Retina & Vitreous Flashcards

1
Q

What is the major risk factor for the development of the condition shown?

A

Systemic hypertension - Retinal arteriolar macroaneurysm

Other risk factors: age in 6th-7th decade, female, HLD, CV disease

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2
Q

Complications and visual prognosis for retinal arteriolar macroaneurysms

A

Macular edema, subretinal hemorrhage, vitreous hemorrhage

Visual recovery good

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3
Q

Eye with penetrating corneal laceration. What is indication to perform immediate vitrectomy?

A

intraocular foreign body

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4
Q

In a patient diagnosed with central serous chorioretinopathy (CSR), what finding might initiate reconsideration of the diagnosis?

A

Intraretinal fluid - if discovered, CSR is incorrect or patient has developed complication of CSR-associated choroidal neovascularization.

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5
Q

Characteristics of CSR?

A

Retinal pigment epithelial detachments

Serous retinal detachements

Macular or inferior peripheral pigmentary changes

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6
Q

Mechanism of CSR

A

Idiopathic serous detachment of the retina related to leakage at the level of the RPE, secondary to hyperpermeability of the choriocapillaris, as seen on indocyanine green angiography

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7
Q

What is the most likely etiology of this macular disease?

A

Idiopathic - full-thickness macular hole

caused by same tractional forces as forces a/w perifoveal vitreous detachment and thus are likely an early stage of age-related PVD

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8
Q

Treatment for this condition?

A

If funciontal visual impairment - pars plana vitrectomy with posterior hyaloid dissection +/- internal limiting membrane removal with postoperative gas tamponade

Full thickness macular hole with a grayish macular rim suggestive of subretinal fluid. Note the retinal pigment epithelial changes at the base of the hole.

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9
Q

Epidemiology of macular holes

A

Idiopathic macular holes occur at a rate of 8 per 100,000 persons per year

female-to-male ratio of 2 to 1

6th to 8th decade of life, younger age in myopic

Bilateral 10%

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10
Q

In an eye with retinoschisis, what exam findings are a/w increased risk of progression to RD?

A

inner and outer layer breaks

outer layer breaks ONLY (but not inner) can lead to localized retinal detachment that does not progress and seldom requires treatment.

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11
Q

% of full-thickness RDs a/w retinoschisis

A

3%

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12
Q

Describe the two types of schisis-related detachements

A

(1) Hole in OUTER but not inner wall of schisis cavity - contents of cavity migrate through hole in outer wall and slowly detach retina. demarcation line with degeneration of RPE. Does not progress. Rarely needs treatment.
(2) Hole in both INNER and OUTER layers. Schisis cavity may collapse and progressive RRD may result. Rapidly progress and usually require treatment. Causative break posterior. May need PPV.

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13
Q

Demarcation line in eye with retinoschisis suggests..

A

that full-thickness detachment is present or was formerly present and has spontaneously regressed.

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14
Q

56-year-old diabetic patient with NPDR in both eyes and blurred vision. OCT image shows the condition that is likely the cause for the visual reduction

A

Cystoid macular edema (DME)

Note: ERMs are often seen in NPDR but there is no contraction or antero-posterior traction at edges of ERM that would cause visual decline in this image.

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15
Q

Mechanism of DME

A

hyperglycemia-induced breakdown of the blood-retina baria which leads to fluid extravasation from retinal vessels into the surrounding neural retina

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16
Q

What are hard exudates?

A

precipitates of plasma lipoproteins

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17
Q

Foveal avascular zone (FAZ) of 1500 microns

A

macular ischemia from capillary occlusion

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18
Q

Vision loss in diabetic retionpathy is commonly a/w the following abnormalities:

A
  • capillary leakage (DME)
  • capillary occlusion (macular ischemia)
  • sequelae from retinal ischemia (retinal neovascularization, vitreous hemorrhage, tractional RD, neovascular glaucoma)
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19
Q

Name the features. Cause?

A

Failure of primary vascular vitreous to regress.

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20
Q

Characteristics of PFV?

A

Persistent fetal vasculature

Mild: prominent hyaloid vessel remnants, large mittendorf dot, bergmeister papilla

Severe: microphthalmia, ciliary process elongation (classic for PFV), retrolenticular plaque, a thick fibrous persistent hyaloid artery, and prominent radial iris vessels.

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21
Q

How frequently is this condition unilateral?

A

two-thirds of cases - persistent fetal vasculature

(cataractous lens, retrolental mesodermal mass, prominent radial iris vessels and ciliary process elongation)

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22
Q

Confiriming feature of circumscribed choroidal hemangioma?

A

echographic reflectivity - highly reflective mass with uniform, high internal spikes

(in Stuge-Weber, may be diffuse and typically have tomato ketchup appearance. For circumscribed choroidal hemangioms, the color can vary from white to red depending on overlying RPE)

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23
Q

Associated disease?

A

choroidal mass with homogenous echotexture and high amplitude spikes. The features were suggestive of a choroidal hemangioma.

Stuge-Weber syndrome (encephalofacial cavernous hemangiomatosis)

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24
Q

What feature characterizes West Nile virus retinopathy?

A

Bilateral, subclinical (febrile illness in 20%)

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25
West nile virus is this type of virus
Single-stranded RNA flavivirus Belons to the Japanese encephalitis virus serocomplex and is endemic to Europe, Australia, Asia, and Africa
26
Most toxic intraocular metal. Why?
Pure copper - acute chalcosis (severe inflammation lead to loss of eye)
27
Least toxic intraocular metal?
Zinc and aluminum - minimal inflammation and can become encapsulated
28
Mode of inheritance for Von Hippel-Lindau syndrome?
Autosomal dominant with incomplete penetrance and variable expression
29
Gene mutation that causes Von Hippel-Lindau
**tumor suppressor gene** mutation on short arm of **chromosome 3** (3p26-p25)
30
Asymptomatic 35-year-old man with BCVA 20/20 OU. Most likely diagnosis?
familial dominant drusen could be confirmed by examination of first-degree relatives
31
Early presenting phenotpyes of macular drusen
Familial dominant drusen Cuticular drusen (basal laminar drusen) Sorsby macular dystrophy Membranoproliferative glomerulonephritis type 2 malattia leventinese (radial drusen, Doyne macular dystrophy, or mutation in EFEMP1)
32
30-50 year old with bilateral presentation.
Adult-onset vitelliform macular dystrophy (pattern dystrophy) (bilateral, symmetrical, grayish-yellow, round or oval-shaped lesions within the macular area. These lesions are mildly elevated and are typically one-third to one-half disc diameter in size and contain a central pigmented spot)
33
Open globe injury that involves large laceration extending posteriorly. Trouble visualizing most posterior aspect and sutering is difficult. Next step?
Leave the most posterior aspect of the wound to heal without sutures. Continuing to dissect risks causing extrusion of ocular contents and further damage.
34
Finding in Vogt-Koyanagi-Harada disease that suggests chronicity?
Choroidal depigmentation or "sunset glow" fundus
35
Signs of acute uveitis phase of Vogt-Koyanagi-Harada disease
Serous retinal detachment Optic disc hyperemia Choroidal thickening
36
FA findings in VKH
multiple RPE leaks in areas of detachment "starry night" or Milky Way" sign
37
Sugiura sign
ocular depigmentation at the limbus (VKH chronic phase)
38
Depigmentation of the trabecular meshwork
"Ohno" sign in VKH (chronic)
39
"sunset glow" sign
Depigmentation of the choroid in VKH
40
Stages of VKH
1. Prodromal 2. Acute uveitic 3. Chronic (convalescent)
41
Characteristics of prodromal phase of VKH
flulike illness with HA, miningismus, tinnitus, and dysacusis
42
Characteristics of acute uveitic phase of VKH
pain, photophobia, vision loss accompanied by onset of **bilateral panuveitis** with **serous retinal detachements**
43
Characteristics of chronic (convalescent) phase of VKH
Depigmentation of skin and uvea: ocular depigmentation at the limbus (Sugiura), TM (Ohno), Choroid (Sunset glow)
44
The DRS demonstrated what treatment to be efficacious to decrease significant vision loss?
panretinal photocoagulation (PRP)
45
Studies that evaluated the impact of blood sugar control, PPV, and intravitreal use of anti-VEGF drugs
EDTRS, DVS, DCRnet.Protocol J
46
Clinical featuer of multiple evanescent white dot syndrome (MEWDS)
**Gray-white, poorly demarcated, patchy, outer-retinal lesions** orange-yellow fovea with granularity optic disc edema mild vitritis mild AC flare RAPD enlaged blind spot
47
**Multiple evanescent whit dot syndrome (MEWDS)** multiple small gray-white dots at the level of the deep retina/RPE in the posterior pole. Foveal granularity when present appears as tiny yellow-orange dots Mild vitritis No RPE scarring because resolves spontaneously over 2-6 weeks
48
Finding that highly suggests MEWDS
transient foveal granularity consiting of tiny yellow-orange flecks at the level of the RPE
49
Major finding of the Branch Retinal Vein Occlusion Study (BVOS)
laser-treated eyes with intact foveal vasculature, macular edema, and VA in the 20/40-20/200 range were more likely to gain 2 lines of visual acuity (65%) than were untreated eyes (37%)
50
Which eyes were observed in the BVOS?
if VA was better than 20/30 All eyes were observed for at least 3 months to permit maximum spontaneous resolution of edema and intraretinal blood
51
Prevelance of SO in patients who had undergone PPV
0.01% increasing to 0.06% when a/w penetrating ocular injuries. Possibly twice this figure.
52
Incidence of SO following nonsurgical ocular trauma
incidence of up to 0.5%
53
Most common complication of pars plana vitrectomy
**Visually significant nuclear sclerotic cataract - 90% of patients over 50** Retinal tear \<5% Intraoperative choroidal hemorrhage \<1% Endophthalmitis 1 out of 2,000 cases
54
What physiologic abnormality is a/w pathophysiology of diabetic retinopathy?
vascular endothelial growth factor (VEGF) upregulation retinal capillary basement membrane thickening increased platelet adhesion retinal capillary pericyte loss
55
For patient with diabetic retinopathy, what is an indication for panretinal (peripheral scatter) photocoagulation?
**High risk PDR** * mild NVD with VH * moderate to severe NVD (1/4 to 1/3DD) +/- VH * moderate (1/2 DD) NVE with VH or any 3 of the 4 * vitreous or preretinal heme * new vessels * new vessels located on or near OD * moderate to severe extent of new vessels
56
Risk of progression of Severe NPDR to high risk PDR?
15% within 1 year
57
Risk of progression of Very Severe NPDR to high risk PDR?
Very severe NPDR (2 or more features of severe NPDR) 45%
58
Can a retinal tear develop in meridional folds?
Yes at posterior edge of fold
59
Zonular traction tufts may cause this
small retinal flap tear
60
Greatest risk of visual loss from toxcitiy for patient taking hydroxychloroquine
renal insufficiency tamoxifen (5-fold) \>1000g hydroxychloroquine and 460 g chloroquine Use \>5 years retinal disease (AMD)
61
Safest recommended dose of hydroxychloroquine and chloroquine
Hydroxychloroquine 5.0 mg/kg/day Chloroquine 2.3 mg/kg/day based on patients real body weight (rather than 6.5 and 3 for ideal body weight)
62
bilateral macular crystals in IVD user diagnosis and complication?
talc emboli may cause **peripheral neovascularization**
63
Which retinal cell is response measured in the pattern ERG mainly generated?
ganglion cells
64
Considered as stand alone treatment for lesion less than this dimension?
Choroidal melanoma, Transpupillary thermotherapy (TTT) for tumors less than 4 mm thick TTT - infrared laser (810 nm) using beam sizes from 0.8 mm to 3.0 mm power 250 mW and 750 mW and 1 minute exposure time.
65
25 yo with transient visual loss. Two days ago noticed "smudge" in central vision with 20/40 VA. no RAPD. Cause for VA decline?
muptiple retinal hemorrhages, disc edema, CWS, tortuous veins from CRVO. Absent RAPD suggests nonischemic. **Macular edema** - result in central scotoma
66
Type of hemorrhage seen in valsalva retinopathy?
**sub-internal limiting membrane (ILM)** hemorrhage raise intraocular venous pressure sufficiently to rupture small superficial capillaries in the macula. Self-limiting, good visual prognosis
67
Accounts for 25% of posttraumatic endophthalmitis
*Bacillus cereus -* rapid and severe course with profound vision loss and often loss of the eye
68
M/c complication of type 1 juxtafoveal retinal telangiectasis?
macular edema
69
What is aneurysmal telangiectasia?
**_Macular telangiectasia type 1 (MacTel 1)_** - unilateral, young males, **aneurysmal dilatations of temporal macular vasculature** with surrounding **CME** and **yellowish exudates**. Macular variant of Coats disease
70
70 year old man with poor vision since childhood has VA 20/200. Maculas mildly atrophic but lack fluid on OCT. Irregular clumps of pigment in the peripheral retinas. Electronegative ERG. Daughter's 10-year-old son recently had "bleeding from some kind of retina problem" Diagnosis?
**Juvenile X-linked retinoschisis** rare hereditary condition with VA 20/200 "spoke-wheel" pattern of radial striae in central macula with schisis Electronegative ERG with b-wave reduced Peripheral retinoschisis in 50% Genetic testing for diagnosis- X-linked
71
Percentage of cones overall reside outside of foveal region?
90%
72
Photocoagulation mechanism and wavelength?
light energy to coagulate tissue - rises above 65 degrees C, denaturation of tissue proteins and coagulative necrosis. Span visible spectrum 400-700 nm (green, yellow, red)
73
Diagnostic device used for evaluation of contrast sensitivity?
Pellli-Robson test (using single large letter size 20/60)
74
What agent for retinal tamponade persists in the eye for approximately 2 months after a PPV?
perfluoropropane gas (C3F8) longer lasting than sulfur hexafluoride gas (SF6) which lasts 2-3 weeks.
75
Temporarily stabilize the retina during dissection and facilitate anterior drainage of subretinal fluid during RD repair
Perfluorocarbon (PFC) liquids which are heavier than water
76
Color (wavelengths) of light readily absorbed by macular xanthophyll?
blue (minimally absorbs yellow, red, infrared)
77
Function of the RPE
Phagocytosis of photoreceptor outer segments absorption of light vitamin A metabolism formation of **outer blood retinal barrier** maintain subretinal space healing of traumatic injuries
78
What 2 entities would be the most likely causative organisms in an otherwise healthy 40-year-old man?
acute retinal necrosis (necrotizing herpetic retinitis) caused by herpes simplex virus and varicella-zoster viruses occurs with occlusive retinal vasculitis and moderate to severe AC and vitreous inflammation
79
Inheritance pattern of Stargardt disease?
Autosomal recessive
80
What mimicking condition must the ophthalmologist also consider?
**Syphilis** Mimic RP - previous ophthalmic artery occlusion, diffuse uveitis, infection, paraneopalstic syndromes, retinal drug toxicity.
81
Electrophysiologic test most useful in detecting primary ganglion cell disease?
Pattern electroretinogram (ERG)
82
Electrophysiology test that can detect whether macular dysfunction is present
multifocal ERG
83
Electrophysiological test that assesses the health of the RPE as well as interaction between RPE and photoreceptors by measuring changes in corneoretinal standing potential that occur during scotopic or photopic adaptation
Electro-oculogram (EOG) (principle use is to diagnose Best vitelliform macular dystrophy)
84
Electrophysiologic test used to diagnos Best vitelliform macular dystrohy
EOG
85
Clinical manifestation of bilateral diffuse uveal melanocytic proliferation (BDUMP)
**serous retinal detachment** diffuse thickening of the choroid reddish or brownish discoloration cataracts
86
Numerous retinal flecks, normalization of rod ERG responses with prolonged dark adaptation
Stationary condition **fundus albipunctatus**
87
Numerous retinal flecks, rod ERG responses do not normalize (are not rescued) with prolonged dark adaptation in this progressive rod-cone dystrophy
**retinitis punctata albescens**
88
Stargardt disease phenotype in which subretinal flecks are widely scattered throughout the fundus
**Fundus flavimaculatus**
89
Most important management step in a patient who presents with a BRAO
Urgent stroke workup (embolic event) Brain imaging, evaluation of carotid arteries, transesophageal echocardiography
90
Acquired night blindness with shimmering photopsias
Melanoma associated retinopathy
91
ERT findings in MAR
preserved dark adapted a-wave followed by strikingly reduced b-wave resulting in electronegative ERG
92
Definition of pleiotropy in the context of ocular genetic disorders. Example?
Diverse phenotypes share mutations in a common causative gene Mutations in a **single gene** that may lead to **multiple phenotypes** ABCA4 (stargardt disease requires both alleles to harbor disease causing mutations but others may cause pattern dystrophy, cone-rod dystrophy or AR retinitis pigmentosa
93
Young myopic woman with acute onset of posterior photopsias with scotomas corresponding to decreased mfERG response and outer retinal layer loss in both eyes
Acute Zonal Occult Outer Retinopathy (AZOOR)
94
Mechanism of purtscher retinopathy
**complement activation** follows acute compression injuries to the thorax or head. injury-induced complement activation which causes granulocyte aggregation and leukoembolization, occludes small arterioles such as those found in peripapillary retina
95
How would this patient present? Findings on DFE and on OCT
Young to middle aged women, UNILATERAL, decreased vision, scotomas, +/- photopsias +/- vitritis **Multiple Evanescent White Dot Syndrome (MEWDS)** multiple small gray, white or yellow-white dots at level of outer retina (RPE) in and around posterior pole. Transient foveal granularity consisting of timy yellow-orange flecks at level of RPE.
96
Treatment for stage 4 ROP? Prognosis?
**lens-sparing vitrectomy** or scleral buckling to alleviate the vitreoreitnal traction causing RD **Stage 4A** rather than later stages 4B or 5 have more favorable outcomes
97
Associated with which condition? What systemic findings would you look for? Complications?
Diffuse choroidal hemangiomas in **Sturge-Weber syndrome** *reddish/orange tumor in postequatorial retina often under macula may produce RD* **Facial cutenous angioma (port-wine stain), cerebral calcifications, seizures, variable mental deficiency** Not genetically transmitted **Glaucoma in 70%**
98
This compoind cuases inner segment ellipsoid distruption apparent on SD-OCT
alkyl nitrate "poppers" inhalation *poppers maculopathy*
99
Verteporfin is approved by the US FDA for what indication?
**Subfoveal CNVs seconadry to myopia, AMD, presumed ocular histoplasmosis syndrome (POHS)** Off label: CSC and circumscribed choroidal hemangiomas
100
Toxicity most likely to cause cherry red spot on fundoscopic exam?
Quinine - retinal ganglion toxciity produces opacification of the ganglion cell layer
101
Causes transient optic nerve head edema and macular edema with optic atrophy
Methanol toxicity
102
In what form of dichromacy is the long-wavelength (L-cone) photopigment absent?
protanopia
103
Individuals who need only 2 primary colors to make a color match
dichromacy (2% males)
104
missing the medium (M-cone) photopigment
deuteranopia (1% of males)
105
Rare form of congenital dichromacy is lack of the short-wavelength (S-cone) photopigment, AD occurs in 0.001% of population
tritanopia
106
Specific sign related to microscope-induced light toxicity
**pirmgentary mottling of the macula 1 to 3 weeks after exposure** **paracentral scotoma may result** mild yellow-white discoloration in the first few days FA shows hyperfluorescence without hypofluorescence
107
Complication of this condition
**retinal ischemia** - retinal vascular occlusions, amaurosis fugax, or VH prepapillary arteriolar loop over the optic disc.
108
The **diabetic retinopathy vitrectomy study** (DRVS) demonstrated efficacy of vitrectomy surgery to decrease SVL (significant vision loss) from what **complication** of DR
**nonclearing vitreous hemorrhage** treat VH and combined with endolaser photocoagulation to treat underlying PDR
109
Presumed h/o refractory central serous choroidopathy however FA shows the following. Diagnosis?
"leopard spot appearance" **Uveal effusion syndrome** - imparied posterior segment drainage causing exudative detachement of retina, choroid, or ciliary body.
110
Risk factors for uveal effusion syndrome
Young age Hyperopia Nanophthalmos
111
Medication a/w development or exacerbation of macular edema
**pioglitazone**
112
What paraneoplastic retinal degeneration is associated with antirecoverin antibodies?
Cancer-associated retinopathy (CAR) Suggestive of systemic malignancy - small cell carcinoma of lung
113
Antienolase and antitransducin antibodies
Antiretinal antibodies present that may cause retinal degeneration ## Footnote **autoimmune retinopathy**
114
Factors that affect treatment prognosis with ocriplasmin injection for VMT syndrome or macular hole.
**AREA** of vitreomacular adhesion (VMA) VMA resolved in 33.6% of eyes if size of adhesions was \< or = 1500 um on OCT VMA resolved in 10.3% of adhesions \> 1500 um
115
In patients with lattice degeneration who do not receive prophylactic treatment, what percentage will have a RD in the affected eye?
**1%** Half of patients with lattice have concomitant atrophic retinal holes. Only 1% to 2% of patients with atrophic retinal holes later develop a clinical retinal detachement
116
Patient with decline in vision in right eye. What patient characteristic is more commonly a/w this condition?
**Trauma** Majority of patients with MH, cause is unknown or idiopathic. For remainder of patients, myopia and direct blunt ocular trauma are associated.
117
Epidemiology of MH
50 to 80 more common in women
118
Peripheral retina with inner retinal atrophy, retinal vascular sclerosis, condensation and adherecne of vitreous to margins with an overlying pocket of liquefied vitreous
lattice degeneration
119
Due to outer retinal ischemia, presumably from occlusions of the choriocapillaris. Scalloped area of retinal atrophy develops with dense adhesions of the inner retina to Bruch membrane. RD do not occur in this area of dense adhesion.
Cobblestone, paving-stone degeneration
120
Loss of the outer plexiform layer forming cystoid spaces that may coalesce
Peripheral cystoid retinal degeneration and retinoschisis
121
Medication a/w exacerbation of macular edema
pioglitazone
122
Paraneoplastic retinal degeneration a/w antirecoverin antibodies
Cancer-associated retinopathy - systemic malignancy such as small cell carcinoma of lung
123
Ocular characteristic affects treatment prognosis for treatment of VMTS or MH for ocriplasmin injection?
**Area of vitreomacular adhesion (VMA)** 1500 um or smaller - 33.6% resolution of VMA \> 1500 um - only 10.3% resolution VMA
124
% of patients with lattice degeneration who do not receive prophylactic treatment that will have a RD?
1%
125
Patient characteristic more commonly a/w this condition?
Unknown or idiopathic is m/c however myopia or **blunt trauma** are associated.
126
Age and sex of patient with MH
50 to 80 years old. More common in women.
127
128
% of population with this condition
10% | (photo shows sclerotic vessels)
129
62 yo phakic man PMHx DM2 x 15 years, HTN c/o 6-months blurred vision of right eye. Diagnosis?
**Diabetic macular edema** - hard exudates in right eye. Early FA confirms vascular leakage and areas of capillary nonperfusion. Later images show cystoid pattern of leakage.
130
FA shows area of bright, lacy, well-defined hyperfluorescence that is identified in the early phase and progressively leaks by the late phases.
Classic CNV
131
Late leakage on FA from an unidentifiable source
Occult CNV
132
What complications of retinal cavernous hemangiomas require treatment?
**Vitreous hemorrhage** - if recurrent VH, laser photocoagulation or cryoptherapy may be used. Note: retinal neovascularization does not develop from cavernous hemangiomas.
133
What retinal degenerative disorder is classified as a retinal ciliopathy that involves mutations that affect cilia of multiple organ systems including those in the photoreceptor outer segments and inner ear?
**Bardet-Biedl syndrome** Other syndromic forms of RP associated with retinal and systemic ciliary dysfunction: Usher Alström Senior-Løken Joubert CEP290-mutation associated Leber's congenital amaurosis Jeune syndromes Screen for *hearing loss* and *renal disease*
134
Risk factor for ROP in premature neonates (other than weight and BGA)
**history of blood transfusion** septic poor postnatal weight gain
135
In patients with intermediate or advanced AMD, to what degree did the nutritional supplementation used in AREDs reduce the risk of progression to more-advanced stages of AMD? How was AREDS2 different?
**25%** risk reduction for progression of more advanced stages with AREDS (vitamin C 500 mg, E 400 IU, beta carotene 15 mg, 80 mg zinc oxide with 2 mg cupric oxide to prevent zinc induced anemia) 19% risk reduction in rates of moderate vision loss (3 lines of VA) at 5 years AREDS2 replaced beta carotene with lutein and zeaxanthin with similar results
136
Surgical practice that improves the prognosis for hole closure for stage 3 full-thickness macular holes?
Peeling of inner limiting membrane for stage 3 and 4 MH improves rate of closure. Vitrectomy advised for all recent MH that are stage 2 or higher. Although up to 50% of stage 1 close spontaneously, prompt intervention is advised for stage 2 or higher given association between hole duration and outcome.
137
What is ocriplasmin?
recombinant protease administered by intravitreal injection designed as alternative to vitrectomy
138
Elevated lesion with B-scan shown. Diagnosis?
**Circumscribed choroidal hemangioma -** high internal reflectivity. orange, dome-shaped lesions a/w exudative RDs Note: choroidal melanoma demonstrates low internal reflectivity
139
Tx for macular edema a/w CRVO
**intravitreal anti-VEGF therapy** **BRAVO -** Study of Efficacy and safety of Ranibizumab injections in patients with ME secondary to **BRVO**. 0.5mg, 0.3mg or sham monthly at 6 months 15 or more letters were 61.1%, 55.2%, 28.8% **CRUISE -** Study of the Efficacy and Safety of Ranibizumab Injection in Patients with Macular Edema Secondary to **CRVO**. 0.5 mg, 0.3 mg, sham. 47.7%, 46.2%, and 16.9% **Aflibercept (VEGF-Trap)** - similar results in **VIBRANT** (BRVO), **COPERNICUS** (CRVO in north america), **GALILEO** (outside north america)
140
Percentage of patients with vitreous hemorrhage and acute symptomatic posterior vitreous detachment (PVD) have a retinal tear
**60%** Retinal tears reported in up to 18% of patients with acute symptomatic PVDs, however if VH then prevalence raises to between 50% and 70%.
141
Percentage of patients with with acute PVD without VH that will nevertheless have a retinal tear?
**10%** Therefore careful exam is mandated even in absence of VH!!!
142
Characteristic of RD following ocular blunt trauma in young eyes
RDs in this setting have a **DELAYED** presentation. Vitreous is still formed (ie not syneretic) which has 3 effects (1) prevents fluid movement under retina by acting like a cork on the break (2) prevents retina from assuming a bullous shape (3) slow progression as vitreous slowly liquefies over time More likely to cause *retinal dialyses* rather than typical retinal tearing at posterior margin of the vitreous base
143
Characteristic aspect of familial exudative vitreoretinopathy (FEVR)
**Failure of temporal retina to vascularize** - retinal folds and peripheral fibrovascular proliferation as well as tractional and exudative RD frequent. Temporal dragging of macula may cause exotropia. Late-onset rhegmatogenous RDs may occur. similar to ROP but patients are born full term and normal respiratory status. Differentiate aided based on FHx and careful exam of all family members.
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Mutation of which gene in which cell type results in nonprogressive dysfunction in patients with nyctalopia and white dots?
**RDH5 gene mutation in the retinal pigmented epithelium** Fundus albipunctatus - RDH5 encoldes 11-*cis-* retinol dehydrogenase, a microsomal enzyme in the RPE that converts 11-*cis-*retinol into 11-*cis-*retinal and is therefore involved in regeneration of rhodopsin. Very delayed rhodopsin regeneration, although normalize, may take many hours in dark. Night blind from birth and usually exhibit yellow-white dots in the posterior pole extending into the midperiphery but spare the fovea
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ERG show a cone-isolated retina pattern, with undetectable rod-specific ERG, and severely reduced bright-flash dark-adapted ERG (arising in dark-adapted cones) that normalizes with sufficiently extended dark adaptation.
**Fundus albipunctatus** (RDH5 encodes 11-cis-retinol dehydrogenase in RPE that regenerates rhodopsin)
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Gene invovled in retinitis punctat albescens
***RLBP1***
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Diseases caused by mutations in *ABCA4*
Stargardt disease fundus flavimaculatus cone-rod dystrophy RP AMD
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POW9 s/p CE/IOL patient develops a steroid-responsive granulomatous uveitis/endophthalmitis with a white plaque on her intraocular lens. Next step in management?
**pars plana vitrectomy (PPV) and partial capsulectomy with intravitreal antibiotic injection 1mg vancomycin adjacent to or inside the capsular bag.** If condition recurs after vitrectomy, removal of entire capsular bag with removal or exchange of IOL. *Propionibacterium acnes* endophthalmitis. Cx positive rate low because obligate microaerophilic bacterium, needs low-oxygen environment. Intravitreal injection of abx clear infection in 50%
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peripheral white plaque within capsular bag and chronic granulomatous inflammation
*Propionibacterium acnes*
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Common species that cause chronic endophthalmitis
*Prpionibbacterium acnes* coagulase-negative *Staphylococcus spp* (S. epidermidis, S. hominis, S. capitis) can penetrate broken skin Funghi
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Patient with AMD and initial new-onset metaphorphopsia, what is the most reasonable sequence of ancillary testing?
Spectral domain oprical coherence tomography, then if needed for diagnostic clarity, fluorescein angiography (to diagnose retinal macroaneurysm, pseudophakic macular edema, BRVO, and other confounders)
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Classic CNV on FA
bright, lacy, well-defined hyperfluorescent lesion that appears in early phase and progressively leaks by late phases
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Occult CNV findings on FA
1 of 2 forms: (1) **PED**, either fibrovascular PED or vascularized serous PED (2) **late leakage** from undetermined source
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Ideal candidate for treatment of a RD with pneumatic retinopexy
phakic patient with macula-involving, superior detachment with a horseshoe tear at 10 o'clock. **Solitary or closely _clustered (1-2 clock-hours)_ retinal breaks located in the _superior 8 clock-hours_ of the retinal periphery** **All breaks _identified_** **_No PVR_ grade C or D** **_Cooperative_ patient who can maintain position** **_Clear media_**
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Patients with poor likelyhood of success for RD using pneumatic retinopexy
post-traumatic, lattice degeneration-associated, and pseudophakic patients
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Contraindications for pneumatic retinopexy
retinal breaks in the **inferior 4 clock-hours** of the retina periphery presence of significant **traction** that would limit the ability of the retina to settle against the choroid (PVR)
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Goal of RD repair and 3 approaches
Bringing the RPE and choroid into contact with the retina long enough to produce choioretinal adhesion that will permanently wall off the subretinal space (1) Scleral buckling (2) Vitrectomy (3) Pneumatic retinopexy
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When is surgery performed urgently in RD repair vs delayed
Urgent - Acute, macula-on retinal detachments Delayed or not needed - chronic RD with pigmented demarcation lines
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Type of identifiable cells that epiretinal membranes contain
**retinal glial cells, RPE, immune cells** glia (Muller cells, microglia, fibrous astrocytes) retinal pigment epithelium immune cells (hyalocytes, macrophages) cells may differentiate over time to form contractile myofibrocytes
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Condition associated with diabetic retinopathy progression
Pregnancy - eye exam recommended in first trimester for patients with DR Hypertension
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Premature infant with immature vascularization in zone I but no evidence of ROP on initial screening. Interval for next screening.
1 week
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ROP screening interval criteria that require 1-week or less follow-up
immature vascularization: zone I - no ROP immature retina extends into posterior zone II, near boundary of zone 1 Stage 1 or 2 ROP: zone I stage 3 ROP: zone II presence or suspected presence of aggressive posterior ROP
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ROP screening interval criteria that require 1- to 2-week follow-up
immature vascularization; posterior zone II stage 2 ROP: zone II unequivocally regressing ROP: zone 1
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ROP screening interval criteria that require 2-week
Stage 1 ROP: zone II immature vascularization: zone II - no ROP unequivocally regressing ROP: zone II
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ROP screening interval criteria that require 2- to 3-week
Stage 1 or 2 ROP: zone III regressing ROP: zone III
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When would you examine an ROP infant from BGA 23 to 31 weeks?
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Stage 1 ROP
**Demarcation Line** This line is a thin but definite structure that separates the avascular retina anteriorly from the vascularized retina posteriorly. There is abnormal branching or arcading of vessels leading up to the demarcation line that is relatively flat, white, and lies within the plane of the retina.
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Stage 2 ROP
**Ridge** The ridge arises from the demarcation line and has height and width, which extends above the plane of the retina. The ridge may change from white to pink and vessels may leave the plane of the retina posterior to the ridge to enter it. Small isolated tufts of neovascular tissue lying on the surface of the retina, commonly called "popcorn" may be seen posterior to this ridge structure and do not constitute the degree of fibrovascular growth that is a necessary condition for stage 3.
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Stage 3 ROP
**Extraretinal Fibrovascular Proliferation** Neovascularization extends from the ridge into the vitreous. This extraretinal proliferating tissue is continuous with the posterior aspect of the ridge, causing a ragged appearance as the proliferation becomes more extensive.
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Stage 4 ROP
**Partial Retinal Detachment** in the initial classification was the final stage and initially known as the cicatricial phase. It was later divided into extrafoveal (stage 4A) and foveal (stage 4B) partial retinal detachments. Stage 4 retinal detachments are generally concave and most are circumferentially oriented. Retinal detachments usually begin at the point of fibrovascular attachment to the vascularized retina and the extent of detachment depends on the amount of neovascularization present.
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Stage 5 ROP
**Total Retinal Detachment** Retinal detachments are generally tractional and usually funnel shaped. The configuration of the funnel itself is used for subdivision of this stage depending if the anterior and posterior portions are open or narrowed.[7] More than one stage may be present in the same eye, staging for the eye as a whole is determined by the most severe stage present.
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Zones in ROP
**Zone I** - A circle is drawn on the posterior pole, with the optic disc as the centre and **twice** the disc-macula distance as the radius, constitutes zone I. Any ROP in this zone is usually very severe because of a large peripheral area of avascular retina **Zone II** - A circle is drawn with the optic disc as the centre and disc to nasal ora serrata as the radius. The area between zone I and this boundary constitutes zone II **Zone III** - The temporal arc of retina left beyond the radius of zone II is zone III
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Explain timing of intraocular development of retinal blood vessels
Normal growth and development of intraocular blood vessels begins at 16 weeks gestational age at the optic nerve head. It is completed at 40 weeks at the temporal side of the retina, and at 36 weeks on the nasal side
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Proven to be effective in reducing the risk of vision loss from intermediate age-related macular degeneration
antioxidants and zinc
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This showed a 25% risk reduction for progression to more advanced stages of AMD and 19% risk reduction in rates of moderate vision loss (\> or = 3 lines of VA at 3 years)
Vitamin C (500 mg) Vitamin E (400 IU) beta carotene (15 mg) Zinc (80 mg) Cupric oxide (2 mg)
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Intermediate AMD
Stage 3 at least 1 large druse (\>125 um) extensive intermediate drusen (63-124 um) nonsubfoveal GA
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Advanced AMD
stage 4 vision loss due to neovascular AMD or subfoveal GA in only 1 eye
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Maximum daily dose of hydroxychloroquine that has been recommended to minimize the risk of retinopathy
**5.0 mg/kg/day** using **total body mass**
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Maximum daily dose of chloroquine that has been recommended to minimize the risk of retinopathy
**2.3 mg/kg/day** based on patients real body weight
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When should an infant be ophthalmoscopically screened for ROP?
All premature neonates with a BW of 1500g or less with a gestational age of 30 weeks or less before hospital discharge, or who have reached 4-6 weeks of age. Selected infants 1500 - 2000 g or \>30 weeks with unstable clinical course
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Timing of first ROP exam
4-6 weeks of postnatal age (or within the 31st to 33rd week of postconceptional or postmenstrual age, whichever is later)
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Where is this 11.5 year old (1-66) child from and how did this infection occur?
Subtropical and tropical climates (including southern USA!) **ocular toxocariasis** from infestation of the retina/choroid with the second stage larva of roundworm parasites *Toxocara canis or Toxocara catis* that complete their life cycles in the small intestines of dogs and cats respectively
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What is an indication for prompt surgical intervention in the setting of a visually significant vitreous hemorrhage secondary to PDR
**(1) Poor visual acuity in the fellow eye secondary to complications of PDR** (2) bilateral vitreous hemorrhage (3) U/S evidence of a retinal tear or RD or TRD that threatens macula
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When do you perform PPV for VH?
fails to clear spontaneously after 4 weeks to 3 months Prompt: monocular, bilateral vitreous hemorrhage, or US evidence of tear, RD, TRD that threatens macula
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Purpose and results of DRVS?
Diabetic retinopathy vitrectomy study - early prospective randomized clinical trial to evalulate role of PPV for severe PDR. Early (1-6 months after VH) vs late (\>1 year after VH) Results: DM1 showed clear benefit from earlier PPV. DM2 did not. well placed PRP with VH can be observed longer.
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Most common systemic condition associated with this condition
**HIV** cytomegalovirus retinitis - most common ocular opportunistic infection in adult patients with advanced AIDS usually when CD4 T-cell count is less than 50/uL
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Eyes at greatest risk for development of an RPE tear after anti-VEGF therapy?
eyes with **fibrovascular pigment epithelial detachment** (PED) \> 600 um in height and presence of **type 1 choroidal neovascularization (CNV)** mechanism: ​contraction of underlying type 1 CNV
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Middle layer of the Bruch membrane composed of this
elastic fibers
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Layers of Bruch membrane
Starting with the innermost: * basement membrane of the RPE * inner collagenous zone * middle layer of elastic fibers * outer collagenous zone * basement membrane of the endothelium of the choriocapillaris
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Seen after uncomplicated cataract surgery. Diagnosis and treatment?
Angiographic cystoid macular edema Tx: **periocular or intravitreal corticosteroid therapy**
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Incidence of CME after cataract surgery
1% to 30%
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Tx for mild CME s/p CE/IOL and for severe or refractory
Mild - topical corticosteroids and NSAIDs Severe/refractory - periocular (posterior sub-Tenon triamcinolone acetonide) or intraocular injection of steroid preparations
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Characteristic clinical finding suggestive of hypertensive choroidopathy
**linear streaks of hyperpigmentation surrounded by margins of hypopigmentation** (Elschnig spots) - lobular nonperfusion of the choriocapillaris or **linear configurations of hyperpigmentation following meridional course of choroidal arteries** (Siegrist streaks)
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Which patients will this finding be seen in?
Elschnig spots - seen in hypertensive choroidopathy from patients who have experienced an episode of acute severe HTN often a/w preeclampsia, eclampsia, pheochromocytoma, or renal hypertension. Results in lobular nonperfusion of the choriocapillaris - tan, lobule --sized patches that become hyperpigmented with margins of hypopigmentation.
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Inheritance pattern of choroideremia
**X-linked recessive** CHM gene on X121.2 that encodes geranylgeranyl transferase Rab escort protein
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Expected condition in postoperative serous choroidal detachment
hypotony
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Pelli-Robson test
contrast sensitivity
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stage 2 AMD
multiple small or few intermediate drusen in 1 or both eyes
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stage 3 AMD
few large drusen or multiple intermediate drusen
200
stage 4 AMD
geographic atrophy
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What is a drusenoid PED?
confluent large drusen that coalesce into a PED (\>350 um diameter)
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4 point grading system for AREDS
* presence of 1 or more large (\>125 um diameter) drusen (1 point) * presence of any pigment abnormalities ( 1 point) * for patients with no large drusen, presence of bilateral intermediate (63-124 um) drusen (1 point) * presence of neovascular AMD (2 points)
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factors predisposing patient to needle penetration of globe during retrobulbar anesthesia
* **axial high myope** * inexperienced practitioner * poor patient cooperation at time of injection * posterior staphyloma * previous scleral buckling surgery * scleromalacia
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Disorder associated with retinal capillary hemangioblastomas
pheochromocytoma Seen in Von Hippel-Lindau - cysts and tumors in viscera and CNS Cerebellar hemangioblastoma, pancreatic cysts, RCC, pheo
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Retinal capillary hemangioblastoma vs Diffuse choroidal hemangioma vs circumscribed choroidal hemangioma
Retinal capillary hemangioblastoma - Von Hippel-Lindau Diffuse choroidal hemangioma - Sturge-Weber circumscribed choroidal hemangioma - No systemic dx or syndrome
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Cause of macroaneurysms
idiopathic systemic HTN Atherosclerotic disease
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Electrophysiologic diagnostic study serves as direct measure of central retinal ganglion cell function
**pattern electroretinogram (PERG)** retinal response to isoluminant alternating checkerboard stimulus to macula 2 main components, P50 and N95, with earlier negative component N35 **N95 arises in the retinal ganglion cells (RGCs) and act as direct measure of central RGC function**
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What does the EOG do?
Electro-oculogram assess the **health of the RPE** by measuring changes in the corneo-retinal standing potential during light adaptation.
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What does multifocal electroretinogram (mfERG) do?
diagnosis of **macular dysfunction** and assesing extent of **central retinal involvement** in generalized retinal disease by producing a topographic electroretinogram (ERG) map of **central retinal cone-system function**.
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What does VEP and VER test?
visual evoked potential or visual evoked response is a measurement of the electrical signals produced in the brain in response to light stimulus recorded at the scalp over the occipital cortex - measures the **integrity of the afferent visual pathway.**
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Severity
Severe NPDR - venous beading and intraretinal microvascular abnormalities (IRMA) are present
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standard ETDRS photo for **hemorrhages with or without microaneurysms (HMA)** eyes with severe NPDR have this in all four midperipheral quadrants
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Standard photograph ETDRS for less severe of two standards for venous beading. 2 main branches of superior temporal vein show beading that is definite but not severe.
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Standard for moderate IRMA (ETDRS). Patients with severe NPDR have moderate IRMA of at least this severity in at least one quadrant.
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Disorder characteristcally a/w negative ERG waveform?
X-linked retinoschisis
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a-wave normal and selectively reduced b-wave in ERG
negative ERG - seen in CSNB, X-linked retinoschisis, paraneoplastic retinopathy, CRAO
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AREDS demonstrated a benefit of antioxidant and zinc nutritional supplementation for what category of AMD?
* bilateral intermediate * unilateral advanced nonexudative AMD
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An eye with high myopia has posterior staphyloma and macular detachement that does not extend beyond the vascular arcades. Peripheral retina is attached without any breaks. Most likely associated macular finding?
**macular hole** MH in an eye with high myopia and posterior staphyloma can lead to rhegmatogenous macular detachment
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Complication from retrobulbar anesthesia
**respiratory depression** from brainstem suppression with respiratory arrest
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Diagnosis
**proliferatie diabetic retinopathy** (NVD with nasal fibrous proliferation along vascular arcades)
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Causes of inherited peripheral retinal ischemia
Norrie disease familial exudative vitreoretinopathy incontinentia pigmenti
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Indication best supports decision to proceed with vitrectomy surgery
individual symptoms and impact on lifestyle - functional vision loss (ERM with macular pucker)
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Ppx laser barricade for lattice should be considered when..
h/o RD in fellow eye - only 1% per year in 11 year f/u for lattice and no symptomatic tears
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Kearns-Sayre syndrome
CPEO + pigmentary retinopathy (ptosis, ocular motility impaired, heart block)
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When atrophy develops in RPE, what other tissue undergoes atrophy?
**choriocapillaris** RPE-outer segment-choriocapillaris are interdependent on each other
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ROP first screening should occur during this time for a 29 week gestation baby?
4-6 weeks after birth **4-6 weeks of postnatal age** or **between 31 and 33 weeks postconceptional or postmenstrual age**, whichever is later.
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Most common cause of dense vitreous hemorrhages
PDR
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Therapy safe and effective for treatment of the condition illustrated in the ETDRS study
focal laser photocoagulation
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Drug used to treat MS that can cause cystoid macular edema with typical late leakage in "petaloid" or flower-shaped pattern
fingolimod
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Pathology of condition shown
elastic degeneration and pathologic calcification of bruch membrane
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"VEGF trap"
aflibercept
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Diagnosis
Exudative AMD
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Binds to VEGF isoform 165
Pegaptanib (Macugen)
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bind to all isoforms of VEGF (nonspecific inhibitors of VEGF)
Ranibizumab (Lucentis) Bevacizumab (Avastin)
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Mechanism of traumatic avulsion of the optic nerve
**sudden increase in IOP causing rupture of the lamina cribosa** extreme rotation and forward displacement of globe penetrating orbital injury causing backward pull on ON
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19 yo acute reduction in central vision, central scotoma, yellow spot in central macula
alkyl nitrites
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Drugs a/w serous macular fluid that may simulate central serous choroidopathy
MEK inhibitors (chemotherapeutics) Sildenafil
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Name 3 recognized categories of this disease
**(1) vitreomacular adhesion (VMA)** **(2) vitreomacular traction (VMT) syndrome** **(3) macular hole** OCT!
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strongest additional indication for treating lattice degeneration wtih laser retinopexy and/or cryopexy
symptomatic retinal tear with or without a PVD
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Where is a retinal tear most likely to be found?
posterior edge of the vitreous base
241
What sickle cell hemoglobinopathy is most commonly associated with proliferative sickle cell retinopathy?
**Sickle cell hemoglobin C (SC)** - **33%** Sickle cell thalassemia (SThal) - 14% Sickle cell homozygote (SS) disease - 3%
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Can induce microcystic form of macular edema that is detected on SD-OCT and does not leak on FA
taxanes (docetaxel, paclitaxel) niacin
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can cause severe pigmentary retinopathy that can develop within a few weeks or months of high-dose utilization
the phenothiazine **thioridazine** concentrated in uveal tissue and RPE by binding to melanin granules
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Feature of choroid that mitigates potential thermal damage a/w light absorption
rapid blood flow
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BRVO at a site other than an arteriovenous crossing
vasculitis
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Preferred surgical intervention
star folds from PVR - complication of RD that occurs in 8% to 10% of cases. RD with PVR represents a combined TRD and RRD which needs a **scleral buckle and vitrectomy**
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In what setting would a scleral buckle and retinal cryopexy with or without drainage of subretinal fluid have a high rate surgical success?
a RD caused by traumatic inferior small dialysis in a young patient without a PVD
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Indication for course of systemic therapy with antimicrobials and corticosteroids?
* retinitis near the macula * immunocompromised patient * dense vitreous opacity * large lesions greater than 1 DD * multiple lesions * decreased VA * frequent recurrences
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Effect that demyelating optic neuritis has with recovered VA on pattern-reversal visual evoked potential (VEP) testing?
delayed pattern-reversal VEP
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A macular “double ring” sign may be seen with the inner ring caused **sub-ILM hemorrhage** and the outer ring caused by **sub-hyaloid hemorrhage**
251
What causes this finding?
Mutliple **intraretinal** and **preretinal** hemorrhages in a patient with Terson syndrome * 33% (BCSC) of **SAH or SDH have intraocular heme** * 9.1% of **intracerebral hemorrhages** * 3.1% of **traumatic brain injury**
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Subhyaloid heme
between posterior vitreous base & internal limiting membrane
253
Pre-retinal heme
posterior to internal limiting membrane & anterior to nerve fiber layer
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Subretinal heme
heme in space between neurosensory retina & retinal pigment epithelium (have amorphous shape due to absence of firm attachments between neurosensory retina & RPE).
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Sub-RPE heme
heme located between RPE and Bruch’s membrane (have well-defined borders because of tight cell junctions between RPE cells).
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“D or boat-shaped retinal heme”
Subhyaloid & pre-retinal hemorrhages
257
dark color retinal heme
**Subretinal & subretinal pigment epithelium (RPE)** hemorrhages * **Subretinal heme** – heme in space between **neurosensory retina & RPE** * **Sub-RPE heme** – heme located between **RPE** and **Bruch’s membrane**
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DDx
* Choroidal neovascular membrane formation * Neurosensory or RPE detachments * Wet age-related macular degeneration * Choroidal tumors * Trauma
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DDx
Subhyaloid & pre-retinal hemorrhages = “D or boat-shaped retinal heme” ## Footnote Location: Subhyaloid heme – between posterior vitreous base & internal limiting membrane. Pre-retinal heme – posterior to internal limiting membrane & anterior to nerve fiber layer. *Possible etiologies:* Retinal neovascularization Posterior vitreous detachment & retinal breaks (result of tearing the major retinal vessels) Terson’s syndrome (vitreous heme + subarachnoid heme) Retinal trauma Valsalva retinopathy
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DDx
Location: within nerve fiber layer (resolve around six weeks) *Possible etiologies:* Hypertensive retinopathy Retinal vein occlusions Papilledema Normal-tension glaucoma Anterior ischemic optic neuropathy
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Location DDx
Location: **inner nuclear & outer plexiform layers** (resolve time is longer than flame-shaped hemes) Diabetic retinopathy Vein occlusion Idiopathic juxtafoveal retinal telangiectasis Ocular ischemic syndrome
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Most common complication of Terson syndrome
**epiretinal membrane 15-78%** Retinal folds/perimacular folds - 20% RD - 9% Ghost cell glaucoma 4%
263
Most common risk factor for development of BRVO
**Hypertension** Cardiovascular disease Hyperlipidemia Glaucoma
264
Mechanism in this disease
**acute rise in intraocular venous pressure resulting in rupture of peripapillary and retinal vessels.** abrupt change in intracranial hemorrhage
265
Mechanism of the following finding
**Purtscher's retinopathy -** Granulocyte aggregation, leukoembolization, fat embolization are not thought to play a role like in purtscher retinopathy * Cotton-wool spots * Peri-papillary retinal whitening with macular involvement * Pre-retinal and/or retinal hemorrhages * Macular edema * Dilated retinal venous system
266
Name of finding for white arrow
Purtscher's flecken - areas of whitening in the inner retina between the retinal arterioles and venules.
267
systemic abnormality present
Retinal neovascularization with a sea fan configuration
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Proliferative sickle cell retinopathy (PSR) is catagorized based on the following pathogenetic sequence
1. **Peripheral arteriolar occlusions** (stage 1) lead to peripheral nonperfusion 2. Peripheral arteriovenular **anastomoses** which are dilated preexisting capillary channels 3. **Preretinal** **sea fan neovascularization** (stage 3) may occur at the posterior border of areas of nonperfusion and lead to 4. **Vitreous hemorrhage** (stage 4) AND 5. **Tractional retinal detachment** (stage 5)
269
complications of PDT
vascular thrombosis (damage to endothelial cells) TVL RPE atrophy Choroidal ischemia CNV
270
Half vs full fluence PDT settings
half - 25 J/cm2 full - 50 J/cm2
271
measurement of electrical signal in the brain in response to a light stimulus recorded at the scalp over the occipital cortex; measure of integrity of afferent visual pathway
VEP or VER
272
40 yo M presents with A. later develops CNVM and disciform scar. Mutation?
TIMP3 - Sorsby macular dystrophy
273
Doyne honeycomb dystrophy, Malattia Leventinese, and radial drusen (all same) mutation?
EFEMP1
274
Mutation in CSNB
NYX
275
mutation in complement factor H with early onset drusen
**cuticular drusen** - small clustured drusen in young aults
276
What 2 adhesion molecules attach the vitreous to the ILM of the retinal surface
**fibronectin and laminin**
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molecule shown to induce PVD
integrin
278
Ciliopathy a/w pigmentary retinopathy + hearing loss
Usher syndrome
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pigmentary retinopathy + hearing loss
Usher syndrome
280
pigmentary retinopathy + bull's eye maculopathy + obesity + polydactyly + hypogonadism + renal disease + MR
Bardet-Biedle syndrome
281
white dot with profound VL followed by full or near-full vision recovery
APMPPE acute posteior multifocal placoid pigment epitheliopathy 'block early' 'stain late'
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white dot with insidious onset and chronic progression
birdshot uveitis
283
white dot that is unilteral, decreased vision, scotomas, +/-photopsias improvement over 2-6 weeks without treatment
MEWDS
284
white dot that is relentlessly progressive, destroys RPE and photoreceptors. Vision recovery atypical.
Serpiginous chorioretinopathy
285
Cx more often seen after laser PRP for proliferative sickle cell retinopathy than for diabetic retinopathy
Retinal tear and subsequent RRD