Vitreoretinal surface diseases

Referral priority: Low or moderate – depending on symptoms and clinical signs.

If a patient develops vitreoretinal surface conditions and are symptomatic, refer them to an ophthalmologist following local guidelines.

Written by
Marko Lukic
Edited by
Svein Tindlund and Jon Gjelle
Published
June 2023
Sections
01
Introduction

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02
Symptoms

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03
Clinical entities

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04
Diagnostic procedures

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05
Management and Treatments

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06
References

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Atlas overview
Sections
01 Introduction
01

Introduction

The vitreous is a clear gel that fills the space between the lens, ciliary body anteriorly, and retina posteriorly.(1) The vitreous comprises approximately 80% of the volume of the eye. It comprises approximately 98% water and 2% proteins, and an extracellular matrix. Collagen is the major structural protein (type II 75% and type IX 15%).(1,2)

The strongest attachment points of the vitreous are at the optic nerve, macula, ora serrata, and major retinal blood vessels.(3) The equatorial and posterior vitreous interface consists of the posterior vitreous cortex, internal limiting membrane (ILM), and the intervening extracellular matrix (ECM). The intervening extracellular matrix is a macromolecular complex that glues the ILM and posterior cortex. It is composed of fibronectin, laminin, chondroitin sulfate, and other structures of the extracellular matrix.(1) The vitreoretinal separation is a normal aging process that starts as the liquefaction of the vitreous gel. Eventually, it ends as a posterior vitreous detachment.(4) The posterior vitreous detachment in terms of vitreoretinal surface appearance means that the posterior cortical vitreous is completely detached from the internal limiting membrane.

There are situations when the separation of the posterior vitreous cortex from the ILM is anomalous, either due to abnormal adhesion or when the liquefaction is faster than the separation of the posterior vitreous cortex.(5) Various conditions may develop secondary to anomalous vitreoretinal interface, such as vitreomacular adhesion, vitreomacular traction, epiretinal membrane, full thickness macular hole, and lamellar macular hole.(5,6) The early recognition and classification of those changes have been significantly improved with the OCT.

02

Symptoms

Vitreomacular adhesion is asymptomatic, and it has been considered a process of normal posterior vitreous detachment (PVD).(4)

Vitreomacular traction causes decreased and blurry vision accompanied by metamorphopsia (distorted vision). Then, patients may complain of micropsia (abnormal visual perception when the objects appear smaller than they are in reality), scotoma, and difficulties with reading.(6,7)

Epiretinal membrane is asymptomatic in a significant number of patients. Those who are symptomatic will have blurry vision, metamorphopsia, and micropsia. Likewise, patients may complain of monocular diplopia.

Macular hole is symptomatic and causes a drop in vision with metamorphopsia. Likewise, it often causes central scotoma.

Lamellar macular holes may present with mild symptoms of blurry vision and metamorphopsia. The vision is stable and very rarely drops significantly over a period of follow-up (only in progression to the full-thickness macular hole).

03

Clinical entities

Vitreomacular adhesion (VMA)
According to the International Vitreomacular Traction Study, vitreomacular adhesion is considered a process of normal PVD (incomplete PVD).(4) The vitreous is detached in the perifoveal area. It is asymptomatic and causes no changes in the retinal architecture. The slit-lamp examination is unremarkable.

VMA can be subclassified according to the adhesion size: focal if the adhesion is less than 1500 microns (area of one disc diameter) or broad if the adhesion is greater than 1500 microns.(4) It may play a role in forming vitreomacular traction or macular hole.(8)

Vitreomacular traction (VMT)
When the abnormal adherence between the posterior cortical vitreous and ILM causes tangential or anteroposterior traction of the macula, this is called vitreomacular traction.(4) It causes anatomical and often functional deficits.

It may also be subclassified as focal or broad according to the aforementioned criteria.

Clinically, the slit-lamp examination may show only subtle changes or, in more significant cases, blunted foveal reflex and cystic spaces. OCT imaging allows early detection of the condition. It shows from very subtle changes to more advanced ones, like cystic spaces due to subfoveal subretinal detachment.(4)

The vitreomacular traction can be isolated or associated with other retinal conditions like age-related macular degeneration, diabetic macular oedema, and retinal vein occlusion. The prevalence of isolated VMT is reported to be 22.5 per 100,000 people. As the use of OCT imaging has been increasing, there is an increased prevalence for both VMT and VMA.(9)

The VMT, if untreated, may cause secondary complications like a full-thickness macular hole, tractional maculoschisis, and tractional foveal/macular detachment.

Image 1. OCT scan of the vitreomacular traction. Pulling of the retinal tissue with secondary schitic changes can be observed.

Epiretinal membrane (ERM)
The epiretinal membrane is semi-translucent fibro cellular tissue found on the surface of the internal limiting membrane (ILM). It has been known under different names, i.e., cellophane maculopathy, epimacular membrane, surface-wrinkling retinopathy, macular pucker, and preretinal macular fibrosis.(10) It has been hypothesised that glial cells form and grow on the residual cortical vitreous remaining on the surface of the ILM during the process of the PVD.

ERM may be primary or often-called idiopathic, which is the most common cause. Likewise, the ERM may be secondary in different retinal conditions, like intraocular inflammation, diabetic retinopathy, myopia, retinal dystrophies, and retinal vein occlusion. Be aware that epiretinal membranes may also be secondary to chronic retinal tears and retinal detachment. Therefore, always check the peripheral retina carefully in every patient with the ERM.

A sheen or abnormal macular reflex is suggestive of the epiretinal membrane. In more advanced cases, you will notice retinal wrinkling. In approximately 20% of patients, the ERM is present in both eyes.(10,11) Likewise, it has been reported that ERM affects 7% of the population.(11)

Image 2. The OCT scan of epiretinal membrane. Increased retinal thickness with retinal wrinkling can be observed. The epiretinal membrane is the hyperreflective line (red arrow) above the ILM (white arrow).
Image 3. The OCT scan of epiretinal membrane. The membrane can be observed in the nasal part of the macula with no macular thickening. The foveal deep is preserved.

Full-thickness macular hole (FTMH)
This entity represents the full defect of the fovea (from ILM to RPE) due to anteroposterior traction of the abnormal vitreomacular adhesion and/or tangential forces secondary to the perifoveal posterior vitreous cortex.(12) The risk factors are age, female gender, myopia, trauma, and history of ocular inflammation.(13) Early stage of the macular hole can be observed as a lipofuscin spot (a yellowish spot) or a ring in the fovea. The more advanced cases are seen as a hole within the foveal zone.

There are two main classifications for macular holes. The Gass’ classification is based on clinical appearance.(14)

  • Stage 1 – loss of foveal depression. Stage 1A represents a yellow dot, and stage 1B represents a yellow ring. It represents a foveal detachment with loss of foveal contour.
  • Stage 2 – represents full thickness macular hole of fewer than 400 µm. The posterior hyaloid membrane is still attached to the ILM, and it is symptomatic.
  • Stage 3 – it represents a macular hole that is ≥400 µm. A greyish ring may be noted around the hole, representing subretinal fluid. The vitreous is detached over the hole, and you may see an operculum as well.
  • Stage 4 –a macular hole that is characterised by features from stage 3 and complete detachment of the posterior vitreous (Weiss ring is clinically seen).

As the OCT imaging brought new insights into the appearance and mechanism of the formation of macular holes, the The International Vitreomacular Traction Study (IVTS) Group proposed classification for macular holes based on the OCT findings based on three factors:(4)

  1. Size: Horizontal diameter at narrowest point: a) small (≤ 250 μm), b) medium (250-400 μm), c) large (> 400 μm).
  2. Cause: primary (due to VMT and abnormal vitreous separation) or secondary (trauma, high myopia, surgical trauma, long-lasting macular oedema, macular telangiectasia type 2).
  3. Presence or absence of VMT.
Image 4. The OCT scan of the full-thickness macular hole. Intraretinal cysts can be observed along with the attached hyaloid membrane (white arrow) and operculum (blue arrow) – this is stage 2 from the Gass classification

Partial thickness macular hole/Lamellar macular hole
The lamellar macular hole can be seen clinically as a reddish-foveal defect. It has been reported that only a minority of cases have been diagnosed clinically as compared to OCT imaging.(15,16) The IVTS Group defined characteristics of the lamellar macular hole that include an irregular foveal contour, a defect or break in the inner fovea, a splitting of the inner and outer retina, lack of a full-thickness foveal defect, and intact photoreceptors.(4,16) The lamellar macular holes are often accompanied by an epiretinal membrane. Some of them may progress to full-thickness macular holes due to tangential forces secondary to the epiretinal membrane.

Image 5. Lamellar macular hole.

Macular pseudohole
The macular pseudohole clinically appears as a round or oval reddish appearance in the fovea, which resembles the macular hole. The appearance is secondary to the contraction of the epiretinal membrane, and the OCT imaging confirms there is no presence of a macular hole. There is the presence of a steep foveal contour seen on the OCT, and there is no loss of retinal tissue. It has been published that 8-20% of ERM may present with the pseudohole.(17)

04

Diagnostic procedures

Slit lamp examination/colour fundus photography – classical ways of observing the fundus. Be aware that many subtle changes or early stages of the conditions can be missed.

Optical coherence tomography –a state-of-the-art imaging tool to recognise the vitreoretinal surface conditions, properly classify them, and monitor the progression or postoperative recovery.

05

Management and Treatments

The management depends on the stage of the lesion and its symptoms. Basically, all VMAs and asymptomatic epiretinal membranes do not require active treatment and can be observed only. Likewise, all lamellar macular holes can be safely observed, and in case of progression or new symptoms, the patient should be referred to an ophthalmologist.

Patient with a full-thickness macular hole and vitreoretinal tractions should be referred to an ophthalmologist – regardless of symptoms. If the lesion is present in the non-dominant eye, the patient may be less aware of the symptoms. Always check the monocular vision and ask the patient to describe the symptoms (i.e., whether they see distorted lines). The symptomatic/progressive cases require a vitrectomy.

06

References

1 S.J. Ryan, C.A. Puliafito, J.L. Davis, J.M. Parel, P. Milne Retina (4th ed.), Elsevier Mosby, Philadelphia, PA (2006)

2 De Smet MD, Elkareem AM, Zwinderman AH. The vitreous, the retinal interface in ocular health and disease. Ophthalmologica. 2013;230(4):165-78.

3 Tsui I, Pan CK, Rahimy E, Schwartz SD. Ocriplasmin for vitreoretinal diseases. Journal of Biomedicine and Biotechnology. 2012 Jan 1;2012.

4 Duker JS, Kaiser PK, Binder S, de Smet MD, Gaudric A, Reichel E, Sadda SR, Sebag J, Spaide RF, Stalmans P. The International Vitreomacular Traction Study Group classification of vitreomacular adhesion, traction, and macular hole. Ophthalmology. 2013 Dec 1;120(12):2611-9.

5 Bottós J, Elizalde J, Arevalo JF, Rodrigues EB, Maia M. Vitreomacular traction syndrome. Journal of ophthalmic & vision research. 2012 Apr;7(2):148.

6 Bottós J, Elizalde J, Arevalo JF, Rodrigues EB, Maia M. Vitreomacular traction syndrome. Journal of ophthalmic & vision research. 2012 Apr;7(2):148.

7 Stalmans P, Duker JS, Kaiser PK, Heier JS, Dugel PU, Gandorfer A, Sebag J, Haller JA. Oct-based interpretation of the vitreomacular interface and indications for pharmacologic vitreolysis. Retina. 2013 Nov 1;33(10):2003-11.

8 John VJ, Flynn Jr HW, Smiddy WE, Carver A, Leonard R, Tabandeh H, Boyer DS. Clinical course of vitreomacular adhesion managed by initial observation. Retina. 2014 Mar 1;34(3):442-6.

9 Jackson TL, Nicod E, Simpson A, Angelis A, Grimaccia F, Kanavos P. Symptomatic vitreomacular adhesion. Retina. 2013 Sep 1;33(8):1503-11.

10 https://eyewiki.aao.org/Epiretinal_Membrane

11 Fraser-Bell S, Guzowski M, Rochtchina E, Wang JJ, Mitchell P. Five-year cumulative incidence and progression of epiretinal membranes: the Blue Mountains Eye Study. Ophthalmology. 2003 Jan 1;110(1):34-40.

12 Chandra A, Charteris DG, Yorston D. Posturing after macular hole surgery: a review. Ophthalmologica. 2011;226(Suppl. 1):3-9.

13 https://eyewiki.aao.org/Macular_Hole

14 Gass JD. Idiopathic senile macular hole: its early stages and pathogenesis. Archives of ophthalmology. 1988 May 1;106(5):629-39

15 Haouchine B, Massin P, Tadayoni R, Erginay A, Gaudric A. Diagnosis of macular pseudoholes and lamellar macular holes by optical coherence tomography. American journal of ophthalmology. 2004 Nov 1;138(5):732-9.

16 Witkin AJ, Ko TH, Fujimoto JG, Schuman JS, Baumal CR, Rogers AH, Reichel E, Duker JS. Redefining lamellar holes and the vitreomacular interface: an ultrahigh-resolution optical coherence tomography study. Ophthalmology. 2006 Mar 1;113(3):388-97.

17 Massin P, Paques M, Masri H, Haouchine B, Erginay A, Blain P, Gaudric A. Visual outcome of surgery for epiretinal membranes with macular pseudoholes. Ophthalmology. 1999 Mar 1;106(3):580-5.