Peripheral Retinal Degenerations and Rhegmatogenous Retinal Detachment

Peripheral Retinal Abnormalities

Each peripheral retinal abnormality poses a different level of risk for retinal detachment. Some of these lesions may be inconsequential manifestations, whereas others represent a high risk for detachment, particularly in patients with high myopia; aphakia or pseudophakia; previous detachment in the fellow eye; or a strong family history of retinal detachment.

This patient has a meridional fold in the nasal ora serrata. Meridional folds, which are elevated pleats of peripheral neurosensory retina, occur in 26% of the population and are bilateral in approximately 50% of patients. This feature is generally not of clinical significance.

A whitish-yellow pearl of the ora serrata is demonstrated in this patient. This is not a high-risk factor for detachment. Observation is warranted.

Retinal Holes

This patient has four asymptomatic round atrophic holes with a localized subclinical retinal detachment. The holes have been observed for over 20 years, and there has been no appreciable change.

This 27-year-old white female has a cystic retinal tuft with localized subretinal fluid. Cystic retinal tufts are chalky white, elevated peripheral lesions composed of glial tissue, with associated traction at its apex from condensed vitreous. They are noted in 5% of the population in autopsy studies and are clinically significant in that they are associated with approximately 10% of primary retinal detachments. Approximately 0.28% of patients with these lesions will have retinal detachments secondary to the tuft. Therefore, due to the high prevalence of these tufts in the general population and the low risk of retinal detachment, cystic retinal tufts are not usually required to have prophylactic laser photocoagulation treatment.

This is a retinal hole without existing traction. There is a surrounding cuff of elevated retina that has lost its transparency.

This larger retinal hole is associated with a cuff of subretinal fluid and early pigmentary demarcating boundary.

This atrophic hole has a cuff of subretinal fluid that is bordered by a ring of pigment epithelial hyperplasia demarcating the extent of the localized detachment.

Lattice Degeneration

Lattice degeneration is a common peripheral vitreoretinal disorder present in 6-10% of the population. While only 0.5-1% of eyes with lattice degeneration will develop a retinal detachment, approximately 20-30% of patients with rhegmatogenous retinal detachments have lattice degeneration. Lattice degeneration has many morphologies, the most common being circumferentially oriented localized round, linear, or ovoid areas of retinal thinning that are sometimes crossed by whitish lines that represent hyalin­ized retinal vessels. Other features include superficial whitish yellow flecks; patches of varying degrees of pigmentation; round or linear red craters; round or linear white patches; and small atrophic round holes. On histopathologic examination, lattice degeneration consists of one of three findings: a localized thinning of the inner retinal layers, vitreous liquefaction overlying thinned retina, and vitreous condensation with exaggerated vitreo-retinal attachments at the margins of the lesion. Although lattice degeneration itself is asymptomatic, it can be associated with retinal tears, detachments, or traction, which may cause floaters, photopsias, or other visual disturbances. In some eyes with lattice degeneration, retinal detachment may occur secondary to retinal tears that develop in areas remote from the lattice degeneration. General practice patterns generally recommend observation for asymptomatic lattice degeneration and only consider treatment of the condition in cases where the fellow eye had a previous retinal detachment.

There is a horseshoe retinal break within a patch of lattice degeneration, which resulted in a gravity dependent retinal detachment. Retinal folds with early proliferative vitreoretinopathy (PVR) are seen inferiorly at the border of the detachment.

This patient has a break within pigmentary lattice. The hole is round, and the retinal detachment is associated with multiple, irregular folds in the retina. The corresponding OCT image shows the retinal break with traction (arrow), and resulting retinal elevation.

These three cases illustrate different morphologies of lattice degeneration. On the left, there are multiple atrophic holes (arrows) bordered by localized fibrosis with a shallow detachment. In the middle, there is radial paravascular pigmentary lattice degeneration with multiple horseshoe breaks (arrows), as well as a localized retinal detachment. In the right image, there is a linear break along the lattice degeneration and localized detachment (arrows). There is also localized hemorrhage within and into the periretinal region.

This patient has pigmentary lattice degeneration without associated pathology.

This patient has lattice degeneration with some atrophic changes, superficial whitish yellow flecks, and sheathing of the retinal vessels.

This lattice degeneration is predominantly pigmentary in nature and extends from a retinal vessel superiorly and temporally in an irregular course. The retinal vessels are associated with sclerotic and sheathing abnormalities (arrows).

This area of lattice degeneration has intraretinal migration of pigment epithelial cells, areas of pigment epithelial atrophy, and sclerotic vascular changes.

The pigmentation in this area of lattice degeneration is quite intense. A lattice configuration overlying the pigment hyperplasia is evident. There is an adjacent zone of atrophy.

Retinal Tears and Localized Detachments

These patients have high-risk retinal breaks with everted edges resulting from vitreous traction and early PVR. Each break has an associated localized detachment, with the detached retina losing its transparency. Bridging retinal vessels are visible traversing the tear in the top four photos, indicative of a high risk for development of vitreous hemorrhage. The choroid can be seen more clearly through the horseshoe breaks, particularly in the lower right photo (arrows).

Retinal Detachments

A rhegmatogenous retinal detachment (RRD) is a sight-threatening condition that occurs in approximately 1 in 10,000 people. Lattice degeneration is the greatest risk factor, but pathologic myopia, previous intraocular surgery, trauma, and family or personal history of retinal detachment are also risk factors. An RRD occurs when fluid accumulates between the sensory retina and retinal pigment epithelium (RPE) through a retinal break caused by vitreous traction. An RRD generally has a classic corrugated appearance and undulates with eye movements. A variety of treatment modalities have evolved to repair RRD, and these include the traditional use of a scleral buckle, pars plana vitrectomy surgery (PPV), or pneumatic retinopexy (PR). Each treatment will influence the appearance of the fundus following treatment, though the general principles for all procedures are essentially the same. The essential steps for repairing RRD are (1) detection of the retinal breaks, (2) closure of the defects, (3) release of vitreous traction, and (4) placement of an adhesive modality (generally photocoagulation and/or cryotherapy). This generally allows for resultant apposition of the retina to the underlying RPE. The most common cause of detachment repair failure, which occurs in 8-10% of patients, is proliferative vitreoretinopathy (PVR), which may require reoperation. There is always the possibility of missing or not fully treating the retinal pathology as well.

This is a quadratic retinal detachment with a retinal break, retinal folds, and gravitating or dependent separation toward the posterior pole but not yet into the macula.

There is a large retinal break superotemporally. It is associated with a descending detachment, which is threatening the temporal macula. At the anterior edge of the break is some pigmentary lattice degeneration (arrows).

Courtesy of Dana Gabel

In this patient a peripheral retinal detachment with retinal breaks and some early pigmentation at the level of the RPE is present.

A retinal detachment is encroaching upon the optic nerve in each eye. Note the loss of retinal transparency in the areas of retinal detachment, which obscures visualization of the underlying choroidal vasculature.

This patient developed a large retinal tear, though with only a limited amount of localized subretinal fluid temporal to the macula. The retina was surgically repaired prior to the detachment extending into the macula.

Chronic Retinal Detachment With Demarcation Lines

This patient has a chronic detachment that extends to the posterior pole, but not into the central macula. Multiple retinal folds are seen, some concentric with the others and some radiating peripherally in a random fashion.

This patient has a dense pigmentary demarcation line bordering a chronic dependent detachment. There are a few retinal breaks (arrows) and areas of lattice degeneration (arrowheads).

This patient has a chronic detachment inferiorly, which is bordered by dense and irregular hyperplastic changes of the RPE and an atrophic demarcation line. There is also a giant retinal cyst inferotemporally (arrows).

Chronic Retinal Detachment with Retinal Macrocyst

A very prominent macrocyst was noted in conjunction with a low lying retinal detachment ( top row ). Echography (middle row, left) confirmed the diagnosis, and SD-OCT showed shallow subretinal fluid encroaching upon the macula. The patient underwent PPV repair of the retinal detachment, with placement of an intraocular air bubble (bottom row, left), though there was no drainage of the cyst. Postoperatively (bottom row, right), the cyst flattened, and vision returned to 20/25.

Retinal Dialysis and Giant Retinal Tear (GRT)

A full-thickness retinal break that involves three or more clock hours is considered a giant retinal tear (GRT). GRTs may be associated with hereditary conditions including Marfan syndrome, Stickler syndrome, and high myopia, though may also occur spontaneously, or result from trauma. Between 80 and 90% of GRTs occur in males.

A retinal dialysis is a tear of the retina that results in disinsertion from the ora serrata. Most retinal dialyses are secondary to trauma and are seen most commonly in the inferotemporal quadrant.

This patient has an inferior retinal detachment caused by a retinal dialysis (arrows). The peripheral retina has separated from the ora serrata and is displaced toward the posterior pole of the fundus. The detached retina has lost some of its transparency due to edema or hydration.

This patient has a retinal detachment from a GRT. The peripheral retina has torn and folded over itself with the anterior edge of the retina now draped over the posterior pole of the fundus (arrows). The detached retina has become hydrated, resulting in loss of retinal transparency.

A 180-degree (six clock hours) GRT. The retina is folded upon itself inferiorly, with bare RPE seen superiorly (left). There was minimal PVR. The retina was repaired via a PPV approach, use of intraoperative liquid perfluorocarbon (to unfold the retina), use of extensive peripheral photocoagulation, and placement of an encircling scleral buckle (right). Anatomic success was achieved.

Retinal Detachment and Macular Hole

This photo exhibits a bullous retinal detachment with multiple retinal folds in a patient with amyloidosis. A macular hole accounts for the detachment (inset) , as no peripheral retinal tears were seen. Most detachments from macular holes occur in pathological myopia or secondary to blunt ocular trauma. This case is an exception.

Jul 30, 2019 | Posted by in OPHTHALMOLOGY | Comments Off on Peripheral Retinal Degenerations and Rhegmatogenous Retinal Detachment
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