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PERIPHERAL RETINAL DISEASES

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Rhegmatogenous Retinal Detachment  
 

Retinal detachment is the separation between the neurosensorial layers of the retina and the retinal pigment epithelium. Rhegmatogenous retinal detachment...

Retinal detachment is the separation between the neurosensorial layers of the retina and the retinal pigment epithelium. Rhegmatogenous retinal detachment is the most common type of retinal detachment. It is caused by a retinal break, allowing access of a liquefied vitreous gel to the potential subretinal space.

Symptoms are variable. Rhegmatogenous retinal detachment is usually preceded by posterior vitreous detachment causing photopsia and floating opacities. Post-detachment symptoms include blurred vision, metamorphopsia, visual field defects and/or loss of central vision.

Predisposing factors for this disease include high myopia, aphakia, pseudoaphakia, family background and inflammation.

Slit lamp examination usually reveals pigment cells suspended in the vitreous cavity (Schaffer sign), being colloquially know as “tobacco dust”. Vitreous hemorrhage can occur as result of a vascular rupture at the tear site. Usually intraocular pressure is lower in the affected eye. The recently detached retina usually has a convex, smooth and mobile appearance. As time passes, if no treatment is established, an inflammatory reaction, known as proliferative vitreoretinopathy (PVR) takes place, causing fibrosis, membrane formation and contraction. Progressively, the retina assumes a folded, convoluted, taut and immobile configuration. PVR adversely affects the prognosis.

Treatment for rhegmatogenous retinal detachment is surgical. Pars plana vitrectomy is the most commonly performed procedure. All retinal breaks are found, vitreoretinal traction is released, the retina and retinal pigment epithelium are brought into apposition, breaks are sealed with either Laser or cryoprobe and finally intraocular tamponade with either gas or silicone oil is performed.

The main factor that determines outcome is the status of the macular region when surgery is performed. If the macula is still attached by the time of surgery, the outcome is significantly superior. Also a longer interval between symptoms and surgery adversely affects the outcome.

Exudative Retinal Detachment  
 

Exudative retinal detachment occurs when fluid passes into the subretinal space due to damage of the retinal blood vessels and...

Exudative retinal detachment occurs when fluid passes into the subretinal space due to damage of the retinal blood vessels and the retinal pigment epithelium. It is generally caused by neoplasia and inflammatory diseases, but any disease affecting choroidal vascular permeability can potentially cause an exudative detachment.

Symptoms include decreased vision, visual field defects, and sometimes pain and leukocoria.

In exudative detachment, the detached retina is smooth in texture. The subretinal fluid responds to the force of gravity and detaches the area in which it accumulates.

Its treatment is usually non surgical.

Exudative retinal detachment may be secondary to preeclampsia and usually resolves after absorption of the subretinal fluid. However, severe eclampsia may cause permanent visual loss due to RPE necrosis.

Choroidal Detachment  
 

The choroidal detachment presents as a smooth, bullous, orange-brown elevation of the retina and choroid, usually extending 360 degrees around...

The choroidal detachment presents as a smooth, bullous, orange-brown elevation of the retina and choroid, usually extending 360 degrees around the periphery in a lobular configuration. The ora serrata is visible without scleral depression.

The suprachoroidal space is normally virtual because the choroid is in close apposition to the sclera. As fluid accumulates, this space becomes real, and the choroid is displaced from its normal position. Fluid accumulation, either a serum-like liquid or blood, can also occur within the choroid, which is a spongy tissue.

As a sequela, linear areas of pigmented epithelium hypertrophy, called Verhoeff lines, indicate the posterior limits of the choroidal detachment after fluid reabsorption.

Choroidal detachment may occur in two forms:

  • Choroidal Effusion: Serous choroidal detachment involves transudation of serum into the suprachoroidal space; it is associated with acute ocular hypotony, post-surgical situations, posterior scleritis, Vogt-Koyanagi-Harada syndrome, trauma, intraocular tumors, or uveal effusion syndrome.
  • Choroidal Hemorrhage: Rupture of choroidal vessels causes bleeding into the suprachoroidal space. It can arise spontaneously (rare), as a consequence of ocular trauma, during or after eye surgery.
Retinal Tears  
 

Retinal breaks are full thickness defects of the neurosensorial retina. When significant vitreoretinal traction is present at the break site...

Retinal breaks are full thickness defects of the neurosensorial retina. When significant vitreoretinal traction is present at the break site and a liquefied vitreous is present, a retinal detachment can occur.

Breaks can be caused by atrophy of the inner retinal layers, vitreoretinal traction or trauma. According to their configuration breaks can be divided in: horseshoe tears, giant tears, operculated holes, dialyses, and atrophic retinal holes.

Horseshoe tears, have a U configuration, with the convexity usually pointing to the optic disc. They are caused by vitreous traction, exerted on the flap (frequently the vitreous remains attached to the flap and traction persists). When tears exceed 90 degrees circumferentially they are called giant tears. Tears are most commonly found in the supero-temporal quadrant.

Operculated holes are also caused by vitreoretinal traction, that is usually stronger and released during the hole formation (vitreous remains attached to the operculum and free from attached retina).

Dialyses are circumferential linear breaks occurring either at the anterior or posterior margin of the vitreous base. They are usually found in the infero-temporal retina.

Atrophic holes are caused by atrophy of the inner retinal layers and are not associated with vitreoretinal traction.

The vast majority of retinal breaks do not cause retinal detachment. Therefore treatment must be tailored to specific situations, bearing in mind that the benefits of treatment must be weighted against potential risks.

Chorioretinal Folds  
 

Chorioretinal folds results from compressive forces on the sclera that produce a series of folds in the inner choroid, Bruch´s...

Chorioretinal folds results from compressive forces on the sclera that produce a series of folds in the inner choroid, Bruch´s membrane, RPE and retina. They may be idiopathic or may be secondary to tumors, hypotony, inflammation (posterior scleritis, idiopathic orbital inflammation, and autoimmune disorders), choroidal neovascular membranes, papilledema, and scleral buckle.

The patients are usually asymptomatic or may have metamorphopsia and decreased vision if folds involve fovea.

The folds appear as alternating bright peaks and dark troughs or valleys predominantly involving the posterior pole. They may be curvilinear, parallel, or circular. Idiopathic folds are usually bilateral and symmetric. Fundus autofluorescence may be useful in detecting the presence of such folds that result from stretching and thinning of the RPE. Similarly, the fluorescein angiogram shows alternate bands of hyper and hypofluorescence.

The most common cause of choroidal folds is disciform scarring of the macula from neovascular age-related macular degeneration.

Differencial Diagnosis

  • Retinal folds due to an epiretinal membrane
  • Rhegmatogenous retinal detachment
  • Toxocariasis
  • Retinopathy of prematurity
Pavingstone Degeneration  
 

Pavingstone (cobblestone) degeneration is characterized by peripheral, small, discrete areas of atrophy of the outer retina. Sometimes, the lesions may...

Pavingstone (cobblestone) degeneration is characterized by peripheral, small, discrete areas of atrophy of the outer retina. Sometimes, the lesions may coalesce into a band like zone of degeneration or may occur singly. It is present in 22-27% of general population and 33% are bilateral.

Pavingstones are characterized by atrophy of the RPE and outer retinal layers, attenuation or absence of the choriocapillaris, and adhesions between the remaining neuroepithelial layers and Bruch's membrane. Large choroidal vessels may be visible beneath the lesions due to the absence of the RPE.

Ophthalmoscopically, they appear yellowish-white and may be surrounded by rim of hypertrophic RPE. The lesions are most common in inferior quadrants, anterior to the equator.

Lattice Degeneration  
 

Lattice degeneration is an atrophic disease of the peripheral retina characterized by oval or linear patches of retinal thinning. It...

Lattice degeneration is an atrophic disease of the peripheral retina characterized by oval or linear patches of retinal thinning. It is bilateral in one third to one half of affected patients. Condensed vitreous at the margins of the lattice lesions may appear as vitreous opacities and represent regions of increased vitreoretinal adhesion. Retinal tears can occur with posterior vitreous separation pulling on the thinned retina, with an increased risk of retinal detachment. However retinal detachment is a relatively rare complication of lattice degeneration.

Lesions may be isolated or multifocal, variable in dimension, and usually are oriented concentric or slightly oblique to the ora serrata, located within the equatorial region of the fundus, typically inferotemporal.

Patients with lattice degeneration are asymptomatic. The acute onset of floaters, flashes, peripheral field loss, or central vision loss may indicate the presence of a retinal tear or detachment. The vast majority of patients will have lesions that are completely stable or slowly progressive.

It occurs more commonly in but is not limited to myopic eyes.

Snail-track retinal degeneration is a clinical variation of lattice degeneration, occurring mainly in young myopic eyes, characterized by long circumferential areas of retinal thinning with a glistening appearance. Many authors consider it to be early stage of lattice degeneration. The lesions consist of retinal degeneration leading to an atrophy of the tissues with lipid deposits in the internal retinal layers and they have the same complications and clinical protocols.

Differential Diagnosis:

  • Retinoschisis
  • Atrophic  holes
  • Chorioretinal scarring
  • Congenital hypertrophy of the retinal pigment epithelium
  • Retinal holes, tears and detachments