BCSC Retina Flashcards
1
Q
What is the ligament of Wieger?
A
A condensation of collagenous fibers attached to the posterior lens capsule.
2
Q
How wide is the vitreous base?
A
2mm anterior and 3mm posterior to the ora serrate.
3
Q
Where does the vitreous attach?
A
The vitreous base, lens capsule, retinal vessels, optic nerve and macula.
4
Q
What is the vitreous made of?
A
Collagen fibrils separated by hydrated hyaluronan molecules.
5
Q
What is the area of Martegiani?
A
The attachment of the vitreous to the disc.
6
Q
Where is the macula clinically and how large is it?
A
The area between the disc and temporal vascular arcades, it is 5.5mm.
7
Q
What defines the macula histologically?
A
The area with 2 or more layers of ganglion cells.
8
Q
Why is the macula yellow?
A
Due to the accumulation of carotenoids, especially zeaxanthin and lutein.
9
Q
What defines the fovea?
A
1.5mm area where the only photoreceptors are cones.
10
Q
What defines the foveola?
A
0.35mm in diameter, where the INL and ganglion cell layer are displaced laterally.
11
Q
What is the umbo?
A
150 micrometers in diameter, the central depression of the foveola, where cones are elongated.
12
Q
What is the parafovea?
A
0.5mm ring around the fovea where ganglion cell layer, INL and OPL are thickest.
13
Q
What is the perifovea?
A
1.5mm wide area around the parafovea.
14
Q
What is the ora serrata?
A
The border between the retina and the pars plana.
15
Q
Where do most retinal tears occur?
A
At the posterior border of the vitreous base, between the ora serrata and the equator.
16
Q
What is a dentate process?
A
Jetties of retinal tissue extending anteriorly towards the pars plana.
17
Q
What are ora bays?
A
Posterior extensions of the pars plana towards the retinal side.
18
Q
What are meridonal folds?
A
Radially oriented thickening of retinal tissue extending to the pars plana,
19
Q
What are the layers of the retina?
A
- Inner limiting membrane 2. nerve fiber layer 3. ganglion cell layer 4. inner plexiform layer 5. inner nuclear layer 6. middle limiting membrane 7. outer plexiform layer 8. outer nuclear layer 9. external limiting membrane 10. rod and cone inner and outer segments
20
Q
Where are rods the most dense?
A
20 degrees from fixation.
21
Q
How many cones does one midget bipolar cell synapse with?
A
One
22
Q
Do bipolar cells synapse with one or many rods?
A
Many
23
Q
What do amacrine cells do?
A
Help in signal processing by responding to changes in retinal stimuli like change in light intensity.
24
Q
What is the ILM (internal limiting membrane)?
A
The foot pads of Muller cells, which attaches to the posterior cortical gel of the vitreous.
25
What is the XLM (external limiting membrane)?
Zonal attachments between photoreceptors and Muller cells, can be seen on OCT but not true membrane.
26
What supplies the inner retina?
The central retinal artery.
27
What supplies the outer retina?
The choriocapillaris.
28
What is the RPE?
Pigmented cells from the outer layer of the optic cup, continuous with pigmented epithelium of the ciliary body and iris.
29
Where is the apex of the RPE cell?
Facing photoreceptors, enveloping the outer segments.
30
What kind of junctions between RPE cells form the blood ocular barrier?
Zonulae occludentes near the apices.
31
What is the RPE function?
1. absorb light 2. phagocytose rod/cone outer segments 3. fatty acid metabolism 4. form outer blood ocular barrier 5. maintain subretinal space 6. heal and form scar tissue.
32
Where is Bruch membrane?
Attached to the basal portion of the RPE.
33
What is the Haller layer of choroidal vessels?
The outer layer of large caliber vessels.
34
What is the Sattler layer of choroidal vessels?
The smaller diameter vessels and precapillary arterioles of the choroid.
35
What are the vortex veins?
Collect blood from the choroid.
36
Where are the vortex veins located and where do they drain?
Leave the eye at the equator, they drain into the superior and inferior ophthalmic veins.
37
Where is the sclera thinnest?
At the limbus, equator and peripapillary area.
38
What is the Hruby lens?
An external planoconcave lens with high negative optical power, gives upright image.
39
How is fluorescein eliminated?
Through the liver and kidneys
40
At what wavelength is fluorescein excited and at what wavelength does it fluoresce?
Excited at 465-490 nm (blue) and fluoresces at 520-530 nm (green).
41
What is autofluorescence?
Fluorescence prior to injection of fluorescein dye, such as optic disc drusen.
42
What are patterns of hypofluorescence?
1. vascular filling defect 2. Blocked fluorescence
43
What are patterns of hyperfluorescence?
1. Leakage 2. Staining 3. Pooling 4. Window defect 5. Autofluorescence
44
What are adverse effects of fluorescein angiography?
nausea/vomiting (10%), extravasation with tissue necrosis, urticarial reaction (1%), anaphylactic reaction (<1/100,000)
45
What does indocyanine green angiography (ICG) image?
Choroidal vessels.
46
What are indications for indocyanine green angiography (ICG) ?
CNV, PED, polypoidal choroidal vasculopathy, RAP, central serous choroiretinopathy, intraocular tumors, chronic inflammatory conditions
47
What allergy do you need to ask about prior to ICG angiography?
Shellfish (ICG has iodine).
48
What is basis for OCT imaging?
Optical reflectivity.
49
What is the axial resolution of SD-OCT?
7 micrometers.
50
What is the source of autofluorescence in the macula?
Lipofuscin, which has a pigment A2E.
51
What are the five different ERG responses?
1. Rod response (dark adapted) 2. Maximal combined response (dark adapted) 3. oscillatory potentials (dark adapted) 4. single-flash "cone response" (light adapted) 5. 30-Hz flicker responses (light adapted)
52
What is the a wave on ERG?
Photoreceptor response, measured from baseline to a-wave trough.
53
What is the b wave on ERG?
Positive waveform measured from the a-wave trough to the b-wave peak which is generated by Muller/bipolar cells.
54
What is the ERG oscillatory potentials response?
Thought to be the result of feedback interactions between integrative cells of inner retina, reduced in retinal ischemia and some types of congenital stationary night blindness.
55
What does the ERG 30-Hz flicker response isolate?
It isolates cones, since rods can respond only up till 20-Hz.
56
Does full field ERG distinguish between macula and peripheral lesions?
No because 90% of cones are outside of the macula, so you can't distinguish a macula lesion based on cone response.
57
What is the c-wave on ERG?
A late positive response occurring 2-4 min after stimulus generated by RPE.
58
What is multifocal ERG?
It tests cone generated responses that subtend 25 degrees from fixation, test for macular dysfunction.
59
What is bright-flash ERG?
Performed with a flash-stimulus brighter than usual to test for retinal function in eyes with opaque media.
60
How does the ERG change with retinal ischemia?
Abnormalities of b-wave and oscillatory potentials such as inversion of b-wave or delay in implicit time.
61
What does the EOG (electro-oculogram) measure?
The voltage differential generated across the RPE, which is measured as the voltage change as a patient looks from left to right with electrodes placed at both canthi.
62
What is a normal and abnormal Arden ratio?
Normal: 1.85
Abnormal: 1.30
63
What is visually evoked cortical potential (VEP)?
Occipital cortex response to stimulation of the retina, because the macula is highly represented in the visual cortex is a mainly test of macular function, can indicate abnormality anywhere along visual pathway from retina to cortex.
64
What can visually evoked cortical potentials be used to test?
Confirm diagnosis of optic neuropathy, estimate VA in infants, detect malingering
65
What are risk factors for AMD?
Family history, cigarette smoking, hyperopia, light iris color, hypertension, hypercholesterolemia, female sex and cardiovascular disease
66
What are druse?
Small yellow lesion at level of RPE, histologically are thickening of inner aspect of Bruch membrane, made of basal laminar deposits and basal linear deposits.
67
What are basal laminar deposits?
Granular, lipid-rich material and widely spaced collagen fibers between plasma membrane and BM of RPE cells.
68
What are basal linear deposits?
Phospholipid vesicles and electron-dense granules within inner collagenous zone of Bruch membrane.
69
What are the sizes of small, medium and large drusen?
Small: <64 micrometers diameter
Medium: 64-124 micrometers diameter
Large: 125 micrometers diameter
70
What are patterns of RPE abnormality in nonneovascular AMD?
Focal hyperpigmentation, nongeographic atrophy and geographic atrophy.
71
What is basal laminar drusen or cuticular drusen?
A clinical syndrome occurring in patients in their 30-40s consisting of innumerable, homogenous round drusen more apparent on angiography.
72
What was the orginal AREDS formulation?
```
500mg vit C
400IU vit E
15mg beta carotene
80mg zinc oxide
2mg cupric oxide
```
73
What were the recommendations of AREDS?
Patients with high risk of advanced AMD and vision loss(extensive intermediate drusen or 1 large druse or non-central geographic atrophy or advance AMD in 1 eye) should be recommended supplementation.
74
Which ingredient of AREDS increased risk of lung cancer in current smokers?
Beta carotene.
75
What is choroidal neovascularization?
Ingrowth of new vessels from the choriocapillaris into the sub-pigment epithelial space through a defect in Bruch membrane.
76
What is classic CNV?
An area of bright, uniform hyperfluorescence identified in early phase of FA that intensifies and demonstrates leakage in late phase.
77
What is occult CNV?
1. Fibrovascular PED
| 2. Late leakage from undermined source on FA
78
What is polypoidal choroidal vasculopathy?
Variant of CNV characterized by multiple serosanguineous RPE detachments, peripapillary multifocal, orange, nodular lesions.
79
What is photodynamic therapy (PDT)?
Administration of photosensitizing drug followed by application of specific wavelength of light to generate reactive oxygen species to cause capillary endothelial cell damage.
80
What did the PDT in AMD studies show?
A benefit in treating predominantly classic lesions (TAP trial) and minimally classic lesions (VIM trial). VIP showed greatest benefit in eyes with occult CNV without classic component in small lesions.
81
What is vascular endothelial growth factor (VEGF)?
A homodimeric glycoprotein that is a heparin binding growth factor for vascular endothelial cells which can induce angiogenesis.
82
What is pegaptanib?
RNA oligonucleotide that binds VEGF165 near the heparin binding domain, does not target all active VEGF-A isoforms.
83
What did the VISION trial show?
Pegaptanib patients lost less vision than sham, and had small gains in vision.
84
What is ranibizumab?
Lucentis, humanized antibody fragment that binds and inhibits all active VEGF-A.
85
What did MARINA show?
Trial of ranibizumab vs sham in minimally classic or occult CNV treated monthly for 2 years, showed vision improvement/stabilization and greater gain of 15 letters compared to sham.
86
What did ANCHOR show?
Trial of ranibizumab and sham PDT vs sham injection and PDT in classic CNV with monthly injections for 2 years, found vision improved or maintained as compared to PDT.
87
What did PIER test?
Ranibizumab monthly for 3 months, then quarterly, demonstrated similar improvement in vision to monthly regimen in initial stage but then decline during quarterly dosing.
88
What are current clinical treatment regimens for wet AMD?
Treat and observe: treat till macula free of exudation then only treat for signs of recurrence
Treat and extend: continue treatment monthly till macula is dry, then extend intervals between treatment (will continue treatment even if appears dry).
89
What did PrONTO show?
Ranibizumab for monthly for 1st three months, then retreat during 1st year for thickening or loss of vision; 2nd year retreat for qualitative increase in fluid, found similar results to monthly regimens.
90
What is VEGF trap?
Soluble protein which combines ligand binding elements of VEGFR1 and VEGR2 extracellular domains.
91
What did VIEW1 and 2 show?
VEGF trap in monthly or bimonthly regimen compared to ranibizumab. Patients getting 2 mg monthly gained more letters than 0.5mg ranibizumab.
92
What is bevacizumab?
Avastin, Monoclonal antibody agains VEG with to antigen binding domains. Shorter half life than ranibizumab.
93
What did CATT show?
RCT comparing ranibizumab and bevacizumab for CNV in AMD, 1 year results showed no difference between two drugs in monthly or as needed delivery schedules.
94
What are the 4 signs of ocular histoplasmosis syndrome?
1. Punched out chorioretinal lesions
2. juxapapillary atrophic pigmentary changes
3. no vitritis
4. CNV
95
Where is ocular histoplamosis endemic?
Ohio and Mississippi River valleys
96
What are angioid streaks?
Dark red to brown bands radiating from optic nerve head representing breaks in Bruch membrane.
97
What conditions are associated with angioid streaks?
pseudoxanthoma elasticum, Paget disease, beta thalassemia, sickle cell anemia, Ehlers Danlos syndrome.
98
What are consequences of angioid streaks?
CNV, subretinal hemorrhage not associated with CNV, choriodal rupture from minor blunt injury.
99
What are signs of pathologic myopia?
Optic disc tilting, peripapillary chorioretinal atrophy, lacquer cracks, subretinal hemorrhages, Forster-Fuchs spots, posterior staphyloma, gyrate atrophy of RPE, cystoid, pavingstone and lattice degeneration, thinning or hole of retina, thinning of sclera, CNV
100
What is the chance of CNV in patients with high myopia?
CNV may develop in 5-10% of eyes with axial length >26.5.
101
What is high myopia?
Axial length >26.5 and spherical equivalent of -6.00 or greater
102
What is pathologic myopia?
Axial length >32.5 and spherical equivalent of -8.00 or greater.
103
What are broad categories of conditions that lead to CVN?
Degenerative (AMD, angioid streaks), Heredodegnerative (Vitelliform mac dystrophy, drusen), Inflammatory (histoplamosis, MCP, toxoplasmosis, VKH, Behcet), tumor, trauma (photocoagulation), idiopathic
104
What is NPDR?
Intraretinal vascular changes without the presence of extraretinal fibrovascular tissue.
105
What is PDR?
Ischemia-induced neovascularization.
106
What did WESDR (Wisconsin Epidemiologic Study of Diabetic Retinopathy) show?
The prevalence of diabetic retinopathy is associated with duration of diabetes in type I and type II DM, mainly in white population.
107
What did NHNES III (National Health and Nutritional Examination Survey III) who/
It showed the frequency of diabetic retinopathy among blacks and Mexican Americans was higher than non-Hispanic whites.
108
What did DCCT (Diabetic Control and Complications Trial) show?
Intensive control of diabetes reduced retinopathy as well as neuropathy and nephropathy in type I DM.
109
What did UKPDS (United Kingdom Prospective Diabetes Study) show?
Intensive control of blood sugar and blood pressure reduced slowed progression of retinopathy in type II DM.
110
Is pregnancy associated with worsening diabetic retinopathy?
Yes, pregnant women need more frequent examination.
111
What are causes of vision loss in diabetic retinopathy?
1. Macular edema
2. Capillary occlusion (macular ischemia, diabetic papillopathy
3. Sequelae from ischemia induced neovascularization (vitreous hemorrhage, tractional RD, neovascular glaucoma).
112
What is the definition of clinically significant macular edema (CSME)?
1. retinal thickening at or within 500 micrometers of the center of the macula
2. hard exudates at or within 500 micrometers of center with associated thickening of retina
3. zone of thickening 1 DD if within 1 DD of center of macula.
113
What study defined CSME and what was recommended treatment?
ETDRS defined CSME and recommended focal laser photocoagulation if met criteria, treated patients had less risk of moderate visual loss and increased chance of visual improvement.
114
What are different options for treatment of diabetic macular edema?
Anti-VEGF drugs, corticosteroids (sub-Tenon's, intravitreal, implant), focal or grid macular laser.
115
What is focal laser treatment?
Green or yellow wavelength spots applied to leaking microaneurysms, 50-100 micrometer spot size, 0.1s duration.
116
What is grid laser treatment?
Green or yellow wavelength laser applied to areas of diffuse leakage more than 500 micrometers from disc or center of macula. 50-100 micrometer spot size, 0.1s duration, spots 1 burn width apart.
117
What are risks of focal laser?
paracentral scotoma, transient increase in edema, CNV, subretinal fibrosis, foveolar burns.
118
What defines severe NDPR?
One of any of the following
4 quadrants of diffuse intraretinal hemorrhages and microaneurysms
2 quadrants of venous beading
1 quadrant of IRMA.
119
Who should be considered for early treatment with PRP?
Patients with severe NPDR, they are at 15% risk of progression to high risk PDR within 1 year.
120
What is high risk PDR?
Any of the following:
- NVD with vitreous hemorrhage
- 1/3-1/4 disc area of NVD
- 1/2 disc area of NVE with vitreous hemorrhage
121
What study defined high risk PDR and what did it show?
Diabetic retinopathy study, showed eyes treated with PRP had less risk of severe vision loss compared to no treatment, high risk PDR had greatest benefit.
122
What are risks associated with anti-VEGF in PDR?
Precipitation of retinal detachment
123
What are the goals of PRP?
1. destroy ischemic retina, reducing production of VEGF
| 2. increase O2 tension by decreasing consumption by retina and increasing diffusion of oxygen from choroid.
124
What is full PRP as defined by DRS?
1200 or more burns, 500micrometer spot size, 1/2 burn width apart, argon green or blue/green laser.
125
What are risks of PRP?
increased vitreoretinal traction, tractional RD, vitreous hemorrhage, decreased night vision/color/contrast, loss of accommodation, photopsias, worsening macular edema.
126
What areas should be spared if possible when treating with PRP?
The horizontal meridians to preserve the long ciliary nerves/vessels and nasal and superior retina to preserve inferior and temporal peripheral vision.
127
What did the DRVS (Diabetic Retinopathy Vitrectomy Study) show?
RCT, showing patients with type I DM and severe vitreous hemorrhage or patients with severe PDR benefitted from early (1-6 mo after onset) vitrectomy compared to late (1 year).
128
When should DM type I patients first be screened?
3-5 years after diagnosis.
129
When should DM type II patients first be screened?
At diagnosis.
130
When should pregnant patients with DM be screened?
First trimester or prior to conception.
131
How often should patients with CSME be followed?
Every 2-4 months.
132
How often should patients with NPDR be followed?`
Every 2-4 months.
133
How often should patients with PDR be followed?
Every 2-4 months.
134
What are Elschnig spots?
Hyperpigmented lobule-sized patches surrounded by hypopigmentation which are signs of hypertensive choroidopathy.
135
What are Siegrist streaks?
Linear hyperpigmentation following meridional course that are signs of acute, uncontrolled hypertension.
136
What are signs of hypertensive optic neuropathy?
Linear peripapillary flame-shaped hemorrhages, blurring of disc margin, florid disc edema and macular exudates
137
Which type of sickle cell disease results is more likely to result in ocular complications?
Sickle cell hemoglobin C (SC) and sickle cell thalassemia.
138
What are signs of nonproliferative sickle cell retinopathy?
Salmon patch hemorrhages, refractile deposits, black "sunburst" lesions.
139
What are salmon patch hemorrhages?
Intraretinal hemorrhage occurring after peripheral retinal occlusion.
140
What is a black sunburst lesions?
Areas of RPE hypertrophy, hyperplasia and pigment migration, due to subretinal extension of hemorrhage.
141
What are the five stages of proliferative sickle cell retinopathy?
1. Pripheral arteriolar occlusion
2. Peripheral arteriovenular anastamoses
3. Sea fan neovascularization
4. Vitreous hemorrhage
5. tractional retinal detachment
142
What are signs of sickle cell disease found outside of the retina?
Segmentation of blood in conjunctival blood vessels, comma shaped thrombi in conjunctiva, dark red spots on disc from intravascular occlusions, angioid streaks.
143
What are patients with sickle cell retinopathy at risk for with treated with PRP?
Higher riskof retinal tears and rhegmatogenous retinal detachment.
144
What are steps to avoid anterior ischemia in surgical management of RD in sickle cell patients?
Avoid epinephrine, avoid buckle, do not remove extraocular muscles, judicious laser use, ensure pt hydration, use supplemental nasal oxygen.
145
Where do BRVOs usually occur?
At an arteriovenous crossing.
146
What should you consider if BRVO occurs away from an arteriovenous crossing?
Underlying retinochoroiditis or retinal vasculitis.
147
Which quadrant is most commonly affected in BRVO?
Superotemporal (63% of time).`
148
What is mean age for occurrence of a BRVO?
Seventh decade.
149
What are risk factors for BRVO?
1. history of HTN
2. cardiovascular disease
3. increase BMI at age 20
4. history of glaucoma
150
What is the prognosis for BRVO?
50-60% of eyes maintain vision 20/40 or better after 1 year
151
In what percent of eyes with BRVO does NVI develop?
1%.
152
What did the Branch Vein Occlusion Study (BVOS) show regarding macular edema?
Argon laser improved treatment in patients with macular edema with vison 20/40-20/200 and intact foveal vasculature.
153
What did the Branch Vein Occlusion Study (BVOS) show regarding PRP?
PRP reduced risk of neovascularization in patients with at least 5 DD of ischemia, and reduced risk of hemorrhage by 1/2 in patients with neovascularization but recommended observing patients with ischemia till they develop neovascularization.
154
What did SCORE (Standard Care Versus Corticosteroid for Retinal Vein Occlusion) show for BRVO?
RCT of laser vs 1mg vs 4 mg triamcinelone showed no difference in visual acuity for patients and increased risk of cataract or high IOP with steroid.
155
What did Branch Retinal Vein Occlusion study (BRAVO) show?
RCT of sham vs 0.3mg vs 0.5mg ranibizumab, found improvement in visual acuity with ranibizumab.
156
What is the pathophysiologic cause of BRVO?
Thickened arterial wall at arteriovenous crossing leads to narrowed vein and turbulent flow leading to endothelial cell damage and thrombus.
157
What are the two types of CRVO?
Nonischemic (good visual acuity, minimal nonperfusion) and ischemic (poor visual acuity, extensive nonperfusion, risk of NVI).
158
What is the ERG pattern associated with CRVO?
Decreased bright flash, dark adapted b-wave:a-wave amplitude ratio.
159
What percent of eyes with ischemic CRVO achieve better than 20/400 vision?
10%
160
What percent of ischemic CRVOs develop NVI?
Up to 60%.
161
When do ischemic CRVOs develop NVI?
Mean of 3-5 months after onset of symptoms.
162
What are risk factors for CRVO?
Hypertension, diabetes mellitus, glaucoma
163
What are conditions/drugs associated with CRVO?
Oral contraceptives, diuretics, polycythemia vera, hypercoagulable conditions (Protein S and C deficiency, hyperhomocysteinemia), vasculitis (sarcoid, lupus), dysproteinemias.
164
What are the complications of CRVO?
Vitreous hemorrhage, anterior segment neovascularization and neovascular glaucoma.
165
What did SCORE (Standard Care Versus Corticosteroid for Retinal Vein Occlusion) show for CRVO?
Patients treated with 1mg or 4mg of triamcinolone demonstrated greater improvement in visual acuity with macular edema than observation.
166
What did the Central Retinal Vein Occlusion (CRUISE) trial show?
Patients receiving 0.5 or 0.3mg of ranibizuman demonstrated greater improvement in visual acuity with macular edema than observation.
167
What did CVOS show?
1. No benefit in visual acuity for patients with macular edema treated with grid laser.
2. No statistically significant decrease in NVI with prophylactic PRP, recommend waiting till 2 clock hours of NVA on gonio prior to PRP.
168
What are symptoms of ocular ischemic syndrome?
Vision loss over weeks to months, aching pain in orbital area, prolonged recovery after exposure to light.
169
What are signs of ocular ischemic syndrome?
Iris neovascularization (2/3 of eyes), AC cellular response (1/5 eyes), low or high IOP
170
What is the definitive treatment for ocular ischemic syndrome?
Endarterectomy.
171
What is the risk with carotid reperfusion in ocular ischemic syndrome?
Reperfusion in eyes with decreased ciliary body perfusion causing decreased aqueous production can lead to rebound high IOPs once the ciliary body is reperfused.
172
What is zone I in ROP?
The area included in a circle centered on the optic nerve whose radius is 2x the distance from the optic nerve to the foveola.
173
What is zone II in ROP?
The area included in a circle centered on the optic nerve with radius from the optic disc to the nasal ora serrata.
174
What is zone III in ROP?
The remainder of the fundus outside of zone I and II.
175
What is stage 1 ROP?
Presence of a demarcation line between vascular and avascular retina.
176
What is stage 2 ROP?
Presence of a ridge demarcating vascular and avascular retina.
177
What is stage 3 ROP?
A ridge with extraretinal fibrovascular proliferation.
178
What is stage 4 ROP?
Partial retinal detachment, A extrafoveal, B including fovea
179
What is stage 5 ROP?
Total retinal detachment with funnel configuration.
180
What is plus disease in ROP?
The presence of retinal vascular dilation and tortuousity in the posterior pole.
181
What is threshold disease?
5 contiguous clock hours of extraretinal NV or 8 cumulative clock hours with plus disease in zone I or II.
182
What is prethreshold disease type 1 ROP?
1. Zone I any stage with plus disease
2. Zone I stage 3 without plus
3. Zone II stage 2 or 3 disease with plus
183
What is prethreshold disease type 2 ROP?
1. Zone I stage 1 or 2 without plus disease
| 2. Zone II stage 3 without plus disease
184
When is retinal vascularization completed?
At 36 weeks of gestation nasally and 40 weeks of gestation temporally.
185
What are conditions associated with eyes with regressed ROP?
myopia with astigmatism, strabismus, amblyopia, cataract, glaucoma, macular pigment epitheliopathy, tractional RD, anomalous foveal anatomy, angle closure glaucoma and pupillary block glaucoma.
186
Which babies need to be screened for ROP?
Babies born 30 weeks or less of gestation or birth weight less than 1500g, or if unstable clinical course and believed to be at risk.
187
When should the first examination for ROP be performed?
At 4-6 weeks of postnatal age or at 31-33 weeks of postmenstrual age, whichever is later
188
When can screening be discontinued for ROP?
1. Full retinal vascularization
2. Zone III vascularization attained without previous Zone I or II ROP.
3. Postmenstrual age of 45 weeks and no prethreshold disease
4. Regressing ROP
189
How closely should prethreshold type II ROP (zone I stage 1 or 2 without plus or zone 2 stage 2 or 3) be followed?
1 week or less follow-up
190
What did Early Treatment of ROP (ETROP) show?
Compared early ablation of retina to cryotherapy for threshold disease, found that patients with high risk prethreshold disease (similar to type 1 ROP classification) benefited from treatment.
191
Which patients with ROP should be considered for ablative laser treatment?
Threshold disease or prethreshold type I.
192
What are risk factors for ROP?
Low birth weight, short gestational age, sepsis, slow weight gain.
193
What did Bevacizumab Eliminates the Angiogenic Threat of Retinopathy of Prematurity (BEAT-ROP) show?
RCT for infants with zone I or II stage 3 disease with plus, bevacizumab versus laser, found significant treatment effect for zone I ROP for anti-VEGF, zone II no difference between laser and anti-VEGF.
194
What is central serous chorioretinopathy?
An idiopathic condition characterized by a well-circumscribed serous detachment of the sensory retina.
195
What is the demographic of patients affected by central serous chorioretinopathy?
25-55 year old males, common in whites, Asians and Hispanics, uncommon in African Americans.
196
What kind of refractive change is associated with central serous chorioretinopathy?
Hyperopic.
197
What conditions are associated with central serous chorioretinopathy?
Exogenous steroid use, endogenous hypercortisolism, organ transplantation, systemic lupus erythematosus, HTN, sleep apnea, GERD, pregnancy.
198
What are the fluorescein angiography patterns noted in central serous chorioretinopathy?
1. Expansile dot pattern
2. Smokestack pattern
3. diffuse pattern
199
What is seen in OCT of central serous chorioretinopathy?
Subretinal fluid and pigment epithelial detachments, patients may have increased choroidal thickness.
200
What is the prognosis of central serous chorioretinopathy?
Generally good, 80-90% of patients experience spontaneous resorption of fluid in 3-4 mo.
201
What are treatment options for central serous chorioretinopathy?
Observation, laser photocoagulation at site of leakage, PDT.
202
What are causes of choroidal vascular abnormalities?
Venous outflow issues (CC fistula), hypertension (malignant, eclampsia, cocaine), inflammation (GCA, Wegener, lupus), thromboembolic disease (TTP, DIC), iatrogenic.
203
What are treatment options for choroidal hemangioma?
Laser photocoagulation, PDT, cryopexy, radiation.
204
What are complications of choroidal hemangiomas?
Cystic retinal edema, neurosensory detachment, epichoroidal membranes.
205
What causes uveal effusion?
Nanophthalmos, scleritis, idiopathic uveal effusion syndrome.
206
What are the findings of bilateral diffuse uveal melanocytic proliferation (BDUMP)?
Diffuse thickening of the choroid, red or brown choroidal discoloration, serous RD and cataracts.
207
What cancers are associated with bilateral diffuse uveal melanocytic proliferation (BDUMP)?
Most common are ovarian, uterus and lung, also found with colon, pancreas, gallbladder and esophagus.
208
What are the demographics of patients affected with APMPPE?
Young adults in 2nd to 3rd decade of life, no sex predilection.
209
What is APMPPE?
Acute posterior multifocal placoid pigment epitheliopathy, a bilateral inflammatory disease presenting with yellow placoid lesions at RPE level in macula.
210
What does fluorescein angiograpy show in APMPPE?
Early blockage, late staining.
211
What is the visual prognosis for APMPPE?
Good, no specific treatment indicated.
212
What are other names for serpiginous choroidopathy?
Geographic choroiditis or helicoid peripapillary choroidopathy.
213
What is serpiginous choroidopathy?
A recurrent bilateral inflammatory disease with gray-yellow lesions spreading centrifugally from posterior pole.
214
What is the prognosis for serpiginous choroidopathy?
Poor.
215
What is the fluorescein angiography pattern for serpiginous choroidopathy?
Early hypofluorescence of lesions, late hyperfluorescence, may appear similar to APMPPE.
216
What is the treatment for serpiginous choroidopathy?
Immunosuppresion.
217
What is MEWDS?
Multiple evanescent white dot syndrome, characterized by multiple white dots in deep retina and RPE.
218
Which white dot syndromes are associated with viral prodromes?
MEWDS and APMPPE.
219
Is MEWDS bilateral or unilateral?
Tends to be unilateral (80%).
220
What is the epidemiology of patients with MEWDS?
Myopic individuals, 2nd to 5th decade of life, F>M.
221
What can be found on pupillary exam of patients with MEWDS?
Relative afferent pupillary defect.
222
What is the fluorescein angiography pattern of MEWDS?
Punctate hyperfluorescent spots in wreathlike cluster.
223
What can be seen on visual field testing of MEWDS?
Enlarged blind spot.
224
What is the prognosis for MEWDS?
Good, no treatment required.
225
What is birdshot choroidopathy?
Bilateral inflammatory disease with vitritis, ovoid birdshot lesions found most in nasal retina, can have disc edema and vascular sheathing.
226
Who is affected by birdshot choroidopathy?
F>M, 4th to 6th decade.
227
What defines birdshot choroidopathy?
All patients have vitritis.
228
What is the HLA associated with birdshot choroidopathy?
HLA-A29 (90% of patients are positive).
229
What is the prognosis of birdshot choroidopathy?
Poor, can have vision loss from CME, optic atrophy or rarely CNV, prone to recurrence.
230
What is treatment for birdshot choroidopathy?
Immunosuppresion; cyclosporine, mycophenolate mofetil, methotrexate, intravitreal steroid.
231
What is MCP?
Multifocal choroiditis and panuveitis syndrome, bilateral disease presenting with vitritis, multiple yellow choroidal lesions which evolved to "punched out" chorioretinal scars.
232
Who does MCP affect?
F>M, from 2nd to 6th decade.
233
What is on the differential for MCP?
Ocular histoplasmosis syndrome (but OHS has no vitritis), must rule out infection like TB and syphilis.
234
What is the prognosis for MCP?
Poor, can be complicated by CNV, ERM formation, CME.
235
What is MCP treated with?
Immunosuppresion after ruling out infectious etiology.
236
What is PIC?
Punctate inner choroidopathy, bilateral inflammatory disease with small round yellow-white lesions, can have optic disc edema, later can develop chorioretinal scars.
237
Who is affected by PIC?
F
238
What is the prognosis for PIC?
Good.
239
What is AZOOR?
Inflammatory disease affecting outer retina, can see mild vitritis or normal fundus, with later depigmentation of RPE.
240
Who is affected by AZOOR?
Young women.
241
What is the prognosis for AZOOR?
Can develop persistent visual field defect, usually good vision in 1 eye.
242
What is treatment for AZOOR?
No treatment proven to have benefit.
243
What is acute macular neuroretinopathy?
Bilateral condition with acute vision loss after viral prodrome, young patients, see wedge shaped red-brown macular lesions, good prognosis.
244
What is acute idiopathic maculopathy?
Unilateral vision loss in young patients after flu, see exudative macular detachment, vit cell, retinal hemorrhage, papillitis, good prognosis.
245
What is ARPE (Acute Retinal Pigment Epitheliitis)?
Krill disease, rapid vision loss in young adults, macula with dark round spots surrounded by depigmented haloes, good prognosis.
246
What is solitary idiopathic choroiditis?
Localized inflammatory condition of choroid, 20-50 year old pts, mild vision loss, one distinct yellow-white choroid lesion.
247
What are the findings in Behcet disease?
Recurrent aphthous oral ulcers, genital ulcers and acute iritis with hypopion.
248
Who does Behcet disease affect?
M>F, common in Japan, Mediterranean, Middle East.
249
What is the HLA association with Behcet disease?
HLA-B5101.
250
What are ocular findings in Behcet disease?
Anterior segment: severe uveitis with hypopion.
Posterior segment: occlusive retinal vasculitis, macular edema, intraretinal heme, retinal necrosis, vitritis, optic neuropathy.
251
What are treatments for Behcet disease?
PRP for neovascularization from ischemia, corticosteroids and immunomodulators.
252
What are signs of retinal disease in systemic lupus erythematosus?
Cotton wool spots, intraretinal hemorrhages, serous elevations of retina and RPE, vasoocclusive disease.
253
What is the differential of intermediate uveitis?
MS, syphilis, Lyme disease, sarcoidosis, pars planitis.
254
Who does pars planitis affect?
Young adult and children, 5-15 years and 25-35 years old.
255
What can you see in pars planitis?
Snowbanking, snowballs, vitritis with spillover to AC, phlebitis, optic nerve swelling, NV along snowbanks.
256
What is the main cause of vision loss in pars planitis?
CME 1/3 of patients.
257
What are complications of pars planitis?
CME, PSC, epiretinal membrane, band keratopathy.
258
What percent of patients with sarcoid have ocular manifestations?
50%
259
What is Vogt-Koyanagi-Harada (VKH) syndrome?
A systemic inflammatory disorder with bilateral granulomatous panuveitis with dermatologic and neurologic manifestations.
260
What HLA is associated with Vogt-Koyanagi-Harada (VKH) syndrome?
HLA-DRB1*0405 in Japanese patients.
261
What are associated systemic symptoms with Vogt-Koyanagi-Harada (VKH) syndrome?
Vitiligo, poliosis, alopecia and meningeal signs.
262
What is Harada disease?
Posterior pole findings of VKH without systemic disease.
263
What are the 4 stages of VKH?
1. Prodrome (flulike illness)
2. Uveitic phase (bilateral granulomatous uveitis)
3. Convalescence phase (depigmentation)
4. Final phase (chronic inflammation)
264
What is the Sugiura sign?
Perilimbal vitiligo seen in VKH.
265
What is seen in the convalescent phase of VKH?
Sunset glow fundus, Dalen-Fuchs nodules, perlimbal vitiligo.
266
What does VHK need to be distinguished from?
Sympathetic ophthalmia.
267
What are the findings in sympathetic ophthalmia?
Bilateral granulomatous panuveitis with optic disc swelling and choroidal thickening.
268
When are patients with AIDS at risk for CMV retinitis?
CD4 count <50
269
What does CMV retinitis look like?
Opacification of the retina with areas of hemorrhage, exudate and necrosis.
270
What does CMV retinitis look like early on?
Cotton wool spots from HIV retinopathy.
271
What is treatment for CMV retinitis?
Intravenous ganciclovir or foscarnet.
272
What is the major complication of CMV retinitis?
Retinal detachment, often requiring vitrectomy.
273
What is acute retinal necrosis (ARN)?
Retinal necrosis secondary to HSV or HZV in healthy patient, presenting with inflammation and areas of retinal whitening and necrosis.
274
What is the major complication of ARN?
Retinal detachment.
275
Is ARN unilateral or bilateral?
20% of patients present with bilateral disease, in unilateral patients onset can occur in other eye without treatment.
276
What is progressive outer retinal necrosis (PORN)?
Necrotizing herpetic retinitis found in immunocompromised patients characterized by rapid progression, absence of vitritis and retinal vessel involvement.
277
What is treatment for ARN?
intravenous acyclovir 800mg 5 times daily, or oral famciclovir 500mg 3 times daily or valaciclovir 1 g 3 times daily.
278
When is risk of retinal detachment highest in ARN?
8-12 week after onset, can use prophylactic laser treatment.
279
What is the most common cause of fungal endophthalmitis?
Candida, patients with history of indwelling catheters, long term antibiotics, immunosuppression therapy, DM, abdominal surgery.
280
What is treatment for candida endophthalmitis?
Intravenous fluconazole or intravenous/oral/intravitreal voriconazole.
281
Is aspergillus endophthalmitis more severe or less severe than candida?
More severe.
282
What are findings in intraocular TB?
Choroidal tubercles (yellow white lesions with indistinct borders), vitritis, papillitis and serous retinal detachment.
283
What is the characteristic finding for ocular syphilis?
Yellow placoid lesions in posterior pole.
284
Can patients with neurosyphilis have negative VDRL or RPR?
Yes, if FTA-ABS is positive and concern for neurosyphilis should consider lumbar puncture.
285
What are the retinal findings in cat-scratch disease?
Macular star formation and optic disc swelling, yellow-white infiltrates.
286
What is the prognosis for cat-scratch disease and what is the treatment?
Good prognosis even without treatment, can treat with doxycycline, ciprofloxacin or erythromycin.
287
How does toxoplasmosis chorioretinitis present?
Unifocal area of acute onset inflammation contiguous with chorioretinal scar. Can also see perivasculitis, arteriolar narrowing and occlusion.
288
What is triple therapy for ocular toxoplasmosis?
Pyrimethamine, sulfadiazine and folinic acid.
289
When can low dose prednisone be started for toxoplasmosis?
On day 3 of antibiotic therapy.
290
What should you consider in HIV patients with ocular toxoplasmosis?
Neuroimaging since 29% of HIV patients with ocular toxo also have intracranial lesions.
291
What are therapies other than triple therapy for toxoplasmosis?
Bactrim, clindamycin or azithromycin.
292
What are the findings in toxocariasis?
Severe uveitis, posterior granuloma with fibrocellular stalk to disc.
293
What is treatment for toxocariasis?
Local and systemic corticosteroids.
294
What can lyme disease present with early on?
Follicular conjunctivitis.
295
What are ocular findings of lyme disease?
Persistent iridocyclitis and vitritis, keratitis, retinal vasculitis, optic neuritis.
296
What is diffuse unilateral subacute neuroretinitis (DUSN)?
Retinal disease secondary to subretinal nematode.
297
What are findings in diffuse unilateral subacute neuroretinitis (DUSN)?
Early: vitritis, papillitis, yellow outer retinal lesions
Late: optic atrophy, retinal arterial narrowing, RPE changes, abnormal ERG.
298
How can diffuse unilateral subacute neuroretinitis (DUSN) be treated?
By photocoagulation of inciting nematode if it can be located.
299
What is the presentation of West Nile Virus Chorioretinitis?
Usually asymptomatic, with linear clusters of creamy circular spots, with target-like inactive lesions.
300
What is trichromatism?
Normal color vision, able to use 3 different wavelengths of colored light to match a given color.
301
What is protanopia?
Missing the long wavelength (L-cone) photopigment.
302
What is deuteranopia?
Missing the medium wavelength (M-cone) photopigment.
303
What is tritanopia?
Missing the short wavelength (S-cone) photopigment.
304
What is anomalous trichormatism?
Have 3 photopigments to match colors but one of the photopigments has an abnormal absorption spectrum.
305
What is deuteranomalous trichromatism?
The M cone photopigment is shifted closer to L-cone photopigment.
306
What is protanomalous trichromatism?
The L-cone photopigment is shifted closer to the M-cone photopigment.
307
Which is the most common color vision defect?
Deuteranomalous trichromatism (shifted red wavelength cone).
308
How are tritanomalous defects color defects inherited?
In an autosomal dominant fashion.
309
How are deuteranomalous or protanomalous color defects inherited?
In an X-linked recessive fashion.
310
What is rod monochromatism?
Photoreceptor disorder in which the cones are absent (may have residual function). Signs are nystagmus, photoaversion and poor acuity. ERG shows reduced cone responses but intact rod responses.
311
How is rod monochromatism inherited?
Autosomal recessive, linked with genes CNGA3, CNGB3 and GNAT.
312
What is blue-cone monochromatism?
X-linked recessive disorder in which only blue cones are present, difficult to identify clinically from rod monochromatism.
313
What is congenital stationary night blindness?
Stable abnormal scotopic vision, usually normal fundus appearance with range in visual acuity.
314
How is congenital stationary night blindness inherited?
X-linked most common, also autosomal dominant and autosomal recessive.
315
What is a negative ERG?
A wave present but reduced b-wave.
316
What is the most common ERG pattern in congenital stationary night blindness?
The negative ERG; in maximal dark-adapted response there is a large a-wave but absent or reduced b-wave.
317
What is fundus albipunctatus?
Disorder in visual pigment regeneration in which rhodopsin takes several hours to recover, with fundus findings of white-yellow dots in posterior pole.
318
What is Oguchi disease?
Patients with slow dark adaptation and characteristic "Mizuo-Nakamura phenomenon" a yellow sheen to fundus after light exposure.
319
What is the enhanced S-cone syndrome?
Rare recessive form of congenital stationary night blindness in which photopic ERG and scotopic are similar. A ring of RPE degeneration is noted at vascular arcades, may have schisis.
320
What is retinitis pigmentosa?
A group of hereditary disorders that diffusely involve photoreceptor and pigment epithelial function characterized by progressive visual field loss and abnormal ERG responses.
321
What are findings seen in retinitis pigmentosa?
Arteriolar narrowing, waxy pallor of the disc, bone spicule-like pigment changes, peripheral retina atrophy, vitreous cell, PSC cataracts.
322
What are the ERG findings in retinitis pigmentosa?
A marked loss of rod and cone signals, with both a and b-wave reduced.
323
What are other conditions on the differential of retinitis pigmentosa?
Ophthalmic artery occlusion, diffuse uveitis, syphilis, paraneoplastic syndromes, retinal drug toxicities.
324
What is sectorial retinitis pigmentosa?
Disease only in 1 or 2 sectors of the fundus, usually symmetric, slowly progressive.
325
What needs to rule out unilateral retinitis pigmentosa?
Vascular occlusion, retinal detachment, trauma, uveitis, infection or retained metallic intraocular foreign body.
326
What are genes found in retinitis pigmentosa?
Rhodopsin, RDS/peripherin.
327
What is Leber congenital amaurosis?
Autosomal recessive pattern, childhood form of retinitis pigmentosa, with visual acuity from 20/200 to NLP, ERG with minimal or undetectable response.
328
What can CME in retinitis pigmentosa be treated with?
oral or topical carbonic anhydrase inhibitors.
329
What defines cone dystrophy?
Progressive loss of visual acuity, hemaralopia, macula bull's eye atrophy, temporal optic atrophy and tapetal retinal reflexes.
330
What is the other name of Stargardt disease?
Fundus flavimaculatus.
331
What is the inheritance of Stargardt disease and the gene?
Autosomal recessive, mutations in the ABCA4 gene as well as RDS/peripherin, STGD4 and ELOVL4.
332
What are the findings in Stargardt disease?
Foveal atrophy with surrounding yellow round or pisciform flecks, dark choroid on fluorescein angiography.
333
What mutation is found in Best disease and what is the inheritence?
VMD2 (Best1) encoding bestrophin, autosomal dominant.
334
What is seen in Best disease?
Yellow yolk-like macular lesion which eventually breaks down leaving geographic atrophy.
335
What is the test specific for Best disease?
EOG, with Arden ratio <1.5
336
What is a complication of Best disease?
CNVM.
337
What is adult vitelliform pattern dystrophy?
A pattern dystrophy with bilateral yellow subfoveal lesions developing in 4th-6th decade, asymptomatic or with mild visual changes.
338
What is familial (dominant drusen)?
Drusen appearing at young ages usually in nasal retina or extending beyond vascular arcades.
339
What are the pattern dystrophies?
Disorders with patterns of yellow/orange/grey pigment deposition at RPE in macula, associated with RDS/peripherin gene and autosomal dominant inheritance.
340
What is Sorsby macular dystrophy?
A dominant disease with development of bilateral subfoveal CNV with early fine drusen deposits.
341
What is the gene associated with Sorsby macular dystrophy?
TIMP3.
342
What is choroideremia?
X-linked dystrophy with night blindness and visual field loss characterized by RPE and choriocapillaris degeneration.
343
What is the gene associated with choroideremia?
CHM gene, a geranylgeranyl transferase Rab escort protein.
344
Do carriers of choroideremia show retinal changes?
Yes, they have patches of subretinal black mottled pigment, usually asymptomatic.
345
What is gyrate atrophy?
Increase levels of ornithine toxic to RPE and choroid lead to loss of RPE and choroid with remaining RPE hyperpigmented.
346
What is the inheritance and gene associated with gyrate atrophy?
Autosomal recessive, OAT gene.
347
How can gyrate atrophy be treated?
Vitamin B6 to lower ornithine levels, helps small percentage of patients.
348
What are the 3 forms of retinoschisis?
1. degenerative peripheral retinoschisis (no inheritance)
2. Congenital X-linked recessive
3. Secondary to traction, optic pits, myopic changes
349
Where does splitting occur in X-linked retinoschisis?
In the nerve fiber layer.
350
What is the ERG pattern found in X-linked retinoschisis?
Negative waveform (intact a-wave, decreased b-wave)
351
What is the gene associated with X-linked retinoschisis?
RS1.
352
What should patients with X-linked retinoschisis be cautioned against?
Contact sports due to trauma worsening vision.
353
What is Goldmann-Favre syndrome?
Dystrophy characterized by night blindness, sensitivity to blue light, pigmentary retinal degeneration, optically empty vitreous, macula schisis.
354
What is the inheritance and gene associated with Goldmann-Favre syndrome?
Autosomal recessive, NR2E3.
355
What are findings in Bardet-Biedl syndrome?
Pigmentary retinopathy, obesity, polydactyly, hypogonadism and cognitive disability.
356
What is the definition of Usher syndrome?
Retinitis pigmentosa associated with congenital sensorineural hearing loss.
357
How is Usher syndrome inherited?
Autosomal recessive.
358
What are neuromuscular disorders associated with pigmentary retinopathies?
Friedreich ataxia, Charcot-Marie-Tooth disease, myotonic dystrophy, neuronal ceroid lipofuscinosis, progressive external ophthalmoplegia, peroxisome disorders.
359
What are findings indicative of lesions associated with familial adenomatous polyposis?
More than 4 widely spaced, small lesions per eye and bilateral involvement.
360
What is incontinentia pigment?
X-linked disorder which causes death in male fetuses, characterized by triphasic dermopathy, involvement of teeth and CNS, with pigmentary abnormalities and deficient peripheral retinal vascularization.
361
What are findings in cancer associated retinopathy (CAR)?
Photopsias, ring scotoma, arterial narrowing or no fundus changes.
362
What are the ERG and field findings in CAR?
Dramatic field loss over short period of time, both a and b-waves decreased on ERG.
363
What is the protein targeted in cancer associated retinopathy (CAR)?
Recoverin.
364
What is the treatment for cancer associated retinopathy (CAR)?
Immunosuppressive therapy.
365
What does the ERG in melanoma associated retinopathy look like?
Negative ERG waveform.
366
What oculocutaneous albinism?
A reduction in melanin biosynthesis affecting eyes, skin and hair follicles.
367
What is ocular albinism?
Reduction in melanin biosynthesis in which hair and skin are normally pigmented and only the eye is affected.
368
What is the inheritance of oculocutaneous albinism?
Autosomal recessive.
369
What is the inheritance of ocular albinism?
X-linked.
370
What are the features of ocular albinism?
Iris transillumination defects, photophobia, nystagmus, hypopigmented fundi, foveal hypoplasia.
371
What are the features of ocular albinoidism?
Same as albinism but with normally developed fovea and normal or minimally reduced visual acuity.
372
What is Chediak-Higashi syndrome?
Albinism with susceptibility to infections.
373
What is Hermansky-Pudlak syndrome?
Albinism with platelet defect leading to easy bruising, found in patients of Puerto Rican descent.
374
What are the neuronal ceroid lipofuscinoses (Batten disease)?
Autosomal recessive diseases with accumulation of lipopigments within neuronal lysosomes characterized by dementia, seizures, vision loss and pigmentary retinopathy.
375
Do the adult versions of neuronal ceroid lipofuscinoses have retinal changes?
No.
376
What is seen in vitamin A deficiency or abetalipoprotenemia which leads to vit A deficiency?
Night blindness and eventual loss of foveal function and diffuse drusen-like spots.
377
What is Zellweger syndrome?
A peroxisomal enzyme disease, characterized by retinal degeneration, hypotonia , seizures, hepatic fibrosis, renal cysts and death during infancy.
378
What is neonatal adrenoleukodystrophy?
A peroxisomal enzyme disease characterized by retinal denegeration, hypotonia and seizures with survival till 7-10 years.
379
What is infantile Refsum disease?
Elevated serum phytanic levels, leading to retinal degeneration, hypotonia and seizures.
380
What is seen in classic Refsum disease?
Elevated phytanic levels, pigmentary retinopathy, cerebellar ataxia, anosmia, hearing loss and cardiomyopathy.
381
Which of the mucopolysaccharidoses are associated with retinal dystrophy?
Those in which heparin sulfate is stored, Hurler syndrome, Scheie syndrome, Hunter syndrome and San Fillipo syndrome.
382
What causes the cherry red spot seen in Tay-Sachs and other ganglioside storage diseases?
Ganglion cells become filled with ganglioside and appear white, in contrast the RPE and choroidal pigmentation in the foveola appear red causing a cherry red spot.
383
What syndromes display a cherry red spot?
Sialidoses, gangliosidoses, and galactosidoses.
384
What form of cystinosis develops the retinopathy?
Nephrotic cystinosis.
385
What is the retinopathy seen in nephrotic cystinosis?
Areas of patchy depigmentation of the RPE alternating with irregularly distributed pigment clumps.
386
What is the retinal finding seen in Kearns-Sayre syndrome?
Pigmentary retinopathy with mottled macular pigmentary changes and rarely bone-spicule changes, variable ERG from normal to extinguished.
387
What are the features of Kearns-Sayre syndrome?
1. Chronic progressive external ophthalmoplegia
2. Retinal pigmentary degeneration
3. Cardiomyopathy
388
What defines low risk for hydroxychloroquine and chloroquine?
Less than 6.5 mg/kg/day or 1000 g total dose for hydroxychloroquine or 3 mg/kg/day or 460 g total dose for chloroquine, especially in the first 5 years of treatment.
389
What are included in the screening examination for hydroxychloroquine and chloroquine?
Ophthalmic exam, fundus photography, HVF 10-2. For patients at risk or with unclear symptoms, OCT, multifocal ERG or fundus autofluorescence.
390
What is seen in hydroxychloroquine toxicity?
Early paracentral field changes and granular depigmenation of the RPE developing to bull's eye maculopathy, with loss of IS/OS on OCT.
391
What is seen in high dose (>800mg/day) thioridazine toxicity?
Pigmentary retinopathy consisting of early coarse retinal stippling and later widespread patchy atrophy of RPE and choroid with nummular pattern.
392
What kind of retinal changes are seen in patients with high dose tamoxifen?
Maculopathy with crystalline deposits and macular edema.
393
What kind of retinopathy can occur with use of canthaxanthine (a drug used to simulate tanning)?
Crystalline retinopathy,
394
What kind of ocular symptoms are seen with IV use of desferrioxamine for hemosiderosis?
Vision loss, nyclatopia, ring scotoma, eventual mottled pigmentary changes.
395
What percent of eyes have a cilioretinal artery?
15-30%
396
What causes cotton-wool spots?
Obstruction in the radial peripapillary capillary net which lead to inhibited axoplasmic transport in the NLF.
397
What are the causes of cotton-wool spots?
Sickle cell retinopathy, radiation retinopathy, HIV associated retinopathy, hypertension, cardiac embolic disease, vasculitis, collagen vascular disease, leukemia, anemia.
398
What are the three types of emboli?
1. Cholesterol emboli (Hollenhorst plaques)
2. platelet-fibrin emboli from arteriosclerosis
3. calcific emboli from diseased cardiac valves
399
What are associations/causes of emboli in BRAO?
Cardiac myxoma, fat emboli from fracture, septic emboli, talc emboli in drug abuse, trauma, coagulation disorder, sickle cell disease, OCP or pregnancy, mitral valve prolapse, arrhythmia, inflammatory/infection, connective tissue disorder.
400
When does a cilioretinal artery occlusion occur?
Most often in setting of CRVO 2/2 to increased hydrostatic pressure reducing blood flow in cilioretinal artery.
401
What should be considered in isolated cilioretinal artery occlusion?
Giant cell arteritis.
402
How long does it take for irreversible damage to retina occur after CRAO?
90 minutes according to studies in primates.
403
What percent of CRAOs are due to giant cell arteritis?
1-2% of cases.
404
In what percentage of eyes with CRAO does NVI develop?
18% within 1-12 weeks.
405
What are causes of ophthalmic artery occlusion?
GCA, dissection of the internal carotid artery, mucormycosis, embolization, complication of surgery or injections.
406
What characterizes ophthalmic artery occlusion?
Vision loss to LP or NLP, often absent cherry-red spot.
407
What characterizes vasculitis?
Early manifestations are perivascular infiltrates and retinal vessel sheathing, often veins involved earlier than arteries, usual combination of vessels involved.
408
What medication can cause vasculitis?
Rifabutin.
409
What is Eales disease?
Priamary occlusive vascular disease involving bilateral peripheral retina and results in extraretinal NV, usually males with an associated tuberculin hypersensitivity.
410
What is Susac syndrome?
Condition with multiple BRAO, hearing loss and strokes, found in woman in 3rd decade, treated with immunosuppression.
411
What is idiopathic retinal vasculitis, aneurysms and neuroretinitis?
Syndrome with retinal vasculitis, macroaneurysms, neuroretinitis.
412
What causes the flower petal pattern of CME?
Pooling in the radial foveal arrangement of glial and Henle inner fibers.
413
What nutritional supplement can cause CME?
Niacin.
414
What are etiologies for CME?
Diabetic retinopathy, CRVO/BRVO, uveitis, retinitis pigmentosa, after ocular surgery, with prostaglandin analogues, X-linked retinoschisis, Goldman-Favre disease, nicotinic acid maculopathy.
415
When is the peak incidence of CME?
6-10 weeks post-op.
416
In what percentage of patients does post-op CME resolve spontaneously?
95%.
417
What can be used to treat CME with retinitis pigmentosa?
Acetazolamide
418
What is Coats disease?
Syndrome defined by vascular dilatation, microaneurysms associated with exudative retinal detachments, usually unilateral and in males.
419
What is parafoveal (juxtafoveal) retinal telangiectasia?
Syndrome of focal retinal gliosis and telengiectasia, three different types.
420
What is type 1 juxtafoveal retinal telangiectasia?
Unilateral parafoveal telangiectasia, typically occurs in males.
421
What is type 2 juxtafoveal retinal telangiectasia?
Bilateral parafoveal telangiectasia, affects men and women, can develop CNV, 1/3 of patients with abn glucose tolerance test.
422
What is type 3 juxtafoveal retinal telangiectasia?
Bilateral parafoveal telangiectasia with retinal capillary obliteration.
423
What can cause vision loss with macroaneurysms?
Embolic or thrombotic occlusion, intraretinal, subretinal or vitreous hemorrhage.
424
What are macroaneurysms associated with?
Systemic arterial hypertension or following CRVO.
425
What is the risk of direct treatment of macroaneurysms?
Rupture or vascular occlusion.
426
What is seen in capillary hemangioblastoma?
Tumor fed by dilated artery and drained by engorged vein, which can lead to serous RD.
427
How is hereditary retinal angiomatosis inherited?
Autosomal dominant, chromosome 3 tumor suppression gene.
428
What is von Hippel disease?
Retina and optic disc capillary hemangioblastomas limited to eye.
429
What is racemose angioma?
Retinal AV malformations with no intervening capillary bed.
430
What are retinal cavernous hemangiomas?
Grapelike clusters of thin-walled sacular angiomatous lesions with slow filling on FA and layering.
431
What is seen in radiation retinopathy?
Cotton wool spots, retinal hemorrhages, microaneurysms, macular edema, disc edema, retinal ischemia.
432
When is retinopathy seen after radiation?
18 months after external beam, earlier with brachytherapy.
433
What is the dose of radiation usually need to induce clinical symptoms?
30-35 grays.
434
What can be seen in valsalva retinopathy?
Intraretinal, subretinal and vitreous hemorrhage.
435
What is seen in Purtscher retinopathy?
Cotton wool spots, hemorrhages and retinal edema secondary to compression injuries to thorax and head.
436
What can cause Purtscherlike retinopathy?
pancreatitis, systemic lupus erythematosus, childbirth, amniotic fluid embolism.
437
What is Terson syndrome?
Vitreous or intraretinal hemorrhage caused by abrupt intracranial hemorrhage.
438
What are the different types of breaks?
Flap/horseshoe tears, giant retinal tears, operculated holes, dialyses, atrophic retinal holes.
439
What is a giant retinal tear?
A tear that extends 3 clock hours.
440
What is a dialysis?
Circumferential linear breaks along anterior and posterior vitreous base.
441
Where are traumatic breaks noted?
Inferotemporal or supranasal quadrant.
442
What is vitreous syneresis?
Collapse
443
What is vitreous synchysis?
Liquefaction.
444
What factors are associated with increased prevalence of PVD?
Increasing axial length and age.
445
What factors are associated with lattice?
Myopia.
446
What kinds of tufts predispose to retinal detachment?
Cystic and zonular traction retinal tufts.
447
Does peripheral cystoid degeneration and paving stone or cobblestone degeneration put patients at risk for retinal detachment?
Very rarely.
448
What is pavingstone or cobblestone degeneration?
Inferior areas of atrophy in outer retina and RPE.
449
What is the goal of prophylactic treatment for retinal breaks?
To create a chorioretinal scar around the break to prevent fluid vitreous from entering subretinal space.
450
Should symptomatic tears be treated?
Yes.
451
Do asymptomatic flap tears need to be treated?
Depends on other risk factors like lattice, myopia, aphakia with detachment in other eye.
452
Does aphasia and pseudophakia put patients at higher risk for retinal detachment?
Yes.
453
What is subclinical retinal detachment?
A detachment in which subretinal fluid extends more than 1 DD from break but not more than 2 DD posterior to equator.
454
What are the 3 types of retinal detachment?
Rhegmatogenous, tractional and exudative.
455
What is the Shafer sign?
"Tobacco dust" or pigmented cells in the vitreous.
456
What are the Lincoff Rules?
1. Sup temp or sup nasal detachments-> 98% of primary break lie within 1.5 clock hours of highest border
2. Sup detachment crossing midline-> 93% primary break is at 12 o'clock or in triangle with apex at ora serrata and sides 1.5 clock hours to either side of 12 o'clock
3. Inferior detachment-> 95% higher side of detachment indicates which side of disc break is in
4. Inferior bullous detachment->originates from superior break
457
What is PVR?
Proliferative vitreoretinopathy, which develops in long standing RRD as glial and RPE cells proliferate, form membranes which contract and cause retinal shrinkage many lead to new breaks.
458
What does the surface of retina look like in rhegmatogenous detachment versus tractional?
Convex in rhegmatogenous, concave in tractional detachment.
459
What are the main causes of large exudative detachment?
Neoplasia and inflammatory disease.
460
What characterizes exudative retinal detachments?
Shifting fluid, smooth surface without folds
461
What is reticular peripheral cystoid degeneration?
Posterior to and continuous with typical cystoid degeneration, with cystoid spaces in NLF can progress to retinoschisis.
462
What is typical degenerative retinoschisis?
Outer plexiform layer split, characterized by smooth elevation, sclerotic vessels, almost never extends to macula.
463
What is reticular degenerative retinoschisis?
Splitting in NFL, more likely to extend posteriorly than typical degenerative retinoschisis.
464
What are the differences between retinoschisis and RRD?
Retinoschisis: absolute scotoma, smooth surface.
465
What is an optic pit?
Small, hypo pigmented colobomatous defect often in inc-temp optic disc margin, can lead to serous RD.
466
What is an epiretinal membrane?
A semilucent avascular fibrocellular membrane adhering to ILM formed by glia, RPE or hyalocytes.
467
What percent of patients have improvement in vision after surgery for ERM?
50-75%.
468
What is vitreomacular traction syndrome?
Incomplete separation of the vitreous from the macula leading to tractional forces causing distortion, cystic changes or shallow detachment.
469
When do idiopathic macular holes occur and who do they affect?
6th-8th decade, women more than men, occur earlier in myopes.
470
What are the stages of macular holes?
Stage 0: PVD with subtle loss of foveal depression
Stage 1: A) foveal pseudocyst B) pseudocyst with break in outer retinal layer
Stage 2: pseudocyst developing into break in inner layer
Stage 3: Fully developed hole
Stage 4: Fully developed hole with complete PVD
471
Should surgery be done for stage 1 holes?
No, because high rate (50%) spontaneous resolution.
472
What is a Mittendorf dot?
Anterior remnant of the hyaloid artery, white plaque on posterior lens capsule.
473
What is a Bergmeister papilla?
Fibroglial tuft of tissue into the vitreous at the margin of the optic nerve head, remnant of hyaloid artery.
474
What are complications of peripapillary vascular loops?
BRAO, amaurosis fugax and vitreous hemorrhage.
475
What is persistent fetal vasculature or persistent hyperplastic primary vitreous?
Failure of primary vascular vitreous to regress, usually unilateral, with dense retrolental fibrovascular tissues, retinal dysplasia and folds.
476
What is anterior PFV?
White vascularized fibrotic membrane behind lens, microphthalmos, shallow anterior chamber, elongated ciliary processes, leukocoria, can lead to glaucoma.
477
What is the treatment for anterior PFV?
Lensectomy and removal of fibrovascular retrolental membrane.
478
What is posterior PFV?
Stalk of tissue emanating from optic disc, usually in inferior quadrant, with normal anterior chamber.
479
What are the hereditary hyaloidoretinopathies?
Vitreous liquification which results in optically empty cavity except for thin layer of vitreous behind lens and threadlike avascular membranes adhering to retina.
480
What is Wagner disease?
Autosomal dominant, not associated with retinal detachment, associated with myopia, strabismus and cataract.
481
What is Stickler syndrome?
Hyaloidoretinopathy (optically empty cavity) with high incidence of retinal detachment, associated with open angle glaucoma, myopia, cataract, Pierre-Robin malformation, joint hyperextensibility, arthritis.
482
What is the genetic inheritance and gene of Stickler syndrome?
Autosomal dominant, COL2A1 (collagen) gene.
483
What is familial exudative vitreoretinopathy (FEVR)?
Usually bilateral, failure of temporal retina to vascularize, leading to retinal folds, tractional/exudative RD, temporal macula dragging.
484
What is the inheritance of familial exudative virtreoretinopathy?
Autosomal dominant (EVR2 for Norrie disease protein) or X linked.
485
What is asteroid hyalosis?
White vitreous opacities made of calcium phospholipids.
486
What can be used for imaging in asteroid hyalosis?
Fluorescein angiography.
487
What is cholesterolosis?
Yellow white or gold cholesterol crystals in eyes with repeated trauma leading to intravitreal hemorrhages.
488
What can be seen in familial amyloidosis?
Bilateral vitreous opacification (glass wool opacification), heme, exudates, cotton wool spots, NV
489
What is commotio retinae?
Damage to outer retinal layers due to shock waves from blunt trauma which appears as retinal whitening, most often in the posterior pole.
490
What is the prognosis for commotio retinae?
Good.
491
What are posterior segment complications of trauma?
Vitreous hemorrhage, commotio retinae, macular hole, sclopetaria, scleral rupture.
492
What is sclopetaria?
Injury produced by high-speed projectile injuries to the orbit due to shock waves, leading to choroidal and retinal rupture with sub retinal and retinal hemorrhage.
493
What are the 2 most common locations for globe rupture?
At the limbus or parallel to and under the rectus muscle insertions.
494
What are diagnostic signs of globe rupture?
Boggy conjunctival chemises with hemorrhage, decrease in ocular auctions, deepened anterior chamber and severe vitreous hemorrhage.
495
Why should posterior wounds be left without closure?
Because attempts at closure might lead to vitreous incarceration during the repair.
496
What are the late complications of penetrating or perforating globe injury?
Fibrous proliferation which can lead to tractional detachment or retinal breaks.
497
Which foreign bodies are tolerated in the eye?
Sterile inert foreign bodies like stone, sand, glass, porcelain, plastic and cilia.
498
What are commonly reactive metals?
Zinc, aluminum, copper and iron. (Copper and iron are the most reactive)
499
What is seen on ERG in siderosis?
Increased a-wave, then diminishing b-wave, with late undetectable ERG.
500
What is the most common pathogen in traumatic endophthalamitis?
Bacillus cereus, especially with soil contaminated injuries.
501
What are ocular signs of non-accidental trauma in babies?
Retinal hemorrhages and cotton wool spots, retinal folds and hemorrhagic schisms cavities.
502
What are signs of avulsion of the optic disc?
Pit-like depression, posterior hemorrhage and contusion necrosis.
503
What are the 3 different types of light injury?
1. Mechanical (power absorbed forms bubbles or shock waves to disrupt tissue) YAG laser
2. Thermal (elevation of tissue temperature leads to coagulation, inflammation, scarring) laser photocoagulation
3. Photochemical (tissue destruction by transfer of energy to molecule resulting in reactivee molecules) solar retinopathy
504
What is solar retinopathy?
Photochemical retinal injury due to sun, thought to be due to blue light, higher risk in young patients or taking photosensitizing drugs
505
What are the retinal findings in solar retinopathy?
Initial yellow white dot in fovea followed by red dot with pigment halo, with lamellar hole after 2 weeks.
506
What increases the risk of photic damage during retinal surgery?
Indocyanine green
507
What wavelengths does melanin absorb?
Yellow, green, red and infrared.
508
What wavelengths does xanthophyll absorb?
Blue, minimal yellow or red.
509
What wavelengths does hemoglobin absorb?
Blue, green and yellow, minimal red.
510
What kind of magnification do planoconcave lenses give and what are they used for?
Used for the macula, upright image with same spot size as selected on slit lamp setting.
511
What kind of magnification do high-plus power lenses give and what are they used for?
Used for peripheral retina, inverted image with wide field of view, spot size that is magnified over laser setting size.
512
What are complications of photocoagulation?
Foveal burns, Bruch membrane rupture, retinal lesions like holes, fibrovascular proliferation, exudative retinal and choroidal detachment, subretinal hemorrhage and choroidal ischemia.
513
What photocoagulation settings increase the risk of Bruch membrane rupture?
Small spot size, high power and short duration.
514
What is transpupillary thermotherapy?
Infrared laser used to raise choroidal temperature with minimal RPE/retina damage, used to treat retinal and choroidal tumors.
515
What is photodynamic therapy (PDT)?
IV administration of photosensitizing drug (verpotin) followed by activation of drug with laser causing photochemical reaction leading to capillary closure, approved for CNV in OHS, myopia, AMD.
516
What are complications of PDT?
Photosensitivity reactions, back/chest/side pain with infusion, severe vision loss.
517
What is indocyanine green used to stain for in retinal surgery?
ILM.
518
What are the half lives of SF6 and C3F8
SF6 is 1 week, C3F8 is 3 weeks.
519
What is triamcinolone used to identify during retinal surgery?
Cortical vitreous.
520
What are the advantages of small gauge vitrectomy?
Shortened operative time, better pt comfort, faster recovery.
521
What are disadvantages of small gauge vitrectomy?
Increased risk of post-op hypotony, endophthalmitis and retinal tears.
522
What percent of patients have an improvement after vitrectomy for ERM?
60-80% of patients.
523
What stage macular holes should be considered for vitrectomy?
2 and up.
524
What did the Submacular surgery trial show?
RCT of observation vs surgery for submacular heme in AMD, found no difference in treatment for improving or stabilizing VA.
525
What are incisional surgical options for submacular hemorrhage?
Vitrectomy or pneumatic displacement of blood with injection of tPA.
526
What pathogens are associated with acute (less than 6 weeks post-op) endophthalmitis?
Coag neg staph, streptococcus and gram negative organisms.
527
What organisms are associated with chronic endophthalmitis (beyond 6 weeks post-op)?
P. acnes, coag neg staph, fungi.
528
What organisms are associated with bleb-associated endophthalmitis?
Streptococcus, Haemophilus, gram-positive organisms.
529
Which specimens are more likely to obtain a positive culture, aqueous or vitreous?
Vitreous.
530
What did the endophthalmitis vitrectomy study show?
Vitrectomy indicated in patients with LP vision or worse, patients with HM or better had similar outcomes with vitrectomy or tap/inject, no difference in outcome with or without systemic abx.
531
What is the treatment for chronic endophthalmitis?
Vitrectomy with partial capsulotomy and injection of vanc bag, followed by removal of entire capsule and IOL if first surgery not effective.
532
What size nuclear pieces should be removed if retained after cataract surgery?
2mm or larger.
533
What are indications for surgery for retained lens fragment?
Poorly controlled IOP or inflammation, fragment >2mm.
534
What is the risk after vitrectomy for retained lens fragment?
Retinal detachment.
535
What are risk factors for suprachoroidal hemorrhage?
Older age, glaucoma, myopia, aphasia, arteriosclerosis, HTN, Sturge-Weber with choroidal hemangioma, tachycardia intraop, hypotony.
536
What are indications for surgical drainage of suprachoroidal hemorrhage?
Pain, increased IOP, retinal detachment, kissing choroidals.
537
What are risk factors for pseudophakic retinal detachment?
Younger age, male sex, longer axial length.
538
What are the indications for pneumatic retinopexy?
Identification of all retinal breaks, all breaks in superior 8 clock hours, single break or multiple breaks within 1-2 clock hours, no PVR grade C or D, clear media and pt who cooperates with positioning.
539
What are complications of pneumatic retinopexy?
Subretinal gas migration, anterior chamber gas migration, recurrent RD, cataract and endophthalmitis.
540
What are complications of scleral buckling?
Myopia, anterior ocular ischemia, diplopia, ptosis, orbital cellulitis, subretinal hemorrhage from drainage and retinal incarceration.
541
What did the Silicone Study show?
RCT of C3F8 vs silicone oil in puts with C3 or more PVR, found no difference in visual acuity, reop or keratopathy whether or not pt had previous vitrectomy. Both better than SF6.
542
What are risks associated with pars plana vitrectomy?
NS cataract, open angle glaucoma, retinal break/detachment, vit heme, retinal incarceration, endophthalmitis.
543
What are the risks associated with silicone oil use?
Glaucoma and band keratopathy.
544
What is the risk of cataract after vitrectomy?
90% within 2 years of vitrectomy.
