Peripheral Retinal Degenerations

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Article initiated by:

Dr Jay Chhablani, Dr. Anupam Basant Bagdi MBBS

All contributors:

Vinay A. Shah M.D., Leo A. Kim, MD, PhD, Robert A Hyde, MD, PhD, Alexander Engelmann, MD, Jennifer I Lim MD, Dr Jay Chhablani, Peter A.Karth, MD, Nikhila Khandwala, Hashem Abu Serhan, Cassie Huang

Assigned editor:

Durga Borkar, MD, MMCi

Review:

Assigned status Update Pending

 by Robert A Hyde, MD, PhD on April 29, 2024.

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Contributing Editors:	add

Peripheral Retinal Degenerations

ICD-10

H35.4

Classification of Peripheral Retinal Degenerations

Peripheral retinal degenerations are classified according to the following criteria:

• Location: equatorial, peripheral, or combined
• Pathomorphology: trophic, tractional, atrophic, or combined
• Depth of retinal changes: intraretinal, retinal, vitreoretinal, or chorioretinal
• Risk for retinal detachment
• Prognosis: progressive or stationary

The following are the commonly used classifications of peripheral retinal degenerations based on the depth of retinal changes observed on optical coherence tomography (OCT):

• Intra-retinal degenerations: senile retinoschisis, white-without-pressure, white-with-pressure, dark-without-pressure, peripheral cystoid degeneration, snowflake degeneration, and pearl degeneration
• Vitreoretinal degenerations: lattice degeneration, snail-track degeneration, retinal tufts, and peripheral retinal breaks
• Chorioretinal degenerations: paving-stone degeneration and peripheral retinal drusen

Utility of OCT in Diagnosis

Optical coherence tomography has been shown to aid in diagnosing peripheral retinal degenerations by characterizing structural elements, including the shape and depth of degeneration. Peripheral OCT may further aid in the differentiation of degeneration types and the identification of associated complications, including subclinical retinal detachment, retinoschisis, retinal tears, and vitreoretinal adhesions or tractions. Therefore, OCT should be utilized in the initial diagnosis, treatment, and follow-up.  

Intra-Retinal Degenerations

Senile Retinoschisis

• Definition: Splitting of layers of the neurosensory retina by thick fluid
• Prevalence: Found in 2%–7% of the general population; more common in people aged ≥40 years and in hyperopic eyes
• Characteristics: Lesion is a bullous elevation of the peripheral retina, most commonly in the inferotemporal quadrant
• Findings: Mostly asymptomatic
• Immobile with movement of the eye; produces an absolute field defect
• Complications: May involve posterior pole and lead to retinal detachment
• Primary cause of retinal detachment in 0.05%–2.5% of cases
• Diagnosis: OCT findings include intraretinal hyporreflective cavities with destruction and thickening of the retinal pigment epithelium (RPE) and separation of the neurosensory retina with splitting at the outer plexiform layer
• Treatment: Argon laser photocoagulation when patients are symptomatic or there is progressive retinal detachment threatening the macula

White-Without-Pressure

• Definition: Distinctive white appearance of the peripheral retina without indentation and without mechanical stimulus
• Prevalence: Found in up to 30% of normal eyes; often bilateral; more frequently diagnosed in younger patients; may be associated with longer axial length
• Characteristics: Whiter than the retina in white with pressure; choroidal markings are almost obscured. Found in the post-equatorial region at the base of the vitreous and ora serrata. Whiteness is further accentuated with scleral depression. Margins are sharply demarcated from normal retina
  • Can also appear as irregular, translucent areas in the retinal periphery with a red-brown border
  • Lesions may have scalloped edges and may appear to move over time due to possible migratory nature
• The exact cause is unknown. One school of thought states it to be a manifestation of peripheral vitreous traction while another believes it to be simply an abnormal reflex from a structurally normal vitreoretinal interface
• Frequently causes confusion with subclinical retinal detachment and retinoschisis, but indentation clearly reveals that the retina is still apposed to the RPE
• Diagnosis: OCT shows white areas corresponding to hyperreflective outer retinal layers and ellipsoid zone, with no vitreous traction
  • Fluorescein angiography may show patterns, including multiple pinpoint areas of hyperfluorescence and increased peripheral fluorescence
  • Fundus autofluorescence may demonstrate relative hypofluorescence within lesions suggestive of potentially reduced lipofuscin density in RPE cells
• Treatment: There is a low risk for association with retinal detachment, so patients are watched and examined every 1–2 years  

White-With-Pressure

• Definition: Distinctive milky white or opalescent appearance of the peripheral retina that is observed in many normal eyes when examined with scleral depression; term used to describe flat peripheral detachment without any retinal break
  • Must be carefully distinguished from a subclinical peripheral retinal detachment
• Prevalence: Seen in around 30%–35% of eyes examined with scleral depression; incidence increases with age; no sex predilection; more commonly observed in myopic eyes during the second decade of life
• Findings: The retina appears normal without depression
  • Inferonasal quadrant is the least likely to be affected
• Complications: Benign condition; not associated with retinal breaks

Dark-Without-Pressure

• Definition: Flat, brown fundus lesion with well-defined margins in the equator of the peripheral retina  
• Findings: Can be found posterior to the area of white-without-pressure and may look like a retinal tear, posterior to ora serrata
• Symptoms: usually none
• Diagnosis: OCT findings show hyporeflectivity of the ellipsoid zone; border corresponds to the site where the ellipsoid zone faded or disappeared
• Treatment: Benign condition; follow the patient with routine examinations

Peripheral Cystoid Degeneration

• Definition: Multiple small intraretinal microcystoid cavities, most frequently seen in the temporal peripheral retina
• Characteristics: Yellow tiny vesicles with blurred borders on a gray background
  • Typical: Occurs in all adults; may be complicated by fusion of cysts, development of cavities in the outer plexiform layer, and flat senile retinoschisis
  • Reticular: Occurs in 18% of adults; may be complicated by bullous retinoschisis with cavities found in the retinal nerve fiber layer. This form is almost always found posterior to typical peripheral cystoid degeneration and tends to follow the retinal vessels
• Diagnosis: OCT findings show microscopic cystoid spaces in the inner to outer plexiform layers

Snowflake Degeneration

• Definition: Small yellowish dots in the peripheral retina that appear white due to light reflection; small crystalline deposits in the retina from the ora serrata to the equator
• Prevalence: Rare
• Findings: May span a wide band in more than one retinal quadrant, most often in the superotemporal quadrant
  • May be combined with other peripheral retinal degenerations (e.g. retinoschisis, lattice, white-without pressure)
• Complications: Retinal tears, retinal holes, and retinal detachment

Pearl Degeneration

• Definition: Congenital retinal degeneration associated with white beads on the retina
• Prevalence: Found in up to 20% of patients
• Characteristics: Ora pearls appear like drusen and are found either between the RPE and Bruch’s membrane or floating above the RPE
• Diagnosis: OCT findings show hyporeflective cavities with vitreous layers attached to the top of the lesion  

Vitreoretinal Degenerations

Lattice Degeneration

• Definition: Retinal thinning with loss of neurosensory layer; vitreoretinal adhesions at the margin of lesion
• Prevalence: Found in 6%–8% of patients
• Characteristics: Retinal thinning with fibrosis and vitreous liquefaction over the lesion; spindle-shaped areas of retinal thinning with or without pigmentation; arborizing white lines; frost-like areas commonly located between the equator and the posterior border of the vitreous base
  • Typical: Well-outlined thinning with white crossing lines
  • Atypical: Found adjacent to vessels in a radial pattern
• Findings: Lesions have an oval or linear pattern; may be a single lesion or multiple lesions; may have yellow deposits, pigment, atrophic holes, or retinal tears
• Complications: Risk for retinal detachment and rhegmatogenous retinal detachment due to vitreous traction
  • Retinal detachment seen in 14%–35% of cases
• Treatment: Prophylactic laser treatment is recommended in patients with vitreous traction and retinal tears associated with flashes and floaters  

Snail-Track Degeneration

• Definition: Groups of glistening white dots (which have been compared to frost granules or salt); may be an early stage of lattice degeneration
• Prevalence: Found in 10% of the general population; more commonly seen in myopic eyes (40%)
• Characteristics: Retinal thinning with vitreous liquefaction above the lesion; associated vitreoretinal tractions
• Findings: More often seen in the superotemporal and superonasal quadrants
• Histologically, this lesion consists of degeneration of the neural elements of the retina, leading to an atrophy of the tissues with lipid deposits in the internal retinal layers
• Complications: May lead to retinal tears in up to 54% of patients; retinal holes may also be seen
• Diagnosis: OCT findings show irregular retinal surface, vitreous destruction, and vitreoretinal adhesions with traction at the margins

Retinal Tufts

• Definition: Area of retinal degeneration caused by attachment to and pulling of the vitreous  

• Types include cystic, noncystic, and zonular traction tufts
• Prevalence: Noncystic tufts are present in up to 72% of adults, cystic tufts are present in up to 5% of adults, and zonular traction tufts are present in up to 15% of adults
• Complications: Vitreous traction can lead to retinal tears and retinal detachment
• Treatment: Prophylactic laser treatment is not recommended by the majority of physicians; routine follow-up examinations are preferred

Peripheral Retinal Tears

• Definition: Isolated tears due to a defect in the sensory retina from vitreous traction after acute posterior vitreous detachment

• Prevalence: 9%–12% of the general population

• Characteristics: Retinal tears or retinal holes; retinal holes are due to atrophic changes in the neurosensory retina

• Complications: High risk of rhegmatogenous retinal detachment, which occurs in 3%–18% of patients

• Treatment: Retinal tears with symptoms on presentation should be treated with prophylactic laser treatment, most commonly transpupillary retinopexy

Chorioretinal Degenerations

Paving-Stone Degeneration

• Definition: Multiple rounded punched-out areas of choroidal and retinal atrophy
• Prevalence: present in 4%–28% of patients; often bilateral; similar prevalence in men and women; increasingly common with age
• Characteristics: Lesions are yellow-white in color and may reveal underlying choroidal vessels due to the sclera being partly visible through the atrophic choroid; discrete margins that may be pigmented; may become confluent
• Findings: Located between the ora and equator with the size of one to several disc diameters
• More common in the inferonasal and temporal quadrants
• Large choroidal vessels may be seen running through the base
• Complications: Benign lesions not associated with complications

Peripheral Retinal Drusen

• Definition: Extracellular protein and fat deposits between the RPE and Bruch’s membrane, caused by degeneration of RPE cells
• Prevalence: Commonly found in people aged ≥40 years
• Characteristics: Resemble crystals
• Findings: Small, round-shaped; clearly outlined
• Complications: Benign degenerations not associated with complications
• Treatment: Prophylactic laser treatment is not indicated