Variable decreased vision (typically around 20/200 level for a full-thickness hole, better for a partial-thickness hole), metamorphopsia, or central scotoma. Three times more likely in women; usually occurs in sixth to eighth decade. Ten percent bilateral.

(See Figures 11.25.1 and 11.25.2.)

Figure 11.25.1: Macular hole.

Figure 11.25.2: Optical coherence tomography of macular hole.

Critical

A full-thickness macular hole appears as a round, red spot in the center of the macula, usually from one-third to two-thirds of a disc diameter in size; may be surrounded by a gray halo/cuff of SRF. VMT demonstrates loss of the normal foveolar depression and often a yellow spot or ring in the center of the macula.

Other

Small, yellow precipitates deep to the retina in the hole or surrounding retina; retinal cysts at the margin of the hole or a small operculum above the hole, anterior to the retina (Gass stage 3 or 4); or both.

Gass Classification of Macular Hole

• Stage 1: An impending (non–full-thickness) hole, yellow spot, or ring in fovea.

• Stage 2: Small full-thickness hole (<400 µm diameter), rim of elevated retina, no PVD.

• Stage 3: Full-thickness hole (≥400 µm diameter), rim of elevated retina, with or without localized operculum over hole, no PVD.

• Stage 4: Full-thickness hole (≥400 µm diameter), with complete PVD.

NOTE A new classification system using OCT has been developed. It is based on size of the full-thickness hole, presence of VMT, and underlying etiology (e.g., primary VMT vs. secondary trauma).

May be difficult to distinguish a macular hole from a pseudohole (no loss of foveal tissue) or a lamellar macular hole (partial thickness).

• Macular pucker with a pseudohole: An ERM (surface-wrinkling) on the surface of  the retina may simulate a macular hole. See 11.26, Epiretinal Membrane (Macular Pucker, Surface-Wrinkling Retinopathy, Cellophane Maculopathy). Look for a sheen from ILM changes or ERM.

• Lamellar hole: Not as red as a full-thickness hole, and a surrounding gray halo is usually not present.

• Intraretinal cysts (e.g., chronic CME with prominent central cyst).

• Solar retinopathy: Small, round, red or yellow lesion at the center of the fovea, with surrounding fine gray pigment in a sun gazer or eclipse watcher. See 11.35, Solar and Photic Retinopathy.

May be caused by vitreous or ERM traction on the macula, trauma, or CME. In early stages of vitreomacular adhesion (VMA)/VMT, the vitreous cortex is attached to the fovea but detached from the perifoveal region, exerting anteroposterior traction on the fovea. Increased tractional forces can allow for eventual progression to full-thickness macular hole.

1. History: Previous trauma? Previous eye surgery? Sun gazer?

2. Complete ocular examination, including a macular examination with a slit lamp and a handheld lens. If a PVD is present, careful examination of the peripheral fundus to rule out peripheral breaks is important.

3. A full-thickness macular hole can be differentiated from a pseudo- or lamellar hole by directing a thin, vertical slit beam across the area in question using a handheld lens with the slit-lamp biomicroscope. The patient with a true hole will report a break in the line (Watzke–Allen test). A pseudohole or lamellar hole may cause distortion of the line, but it should not be broken.

4. OCT is essential for evaluating the vitreoretinal interface and determining the degree of traction from vitreous or the presence of any ERM. It is also useful in staging macular holes, differentiating from pseudo- or lamellar holes, and evaluating for progression (see Figure 11.25.3).

   Figure 11.25.3: Optical coherence tomography of vitreomacular traction.

   

5. IVFA sometimes may be helpful in identifying exudative retinal vascular disease (i.e., diabetic retinopathy, vein occlusion, pseudophakic CME) in cases that also have VMA/VMT.

1. Stage 1 macular holes can be observed, as 50% resolve spontaneously.

2. For symptomatic macular holes, PPV with ILM peel and gas tamponade remains the gold standard for treatment. It is preferable to operate within the first 6 months of onset for the highest chance of anatomic success and best visual recovery. Serious complications  are rare, but cataract progression in phakic patients is almost universal.

3. Ocriplasmin is a recombinant protease with activity against components of the vitreoretinal interface (fibronectin and laminin). It is FDA-approved for the treatment of symptomatic VMA, VMT, and macular hole. Adverse effects include ERG changes with vision loss, lens subluxation, and dyschromatopsias. It is no longer available in the United States.

1. Follow-up intervals vary depending on symptoms, examination, and surgical management.

2. All patients are seen promptly if RD symptoms develop.

3. Because there is a small risk that the condition may develop in the contralateral eye, patients are given an Amsler grid for periodic home monitoring.