Lattice Degeneration

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Practice Essentials

Lattice degeneration elevates the risk of retinal detachment but does not require treatment if it is asymptomatic.

Background

Lattice degeneration is a common, atrophic disease of the peripheral retina characterized by oval or linear patches of retinal thinning.[1, 2] The prevalence peaks by the second decade and is believed to be minimally progressive but may be complicated by retinal breaks and retinal detachment.[3]

Pathophysiology

The pathogenesis of lattice degeneration is not well understood, although several theories have been proposed.[4] Regional maldevelopment or absence of the internal limiting membrane versus abnormal vitreoretinal traction dynamics appear to be the most cogent arguments proposed.

Epidemiology

Frequency

United States

Lattice degeneration affects approximately 10% of the population and is bilateral in 30-50% of patients who are affected. A variable familial risk may be present on the basis of various autosomal dominant pedigrees.[5] An increased prevalence exists in myopic eyes, and its prevalence may be associated with increasing axial length, reaching 15% in the longest eyes.

International

No information is available regarding the international occurrence of lattice degeneration.

Mortality/Morbidity

See discussion of retinal detachment in History and Physical.

Race

No reported racial differences exist in lattice degeneration.

Sex

No reported sex differences exist in lattice degeneration.

Age

See History regarding early onset and progression with age.

Prognosis

The prognosis of lattice degeneration is generally good.

Patient Education

Educate the patient on signs of retinal detachment.

Encourage annual follow-up for dilated eye examinations.

Patients with lattice degeneration need to be made aware of their condition and should be warned about the increased lifetime risk of retinal tears or detachment. They should be advised to see an eye doctor immediately if they develop symptoms of retinal tears or detachments, including new floaters, flashes of light, decreasing vision, or a "curtain" in their vision.

History

Patients with lattice degeneration are typically asymptomatic, and the lesions are usually an incidental finding of dilated ophthalmologic examination. A presenting complaint of blurriness in the distance may be the result of myopia, a common association with lattice degeneration. The acute onset of floaters, flashes of light, peripheral field loss, or central vision loss may indicate the presence of a retinal tear or detachment, a complication of lattice lesions.[6]

Physical

Clinical features

Lattice degeneration is characterized by oval or linear patches of atrophic retina with a reddish base and is variably located within the equatorial region of the fundus, typically inferotemporal.[2]

Lesions may be isolated or multifocal, variable in dimension, and usually are oriented concentric or slightly oblique to the ora serrata.

Condensed vitreous at the margins of the lattice lesions may appear as vitreous opacities and represent regions of increased vitreoretinal adhesion; overlying vitreal opacities alternatively may be explained by glial proliferation.[7]

Crisscrossing fine white lines that account for the name lattice degeneration are present in roughly only 10% of lesions and most likely represent hyalinized blood vessels; this is shown in the image below.



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Example of a lattice lesion containing white crisscrossing wicker lines, which are seen in about 10% of lattice lesions. This lesion is complicated by....

Various pigmentary disturbances, shown in the image below, occur in more than 80% of lattice lesions. White-yellow occasionally refractile flecks, similar to that seen with degenerative retinoschisis, are an additional common associated feature.



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An example of a heavily pigmented lattice lesion.

Atrophic retinal holes, shown in the image below, and tractional retinal tears may complicate lattice degeneration and increase the risk of retinal detachment.



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Lattice lesion containing small atrophic holes.

Clinical variations

Snail-track degeneration is a morphologic descriptive term for retinal lesions with the same characteristic size, shape, orientation, and location as lattice lesions and is associated with the aforementioned yellowish flecks. Examples of snail-track degeneration are shown in the images below.



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A peripheral lattice lesion demonstrating the typical snail-track appearance, with overlying vitreal opacities, which may represent glial proliferatio....



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Another example of a peripheral lattice lesion with a snail-track appearance.

Vitreous base excavations represent lesions of similar shape, size, and orientations as lattice lesions having a uniform reddish base and located within the vitreous base.

Pigmentary degeneration is a term that most likely represents lattice lesions with prominent but variable pigmentary changes, including clumps of pigment sometimes heavily scattered at the base of lattice lesions and demarcation lines circumscribing cuffs of subretinal fluid.

When retinal thinning and pigmentary disturbances are found along retinal vessels, these lattice lesions are referred to as radial perivascular chorioretinal degeneration and are classic findings in Wagner and Stickler disease, a familial vitreoretinal degenerative syndrome. An example of this is shown in the image below.



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Radial perivascular chorioretinal degeneration with retinal tear at the margin. These lesions run along vessels and may be found in Wagner's and Stick....

Clinical examination

Identification of lattice lesions depends largely upon the experience of the examiner and the method of examination used.

In patients with symptomatic lattice degeneration (presence of newly onset flashes or floaters), the retinal periphery must be viewed for 360° of its circumference to identify any retinal breaks, if present. If the general ophthalmologist is unable to do so, referral should be promptly made to a vitreoretinal subspecialist.

The most convenient and frequently used method of examination to detect lattice is with binocular indirect ophthalmoscopy with scleral depression (indentation).

Slit lamp examination with a Goldmann contact lens is used less frequently. This method allows for high magnification of the lattice lesions and the associated vitreoretinal relationships, but evaluation with scleral depression, a critical element of the peripheral examination, becomes technically difficult when using a contact lens.

Clinical course

Lattice lesions are believed to develop early in one's lifetime with minimal progression thereafter. Associated features, such as crisscrossing sclerotic vessels, pigmentation, and atrophic retinal holes, may subsequently develop.

Retinal detachment is a relatively rare complication of lattice degeneration (< 1% of patients with lattice degeneration), but lattice degeneration is associated with as many as 40% of all rhegmatogenous retinal detachments. Lattice degeneration is present in 11% of fellow eyes in patients with rhegmatogenous retinal detachments.[8, 9, 10] Rhegmatogenous retinal detachment is shown in the image below.

A 2015 study reported a higher risk (3.4%) of retinal tears and detachments in myopic eyes (>6 D) over a 10-year period.[11]



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An acute rhegmatogenous retinal detachment that may be associated with lattice degeneration. (Lattice lesion not seen in this image.)

Retinal detachments caused by lattice degeneration occur most commonly by a tractional tear at the cuff or posterior margin of the lattice lesion or less commonly by means of an atrophic hole within the zone of lattice.

Tractional tears

Tractional tears located at the margin of lattice account for 55-70% of retinal detachments in lattice degeneration and are the result of a posterior vitreous detachment (PVD).

These patients are typically older than 50 years, and only 40% of such eyes are myopic.

An acute PVD complicated by retinal tear formation usually is signaled by the complaint of new-onset floaters and/or flashing lights (referred to as photopsia)

As in all retinal tears, preretinal or vitreous hemorrhage may be present if the tear extends through peripheral retinal blood vessel.

Patients with these complaints constitute a true ophthalmologic emergency and need urgent ophthalmic examination.

PVD-related lattice detachments typically are superotemporal and not associated with demarcation lines. Surgical repair is anatomically successful in 90% of such cases.

Atrophic holes

Atrophic holes account for 30-45% of retinal detachments associated with lattice degeneration. In another retrospective case-controlled study, 23.6% of patients with retinal detachments had atrophic holes associated with lattice degeneration.[12]

Seventy percent of patients with rhegmatogenous retinal detachment secondary to round atrophic holes in lattice degeneration occur in patients younger than 40 years, and 70% of these detachments occur in myopic eyes. Sixty percent are found in females.

Fellow eye pathology is found in a significant (96%) portion of these patients. Thus, examination of the fellow eye is important in these cases.

The posterior hyaloid gel usually is attached, excluding a PVD-related mechanism. These detachments are typically inferior, occur slowly, and demonstrate demarcation lines on examination resulting from the slow progressive accumulation of subretinal fluid.

A 98-100% success rate exists in repairing such inferior retinal detachments with demarcation lines with an excellent visual prognosis in the absence of macular detachment.

Causes

See Pathophysiology.

Physical Examination

A 360° evaluation of the peripheral retina is conducted using scleral depression or the Goldmann 3-mirror lens. 

Complications

Potential complications of lattice degeneration include development of retinal breaks or detachments.

Approach Considerations

A dilated eye examination with careful examination of the peripheral retina may be performed.

Laboratory Studies

No appropriate laboratory studies exist for lattice degeneration.

Imaging Studies

Optos camera fundus photography may be useful for annual follow-up and documentation of the condition.

Procedures

Examination by direct and indirect ophthalmoscopy

Histologic Findings

Histologic studies of autopsy cases demonstrate that lattice lesions are characterized by 3 invariable features: thinning or atrophy of the inner retinal layers, vitreous liquefaction overlying the area of thinned retina, and vitreous condensation and exaggerated vitreoretinal attachments at the borders of the lesions.

The blood vessels within the lesions are usually patent, but they often show fibrous thickening of their walls, which correlates to the white lattice lines seen clinically. Melanin-laden macrophages may explain the pigmentation seen clinically. Glial proliferations may represent overlying preretinal opacities.

Electron microscopic studies have demonstrated retinal thinning, loss of retinal neurons, internal limiting lamina absence, fibrosis of blood vessels, and accumulation of pigment and/or glial elements.

Medical Care

The presence of uncomplicated lattice does not interfere with visual function and does not constitute a high risk for future development of retinal detachment. Prophylactic treatment is clearly indicated only in the context of specific circumstances.[13, 14, 15]

Indications for prophylactic treatment

Lattice degeneration complicated by tractional tears as the result of an acute, symptomatic posterior vitreous detachment represents a high-risk situation for future retinal detachment and is an urgent indication for laser retinopexy. Lattice and atrophic holes complicated by progressively increasing subretinal fluid represents an additional indication for surgical intervention.

The presence of lattice lesions in fellow eyes of patients who have sustained retinal detachment in the first eye may be treated prophylactically. Exceptions may include eyes with greater than 6 clock hours of lattice lesions and eyes with myopia greater than 6 diopters (D). Strong evidence suggests that subsequent retinal detachments may occur as a result of lesions developing in previously healthy retina.[16] Moreover, laser scars may increase vitreoretinal adhesion and increase the risk of future retinal tears. Therefore, this indication is controversial. In the absence of the aforementioned features, convincing evidence does not exist to clearly indicate prophylactic laser treatment of fellow eye lattice lesions.

Although prophylactic laser treatment may not convincingly prevent subsequent retinal detachment, some authors believe that laser demarcation may limit the extent of future detachments and help preserve the macula.

Methods of prophylactic treatment

Subclinical retinal detachment (>1-disc diameter of subretinal fluid but <2-disc diameters posterior to the equator) may be treated more effectively with a scleral buckle approach versus a laser barrier. 

Laser photocoagulation is the primary method of prophylactic treatment. Recommended laser settings include the following: green, yellow, or red wavelengths via biomicroscope/contact lens or indirect ophthalmoscope delivery systems, duration of 0.1-0.2 seconds, and spot size of 100-200 micrometers. Apply laser in 3 confluent 360° rings around the lesion. Care should be taken to avoid bare retinal pigment epithelium.

Cryotherapy may be a necessary alternative in cases in which significant hemorrhage prevents laser administration.

Retinal detachment treatments

Frank rhegmatogenous retinal detachment may be treated with a scleral buckling procedure and/or pars plana vitrectomy with gas administration.[17] All areas of lattice and retinal breaks should be meticulously sought after and barricaded with laser or cryotherapy.

Surgical Care

Treatment of rhegmatogenous retinal detachment as described under Medical Care.

Complications

Retinal detachment may still occur after treatment.

Consultations

A retinal surgeon may need to be consulted.

Long-Term Monitoring

Patients with lattice degeneration should undergo annual dilated eye examinations with close attention the peripheral retina.

Further Outpatient Care

Educating patients about the symptoms (eg, new-onset floaters, flashes, visual field defects) of a retinal tear or detachment is critical. The necessity of being examined on an emergent basis if symptoms of flashes or floaters occur should be stressed.

Complications

Complications are rare with laser retinal treatment, but epiretinal membrane, macular pucker, and/or retinal detachment may occur.[18]

Prognosis

The prognosis is favorable.

Patient Education

Educating patients about the symptoms (eg, new-onset floaters, flashes, visual field defects) of a retinal tear or detachment is critical.

Author

Hemang K Pandya, MD, Fellow in Vitreoretinal Disease and Surgery, Dean McGee Eye Institute, University of Oklahoma College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Ilya (Eli) M Sluch, MD, Fellow in Cornea, Bascom Palmer Eye Institute

Disclosure: Nothing to disclose.

Specialty Editors

Simon K Law, MD, PharmD, Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

Steve Charles, MD, Founder and CEO of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

Chief Editor

Andrew A Dahl, MD, FACS, Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Disclosure: Nothing to disclose.

Additional Contributors

Alex Yuan, MD, PhD, EyeSTAR Resident and Postdoctoral Fellow, Department of Ophthalmology, Jules Stein Eye Institute at University of California, Los Angeles

Disclosure: Nothing to disclose.

David Sarraf, MD, Associate Clinical Professor of Ophthalmology, Retinal Disorders and Ophthalmic Genetics Division, Jules Stein Eye Institute, UCLA Geffen School of Medicine, Greater Los Angeles Veterans Affairs Healthcare System

Disclosure: Nothing to disclose.

Stanley M Saulny, MD, Consulting Staff, Department of Ophthalmology, Ophthalmology Associates of the Valley

Disclosure: Nothing to disclose.

V Al Pakalnis, MD, PhD, Professor of Ophthalmology, University of South Carolina School of Medicine; Chief of Ophthalmology, Dorn Veterans Affairs Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

This review was partly supported by a Research to Prevent Blindness Grant #OP 31 for David Sarraf, MD.

References

  1. Straatsma BR, Zeegen PD, Foos RY, et al. Lattice degeneration of the retina. XXX Edward Jackson Memorial Lecture. Am J Ophthalmol. 1974 May. 77(5):619-49. [View Abstract]
  2. Byer NE. Lattice degeneration of the retina. Surv Ophthalmol. 1979 Jan-Feb. 23(4):213-48. [View Abstract]
  3. Lewis H. Peripheral retinal degenerations and the risk of retinal detachment. Am J Ophthalmol. 2003 Jul. 136(1):155-60. [View Abstract]
  4. Meguro A, Ideta H, Ota M, Ito N, Ideta R, Yonemoto J, et al. Common variants in the COL4A4 gene confer susceptibility to lattice degeneration of the retina. PLoS One. 2012. 7(6):e39300. [View Abstract]
  5. Edwards AO, Robertson JE Jr. Hereditary vitreoretinal degenerations. Ryan SJ, ed. Retina. 3rd ed. St. Louis: Mosby; 2001. 482-98.
  6. Byer NE. Long-term natural history of lattice degeneration of the retina. Ophthalmology. 1989 Sep. 96(9):1396-401; discussion 1401-2. [View Abstract]
  7. Foos RY, Simons KB. Vitreous in lattice degeneration of retina. Ophthalmology. 1984 May. 91(5):452-7. [View Abstract]
  8. Gonzales CR, Gupta A, Schwartz SD, Kreiger AE. The fellow eye of patients with rhegmatogenous retinal detachment. Ophthalmology. Mar 2004. 111:518-521. [View Abstract]
  9. Gonzales CR, Gupta A, Schwartz SD, Kreiger AE. The fellow eye of patients with phakic rhegmatogenous retinal detachment from atrophic holes of lattice degeneration without posterior vitreous detachment. Br J Ophthalmol. 2004 Nov. 88(11):1400-2. [View Abstract]
  10. Folk JC, Arrindell EL, Klugman MR. The fellow eye of patients with phakic lattice retinal detachment. Ophthalmology. 1989 Jan. 96(1):72-9. [View Abstract]
  11. Ho TC, Ho A. Long-term natural course of lattice degeneration of the retina in high myopic eyes - A ten-year long term study. Investigative Ophthalmology & Visual Science. 2015. 56 (7):
  12. Ung T, Comer MB, Ang AJ, Sheard R, Lee C, Poulson AV, et al. Clinical features and surgical management of retinal detachment secondary to round retinal holes. Eye. Jun 2005. 19:665-669. [View Abstract]
  13. Avitabile T, Bonfiglio V, Reibaldi M, et al. Prophylactic treatment of the fellow eye of patients with retinal detachment: a retrospective study. Graefes Arch Clin Exp Ophthalmol. 2004 Mar. 242(3):191-6. [View Abstract]
  14. Wilkinson CP. Evidence-based analysis of prophylactic treatment of asymptomatic retinal breaks and lattice degeneration. Ophthalmology. 2000 Jan. 107(1):12-5; discussion 15-8. [View Abstract]
  15. Wilkinson C. Interventions for asymptomatic retinal breaks and lattice degeneration for preventing retinal detachment. Cochrane Database Syst Rev. 2005. CD003170. [View Abstract]
  16. Mastropasqua L, Carpineto P, Ciancaglini M, Falconio G, Gallenga PE. Treatment of retinal tears and lattice degenerations in fellow eyes in high risk patients suffering retinal detachment: a prospective study. Br J Ophthalmol. Sep 1999. 83:1046-1049. [View Abstract]
  17. Orlin A, Hewing NJ, Nissen M, Lee S, Kiss S, D'Amico DJ, et al. Pars plana vitrectomy compared with pars plana vitrectomy combined with scleral buckle in the primary management of noncomplex rhegmatogenous retinal detachment. Retina. 2014 Jun. 34(6):1069-75. [View Abstract]
  18. Wilkinson CP. Interventions for asymptomatic retinal breaks and lattice degeneration for preventing retinal detachment. Cochrane Database Syst Rev. 2014 Sep 5. 9:CD003170. [View Abstract]

Example of a lattice lesion containing white crisscrossing wicker lines, which are seen in about 10% of lattice lesions. This lesion is complicated by an extensive retinal tear at the cuff of the lesion.

An example of a heavily pigmented lattice lesion.

Lattice lesion containing small atrophic holes.

A peripheral lattice lesion demonstrating the typical snail-track appearance, with overlying vitreal opacities, which may represent glial proliferations or regions of increased vitreoretinal condensation.

Another example of a peripheral lattice lesion with a snail-track appearance.

Radial perivascular chorioretinal degeneration with retinal tear at the margin. These lesions run along vessels and may be found in Wagner's and Stickler's disease.

An acute rhegmatogenous retinal detachment that may be associated with lattice degeneration. (Lattice lesion not seen in this image.)

Example of a lattice lesion containing white crisscrossing wicker lines, which are seen in about 10% of lattice lesions. This lesion is complicated by an extensive retinal tear at the cuff of the lesion.

Another example of wicker lines seen within a lattice lesion. Prophylactic retinopexy has been performed around this lesion.

An example of a flap tear at the edge of a lattice lesion and three adjacent holes. This area of lattice degeneration has been barricaded by laser retinopexy.

A large horseshoe tear at the opposite edge of the lattice lesion pictured above. Laser retinopexy surrounds the tear and lattice lesion.

A peripheral lattice lesion demonstrating the typical snail-track appearance, with overlying vitreal opacities, which may represent glial proliferations or regions of increased vitreoretinal condensation.

An example of a heavily pigmented lattice lesion.

An acute rhegmatogenous retinal detachment that may be associated with lattice degeneration. (Lattice lesion not seen in this image.)

Another example of a peripheral lattice lesion with a snail-track appearance.

Lattice lesion containing small atrophic holes.

Radial perivascular chorioretinal degeneration with retinal tear at the margin. These lesions run along vessels and may be found in Wagner's and Stickler's disease.