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MACULAR DISEASES

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Epiretinal Membranes  
 

Epiretinal membranes are the result of cellular proliferation along the internal limiting membrane and retinal surface. They are hypo-cellular largely...

Epiretinal membranes are the result of cellular proliferation along the internal limiting membrane and retinal surface. They are hypo-cellular largely collagen structures and can be either idiopathic or secondary. They vary in severity from very subtle membranes to thick membranes covering the macular region.

The so-called idiopathic type of epimacular membrane has been shown to be caused by glial proliferation through a defect in the internal limiting lamina, usually created by a posterior vitreous separation. Secondary causes include retinal vascular occlusions, diabetic retinopathy, the aftermath of surgical procedures such as like retinal detachment surgery, ocular inflammatory conditions and the after-effects of laser or cryotherapy.

Contraction of these membranes causes retinal wrinkling and several types of retinal detachments, usually micro-detachments.

Patients with macular epiretinal membranes can experience a relative loss of vision accompanied by metamorphopsias.

Those patients with recent loss of visual acuity, metamorphopsias and/or angiography leakage, must be submitted to surgery, particularly if they need a cataract removal.

Epiretinal Membranes in Children  
 

Epiretinal membranes consist of proliferations of nonvascular retinal cells capable of inducing distortion of the macular architecture, with consequent reduction...

Epiretinal membranes consist of proliferations of nonvascular retinal cells capable of inducing distortion of the macular architecture, with consequent reduction of visual acuity and metamorphopsias.

Their occurrence in the pediatric age group is rare and the diagnosis is often delayed. Their treatment is urgent because of the potential risk of amblyopia.

Surgery is indicated if the eye presents micro-detachments of the neurosensorial retina, changes at the internal blood-retinal barrier, metamorphopsias, or amblyopia. It can significantly improve retinal structure and visual acuity.

These membranes can be found in retinopathy of prematurity, traumatic or non-traumatic retinal detachments, uveitis, familial exudative vitreoretinopathy, Coat’s disease and other vasculopathies with or without vitreous hemorrhage.

Idiopathic Macular Hole  
 

A macular hole is a full-thickness defect of retinal tissue involving the anatomic fovea of the eye.

There are various...

A macular hole is a full-thickness defect of retinal tissue involving the anatomic fovea of the eye.

There are various causes of macular hole: it can be idiopathic or due to trauma, accidental foveal burns with laser, long-standing cystoid macular edema and myopia.

The underlying pathophysiology for the formation of these holes is not well understood, although epiretinal membrane formation, vitreous traction and hydraulic forces may play a role in a few cases. The anteroposterior and “tangential” vitreous traction on the fovea may be a primary cause off macular holes in the majority of cases.

Differential Diagnosis

  • Central serous retinopathy
  • AMD
  • Cystoid macular edema
  • Epiretinal membrane with pseudo-hole
  • Lamellar hole
  • Solar retinopathy
  • Solitary drusen
  • Vitreomacular traction syndrome

References:

Johnson RN, Gass JD. Idiopathic macular holes. Observations, stages of formation, and

implications for surgical intervention. Ophthalmology. Jul 1988;95(7):917-24. [Medline].

Vitreomacular Traction Syndrome  
 

Vitreomacular traction syndrome is a disorder caused by incomplete posterior vitreous detachment with persistent traction on the macula. It usually...

Vitreomacular traction syndrome is a disorder caused by incomplete posterior vitreous detachment with persistent traction on the macula. It usually produces cystoid changes and decreased visual acuity.

Epimacular membrane formation without posterior vitreous detachment may predispose patients to a vitreomacular traction syndrome. Fibrocellular proliferation between the inner surface of the retinal and posterior surface of the vitreous results in increased vitreoretinal adhesion.

Vitrectomy is effective in releasing vitreomacular traction and, in many cases, restores macular anatomy and induces regression of cystoid macular edema.

Degenerative Myopia  
 

Degenerative myopia is characterized by a refractive error of at least -6.00 diopters, with an axial length of the globe...

Degenerative myopia is characterized by a refractive error of at least -6.00 diopters, with an axial length of the globe of more than 26 mm. Progressive choroidal degeneration is then usually associated in the posterior pole.

The overall expansion of the globe with sclera thinning results in the hallmark ectasia of degenerative myopia: the posterior staphyloma. Progressive axial elongation appears directly involved in the occurrence of the posterior vitreous detachment, peripheral breaks, and posterior pole degeneration. Central visual impairment can be due to lacquer cracks, atrophic areas, new choroidal vessels, or a macular hole with posterior retinal detachment.

Pathogenesis is not clearly understood and could be due to heredo-degenerative factors.

Degenerative myopia is associated with an increased risk of developing a choroidal neovascular membrane and a rhegmatogenous retinal detachment.

Angioid Streaks  
 

Angioid streaks are usually bilateral and represent visible breaks in the Bruch’s membrane. The elastic fibers of Bruch’s membrane are...

Angioid streaks are usually bilateral and represent visible breaks in the Bruch’s membrane. The elastic fibers of Bruch’s membrane are charged with calcium, and this accumulation makes the layer brittle and susceptible to breaks.

Clinically they consist of dark red to brown linear bands of irregular contour, originating radially from the optic disc and extending into the periphery. Near the optic disc, a circular ring sometimes interconnects them. Retinal pigment epithelium contiguous to the borders of the streaks, can be hypopigmented or thinned. The transition regions between normal and pathological retina may have a fine stippled appearance, known as “peau d’orange”. In the mid-periphery, small depigmented subretinal deposits, known as crystalline spots, are frequently present. Other ocular findings include optic disc drusen and peripheral round atrophic scars.

In FAF, recent angioid streaks produce no changes in the autofluorescence pattern. Chronically, as the RPE/photoreceptor complex becomes atrophic, irregular hypofluorescent lines with an enhanced autofluorescence border appear.

The most frequent association with systemic disease is pseudoxanthoma elasticum (PXE) also known as Gronblad-Strandberg syndrome. Several other systemic diseases have been associated with angioid streaks (see below) however similar changes can be seen in healthy older patients.

Patients are usually asymptomatic. Angioid streaks can, however cause subretinal hemorrhages associated or not with CNV. Therefore, it is essential to confirm the presence of CNV before initiating treatment.

There is no proven treatment to prevent this condition in eyes at risk. When CNV is present, therapeutic options include Laser (if the lesion is extrafoveal), PDT or intravitreal injection of an anti-VEGF agent.

Patients should be informed that their eyes must be adequately protected, due to their susceptibility to choroidal rupture even after a mild blunt trauma.

Systemic Diseases Associated With Angioid Streaks:

  • Pseudoxanthoma elasticum
  • Ehlers-Danlos syndrome
  • Hereditary spherocytosis
  • Paget’s disease
  • Sickle cell disease
  • Thalassemia
Hypotonic Maculopathy  
 

Hypotonic maculopathy is a condition in which chorioretinal folds develop in the posterior pole of patients with chronically low intraocular...

Hypotonic maculopathy is a condition in which chorioretinal folds develop in the posterior pole of patients with chronically low intraocular pressure (usually lower than 5 mmHg). Fundus abnormalities also include papilledema and vascular tortuosity.

It usually occurs after anti-glaucoma surgery or when perforating eye injuries are sustained. Young age, myopia, primary filtering surgery, systemic illnesses and elevated preoperative intraocular pressure have been found to be associated with hypotonic maculopathy.

Idiopathic Choroidal Neovascular Membrane  
 

Idiopathic choroidal neovascular membranes have been found in a significant number of young or middle-age adult patients, with no apparent...

Idiopathic choroidal neovascular membranes have been found in a significant number of young or middle-age adult patients, with no apparent cause.

Ultrastructural features of some excised submacular idiopathic neovascular membranes were similar to those observed in patients with Presumed Ocular Histoplasmosis Syndrome. Idiopathic NV membranes often occur in association with a pigment ring or gray mound in the fundus.

Patients may develop loss of central vision secondary to serous and hemorrhagic detachment of the macula caused by choroidal neovascularization.

There is no evidence of vitreous cells, condensation or vasculitis, or any systemic disease in this group of patients. The cause of these choroidal neovascular membranes remains unknown.

Differential Diagnosis

  • Degenerative Myopia
  • Angioid streaks
  • ARMD, exudative
  • Central serous chorioretinopathy
  • Presumed ocular histoplasmosis syndrome
  • Birdshot retinopathy
  • White Dot Syndromes