Basic Concepts of Final Flashcards
What is a pre-retinal/epi macular membrane? (EMM)
Proliferation of retinal glial cells that forms a fibrotic sheet on the ILM.
Benign.
Unilateral or bilateral.
Will not worsen, will stabilize over time.
Two types- Simple/non contractile/cellophane and complex/contractile/macular pucker.
Cellophane maculopathy
Simple, non contractile type of EMM.
Very mild symptoms or asymptomatic. Ok to monitor.
Macular picker
Complex, contractile type of EMM.
Pt will be symptomatic with metamorphopsia, decreased VA, BV tortuosity, and retinal wrinkling.
May lead to pseudo hole (contracture makes it look like there is macular hole, but not actually.) or lead to CME or serous detachment.
Proliferative vitreoretinopathy (PVR)
Intense EMM. Dense and will develop quickly.
involves all layers of the retina- pre, intra, and sub- fibrous proliferation.
Symptomatic
Associated with high myopia.
Main difference between EMM and PVR
EMM is fibrous proliferation of glial cells. Benign, common in older population.
PVR is fibrous proliferation of pre, intra, and sub retinal cells. Associated with high myopia.
Tx for EMM and PVR
Same if symptomatic.
Pars Plana vitrectomy, epi macular membrane peel and ILM peel.
Cystoid Macular Edema (CME)
Cystic pattern around macula. Isolated, acute event. Usually not recurrent.
No FLR, yellow macula, decreased VA and metamorphopsia.
Cause- surgery, chronic inflammation, vascular disease, retinal dystrophies, CNV.
Tx: Topical NSAID + steroid. RTC 2 weeks.
PVD
Vitreous condenses and separates from ILM. Now called posterior hyaloid membrane.
Can be partial- See annular ring, but no collapsed vitreous.
Full- Collapsed vitreous + annual ring.
PVD –> ____ –> ___ –> ____
PVD
VMA (vitreomacular adhesion)
VMT (Vitreomacular traction syndrome)
MH (Macular hole)
PVD may take years to progress to VMA.
PVD and VMA are asymptomatic.
VMT and MH are symptomatic.
VMA (vitreomacular adhesion)
Attachment of posterior hyaloid membrane to ILM.
No symptoms, watch and monitor.
Classification-
Focal vs broad (adhesions +/-1,500)
isolated vs concurrent (another disease present or no?)
VMT (Vitreo-macular traction syndrome)
Continuation of VMA. Pulling of the ILM by the vitreous?
Symptomatic!!!! Blurry VA and metamorphopsia.
Lack of foveal contour (OCT), Absent FLV and yellow macula. May present with cyst or foveal detachment.
Monitor, may resolve. Or could do pars plana vitrectomy + ILM + epi macular membrane peel.
JETRA not a good option
When is JETRA indicated.
VMT. Intravitreal injection but may cause severe toxicities.
VMT treatment options (3)
Monitor - may resolve. Do amsler daily.
Surgery- Vitrectomy and peels
JETRA- not good option bc toxic.
Macular hole
Combination of VMA and VMT
Signs- red macula appearance, + Watzke Allen sign. (Beam is broken, missing, or tapered).
Symptoms- Decreased VA, metamorphopsia.
OCT to confirm Dx.
Two classification systems- International and GASS.
Surgery- Vitrectomy, peels.
International classification of MH
0- VMA
1a and 1b- VMT with foveal detachment
2- FTMH less than 400 micrometers with VMT
3- FTMH more than 400 micrometers with VMT
4- FTMH without VMT. PVD is complete.
0-3 are partial PVD
4 is PVD is complete
GASS classification of MH
1a- impending, or cyst. 1b- Occult. 2- early FTMH or lamellar 3- Established FTMH 4- Full thickness MH
How to know if eye is at risk for macular hole?
no or partial PVD? At risk.
Full PVD? No risk of developing MH.
Macular pseudohole
Contracture of EMM. Distorts the normal foveal appearance- intact photoreceptors without loss of foveal tissues.
Minimal symptoms.
Difference between macular pseudo hole and lamellar macular hole
Pseudohole- intact photoreceptors without loss of foveal tissues. Minimal symptoms. Surgery to tx EMM.
Lamelllar- DO have inner retinal splitting with atypical contour. Minimal symptoms. Monitor.
When to do this surgery- pars plana vitrectomy, epi macular membrane peel and ILM peel.
EMM PVR VMT MH Pseudo macular hole
ICSC
Localized detachment of the sensory retina from RPE at the macula or PP. WITHOUT drusen. Fluid can see through and cause further detachment.
(sensory retina and RPE have loose attachment)
2 presentations- smoke stack and ink blot.
Symptoms: Unilateral blurred vision, decrease contrast, size changes, accepts plus.
Signs: Oval detachment of retina with sub retinal fluid. If the fluid is clear= acute. Turbid= long standing.
OCT to confirm Dx. FA to find location for laser tx.
Tx: Monitor (if not near macula), anti veg F, laser, or epilerone (diuretic).
Associated with: h pylori, oral steroids, HTN, autoimmune, type A, anxiety. `
Drusen
Deposition of abnormal material in bruchs. Bruchs will thicken and RPE will thin.
- Located BETWEEN CELLS.
- Due to compromised RPE metabolism.
- Hyper with FA.
- Associations: Bruchs or RPE disease, choroid disease, choroid melanoma, macular degeneration.
3 types of drusen
- Hard and small. Yellow/white. Well defined. Less than 63 micrometers. No threat to vision.
- Soft and large. Irregular. Pale or dull yellow. Associated with neovasc. –> CNV. Can clump and cause confluent drusen.
- Calcific drusen. Long standing hard or soft. Golden/glistens. Surrounded by RPE changes.
Pseudo drusen/ reticular drusen
Between PR and RPE. Associated with geographic atrophy (The breakdown of RPE causing dry AMD)
Geographic atrophy
The breakdown of RPE. may lead to dry AMD.
Basal laminar drusen
Congenital, hereditary. Located at equator and around arcades.
Risk prediction for transition to AMD in 5 years
Soft drusen greater than 125 micrometers
and/or
Pigmented hyperplasia of RPE
max score per eye- 2
max score total- 4
0: 0.3%
1: 3%
2: 12%
3: 25%
4: 50%
ARM (Age related maculopathy)
“Pre-AMD”
May or may not progress to macular degeneration or VA loss
Variation of normal.
May present with drusen alone, RPE hyper or hypo changes.
ARMD
Sight threatening. More advanced form of ARM.
Will have drusen- may or may not have PED.
Can have geographic atrophy (dry) or CNV (wet)
Tx for geographic atrophy
Early tx: Stop smoking, diet changes, fish, less red meat, L & Z.
Intermediate/advanced tx: Supplement from AREDS2.
Tx for CNV/wet AMD
Anti VegF
PED is associated with what type of drusen
Soft drusen.
PED, soft drusen, CNV all associated with wet AMD.
PED is associated with what type of AMD
Wet. Associated with soft drusen and CNV.
When it settles and re-attaches to Bruch’s, then it can cause geographic atrophy as well.
AKA can have wet CNV with geographic atrophy.
PED
Detachment of RPE from Bruchs
Associated with soft drusen, CNV, and wet AMD.
May cause geographic atrophy when it settles.
Atrophic/dry AMD
90% of cases. More common, less devastating.
Must have drusen
Oxidative stress–> inflammation triggered by drusen –> complement cascade.
Signs- Focal hypo or hyper pigmentation and possible RPE/PEd detachment.
Early tx: Stop smoking, diet changes, fish, less red meat, L & Z.
Intermediate/advanced tx: Supplement from AREDS2.
Neovasc/wet AMD
- Percent of time
- ASsociated with what
- Symptoms
- Associated findings
- Tx
10% Less common but more devastating.
ALWAYS associated with soft drusen (greater than 125 micrometers) and CNV (thickening of bruchs –> bleeding).
RPE/PED detachment is common.
Symptoms- Metamorphopsia, sudden painless VA loss secondary to CNV leaking.
Associated findings: Hemorrhagic PED, sub retinal heme with disci form scarring, vitreal heme.
Tx: Surgery, anti veg F, Steroid injection to decrease inflammatory response, Visudye that attaches to CNV then laser, Meritage (radiation + anti VegF)
Risk factors of Arm
Age**** most important 89% caucasian Smoking Obesity HTN High cholesterol
AREDS reduces risk of severe loss by
25-31%
Changes from ARED1 to 2
Removing beta carotene- Improved results. Decreased AMD progression.
Decreasing Zinc and adding zeaxanthin had no changes.
Fish oil and lutein decreased AMD progression.
Overview:
No effect: Decreasing ZINC and adding zeaxanthin.
Improved effect: removing beta carotene and adding lutein and omega 3
classification of AMD
early
Intermediate
Advanced
Early- small or medium drusen without pigmentary changes.
Intermediate- medium drusen with pigment changes OR any large drusen (greater than 125)
Advanced- Geographic atrophy or CNV.
Difference between RPE/PED detachment and ICSC
RPE/PED is always associated with drusen.
ICSC is NOT associated with drusen.
CNV
- What is it
- Tx
- 3 outcomes
Develops from choriocapillaris and grows into compromised Bruchs due to drusen.
Anti Veg F no matter the type of location. Numb, inject, BIO, rx antibiotics, 1 week eval, 1 month eval.
Regression, exudate(likely), disciform scar (ultimate)
CNV type 1
Sub RPE (between RPE and bruchs) occult Poorly defined, grey/green Hard to locate with FA Common in AMD
CNV type 2
Sub retinal (between sensory retina and RPE)
Classic
Well defined and contained, pink/yellow.
Common in young pts who develop CNV from myopia.
Locations of CNV
Subfoval- poor outcome
Juxtafoveal- 1 to 199 micrometers away from FAZ
Extrafoveal- greater than 200 micrometers from FAZ
RPE/PED detachment
- What is it
- What does it look like
- Outcomes
- Tx
Fluid between RPE and bruchs due to drusen damage.
Dome with distinct boarders. Opaque/yellow with ring around them.
Neo, geographic atrophy, RPE tear, resolution.
Monitor
Polypoidal Choroidal Vasculopathy
- What is it
- Prognosis
- Demographics
- Appearance
- Tx
Idiopathic hemorrhagic disorder of the macula.
Drusen causes CNV. Choroidal vessels form polyps (aneurysmal dilation).
Similar to AMD but better prognosis. Good outcome.
Demographics: AA, younger, females.
Must have drusen. Appears as red/orange branching vascular networks in macula.
Tx: PDT is more effective than PDT + lucentis or lucentis alone.
3 things that are always associated with drusen
RPE/PED detachment (usually soft drusen –> CNV/wet)
AMD (dry or wet)
polypoidal choroidal vasculopathy (Drusen –> CNV –> polyps –> tx with PDT –> Good outcome)
Geographic atrophy can be caused by which 2 things
AMD or previous RPE detachment
Choroidal rupture
Break in Bruchs secondary to blunt trauma.
Yellow concentric lines radiating away from ON
Hemorrhages when acute
RPE hyperplasia over time as boarder. Dark color.
Look for Neo- can develop CNV. Tx anti veg F.
Degenerative myopia
Progressive choroidal thinning.
Ref error greater than -6.00D or Axial length greater than 26 mm (avg is 23-24)
- Stationary. Associated with rapid body growth.
- Late. Environmental. Ex: optometry school.
- Pathological
3a: Developmental. Globe is elongating. RPE/choroid thins.
3b: Degenerative. Bruchs changes –> thinning –> CNV –> choroid atrophy
Angiod Streaks
Bruchs alteration with RPE and choriocap disruption.
Crack like formation in Bruchs that thickens and calcifies.
Bike spokes radiating away from ON.
Solar Maculopathy
Foveal burn/injury from solar radiation.
Presumed ocular histoplasmosis syndrome (POHS)
Multifocal, bilateral chorioretinitis caused by soil fungi/mold.
Triad:
Punched out chorioretinal scars.
Peripapillary chorioretinal atrophy around ON.
Acute or chronic macular exudative changes from CNV.
If macula involved –> anti veg F.
If not–> monitor. Good prognosis.
Ocular Toxoplasmosis
Most common cause of posterior uveitis in non immunocompromised pts.
Unilateral.
Focal chorioretinitis the involves full thickness inflammation that results in pigmented scar.
Due to toxoplasma gondii- parasite in cats.
Self limiting but destructive. Poor prognosis.
Symptoms- granulomatous uveitis, blurred VA, floaters, large KPs.
Intravitreal steroid injections or bactrim.
Ocular toxocariasis
Unilateral
Nematode infection from roundworms in dogs and cats.
Toxocaracanis or cati.
Blurred vision, pain, develop strab or leukocoria
Fibrocellular stalks extending to the disk and causing retinal wrinkling.
Increase in eosinophils.
Anti worm therapy if you see live worms. If not, steroid injection.
ON blood supply
Short posterior ciliary artery
Posterior ciliary artery
Pial artery
Superficial ON receives blood from small branch of CRA.
Blood supply of ON is compartmentalized. Can have sectors of ON that are pale. Similar to choroid.
NFL –> Pre laminar/glial –> laminar –> retro laminar
NFL: Ganglion cell axons that converge to form the ON. Non myelinated. No ILM at disc.
Pre laminar: Provides support and nutrients. Surrounded by tissue of Elschnig that separates the ON from choroid.
Laminar: Cribriform plate of collagen and glial tissue.
Retro Laminar: NF are myelinated and larger in diameter. Covered by pia, arachnoid, and dura.
ON drusen
Bilateral!!!!! progressive calcific bodies that 10% of the population has.
Covert or overt.
HF on FAF (Test of choice for dx)
Pt will have no symptoms. Excellent outcome.
Signs- small c/d, scalloped ON boarders, well defined vasculature, + SVP, Might have occlusion if pressing on arteries or veins. random VF defect.
- May cause anomalous elevation of the disc- pseudopapilledema but no true edema present
Educate pt about symptoms for artery/vein occlusion.
Papilledema
Medical emergency
Bilateral !!!!!!
ON edema due to increased intracranial pressure.
Due to tumor, ventral obstruction, IIH, thrombosis, stage 4 HTN retinopathy.
Signs: ON hyperemia, blurred margins, obscured and tortuous vessels, NFL opacities and thickened due to edema, loss of SVP, ON hemes, patent lines, Red cap problem.
Symptoms- HA, nausea, tingling, fatigue.
Send for MRI/MRV. If normal, suspect IIH. Check exit lumbar pressure. If greater than 250 mm h20, dx IIH.
Papilledema Stages
Bilateral!!!!!
Incipient/Early- Hyperemic ON, minimal NFL opacities.
Acute/well developed- NFL opacities and hemes.
Chronic- Decrease in hyperemia, hemes, white concretions with folds around ON, shunt vessels, decrease in VA.
Atrophic- pale, edematous. Significant VA decrease.
How to differentiate papilledema from ONH drusen on an OCT
Papilledema- Lazy V disc. RNFL very thick because edema and out pocketing of bruchs.
ONH drusen- Minimal or no c/d ratio. Normal RNFL thickness.
IIH
Increase in intracranial pressure for no known reason.
Bilateral !!!!!!!
Females, child bearing age, obese.
Symptoms- fluctuating VA
Signs- ONH edema, obscured BV and tortuosity, hyperemia, diminished sVP.
Tx: Diamox and vigorous weight loss program.
Define
Atrophy
Neuritis
Neuropathy
Atrophy- something likely already occurred. Primary and secondary causes. Unilateral or bilateral.
Neuritis- may lead to atrophy but usually good prognosis.
Neuropathy- will lead to atrophy. Poor prognosis.
Primary ONH atrophy
Pale, flat disc with distinct margins. No swelling. Appears normal! No swelling.
Unilateral if anterior to chiasm and bilateral if posterior to chiasm.
Could be due to retrobulbar optic neuritis, tumor, hereditary, alcohol toxicity, poor nutrition.
Atrophy could be:
temporal- involving papillomacular bundle or
band- temporal and nasal involved, superior and inferior sparred.
Secondary ONH atrophy
long standing ONH swelling with a raised disc and indistinct margins. Pale ON.
Can be unilateral or bilateral.
Due to chronic papilledema or ischemic optic neuropathy.
Two main categories of Optic Neuritis
Ophthalmic classification- retrobulbar, papillitis, and neuroretinitis.
Etiology classification- Demyelinating, parainfectious, infectious, autoimmune.
Retrobulbar optic neuritis / demyelinating optic neuritis
Signs: + APD Color vision defect decreased red cap decreased VA ON appears normal! Loss of insulating myelin layer- conduction disruption. UNILATERAL!!!!!!!!!!!!!!!!!! Associated with MS
Prognosis- good recovery based on ONTT.
Define papillitis and neuroretinitis
Papilitis- Inflammation of ON. Hyperemia, flame heme and vitritis.
Neuroretinitis- Papillitis + NFL inflammation. Could involve macular star. Ex: HTN grade 4
Causes of neuroretinitis
Idiopathic 25% of the time
Could be infectious or parainfectious
Ex: HTN grade 4
Excellent prognosis. Manage cause. Idiopathic will resolve on own!
Examples of infectious, parainfectious, and non infectious optic neuritis
Para- Viral or after immunization. Can be bilateral.
Infectious- Bacterial or zoster. Sinus, cat scratch, syphillis, lyme.
Non infectious- Sacroid
ONTT three groups and outcomes
- IV (1x per day for 3 days) followed by oral pred (11 days, 4 day taper).
- Placebo
- Oral pred
Group 1: increased recovery time in 3-4 weeks.
Group 2: recovered in 6-8 weeks.
Group 3: Recovered in 6-8 weeks but 2x probability for recurrence!!!!!! + side effects of hyperglycemia and GI
ONTT inclusion criteria
15 year follow up data
Acute unilateral optic neuritis
Determined risk of developing MS by tracking white matter lesions by MRI that predict MS
ONTT stands for
Optic neuritis/nerve treatment trial
NAION
Elderly. 55-65 years.
Partial or total occlusion of the SPCA that supplies the ON- will get altitudinal defect.
Due to: HTN, high cholesterol, DM, hypotension, sleep apnea, meds for arrhythmia or viagra.
Disc at risk- small, congested.
Symptoms- Sudden painless monocular VA loss with altitudinal defect.
Signs- ON edema with splinter hemorrhages (flame shape at ON). Hyperemia of disc.
No tx- poor prognosis. Will stabilize.
Refer to get blood work: ESR, CRP, and platelets.
Normal values
ESR
CRP
Platelets
ESR
males = age/2
females age + 10/2
CRP: less than 5mg/L
Platelets: 150,00-400,000
AION/Giant cell arteritis
Sudden, profound, PAINFUL vision loss.
Edematous, pale nerve with cupping changes after time.
Send to ER for IV methyl pred. Will not help eye involved, just stabilize it and prevent it from moving to the other eye.
Dx: Gold standard is biopsy of temporal artery. Could also check platelet, ESR, and CRP levels.
Diabetic Ischemic Optic Neuropathy
Sudden VA loss, painless
Mild optic nerve edema with hyperemia.
Surface telangiectasia (easily confused with NVD)
Could be unilateral or bilateral. Due to DM 1 or 2.
Monitor, may take months to go away. Good prognosis.
Myelinated nerve fibers
Unilateral Asymptomatic, maybe slight VF defect. Follows NFL. DD- CWS, Artery occlusion, coats disease. Good prognosis, monitor.
Crescents
Choroidal- dark
Scleral- white
RPE or choroid does not contact optic disc in the prelaminar/glial region.
Associated with ROP, high myopia, Sickle cell
Malinsertions
Tilted down temporally, tilted up nasally.
Common with greater than -6.00D refractive error.
Due to oblique insertion of the ON thru the scleral canal.
Temporal crescents common.
Tilted ON
Tilted Inferior or IN. Boarders may be hidden/less pigmented but still have pronounced choroidal vasculature radiating away from ON.
Raised superior or superior temporal.
associated with staphyloma
BILATERAL
VF defect
Difference between malinsertion and tilted ON
Malinsertion: Tilted temporally, raised nasally. Associated with high myopia, ROP, sickle cell. No VF defect.
Tilted: Tilted inferior, raised superior. Associated with staphyloma and likely to have VF defect. Bilateral.
Staphyloma
Posterior Sclera fails to fully develop in PP leading to elongation and tessellated fundus.
Disc appears round and normal.
VA variable, high myopia, VT WILL NOT BE HELPFUL.
Pigment changes around staphyloma.
Do B Scan.
ON coloboma
Large white excavation at disc- inferior. Abnormal blood vessels. Non rhegmatogenous retinal detachment. Variable VA and VF. ****Associated with cardiac issues.
REtinochoroidal coloboma/Bridge coloboma
Dual coloboma with normal tissue between.
Associated with systemic disease- cardiac, CNS, GI, musculoskeletal, chromosome abnormalities.
Rhegmatogenous detachment due to tear.
Morning Glory
Unilateral.
Enlarged, excavated disc.
White glial tissue central and black papillary ring around ON like a funnel.
Abnormal blood vessels.
Non rhegmatogenous detachment.
ON pit
Round localized depression in the disc.
VA= normal
VF= defects common
Good prognosis
Size difference between ONs
ON hypoplasia/Aplasia
Increased distance between temporal ON and fovea.
Surrounded by white ring.
Normal blood vessels.
strab, nystagmus. Poor outcome.
May have brains effect.
