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< Drugs and Disease

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

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 cu of subretinal uid.

View Media Gallery

A macular hole was rst 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 dierentiated 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.
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,
rst described a classication 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 characteristic yellow spot at the center
of the fovea.

View Media Gallery

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 rst stage when a full-thickness break in the retina
exists. It is dened as a full-thickness macular hole less than 400 m 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 m 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 cu of subretinal uid with
associated photoreceptor atrophy.
See Causes.
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
aected 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
signicant 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 aected more than men.
Age
Peak incidence is in the seventh decade of life.

Clinical Presentation

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