Macular Hole

A macular hole is a small break or opening through all the layers of the retina at its very center—the macula. The word macula comes from the Latin word for spot, referring to the central 5 mm region of the retina responsible for sharp, detailed, and color vision. Within the macula sits the fovea—from the Latin word for pit—a tiny 1.5 mm zone packed with the cone photoreceptors that allow us to read, recognize faces, drive, and see fine detail. When a full-thickness break develops through all the retinal layers at this critical location, the result is a characteristic loss of central vision that glasses cannot correct.

Macular hole is classified as a vitreoretinal disorder—a condition involving the interface between the vitreous (the gel filling the interior of the eye) and the retina. It is not a single entity but an umbrella term covering several subtypes that share the same anatomical problem—a defect at the foveal center—but differ in their cause, their rate of progression, and their surgical approach. The most common subtype by far is idiopathic macular hole, which develops spontaneously in older adults due to age-related changes in the vitreous gel. Macular holes affect approximately 3.3 per 1,000 people in the general population, with an annual incidence of about 8.69 eyes per 100,000. They peak in the sixth through eighth decades of life—the 55 to 75 age range—and are approximately three times more common in women than in men.

Macular holes are progressive in the majority of cases. A stage 2 hole progresses to a larger, more advanced stage in about 74% of cases within 12 months without treatment. However, the prognosis with surgery is excellent: vitrectomy achieves anatomical closure of the hole in 85–100% of patients, and vision improves in about 58% of patients at one year after surgery. Without treatment, advanced macular holes typically leave vision at 20/200 or worse—the threshold for legal blindness—making timely diagnosis and treatment critically important.

Macular holes are classified by stage of development, by size, and by what caused them. Understanding the type helps determine the urgency and approach to treatment.

By stage of development (Gass classification), doctors recognize four stages reflecting the progressive nature of the condition. A stage 1 hole is an impending or early hole, in which the vitreous is still pulling on the fovea, but a complete break has not yet formed. A stage 2 hole is a small full-thickness break (400 micrometers or less in diameter) with the vitreous still attached. A stage 3 hole is larger than 400 micrometers, with the vitreous detached from the back of the eye but still partly present. A stage 4 hole is the same as stage 3 hole, but with complete separation of the vitreous. The most important subtypes by cause include:

• Idiopathic macular hole: the most common type, accounting for about 96% of all cases. It develops spontaneously as a result of age-related vitreous traction on the fovea. It affects primarily adults aged 55 to 75 and is about three times more common in women. The fellow (opposite) eye has approximately a 7–13% lifetime risk of also developing a macular hole.
• Lamellar macular hole: a partial-thickness defect in which the retina at the fovea is disrupted, but the deepest layers (the photoreceptors) remain intact. It produces milder visual symptoms than a full-thickness hole and is often associated with an epiretinal membrane—a thin layer of scar tissue on the retinal surface. It affects approximately 1–4% of people referred for macular assessment.
• Myopic macular hole: occurs in eyes with high axial myopia (very severe nearsightedness, with an eye that is longer than normal from front to back and may have a structural bulge at the back wall called a posterior staphyloma). These holes are harder to close surgically and carry a higher risk of the entire macula detaching from the back of the eye.
• Traumatic macular hole: caused by a blunt injury to the eye that transmits force through the vitreous to the fovea. It is more common in young males and has a higher spontaneous closure rate (about 23%) than idiopathic holes; observation for up to three months before offering surgery is reasonable.
• Secondary macular hole: caused by another eye condition rather than vitreous traction alone. Known causes include diabetic macular edema, retinal vein occlusion, macular telangiectasia (an abnormality of the tiny blood vessels at the foveal center), and certain medications such as tamoxifen (used to treat breast cancer) and toremifene.
• Refractory or recurrent macular hole: —a hole that fails to close after surgery (approximately 10% of cases) or that reopens after an initial successful closure. These cases require more advanced surgical techniques and carry a lower visual prognosis.

The formation of a macular hole involves a combination of abnormal mechanical forces applied to the central retina and structural vulnerability of the foveal tissue. Most idiopathic macular holes develop from the same age-related process: as the vitreous gel degenerates and liquefies over time, it begins to separate from the retinal surface—a normal process called posterior vitreous detachment (PVD). In most people, this separation proceeds smoothly. In some people, however, the vitreous remains abnormally adherent to the fovea as it pulls away from the surrounding retina, transmitting anteroposterior and tangential traction forces to the foveal center. This tractional pulling can tear or stretch the delicate foveal layers until a full-thickness hole forms. A specialized supporting cell type—called the Müller cell cone—forms a structural plug at the very center of the fovea, and its disruption or degeneration is thought to remove the structural support that normally keeps the foveal tissue intact under this traction. The specific causes and contributing factors include:

• Age-related vitreous traction: the primary mechanism in idiopathic holes. Incomplete posterior vitreous detachment leaves residual adhesion at the fovea, transmitting damaging mechanical forces to the central retina.
• Female sex and postmenopausal estrogen loss: The 3:1 female predominance in idiopathic macular hole is thought to reflect estrogen’s neuroprotective role. Estrogen supports the high-energy-consuming foveal cone photoreceptors and Müller glial cells through mitochondrial and cellular mechanisms; loss of estrogen after menopause may increase foveal vulnerability to vitreous traction.
• High myopia: The elongated, structurally stressed eye in severe nearsightedness places the retina under additional mechanical tension, predisposing to macular hole formation independent of standard vitreous traction mechanisms.
• Ocular trauma: Blunt force to the eye transmits compressive and tangential forces through the vitreous to the fovea, directly disrupting foveal tissue.
• Secondary conditions: Diabetes, retinal vein occlusion, inflammation (uveitis), abnormal foveal blood vessels, and certain medications can each cause structural changes at the fovea that result in a secondary macular hole through mechanisms distinct from vitreous traction.

The following factors are associated with a higher risk of developing a macular hole or of having a more complex clinical course.

• Older age: The peak incidence is between ages 55 and 75. Age-related vitreous liquefaction and incomplete PVD become more common with each decade.
• Female sex: Women develop idiopathic macular holes approximately three times more often than men, likely reflecting postmenopausal loss of estrogen’s protective effects on foveal photoreceptors and Müller cells.
• High myopia: Severe nearsightedness (particularly with axial length greater than 26 mm) carries a higher risk of macular hole and the more serious complication of macular hole-induced retinal detachment.
• Fellow eye involvement: Having a macular hole in one eye confers approximately a 7–13% lifetime risk of developing one in the other eye.
• Vitreomacular adhesion: Abnormal, persistent vitreous adhesion at the fovea detected on optical coherence tomography (OCT) imaging is a recognized precursor to macular hole formation and can be monitored for progression.
• Prior ocular surgery or trauma: This can introduce mechanical forces or structural changes that predispose to macular holes.
• Certain medications: Long-term tamoxifen (used in breast cancer treatment) and toremifene can cause a secondary macular hole as a recognized drug toxicity.

There is no population-wide screening program for macular holes. Most cases are diagnosed when a patient develops visual symptoms and seeks evaluation, or when a doctor detects early macular changes during a routine dilated eye examination. Adults over age 50 should have comprehensive dilated eye examinations at least every one to two years. Patients with known high myopia, a macular hole in the fellow eye, or a finding of vitreomacular adhesion on OCT imaging—a recognized precursor—should be monitored more frequently, as directed by their ophthalmologist.

Macular holes cannot be prevented in the general sense, as the primary cause—age-related vitreous degeneration—is a normal part of aging. No lifestyle modification has been proven to prevent the development of idiopathic macular holes. However, the following steps reduce specific risks:

• Protect eyes from injury: Wearing appropriate eye protection during sports, occupational hazards, and any activity involving risk of blunt force to the eye reduces the risk of traumatic macular hole.
• Manage diabetes carefully: Maintaining good blood sugar control reduces the risk of diabetic macular disease, including secondary macular holes from cystoid macular edema.
• Regular dilated eye exams for high-risk individuals: People with high myopia, a macular hole in one eye, or vitreomacular traction visible on imaging benefit from close surveillance so that any progression is caught at the earliest stage, when treatment is most effective.

The hallmark symptom of a full-thickness macular hole is blurred or distorted central vision—the part of vision used for reading, recognizing faces, watching television, and driving. Because the macula is the center of fine detail and high-contrast vision, damage at this specific location produces a characteristic pattern: peripheral vision remains normal while central vision is impaired, sometimes to the point of a central blank or dark spot (scotoma) in the affected eye. Symptoms typically develop gradually in idiopathic holes and may be noticed when one eye is accidentally closed or covered.

Common signs and symptoms of macular hole include:

• Blurred or reduced central vision: Fine detail that was previously clear—printed text, faces, fine needlework—becomes blurry or unclear. Distance and near vision are both affected.
• Central distortion (metamorphopsia): Straight lines appear wavy, curved, or bent when viewed with the affected eye. This is a highly characteristic early symptom that prompts patients to seek care. A grid of straight lines (such as an Amsler grid) is a classic screening tool for this symptom.
• A central blind spot or gray area: In more advanced holes, a central dark or blank spot develops in the center of vision in the affected eye.
• Micropsia: Objects may appear smaller than expected when viewed with the affected eye, due to photoreceptor displacement at the foveal margin.
• Reduced color perception: Some patients notice that colors appear less vivid or saturated in the affected eye, reflecting cone photoreceptor involvement at the foveal center.

It is important to note that early-stage or small macular holes may cause only subtle symptoms that are easily overlooked, particularly if the other eye compensates well. Any new or progressive central visual distortion or blurring in one eye—especially in adults over 50—warrants a prompt dilated eye examination.

A macular hole is diagnosed by an ophthalmologist or a vitreoretinal specialist—a subspecialist in diseases of the retina and vitreous. The diagnosis is primarily made by clinical examination and confirmed with imaging, most importantly, OCT. A careful history of symptoms, visual acuity testing, and dilated fundus examination are the first steps, followed by the imaging studies below.

• Optical coherence tomography: the essential diagnostic tool for macular hole. OCT is a noninvasive scan that uses light waves to produce high-resolution, cross-sectional images of every retinal layer. It confirms the presence of a full-thickness hole versus a partial-thickness (lamellar) defect, measures the hole’s minimum linear diameter (used for the International Vitreomacular Traction Study—IVTS size classification as small—up to 250 micrometers, medium—250 to 400 micrometers, or large—over 400 micrometers), visualizes the status of vitreomacular traction, identifies any associated epiretinal membrane, and provides baseline measurements against which treatment response is compared.
• Dilated fundus examination: The ophthalmologist examines the back of the eye using a lens and bright light after the pupil has been dilated with drops. A macular hole appears as a round, reddish spot at the center of the retina. Surrounding retinal changes, any epiretinal membrane, and the status of the peripheral retina are also assessed.
• Amsler grid test: a simple self-monitoring tool in which the patient looks at a grid of straight lines and reports any distortion, waviness, or missing areas. It is used both as a diagnostic prompt and as a home monitoring tool for patients with early macular changes.
• Fundus photography: Detailed color photographs of the retina document the size and appearance of the macular hole for the medical record and for comparison at follow-up visits.
• Fluorescein angiography and OCT angiography: These are dye-based or noninvasive vascular imaging studies used when secondary causes of macular hole are suspected—such as macular telangiectasia, diabetic changes, or abnormal foveal blood vessels—or when the diagnosis is uncertain.

Macular holes are highly treatable, and the goal of treatment is both anatomical closure of the hole and improvement in visual acuity. The appropriate treatment depends on the stage, size, cause, and duration of the hole, as well as the status of the vitreous attachment. Three treatment approaches are used: observation, pharmacologic therapy, and surgery.

Observation alone is appropriate in select circumstances. Very small stage 1 or stage 2 holes—particularly those smaller than 250 micrometers in diameter and in eyes where the vitreous has not yet fully separated—have a meaningful chance of spontaneous closure. Published data suggest approximately 3–15% of all idiopathic holes close without treatment, with higher rates (roughly 23%) in traumatic holes and in very small holes averaging 177 micrometers or less. When spontaneous closure does not occur within three months for traumatic holes or within a shorter window for idiopathic holes, surgery is typically recommended because prolonged waiting worsens the prognosis for visual recovery. For patients who are not surgical candidates due to health conditions or personal preference, observation with regular monitoring is continued.

The only pharmacologic treatment specifically approved for macular hole is ocriplasmin (brand name Jetrea®, approved by the U.S. Food and Drug Administration—FDA in 2012). Ocriplasmin is a recombinant proteolytic (protein-dissolving) enzyme injected into the vitreous cavity in a single outpatient procedure. It dissolves the vitreous protein attachments at the vitreomacular interface, releasing the tractional forces that are driving hole formation. It is most effective for small holes (250 micrometers or less) in eyes where the vitreous is still adherent to the fovea (vitreomacular traction is present), and it is particularly well-suited for eyes that do not yet have a complete posterior vitreous detachment and do not have an epiretinal membrane. In appropriately selected patients, ocriplasmin achieves macular hole closure in approximately 40% of cases—a substantially higher rate than the spontaneous closure rate in comparable patients. Side effects can include transient vitreous floaters, transient decreased vision or visual disturbance, and, in rare cases, subretinal fluid or transient changes in the electroretinogram. The procedure avoids surgery and its associated risks.

For the majority of patients—particularly those with medium or large holes, or holes that have not responded to pharmacologic treatment—pars plana vitrectomy (PPV) is the definitive and most effective treatment. This outpatient surgical procedure is performed under local or general anesthesia. The vitreoretinal surgeon inserts fine instruments through tiny incisions in the sclera (the white of the eye), removes the vitreous gel, and carefully peels the internal limiting membrane (ILM)—the innermost structural layer of the retina—from the macular surface. This ILM peel is a critical step: it releases all residual tangential traction on the fovea and provides a stimulus for the Müller cells to proliferate and bridge the hole. The eye is then filled with a gas bubble (typically sulfur hexafluoride or perfluoropropane) that holds the edges of the hole together while healing occurs. After surgery, patients must maintain a face-down posture for one to five days—the most demanding part of recovery—so the gas bubble remains over the macular hole. Vision is blurry while the gas is present and improves as the gas gradually absorbs over two to eight weeks, replaced by the eye’s own fluid. Vitrectomy achieves anatomical closure in 85–100% of cases. For large holes over 500 to 650 micrometers—particularly those in high-myopia eyes or recurrent cases—the inverted ILM flap technique is used: instead of completely removing the ILM, the surgeon creates a flap of ILM that is folded over the hole, providing additional cellular scaffolding to support closure. Other scaffold-augmenting modifications include using the patient’s own blood products (autologous platelet concentrate or autologous serum) or amniotic membrane tissue to assist healing in the most challenging refractory cases. The main surgical risks include accelerated cataract formation (very common in the natural lens, typically requiring cataract surgery within one to two years) and a small risk of retinal detachment (approximately 1%) and infection.

A macular hole diagnosis, while alarming, carries an excellent outlook with timely treatment. Most patients who undergo surgery achieve meaningful improvement in central vision—reading ability typically improves by two to four lines on the eye chart, and the distortion of central vision often substantially resolves as the hole closes and the photoreceptors reorganize over months following surgery. Full recovery takes time—visual improvement continues for up to 12 months after the procedure—and the final level of vision is best predicted by how long the hole was present before surgery and the patient’s vision at the time of the operation. Holes treated within six months of symptom onset generally achieve better visual outcomes than those treated later.

During the recovery period after surgery, face-down positioning is required for a few days, and flying is prohibited until the gas bubble has fully absorbed. The fellow eye should be examined carefully and monitored with an Amsler grid at home—any new central distortion in the opposite eye should prompt an immediate call to the ophthalmologist rather than waiting for a scheduled appointment. People at higher risk for macular hole in the second eye—particularly those with high myopia or a detected vitreomacular traction on OCT—should maintain regular dilated eye exam surveillance. For patients who do not achieve full visual recovery after surgery, low-vision rehabilitation specialists and assistive technologies (magnification devices, high-contrast reading aids, voice-activated technology) can help maintain independence and quality of life.

To further your understanding of your diagnosis and to contribute to cutting-edge research, consider participating in a clinical trial so clinicians and scientists can learn more about the causes, symptoms, treatment, and prevention of macular holes and related retinal disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.