Macular epiretinal membranes may be either idiopathic or secondary to other intraocular abnormalities, including retinal breaks, retinal vascular disease, uveitis, blunt and penetrating trauma, and surgery. Idiopathic macular epiretinal membranes are most common after 50 years of age.

Males and females are affected equally.

Bilateral macular epiretinal membranes occur in approximately 20% of patients, with the severity of the membranes usually asymmetric.

Most patients have posterior vitreous detachment (PVD), and it is thought that this phenomenon may contribute to formation of the membrane. After PVD, a portion of posterior cortical vitreous may be left behind on the macula, or the internal limiting membrane may be disrupted, allowing glial cells to proliferate on the surface of the retina. These proliferating glial cells form the epiretinal membrane. After retinal break formation and trauma, retinal pigment epithelial cells may escape into the vitreous cavity and settle upon the macula. These cells may then undergo metaplasia to glial cells, allowing the formation of macular epiretinal membranes.

If the macular epiretinal membrane is thin and not producing distortion of the retina, then the patient is usually asymptomatic. With progressive thickening and contraction of the membrane, patients begin to notice decreased vision, metamorphopsia, and macropsia or micropsia.

Most patients maintain vision better than 20/50.

Most epiretinal membranes progress slowly and occasionally improve spontaneously.

Macular epiretinal membranes initially appear as translucent, glistening membranes over, or adjacent to, the central macula (Fig. 2-1). There may be a “pseudohole” appearance over the fovea. As the membrane matures, it becomes more opaque (Fig. 2-2A).

There may be associated superficial hemorrhages or nerve fiber layer infarcts.

The membranes contract centrally and cause wrinkling and distortion of the underlying retina. This wrinkling and distortion gives rise to other names for this condition: cellophane maculopathy, surface wrinkling retinopathy, and macular pucker. If the distortion is severe, cystoid macular edema and even shallow tractional retinal detachment can occur.

Cystoid macular edema

Choroidal neovascularization (CNV)

Macular hole

Choroidal folds

Combined hamartoma of the retina

Retinal pigment epithelium

Usually measurement of visual acuity, Amsler grid testing (to assess for metamorphopsia, macropsia, or micropsia) and ophthalmoscopy suffice to make the diagnosis of macular epiretinal membrane. Slit-lamp biomicroscopy with either a noncontact or a contact lens greatly facilitates diagnosis.

Optical coherence tomography (OCT) has become standard in documenting the presence of an epiretinal membrane. OCT reveals a hyperreflective band on the surface of the retina, associated with retinal distortion, thickening, and/or cystoid edema in advanced cases.

Fluorescein angiography will clearly delineate retinal vascular distortion and leakage that may occur from cystoid macular edema (Fig. 2-2B, C).

Most patients with uniocular macular epiretinal membrane and vision better than 20/50 are usually not significantly symptomatic and do not require intervention. Patients with vision of 20/60 or worse and those patients with intolerable distortion may benefit from pars plana vitrectomy with membrane peeling and removal (Fig. 2-3).

Rarely, the membrane may spontaneously release, leading to resolution of symptoms.

Idiopathic macular holes typically occur in the sixth through eighth decades of life with a 3:1 predominance in women.

The incidence of bilaterality is 5% to 10%.

Tangential vitreoretinal traction is the presumed cause of development of idiopathic macular hole.

The Gass classification of the stages of idiopathic macular hole development is helpful in understanding the progression of the disease and the biomicroscopic findings (Table 2-1).

Patients with a full-thickness macular hole in one eye and an impending macular hole with no PVD in the fellow eye are at substantial risk of progression to stage 2 macular hole in the second eye. Patients with a full-thickness macular hole in one eye and a normal retina with PVD in the fellow eye are at very little risk of progressing to macular hole in the second eye.

Patients usually report decreased visual acuity with central scotoma. There may be metamorphopsia.

Often patients notice decreased vision in the affected eye when the fellow eye is incidentally covered as for a routine eye examination.

Depending on the stage and severity of the macular hole, the visual acuity may be near normal or severely reduced to less than 20/400.

Amsler grid testing will often reveal metamorphopsia or a central scotoma. Ophthalmoscopy and slit-lamp biomicroscopy reveal findings consistent with the stage of the macular hole.

There are often small yellow dots in the center of the hole at the level of the retinal pigment epithelium (Fig. 2-8).

The Watzke–Allen sign is the patient’s description of discontinuance in the center of a thin slit beam shone over the fovea.

Macular epiretinal membrane with pseudohole

Cystoid macular edema

Central serous retinopathy (CSR)

Choroidal neovascular membrane

Solar retinopathy

Adult vitelliform dystrophy

Clinical examination alone is often diagnostic. Fluorescein angiography in eyes with stage 2, 3, or 4 macular holes will reveal early central hyperfluorescence in the fovea corresponding to loss of xanthophyll pigment and retinal pigment epithelial depigmentation and atrophy at the base of the hole. OCT clearly distinguishes between partial- and full-thickness holes. (Fig. 2-9).

No treatment is recommended for stage 1 macular holes because these resolve spontaneously in 50% of cases. Spontaneous resolution of more advanced stages of macular hole can occur, but it is rare.

Vitrectomy can be performed for more advanced stages of macular hole. The surgery consists of a standard pars plana vitrectomy, peeling of the posterior hyaloid, and injection of a long-acting gas such as perfluoropropane. Peeling of the internal membrane may also be done. Patients must then maintain face-down positioning for 1 to 2 weeks to allow the gas bubble to tamponade the hole. Recent evidence suggests that face-down positioning is not as critical as once believed, but this needs to be studied further. The success rate for macular hole surgery approaches 80% to 90% with closure of the hole and improvement in visual acuity (Fig. 2-10).

Negative prognostic indicators include a long duration of hole presence (i.e. greater than 1 year) and larger size of the hole.

VMTS occurs in the same age group as those who develop PVD. PVD is uncommon before 50 years of age and is present in over 50% of people aged 70 years and older.

Patients who have VMTS experience progressive distortion and visual loss, which is often more severe than that occurring with macular epiretinal membrane.

The posterior hyaloid is visibly thickened. There is macular distortion, often with tractional retinal detachment in the macula. Retinal striae may be present. There may be traction in the peripapillary region. An epiretinal membrane may be seen clinically (Fig. 2-11).

Macular epiretinal membrane

Combined hamartoma of the retina and retinal pigment epithelium

Fluorescein angiography may reveal retinal vascular distortion and leakage. There may be cystoid macular edema and optic nerve edema.

Spectral-domain OCT helps determine the presence of VMTS as well as associated macular edema and tractional detachment.

Occasionally patients may experience spontaneous improvement if PVD occurs.

Surgical intervention is indicated if visual acuity is reduced to 20/70 or worse. During vitrectomy surgery, the posterior hyaloid is removed, as are any epiretinal membranes in the macular region. The retinal architecture can be restored to a normal appearance. The vision can be improved but usually complete recovery does not occur due to residual macular edema.

Pharmacologic vitreolysis with microplasmin is currently under investigation for the treatment of VMTS, with promising initial results.

CME is most commonly seen after cataract surgery. Other types of ocular surgery, such as trabeculectomy, laser photocoagulation and cryoretinopexy, may also give rise to CME.

Less commonly, CME is seen in association with diabetic retinopathy, CNV, uveitis, retinal vein obstruction, perifoveal telangiectasis, retinitis pigmentosa, and other entities.