the posterior hyaloid has been confirmed to be still attached to the fovea on OCT analysis. Stage 3 MH is further progression to a hole ≥400 µm in size. Nearly 100% of stage 2 MHs progress to Stage 3 and the vision further declines. A grayish macular rim often denotes a cuff of subretinal fluid. The posterior hyaloid is noted to be detached over the macula with or without an overlying operculum. Stage 4 MH is characterized by a stage 3 MH with a complete posterior
  9. 9. Figure 1: Clinical photo demonstrating a full thickness macular hole with a grayish macular rim suggestive of subretinal fluid. Note the retinal pigment epithelial changes at the base of the hole
  10. 10. Full-thickness macular hole showing a surrounding cuff of subretinal fluid
  11. 11. Fundus photograph of a stage 1a macular hole with characteristic yellow spot at the center of the fovea.
  12. 12. Gass Biomicroscopic Classification Stage 1a Seen as a yellow spot. This is not specific for macular hole - can be associated with central serous chorioretinopathy, cystoid macular oedema, and solar maculopathy. Stage 1b Occult hole: doughnut-shaped yellow ring (approximately 200-300 μm) centred on the foveola. Approximately 50% of holes progress to stage 2. Stage 2 Full thickness macular hole (<400 μm). Prefoveolar cortex usually separates eccentrically creating a semi-transparent opacity, often larger than the hole, and the yellow ring disappears. These generally progress to stage 3. Stage 3 Holes >400 μm associated with partial vitreomacular separation. Stage 4 Complete vitreous separation from the entire macula and optic disc
  13. 13. Figure 3: This figure demonstrates the stages of macular holes based on the 1995 paper by J. Donald M. Gass.(9) This figure shows the range of pathology between cystic changes (Stage 1) to full thickness defects with a complete posterior vitreous detachment (Stage 4) Gass Stages: Stage 1 - cystic foveal change Stage 2 - 100-300 micron full-thickness retinal defect with pseudo-operculum Stage 3 - 250-600 micron full-thickness retinal defect with a persistent hyaloid attachment Stage 4 - stage 3 with complete PVD
  14. 14. Figure 2. Stages of macular hole as seen by optical coherence tomography. A) Stage 1B occult hole, vision 20/40. B) As the pseudo-operculum lifts, the hole goes to stage 2 and becomes apparent on clinical examination, vision 20/70. C) The pseudo- operculum is now separated from the retina in this stage 3 macular hole, vision 20/160. D) The macular hole is stage 4 when the posterior vitreous detaches, vision 20/200. E) Two months after surgery with vitrectomy, fluid-gas exchange and face- down positioning, the hole is closed and the vision has improved to 20/30.
  15. 15. Stage 0 Minimal changes in the foveal contour with detachment of the perifoveal vitreous cortex without traction. VMA Stage 1A: Imminent MH Foveal cysts and sensory foveolar detachment associated with perifoveal detachment with traction of the posterior vitreous on the foveal internal limiting membrane. VMT Stage 1B Cyst in the outer retina causing rupture of the cones layer. Perifoveal detachment of posterior vitreous. VMT Stage 2: Small MH Full-thickness MH of small diameter, with partial rupture of the internal wall of the cyst. Partial detachment of the posterior vitreous, which remains adhered to the operculum. FTMH small / medium with VMT Stage 3: Large MH MH of a larger size. Total detachment of the posterior vitreous at the level of the macular area, which persists adhered to the papillae. Occasionally, a free operculum adhered to the posterior vitreous can be seen. FTMH medium / large with VMT Stage 4: Full-thick MH with PVD Total detachment of the posterior vitreous. In some cases, the vitreous is not observed on OCT scans. Larger diameter of the hole with halo of outer retinal detachment in many occasions. FTMH small / medium / large without VMT Table 1. Comparison of Gass and IVTS classification from Garcia-Lavana et al 20152 (FTMH: full-thickness macular hole; MH: macular hole; PVD: posterior vitreous detachment; VMA: vitreomacular adhesion; VMT: vitreomacular traction2)
  16. 16. figure 1 showing a stage 1B macular hole. There is a defect in the outer layers of the retina.
  17. 17. OCT of a stage 2 macular hole with a break in the roof and cystoid hydration.
  18. 18. Full thickness stage 3 macular hole with overlying operculum. This macular hole would be classified as stage 4 if the posterior vitreous completely detached from the macula and optic nerve
  19. 19. More recently, the The International Vitreomacular Traction Study (IVTS) Group also formed a classification scheme of vitreomacular traction and macular holes based on OCT findings[5]: Vitreomacular adhesion (VMA): No distortion of the foveal contour; size of attachment area between hyaloid and retina defined as focal if </= 1500 microns and broad if >1500 microns Vitreomacular traction (VMT): Distortion of foveal contour present or intraretinal structural changes in the absence of a full-thickness macular hole; size of attachment area between hyaloid and retina defined as focal if </= 1500 microns and broad if >1500 microns Full-thickness macular hole (FTMH): Full-thickness defect from the internal limiting membrane to the retinal pigment epithelium. Described 3 factors: 1) Size -- horizontal diameter at narrowest point: small (≤ 250 μm), medium (250-400 μm), large (> 400 μm); 2) Cause -- primary or secondary; 3) Presence of absence of VMT Other features to note on exam include yellow deposits at the base of the hole, retinal pigment epithelial changes at the base of the hole, and epiretinal membrane adjacent to hole.
  20. 20. Figure 2: Optical coherence tomography image of a macular hole with an overlying operculum. The classic “anvil-shaped” deformity of the edges of the retina is noted due to intraretinal edema.
  21. 21. igure 3: A small full thickness macular hole on optical coherence tomography with intraretinal and subretinal fluid.
  22. 22. Diagnostic procedures Fluorescein angiography demonstrates a hyperfluorescence pattern consistent with a transmission defect due to loss of xanthophyll at base of the MH. It typically shows a window defect early in the angiogram that does not expand with time, and there is no leakage or accumulation of dye.There may be Amsler grid abnormalities. However, plotting small central scotomas is often difficult. However, OCT is the gold standard in the diagnosis and management of this disorder. This high-resolution image can allow evaluation of the macula in cross-section and three-dimensionally. OCT can be helpful detecting subtle MHs as well as staging obvious ones. OCT can also help guide management. OCT can assist in the determination of whether there is an associated epiretinal membrane or if the posterior hyaloid is still attached or not, which can be critical in deciding on the surgical approach. It can also be used to aid in gauging the prognosis of the fellow eye.
  23. 23. The Amsler Grid is a simple test that will help you determine if your vision is distorted in this way Left: An Amsler Grid with straight lines as seen by a normal-sighted person Right: A person with macular problems may notice distortion of the grid pattern such as bent lines and irregular box shapes or a grey shaded area
  24. 24. Differential diagnosis The clinical appearance of a MH is fairly distinctive. However, epiretinal membrane with a pseudohole, lamellar hole, and vitreomacular traction must also be considered. Cystoid macular edema, subfoveal drusen, central serous chorioretinopathy, or adult vitelliform macular dystrophy are also in the differential diagnosis of a stage 1 MH.
  25. 25. SD-OCT demonstrating lamellar hole features described by Witkin et al.8 The OCT demonstrates an irregular foveal contour, a break in the inner fovea, an intraretinal split with bridging columns and the absence of a full thickness foveal defect. An ERM is also present.
  26. 26. SD-OCT demonstrating partial PVD with vitreo-foveal traction and concurrent ERM.
  27. 27. SD-OCT of foveal detachment. This has variously been classified as stage 1b macular hole by Altaweel and Ip2 and also type 1A impending hole by Yeh et al.10 and thought to be the precursor of FTMH.
  28. 28. Stratus OCT of vitreofoveal traction associated with inner retinal pseudocyst formation (type 1B impending hole), which is suggested by Yeh et al.10 to be a precursor of LMH.
  29. 29. Stratus OCT demonstrating foveal detachment. The presence of a FTMH cannot be ruled out because of the presence of artifact that may mask the presence of a combined inner and outer retinal break (arrowhead).
  30. 30. Flowchart of evolution of macular interface pathology and reported mechanisms of pathogenesis.
  31. 31. SD-OCT demonstrating MPH (ERM and steepened foveal contour), but with reduced foveal retinal thickness more commonly associated with LMH
  32. 32. SD-OCT of FTMH with LMH features of intraretinal split and ERM.
  33. 33. Common features of lamellar macular hole are depicted, including an irregular foveal contour, a defect of the inner fovea (between bars), a separation between the inner and outer retina, and an intact outer retina (lack of full-thickness hole). There is also an associated ERM present,
  34. 34. Additional typical examples of lamellar macular hole,
  35. 35. In a lamellar macular hole, the defect between the inner and outer retina often conforms to an anvil shape. This area also may have schisis-like clefts.
  36. 36. Depending on the exact cross section imaged, the defect between the inner and outer retina in a lamellar macular hole may be asymmetric.
  37. 37. Macular hole. (A) Stage 1 macular hole in a 63-year-old woman with a 3-month history of decreased visual acuity (20/60). An outer retinal defect can be observed in the B-scan (arrow). (B) A full-thickness retinal defect developed after 2 months of follow-up with worsening in the visual acuity (20/80). The posterior vitreous remains adhered to the edge of the macular hole. (C) One month after surgery, the macular hole was closed and the visual acuity improved to 20/50, but a persistent foveal outer defect could be observed (arrowhead).
  38. 38. Preoperative OCT of an eccentric stage 2 macular hole with vitreous adhesion to the roof of the macular hole
  39. 39. Fundus photo of the traumatic macular hole with associated subretinal hemorrhage and choroidal rupture
  40. 40. Medical therapy In general, most Stage 1 MH can be followed conservatively given approximately 50% chance of spontaneous closure[. However, if the patient has symptomatic VMT or even a full-thickness macular hole with associated VMT, some may consider one of the following treatment options: Intravitreal ocriplasmin. Ocriplasmin is a 27 kilodalton serine protease that essentially performs pharmacolytic vitreolysis, separating the hyaloid from the underlying retina. In the registration MIVI-TRUST clinical trials. At the day 28 post injection time-point, eyes receiving ocriplasmin (injected intravitreally) exhibited greater release of the vitreoretinal attachment (primary endpoint, 26.5% vs. 10.1% in controls who received an intravitreal injection of drug vehicle, p < 0.001) and closure of macular hole (40.6% vs. 10.6%, p < 0.001). There have been some rare adverse effects reported in association with use of this drug including electroretinography changes, lens subluxation, and dyschromatopsia. Intravitreal gas or air. More recently, some have found success in treating patients with a small bolus of injected intravitreal gas or air. Research studies are ongoing, but some have reported success rates as high as 83% for the closure of FTMH