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Case report – female with symptoms of “spiders & cobwebs”

Dirk graduated from Witwatersrand Technicon with a Diploma in Optometry in 1984. He continued his studies at Ashton University, Birmingham, UK, to obtain a Professional Doctorate, D Optom. He also holds the MC Optom, from the Institute of Optometry, a TMOD, Concentrated Ocular Therapeutics, from Pennsylvania College of Optometry and a Certificate of Advanced Study from New England College of Optometry. He is currently in private practice, with a part time teaching position at the University of Johannesburg department of optometry. He is an Independent Consultant to several corporations in the optical industry. He authored the book “South African guide to topical ophthalmic drugs”. Dirk has written, published and lectured extensively on a many different topics relating to optometry. He loves flying vintage aircraft and is a scuba diving instructor.

This 60 year old Caucasian female was seen with symptoms of “spiders and cobwebs” which appear and disappear. She also noted a slight decrease in her vision since her last visit 12 months ago. She is healthy and uses no meds.

Refraction and vision:

1. +0.25/-1.00×70 20/25-
2.+0.25/-1.25 x 90 20/20+ Add +2.25

Pupil PERRLA
IOP 12mmHg OU
Discs normal with C/D 0.30 OU
Media clear, some floaters evident
Dilated exam shows changes in the right macular area
Fundus photos and FAF images are attached.
Your DDx and recommendation for additional tests as well as treatment strategy.

Figure 1. Fundus photo right eye
Figure 2. Fundus photo left eye
Figure 3. FAF image right eye
Figure 4. FAF image left eye

DDx Idiopathicpreretinal macular gliosis (IERM) ICD 10 H35.3. Treatment includes observation and possible surgery, membrane stripping and vitrectomy, complications include macular hole formation. Due to the good acuity as well as OCT appearance this patient was observed regularly until the vitreous detaches without damaging the retina. She was been given an Amsler grid and asked to return immediately if things change, routine follow-up was scheduled for 3/12 months which includes mydriatic examination.

“Idiopathic preretinal macular gliosis (IERM).

Fig 5. Herewith the OCT of this patient showing the epiretinal membrane in the right eye more clearly. OCT of the right macular, note the epiretinal membrane as well as the disturbance in the anatomy of the macula due to the traction caused by the membrane.similar in males and females5, and is bilateral in 20-30% of cases7,1. Idiopathic epiretinal membranes are

Common synonyms for preretinal macular gliosis include epiretinal membrane, macular pucker, premacular fibrosis, cellophane retinopathy, epimacular membrane7, primary retinal folds, and silent central retinal vein obstruction5. The majority of patients with idiopathic epiretinal membranes are over 50 years old but young adults and children are occasionally affected7, 5. The prevalence of idiopathic epiretinal membranes in the 50-year and older group is 6% with increasing prevalence with advancing age7,5,1. Prevalence was significantly more common in Chinese persons compared with whites, blacks, and Hispanics. Risk factors for epiretinal membranes were increasing age, presence of diabetes, and hypercholesterolemia6. Mild epiretinal membrane formation occurs commonly in association with blunt or penetrating ocular trauma, vitreous inflammatory conditions, retinal vascular disease, and long standing vitreous haemorrhage7. Posterior vitreous detachment is present in 90% of eyes with idiopathic epiretinal membranes (IERM)7,5,2. IERM are produced by retinal glial cells that migrate through defects in the internal limiting membrane and from the optic nerve head to proliferate and contract on the inner retinal surface forming the fibro vascular tissue termed IERM7,5,2,1.  The cellular elements of IERM include one or more of the following; retinal pigment epithelium cells (RPE), fibrous astrocytes, fibrocytes, inflammatory cells, and macrophages. The observation of RPE cells in the IERM is poorly understood but possibly involves the Trans retinal migration of the cells due to biochemical stimuli7,5,2.

The clinical appearance of the IERM depends on its thickness and the extent to which it has undergone shrinkage or contraction. In the milder form, it is referred to as cellophane maculopathy (CMR). The membrane is thin and transparent, produces no distortion of the inner retinal surface, leaving the patient asymptomatic. It is often only detected by a glistening light reflex from the inner retinal surface with indirect ophthalmoscopy7, 5.  Thicker or more contracted IERM’s produce traction on the neural retina and may be more opaque or grey white partially obscuring visualization of the underlying retinal structures. The white appearance can be attributed to whitening of the inner retina due to traction induced axoplasmic stasis in the nerve fibre layer.  This form is known as preretinal macular fibrosis (PMF)7,5. Full thickness macular distortion, macular oedema, puckering, and folding leads to metamorphopsia, loss of visual acuity and occasional central photopsia7,5,1. Tractional effects may cause macular oedema, preretinal and or intraretinal haemorrhages, tractional macular detachments, pseudo hole’s, and or full thickness macular holes7.

Differential diagnosis of epiretinal membranes.7
Vitreomacular traction syndrome
Combined retinal pigment epithelium and hamartoma traction syndrome
Prominent macular reflex in young patients
Cystoid macular edema
Optic disc swelling
Idiopathic macular hole

Treatment of IERM is not normally indicated unless the membranes cause significant visual symptoms. The goal of treatment is to reduce the most common mechanisms of visual loss, including macular distortion, traction macular detachment, foveal ectopia, tissue that covers the fovea, retinal vascular leakage with macular oedema, and traction-induced obstruction of axoplasmic flow7,1.  Vitreous surgery with peeling of the membrane from the surface of the macula is generally recommended in the more severe cases7.

References

  1. Alexander L J. 1989. Primary care of the posterior segment. Connecticut. Appleton & Lange
  2. Fraser-Bell S, Guzowski M, Rochtchina E et al. Five-year cumulative incidence and progression of epiretinal membranes. The Blue Mountains eye study. Ophthalmology 2003:1101) 34-40
  3. Jones W L, Reidy R W. 1985. Atlas of the peripheral ocular fundus. Boston. Butterworth publishers
  4. Kanski J J ed. 2003. Clinical ophthalmology, a systematic approach. 5th edition. Edinburgh. Butterworth-Heinemann
  5. McCarty D J, Mukesh B N, Chikani V et al. Prevalence and associations of epiretinal membranes in the visual impairment project. American Journal of ophthalmology 2005:1402) 288.e1-288.e8
  6. Ng C H, Cheung N, Wang J J et al. Prevalence and risk factors for epiretinal membranes in a multi-ethnic United States Population. Ophthalmology 2011:118 694-699
  7. Yanoff M, Duker JS eds. 1999. Ophthalmology. London. Mosby”

Final comment: technology is everything in diagnostics, invest in some decent equipment!

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