Macular Hole

Updated: Apr 21, 2016
  • Author: Kean Theng Oh, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

Background

A macular hole is a defect of the foveal retina involving its full thickness from the internal limiting membrane (ILM) to the outer segment of the photoreceptor layer.

See the image below.

Full-thickness macular hole showing a surrounding Full-thickness macular hole showing a surrounding cuff of subretinal fluid.

A macular hole was first described by Knapp in 1869 in a patient who sustained blunt trauma to the eye. Subsequent case reports and series pointed to antecedent episodes of ocular trauma such that the two were customarily linked to each other. However, throughout this century, ophthalmologists increasingly have recognized that this condition more commonly occurs in atraumatic settings and have differentiated these macular holes from trauma-induced holes by describing them as idiopathic full-thickness macular holes. In fact, case series as far back as the 1970s reported that more than 80% of macular holes are idiopathic and that only less than 10% have associated history of trauma to the eye.

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Pathophysiology

The causes underlying trauma-related macular holes and idiopathic macular holes are understandably divergent.

Trauma-related macular holes are suspected to be related to the transmission of concussive force in a contrecoup manner, which results in the immediate rupture of the macula at its thinnest point. Patients who underwent successful repair of a rhegmatogenous retinal detachment were also found to infrequently develop macular holes (< 1% incidence). The underlying pathophysiology for formation of these holes is not well understood, though epiretinal membrane formation, foveal photoreceptor atrophy, and hydraulic forces may play a role.

While the vitreous was suspected to be involved in the causation of idiopathic macular holes by Lister in 1924, Johnson and Gass, in 1988, first described a classification system that focused on anteroposterior and tangential vitreous traction on the fovea as a primary underlying cause for idiopathic macular holes. [1, 2] Shrinkage of prefoveal cortical vitreous with persistent adherence of vitreous to the foveal region results in the causative traction.

Gass macular hole stages are described below.

Stage 1a (foveal detachment; macular cyst): Tangential vitreous traction results in the elevation of the fovea marked by increased clinical prominence of xanthophyll pigment. This stage is occasionally referred to as the yellow dot stage and can also be seen in cases of central serous chorioretinopathy, cystoid macular edema, and solar retinopathy as depicted below.

Fundus photograph of a stage 1a macular hole with Fundus photograph of a stage 1a macular hole with characteristic yellow spot at the center of the fovea.

Stage 1b: As the foveal retina elevates to the level of the perifoveal, the yellow dot of xanthophyll pigment changes to a donut shaped yellow ring. Persistent traction on the fovea leads to dehiscence of deeper retinal layers at the umbo.

Stage 2: This is the first stage when a full-thickness break in the retina exists. It is defined as a full-thickness macular hole less than 400 µm in size. The full-thickness defect may appear eccentric, and there may be a pseudo-operculum at this stage if there has been spontaneous vitreofoveolar separation. These opercula have been examined and found to be vitreous condensation and glial proliferation without harboring any retinal tissue.

Stage 3: A full-thickness macular hole in the retina exists. It is greater than 400 µm in size and is still with partial vitreomacular adhesion/traction.

Stage 4: A full-thickness macular hole exists in the presence of a complete separation of the vitreous from the macula and the optic disc. There is evidence, however, that, even in the presence of an apparent posterior vitreous detachment, a thin shell of residual cortical vitreous may still remain and contribute to the macular hole.

The advent of ocular coherence tomography (OCT) has provided in vivo structural support to hypotheses focused on vitreous traction underlying idiopathic macular holes. The OCT has allowed careful evaluation of the vitreoretinal interface demonstrating persistent adhesion on the fovea resulting in oblique traction on the fovea even with a partial posterior vitreous detachment. The persistent traction on the fovea prior to anatomic changes to the fovea has been referred to as Stage 0. This clinical appearance may resolve without progression in 40-50% of patients. [3]

Visual dysfunction in patients with macular hole is directly related to the absence of retinal tissue in the fovea. However, visual dysfunction may seem out of proportion to the size of the macular hole and potentially may also be related to the presence of a cuff of subretinal fluid with associated photoreceptor atrophy.

See Causes.

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Epidemiology

Frequency

United States

The overall prevalence is approximately 3.3 cases in 1000 in those persons older than 55 years. Peak incidence of idiopathic macular hole development is in the seventh decade of life, and women typically are affected more than men. Reasons for this, at best, are speculative at this point. Some epidemiologic risk factors, such as cardiovascular disease, hypertension, and a history of hysterectomy, have been reported by other studies. However, none of these have been proven to have any significant association with macular hole formation.

International

The prevalence rate of macular hole in India is a reported 0.17%, with a mean age of 67 years.

The Beijing Eye Study found the rate of macular holes to be 1.6 out of 1000 elderly Chinese, with a strong female predilection.

Mortality/Morbidity

The natural history of a macular hole varies based on its current clinical stage. It has been reported that around 50% of stage 0 and stage 1 macular holes may resolve both in the anatomic changes and the symptoms produced. Stage 2 holes progress and worsen in most cases to stage 3 or stage 4, resulting in worsening vision. Best estimates for the incidence of development of an idiopathic full-thickness macular hole in the fellow eye are approximately 12%. In rare instances (0-10%), a full-thickness macular hole may spontaneously close with resultant good vision.

Race

There is no racial predilection reported, though prevalence rates for the epidemiologic studies in India and China are consistent with reported data.

Sex

Women typically are affected more than men.

Age

Peak incidence is in the seventh decade of life.

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