Retina Flashcards
(187 cards)
1
Q
What are the effects of epiretinal membrane (ERM) and its treatment?
A
Effects of ERM
● Cystoid macular edema (CME) → occurs due to tractional forces exerted by ERM on retinal blood vessels running through nerve fiber layer
Treatment
● Vitrectomy with membrane peel +/-internal limiting peel
● Removing internal limiting membranes prevents recurrence of ERM
2
Q
What is central areolar choroidal dystrophy?
A
● Autosomal dominant
● Mutations in peripherin/RDS gene
● Onset -middle age
● Electrooculography and electroretinography are normal
● Normal choroidal flush
● Visual acuity ranges from 20/25 to 20/200
● Mild nonspecific granularity of fovea → slowly progresses to pathognomonic round zone of neurosensory RPE and choriocapillary atrophy
3
Q
What are the electrooculography (EOG) and electroretinography (ERG) findings in Best’s disease?
A
● EOG abnormal
● ERG normal
4
Q
What is Stargardt’s disease?
A
● Autosomal recessive (most common); ABCA4 gene mutation -codes for ATP binding cassette transporter protein expressed by rod outer segments → accumulation of lipofusin in RPE → limits RPE function and viability
● Diffusely “dark choroid” on fluorescein angiogram -retinal vessels are highlighted against a hypofluorescent choroid due BLOCKAGE from diffusely distributed lipofuscin. >80% have this
● Visual acuity ranges from 20/50-20/200
● Macular atrophy (beaten bronze) discrete yellowish pisciform flecks at level of RPE
● Flecks widely distributed in fundus = fundus flavimaculatus
● Vitamin A increases rate of lipofuscin pigment accumulation in RPE, so avoid!
5
Q
What age does North Carolina macular dystrophy present and what is its range of visual acuity?
A
● Presents in late puberty
● Visual acuity ranges from 20/20-20/200
6
Q
What is central serous chorioretinopathy?
A
● Well-delineated serous retinal OR RPE detachments
● More common in males; age 25 to 55
○ Symptomatic if macula is affected → central vision loss, metamorphopsia, hyperopic shift, central scotoma, decreased color vision
○ Most commonly bilateral and asymmetric
● Risk factors
○ Type A personality
○ Exogenous or endogenous steroids
○ Stress
○ Organ transplantation
○ Systemic lupus erythematosus
○ Systemic hypertension
○ Sleep apnea
○ GERD
○ Psychopharmacologic medications
○ Pregnancy
● Visual prognosis is good except → chronic, recurrent or bullous
● 80-90% undergo spontaneous resorption of subretinal fluid within in 3 months
○ Observe initially → if fluid persists > 3-6 months → treatment with thermal laser photocoagulation, photodynamic therapy or systemic medications
● Most common fluorescein angiogram pattern “expansile dot” pattern
● Least common fluorescein angiogram pattern “diffuse pattern”
● Fluorescein angiogram pattern “smokestack” pattern occurs in 10% of cases
● OCT macula shows serous retinal detachment, serous RPE detachment, and thickened choroid
7
Q
What is the differential diagnosis for bull’s eye maculopathy?
A
● Cone or cone-rod dystrophies
● Hydroxychloroquine and chloroquine toxicity
● Chronic macular hole
● Central areolar choroidal dystrophy
● Olivopontocerebellar atrophy
● Age-related macular degeneration
● Ceroid lipofuscinosis
● Bardet-Biedl syndrome
● Stargardt disease
● Traumatic maculopathy
● Leber congenital amaurosis
8
Q
What are the screening methods for chloroquine and hydroxychloroquine retinopathy?
A
Visual fields
● 10-2 SITA for non-Asians
● 24-2 or 30-2 SITA for Asians patients; toxicity often manifests beyond macula in Asians
ERG
● Multifocal ERG
Autofluorescence
● Increased autofluorescence in parafoveal or extramacular areas may precede areas of thinning on OCT. Late RPE loss appears as area of reduced autofluorescence
● Most frequent region of retina to show early damage is inferotemporal -corresponds to superonasal field defect
SD-OCT macula
● Localized thinning of photoreceptor layers in parafoveal in non-Asians; near arcades in Asian
9
Q
What is the differential diagnosis for cystoid macular edema?
A
● Post-op cataract surgery
● Diabetes
● BRVO or CRVO
● Uveitis
● Post PRP
● Retinitis pigmentosa
● Subretinal disease
● Niacin supplementation
10
Q
What is an optic pit?
A
● Small, hypopigmented, round, excavated colobomatous defects of the optic nerve
● Most commonly located inferotemporal
● Associations
○ Serous macular detachments (fluid contained is either liquid vitreous or CSF) occurs in 25-755 of cases → poor prognosis if left untreated
○ Paracentral or arcuate scotoma
○ Macular schisis
● Treatment
○ Vitrectomy with gas-bubble placement
11
Q
What are the 5 most common indications for pars plana vitrectomy in diabetic patients?
A
● Non-clearing (dense) vitreous heme
● Tractional retinal detachment involving or threatening macula
● Diffuse diabetic macular edema associated with posterior hyaloid traction
● Combined tractional and rhegmatogenous RD
● Significant recurrent vitreous hemorrhage despite maximal PRP
12
Q
What is the histologic definition of macula?
A
Region with more than one ganglion cell layers
13
Q
What is the histological definition of fovea?
A
● Depression in the inner retinal surface and consists only of cones
● 1.5 mm diameter
14
Q
What is the histologic definition of foveola?
A
● Central floor of fovea
● Inner nuclear layer and ganglion cell layer are absent
● 0.35mm in diameter
15
Q
What is the histologic definition of umbo?
A
Central concavity of floor of foveola
16
Q
What is abetalipoproteinemia?
A
Etiology
● Inability to synthesize apolioprotein B → fat malabsorption → fat-soluble vitamin deficiency (A, D, E, K), and retina and spinocerebellar degeneration
Genetics
● Autosomal recessive
● Mutation in microsomal triglyceride transfer protein
Clinical findings
● Pigmentary retinopathy
● Foul-smelling stools/fat malabsorption
● Ataxia
● Growth retardation
Work up
● Vitamin A levels
Treatment
● Supplementation of vitamin A and E
17
Q
What is the most important indicator of visual prognosis in BRVO?
A
Extent of capillary nonperfusion → predictor of NV
18
Q
What are idiopathic epiretinal membranes?
A
● Transparent, avascular, fibrocellular membrane on the inner retinal surface that adheres to and covers internal limiting membrane (ILM) of retina
● Always associated with posterior vitreous detachment
● 2% over age 50
● 20% over age 75
● Histologic findings: Muller cells (glial cells), fibrous astrocytes, fibroblasts, macrophages, hyalocytes, RPE
● Treatment
○ Vitrectomy, epiretinal membrane peel, ILM peel
● Cannot regrow on surface of nerve fiber layer
19
Q
What is Gyrate atrophy?
A
Genetics
● Autosomal recessive
● Mutation of OAT gene, chromosome 10
Cause
● Elevated ornithine which is toxic to retina
Clinical presentation
● Geographic paving-stone-like areas of atrophy that coalesce to form scalloped border between abnormal and normal RPE
● Hyperpigmentation of remaining RPE
● Diffuse loss of RPE and choroid
● No retinal vessel attenuation, unlike RP
● Posterior subcapsular cataract
● High myopia
● Vision (night blindness) becomes abnormal around age 10
Treatment
● Arginine and B6 restriction
20
Q
What is uveal effusion syndrome?
A
Definition
● Reduced transsclearal aqueous outflow → alteration in net water movement across the vitreous cavity and posterior eye wall
Etiology
● Abnormal scleral composition or thickness
Associations
● Nanophthalmos
● Scleritis
● High hyperopia
● Idiopathic uveal effusion syndrome
● Glaucoma
Clinical findings
● Abnormal episcleral vessels
● Choroidal and ciliary body thickening
● RPE alterations
● Exudative retinal detachment; ciliochoroidal detachment
● “Leopard-spot” pattern without leakage on fluorescein angiography
21
Q
What is associated with Alagille syndrome?
A
● Cholestasis
● Posterior embryotoxon, Axenfeld anomaly
● Pigmentary retinopathy
● Congenital heart disease
● Flattened facies
22
Q
What is associated with myotonic dystrophy?
A
● Ptosis
● Cardiac conduction defects
● frontal balding
● Christmas-tree or polychromatic cataract
● Ophthalmoplegia
● Pigmentary retinopathy
23
Q
What is Charcot-Marie-Tooth disease associated with?
A
● Degeneration of lateral horn of spinal cord
● Distal muscle weakness or wasting
● Kyphosis or scoliosis
● Pigmentary retinopathy
● Optic atrophy
24
Q
What is cystinosis?
A
● Autosomal recessive
● Defect in transport out of lysosomes → intralysosmal cystine accumulates
● Three forms:
○ Benign
○ Late-onset
○ Nephropathic → retinopathy is only found in this form which is not visually significant
○ All forms have corneal and conjunctival crystals
● Treatment
○ Cysteamine
25
What are the preferred argon laser color treatments for different retinal pathologies?
● Vitreous hemorrhage: red
● Macular edema due to diabetes or vein occlusion: green or yellow
● Choroidal neovascularization: green; red if blood present
● Coats disease: yellow
26
What are the risk factors for retinal detachment (RD) following cataract surgery?
● Younger age (attached vitreous)
● Vitreous loss during surgery (20-fold increase)
● Zonular dehiscence
● RD in fellow eye
● Axial length >23mm
● Male gender (PVD less common)
27
What are signs of intraocular copper toxicity, "chalcosis"?
● Very toxic to the eye
● Deposits in Descemet's membrane
● Sunflower cataract
● Greenish color of iris
● Brown vitreous opacities
● Metallic flecks on retinal vessels
● Acutely toxic to retina → can lead to loss of an eye
28
What are the side effects of intravenous fluorescein?
● Temporary yellowing of skin and conjunctiva: All patients
● Bright orange discoloration of urine for the first 24 to 36 hours: All patients
● Nausea and vomiting: 10%
● Urticarial reactions: 1%
● Anaphylactic reaction: < 1:100,000
29
What is the differential diagnosis for neuroretinitis?
● Idiopathic (most common) AKA Leber's idiopathic stellate neuroretinitis
● Sarcoidosis
● Syphilis
● EBV
● Lyme
● Histoplasmosis
● Toxocariasis
● Toxoplasmosis
● Bartonella henselae
30
What is associated with Bardet-Biedl syndrome?
● Macular pigment mottling
● Polydactyly
● Obese
● Intellectual disability
● Hypogonadism
● Renal failure
31
What are the differences between Laurence-Moon syndrome and Bardet-Biedl syndrome?
Laurence-Moon syndrome is the same as Bardet-Biedl syndrome except it has spastic paraplegia and does not have polydactyly
32
What is Alstrom syndrome?
● Autosomal recessive
● Tapetoretinal degeneration
● Central vision is lost early
Associations
● Obesity
● Diabetes
● Hearing loss
● Renal failure
● Dilated cardiomyopathy
33
Which forms of sickle cell disease result in the most serious ocular complications (i.e. tractional retinal detachment, vitreous hemorrhage)?
Sickle cell hemoglobin C (SC) and sickle cell thalassemia (SThal)
34
What is Usher syndrome?
● Sensorineural deafness plus retinitis pigmentosa (RP)
● 10% cases of RP
● Deafness is always congenital
35
What are the treatment options for cystoid macular edema associated with retinitis pigmentosa?
● Oral carbonic anhydrase inhibitors (CAIs)
● Topical CAIs
● Topical NSAIDs
36
What is the most common cause, presentation, and prognosis of post traumatic endophthalmitis?
Cause
● Bacillus cereus
● 25% of post traumatic endophthalmitis is due to Bacillus cereus
Presentation
● Rapid and severe course
Prognosis
● Very poor
37
What is a Birdshot fluorescein phenomenon?
"Quenching" of fluorescein dye → quick disappearance of fluorescein dye from retinal circulation
38
What is an APMPPE fluorescein phenomenon?
Blockage in early frames with diffuse late staining
39
What is a serpiginous fluorescein phenomenon?
Blockage in early frames but early hyperfluorescence at edges of lesions
40
What is a MEWDS fluorescein phenomenon?
"Wreath-like" clusters of spots associated with each retinal lesion
41
What is a sugiura sign?
● Perilimbal vitiligo
● Seen in convalescent phase of VKH syndrome
42
What are foster-fuchs spots?
Spots in the macula of pathologic myopia due to RPE hyperplasia after subretinal neovascularization or hemorrhage
43
Dalen fuch nodules are seen in which conditions?
VKH and sympathetic ophthalmia
44
What is bilateral diffuse uveal melanocytic proliferation (BDUMP)?
● Bilateral diffuse thickening of choroid
○ Reddish/brownish choroidal discoloration
● Resemble large choroidal nevi
● Associations
○ Serous retinal detachment
○ Cataracts
○ Most commonly seen in the following cancers: ovarian cancer, uterine cancer, lung cancer, colon cancer, pancreatic cancer, gallbladder cancer, esophageal cancer
45
What is the mechanism of mechanical light damage to retina and when is it seen?
● Absorbed light is strong enough to produce gas bubbles → result in shock wave to mechanically disrupt tissues
● Seen with YAG capsulotomy
46
What is the mechanism of thermal light damage to retina and when is it seen?
● Absorbed light causes rise in temperature of surrounding tissues
● Seen with PRP
47
What is the mechanism of photochemical light damage to retina and when is it seen?
● Biochemical reactions that cause destruction without rise in temperature
● Seen with solar retinopathy and overexposure from operating microscope
48
What are the ocular associations with pseudoxanthoma elasticuma (PXE)?
● Peau d'orange fundus
● Angioid streaks
● Optic drusen
● Round atrophic scars
● Vision loss due to angioid streaks from submacular hemorrhages or choroidal neovascular membranes
● Higher risk of choroidal rupture even after minor ocular trauma
49
What is Behcet's disease?
Systemic manifestations:
● Recurrent oral ulcers
● Epididymitis
● Skin lesions (erythema nodosum)
● CNS involvement
● Arthritis
Ocular manifestations:
● Hypopyon
● Occlusive vasculitis
● Retinal necrosis
● Ischemic optic neuropathy
● Severe vitritis
Associations
● HLA-B51
50
What is Best disease?
Genetics
● Autosomal dominant
● Mutation in VMD2/BEST 1 gene
● Protein codes for bestrophin → transmembrane chloride channel located in basolateral membrane of RPE
Clinical findings
● Yolk-like (vitelliform) macular lesion in childhood and eventually breaks down into atrophic appearance
● Good VA in majority of cases
● 20% will develop choroidal neovascular membrane which reduces vision to 20/200
● Electroretinogram normal
● Electrooculogram (EOG) abnormal (Arden ratio <1.5): Abnormal pumps on RPE, although 37% with Best disease can have normal EOG
51
What is the gene that is mutated in Sorsby macular dystrophy?
TIMP3
52
What is the Geneva study?
● Randomized clinical trial, 1267 patients
● Evaluated dexamethasone (Ozurdex) intravitreal compared to sham intravitreal in eyes with vision loss due to macular edema (ME) associated with BRVO or CRVO
● Results: Ozurdex reduces risk of vision loss, improve speed and incidence of visual improvement in eyes with ME associated with BRVO or CRVO
53
What is the timing of retinopathy of prematurity (ROP) screening?
Dilated fundus exam 4 to 6 weeks of postnatal age or within 31st to 33rd week of postconceptional or postmenstrual age, whichever is later
54
What are the clinical findings most predictive of progression to proliferative diabetic retinopathy?
● Venous beading
● Intraretinal microvascular abnormalities (IRMA)
55
What are the most common causes of spontaneous vitreous hemorrhage, in decreasing order?
● Diabetic retinopathy
● Retinal break without detachment
● Posterior vitreous detachment
● Rhegmatogenous retinal detachment
● Neovascularization after BRVO or CRVO
56
What is different about the temporal nerve fibers in all forms of albinism?
The fibers decussate instead of projecting to ipsilateral lateral geniculate nucleus
57
What is Chediak-Higashi syndrome?
● Life threatening condition associated with albinism
● Autosomal recessive
● Mutation in lysosomal trafficking regulator in CHS1/LYST gene
● Albinism plus neutropenia
Associations
● Susceptibility to infections
● Bleeding and coagulation defects
● Ataxia
● Peripheral neuropathy
● Mucosal defects: gingivitis, oral ulcers, periodontal disease
● Many patients do not survive past early childhood
58
What is Hermansky-Pudlak syndrome?
“Four P’s”
Platelet defect
Pulmonary fibrosis
Puerto ricans
Pigmentless
● Life threatening condition associated with albinism
● Albinism plus platelet defect
Associations
○ More common in Puerto Rican descent
○ Pulmonary fibrosis
○ Granulomatous colitis
○ Immunodeficiency
59
What are the similarities and differences between AREDS versus AREDS-2 formulation?
● AREDS reduced risk of progression from intermediate or advanced to more advanced ARMD by 25%
● 19% risk reduction in rate of moderate vision loss at 5 years
60
What are the clinical findings in pathologic myopia (-8.00 or more, axial length >32.5mm)?
● Foster-Fuchs spots
● Optic disc tilt
● Peripapillar atrophy
● Lacquer cracks
● Posterior staphyloma
● Atrophy of RPE or choroid
● Elongation or atrophy of ciliary body
● Lattice
● Paving-stone degeneration
● Choroidal neovascularization
● Peripheral retinal holes
61
What is the clinical feature suggestive of exudative retinal detachment?
Shifting subretinal fluid
62
What are the clinical features suggestive of rhegmatogenous retinal detachment?
● Low IOP
● Early extension from ora serrata to disc
63
What is retinal racemose angioma or congenital retinal arteriovenous malformation?
● Unilateral
● Non-hereditary
● No intervening capillary bed between retinal arteries and veins
● Poor vision if fovea is involved
● Risk for obstruction and neovascularization
● Rapid venous filling on fluorescein angiogram; does not leak
● Associated with Wyburn-Mason syndrome → ipsilateral vascular malformations of brain, orbit, and mandible
64
What is type 1 parafoveal (juxtafoveal) retinal telangiectasia?
● AKA Leber miliary aneurysm
● Occurs greater in males than females
● Unilateral
● Congenital or acquired
● Resembles macular variant of Coats disease with circinate type of exudate
● Photocoagulation treatment can be successful in resolving exudation
65
What is type 2 parafoveal (juxtafoveal) retinal telangiectasia?
● No sex predilection
● Bilateral
● Perifoveal retinal changes (more pronounced in temporal fovea)
○ Thickening of fovea
○ Loss of transparency with grayish appearance of fovea
○ Small telangiectatic vessels in the fovea
○ Cystic appearance of fovea
● 1/3 patients have abnormal glucose tolerance test
● Does not respond to photocoagulation treatment because leaky vessels are not predominant feature
● May respond to anti-VEGF
66
What is type 3 parafoveal (juxtafoveal) retinal telangiectasia?
● Bilateral
● Vision loss from retinal perifoveal capillary obliteration
● On fluorescein angiography, telangiectatic vessels are readily apparent and leak
● OCT mac with thinned central macular retina, including fovea with inner lamellar oblong foveal cavitations in which long axis is parallel to retinal surface
● Does not respond to photocoagulation treatment because leaky vessels are not a predominant feature
● May respond to anti-VEGF
67
What are the common features among all 3 types of parafoveal (juxtafoveal) retinal telangiectasia?
● Focal retinal gliosis and telangiectasia of capillary bed
● Vision loss from capillary incompetence and exudation
● Refractile changes (“retinal crystals”) in advanced cases
● Histologic changes: not true telangiectasia but rather structural abnormalities similar to diabetic microangiopathy with deposits of excess basement membrane within the retinal capillaries
68
What are the most common causes of diffuse unilateral subacute neuroretinitis (DUSN)?
● Baylisascaris procyonis
● Ancylostoma caninium
● Toxocara canis
69
Which retinal findings increase the risk of retinal tear or retinal detachment?
● Cystic retinal tufts
● Zonular traction retinal tufts
● Lattice degeneration
● Meridional folds
● Enclosed ora bays
● Peripheral retinal excavations
70
Which retinal findings do not predispose to retinal tear or retinal detachment?
● Non-cystic retinal tufts
● Cobblestone or pave stone degeneration
● RPE hyperplasia or hypertrophy
● Peripheral cystoid degeneration
71
What is the chance of severe non-proliferative diabetic retinopathy (NPDR) progressing to high risk proliferative diabetic retinopathy (PDR) over 1 year?
0.15
72
What is the chance that very severe non-proliferative diabetic retinopathy (NPDR) will progress to high risk proliferative diabetic retinopathy (PDR) over 1 year?
0.45
73
What is another name for birdshot retinochoriodopathy?
Vitiliginous chorioretinitis
74
Which conditions are associated with HLA-DR4?
● VKH
● Sympathetic ophthalmia
● Ocular cicatricial pemphigoid
75
What is the differential diagnosis for angioid streaks?
“PEPSI-HAM”
● Pseudoxanthoma elasticum
● Ehlers-Danlos syndrome
● Paget disease of bone
● Sickle cell anemia or other hemoglobinopathy (i.e. beta thalassemia)
● Idiopathic
● Homocysteinuria
● Acromeglay
● Marfans syndrome
Angioid streaks are breaks or irregularities of Bruch's membrane → leads to spontaneous subretinal hemorrhage. Choroidal neovascularization is major cause of permanent vision loss
76
When does retinal vascularization begin and end in a fetus or newborn?
● Process begins at 4 to 5 months of gestation
● Involves outward spread of mesenchymal cells from optic disc
● Vascularization is complete in the nasal quadrants at 36 weeks and temporal quadrants at 40 weeks
77
What is the clinical presentation and enzyme affected in Tay-Sachs disease?
● Defect in hexosaminidase A enzyme → leads to accumulation ganglioside GM2
● Cherry red spot due to abnormal protein in ganglion cells surrounding fovea
78
What is the material that accumulates in Batten disease?
Ceroid lipofusin
79
What is the treatment for bacillus cereus endophthalmitis?
● Intravitreal vancomycin (preferred) or intravitreal clindamycin
○ Resistant to penicillins and cephalosporins
80
What are the indications for intravitreal clindamycin?
● Ocular toxoplasmosis
● Bacillus cereus endophthalmitis
81
What is proliferative vitreoretinopathy (PVR)?
● PVR occurs when liberated RPE and glial cells grow on retinal surface to form membranes
● Cellular elements
○ Muller cells
○ Fibrous astrocytes
○ Macrophages
○ Fibroblasts
○ Myofibroblasts
● These membranes can contract leading to secondary breaks, fixed retinal folds, retinal shrinkage, and recurrent retinal detachments
82
What is melanoma associated retinopathy (MAR)?
● Paraneoplastic syndrome
● Predominantly affects rods
● Seen in patients with known history of melanoma
● Antibodies against bipolar cells in inner nuclear layer
● Color vision and central vision preserved
● Electronegative ERG
83
What is cancer associated retinopathy (CAR)?
● Paraneoplastic syndrome
● Affects both cones and rods (cones > rods)
● Most commonly associated cancers: small cell lung cancer, non-small cell lung cancer, endometrial cancer, ovarian cancer, cervical cancer, breast cancer, prostate cancer, colon cancer
● Visual field with ring scotoma
● Screening serology: anti-recoverin (most specific), anti-alpha enolase, anti-transducin
● Electronegative ERG
84
What are the causes of electronegative ERG (preserved a-wave and reduced b-wave)?
● Congenital stationary night blindness
● X-linked retinoschisis
● Paraneoplastic retinopathy
● Central retinal artery occlusion
● Birdshot retinochoroidopathy
● Methanol and Quinine toxicity
85
How is indocyanine green metabolized and what is its contraindication?
Metabolism
● Hepatically metabolized
● Stop metformin 1 to 2 days prior to ICG administration
Contraindication
● Allergy to iodine or shellfish
86
What is category 1 age-related macular degeneration (ARMD)?
● None or few small drusen (<63 microns)
● 0.45% chance of progression to advanced ARMD
87
What is category 2 age-related macular degeneration (ARMD)?
● Early ARMD
● Extensive small drusen or pigment abnormalities or at least 1 intermediate sized drusen (63 to 124 microns)
● 1.3% chance of progression to advanced ARMD by 5 years
88
What is category 3 age-related macular degeneration (ARMD)?
● Intermediate ARMD
● Extensive intermediate drusen or at least 1 large drusen (125 microns) or non-central geographic atrophy
● 18% chance of progression to advanced ARMD by 5 years
89
What is category 4 age-related macular degeneration (ARMD)?
● Advanced ARMD
● Clinical findings
○ Central geographic atrophy
○ Choroidal neovascularization
○ Serous or hemorrhagic detachment of neurosensory retina or RPE
○ Subretinal and sub-RPE fibrovascular proliferation
○ Disciform scar
● If advanced ARMD in 1 one eye then 43% chance of advanced ARMD in fellow eye at 5 years
90
What is vitreomacular traction?
● Subset of epiretinal membrane
● Predominantly involves glial cells
● Treatment
○ Surgical correction → visual improvement of 2 lines or more in 75%
○ Ocriplasmin: protease that targets fibronectin and laminin. Intravitreal injection
91
What are hereditary hyaloideoretinopathies?
● Premature vitreous breakdown → "optically empty vitreous cavity" except for thin layer of cortical vitreous behind the lens and threadlike, avascular membranes that run circumferentially and adhere to the retina
● High incidence of retinal detachment (RD) secondary to scattered lattice and retinal thinning
● Examples
○ Stickler syndrome (most common): Autosomal dominant. Mutation of COL2A1 encodes type II procollagen. High incidence of RD. Associated with cortical vitreous condensation firmly adherent to retina, myopia, open angle glaucoma, cataract, hyperextensibility and enlargement of joints, arthritis, and mild spondyloepiphyseal dysplasia. Orofacial findings -midfacial flattening, Pierre Robin malformation complex of cleft palate, micrognathia, and glossoptosis. RD’s are difficult to treat and tendency toward proliferative vitreoretinopathy
○ Wagner disease: Autosomal dominant. Not associated with systemic findings. Does not have predilection to RD. Associated with myopia, strabismus and cataract
○ Weill-Marchesani syndrome: Associated with systemic findings
○ Some varieties of dwarfism: Associated with systemic findings
92
Which choroidal neovascularization (CNV) pattern responded most to photodynamic therapy in Treatment of Age-Related Macular Degeneration PDT trial?
Classic CNV
93
What are the indications for prophylactic laser treatment for lattice degeneration?
● High myopia
● History of retina detachment in the fellow eye
● Flap tears within lattice
● Aphakia
94
What is cholesterolosis (synchysis scintillans)?
● Accumulation of yellow-white, gold, or multicolored cholesterol crystals in the vitreous and anterior chamber of eyes that have undergone previous accidental or surgical trauma causing large intraocular hemorrhage
● Crystals are highly refractile
● Frequently have posterior vitreous detachment → allows crystals to settle inferiorly (in contrast to asteroid hyalosis in which opacities are evenly distributed through the vitreous)
95
What are the susceptibility genes for age-related macular degeneration?
CFH and ARMS2
96
What is choroideremia?
Genetics
● X-linked recessive
● Mutation of CHM which encodes for geranylgeranyl transferase Rab escort protein
Clinical findings
● Widespread degeneration of RPE and choriocapillaris
● Night blindness and progressive peripheral visual field loss over 3 to 5 decades
● Most patients maintain good visual acuity
Treatment
● No treatment
● Promising results in gene therapy clinical trials, pending FDA approval
97
Which ERG tests are used to evaluate rod and cone function?
Cone function
● Measured with 30 Hz flicker response (rods do not respond over 20 Hz)
● Measured with photopic ERM, when the eye is light-adapted
Rod function
● Measured with dark adapted scotopic ERG
98
How is fluorescein angiography performed?
● Photographs of retina are taken following intravenous injection of sodium fluorescein, 2-5 mL of 25% solution or 5 mL of 10% solution
● A camera flash emits white light
● White light passes through blue (excitatory) filter -only blue light enters the eye
● Blue light (465-490 nm wavelength) excites unbound fluorescein molecules circulating in retinal and choroidal circulations
● Fluorescein molecules emit photons of light at 520-530 nm (yellow-green)
● Yellow-green light filter on the camera lens blocks the reflected blue light → only yellow-green light emitted by fluorescein molecules is imaged
99
What are the features of retinoschisis that differ from a retinal detachment?
● Absolute scotoma
● Positive laser uptake with photocoagulation
● Absence of corrugation
100
What are the differences between typical and reticular degenerative retinoschisis?
● Typical
○ Splitting in outer plexiform layer
● Reticular
○ Splitting in nerve fiber layer
○ More common to have posterior extension of schisis
101
What are the features of retinoschisis that would indicate treatment?
If there are both inner and outer layer holes
102
Foveal avascular zone beyond what diameter would cause vision loss?
> 1000 microns
103
Which clinical findings have the best prognosis for retinal reattachment after a retinal detachment?
● Small holes
● Retinal dialysis
● Associated with demarcation lines
104
Which clinical findings have the worst prognosis for retinal reattachment after a retinal detachment?
● Proliferative vitreoretinopathy
● Giant tears
● Choroidal detachment
● Inflammation
● Traumatic posterior breaks
105
What are the complications of excessive or misdirected PRP and its treatment?
Complications
● Corneal burns → lead to opacities
● Iritis, iris atrophy, anisocoria → thermal damage to long ciliary nerves in suprachoroidal space or iris sphincter muscle
● Lenticular burns and opacities
● Chorioretinal edema
● Exudative retinal and choroidal detachment
● Bruch membrane ruptures
● Cystoid macular edema
● Optic neuropathy
● Foveal burns
● Creation of retinal or choroidal lesions
Treatment
● Close observation with or without systemic steroids
106
What color of light is absorbed and not absorbed by xanthophyll?
● Xanthophyll readily absorbs blue
○ Avoid blue light laser photocoagulation in the macula
● Xanthophyll does not absorb red or yellow
107
What color of light is absorbed by hemoglobin?
Hemoglobin readily absorbs blue, yellow, and green
108
What color of light is absorbed by melanin?
Melanin readily absorbs yellow, green, red, and infrared
109
What is another name for tuberous sclerosis?
Bourneville-Pringle disease
110
What are the various intravascular plaques and their sources?
● Calcified plaque: heart valve or aorta calcification
● Hollenhorst plaque: carotid stenosis, aortic arch atheroma
● Platelet-fibrin plaques: aortic arch atheroma
111
What is Schwartz-Matsuo syndrome?
● Retinal tears involving vitreous base, shed photoreceptor outer segments
● Photoreceptors travel to anterior chamber
● Then photoreceptors are phagocytosed by endothelial cells of trabecular meshwork
● Results in secondary open angle glaucoma
112
What is the etiology of shunt vessels?
As venous return is obstructed, shunt vessels develop from dilation of pre-existing venous shunts between retina and choroid
113
What is the differential diagnosis for non-leaking cystoid macular edema on fluorescein angiogram?
● X-linked retinoschisis
● Niacin toxicity
● Taxanes (docetaxel, paclitaxel → anti-microtubule)
● Goldmann-Favre
● Phototoxicity
● Certain types of retinitis pigmentosa
114
Which medications cause pigmentary retinopathy?
● Thioridazine
● Phenothiazine
● Chlorpromazine
115
What is the differential diagnosis for crystalline retinopathy?
Systemic diseases
● Primary hereditary hyperoxaluria (primary oxalosis)
● Cystinosis
● Sjogren-Larsson syndrome
Drug-induced causes
● Tamoxifen
● Canthaxanthine (oral tanning agent)
● Talc retinopathy (methadone or meperidine)
● Nitrofurantoin toxicity
● Methoxyflurane (anesthetic) -secondary oxalosis
● Ethylene glycol ingestion (anti-freeze) -secondary oxalosis
● Intravitreal triamcinolone
● Long-term Kola nut ingestion
Ocular disease
● Bietti crystalline coreneoretinal dystrophy
● Calcified drusen
● Gyrate atrophy
● Retinal telangiectasis
116
What is poppers maculopathy?
● Due to alkyl nitrite
○ Class of recreational drugs
○ Inhaled to induce euphoric effect and enhance sexual arousal
● Present with central scotoma or photopsia
● Exam with yellow spot on the fovea
● Disruption of inner segment ellipsoid layer seen on OCT macula
● May or may not improve with time
117
What are complications of pars plana vitrectomy?
● Visually significant nuclear sclerosis cataract is seen in more than 90% of patients over 50 years of age within 2 years of vitrectomy
● Bacterial endophthalmitis in 1 out 2,000 cases
● Increased long term risk of open-angle glaucoma in 10-20%
● Retinal tears occur in less than 5%
● Intraoperative choroidal hemorrhage occur in less than 1%
118
What is the ERG finding in fundus albipunctatus?
Normalization of rod ERG with prolonged dark adaptation
119
What are the causes of chronic endophthalmitis?
● P. acnes
● Coagulase negative Staph
● Fungus (i.e. candida, aspergillus, torulopsis)
● Mycobacterium
120
What are the side effects of extensive, high power, small spot size, short duration PRP?
● Vitreous hemorrhage (perforation of blood vessels)
● Rupture of Bruch membrane (hemorrhage from choriocapillaris and chorioretinal neovascularization)
● Chorioretinal edema → serous retinal or choroidal detachment
● Tears of RPE
121
What is infantile Refsum disease?
Genetics
● Autosomal recessive
Etiology
● Phytanic acid storage disease → elevated serum phytanic acid level
Clinical findings
● Pigmentary retinopathy
● Reduced or extinguished ERG signals
● Nyctalopia -early symptom
● Cerebellar ataxia
● Polyneuropathy
● Anosmia
● Hearing loss
● Cardiomyopathy
Diagnosis
● Elevated plasma levels of phytanic acid
● Reduced phytanic acid oxidase activity in cultured fibroblasts
Treatment
● Dietary restriction of phytanic acid precursors → slow or stabilize the neuropathy but not retinal degeneration
122
What are predictive risk factors for malignant transformation of choroidal nevus?
● Nevus thickness (>2mm) -most important risk factor
● Subretinal fluid
● Symptoms -VF loss, photopsia, metamorphopsia
● Overling orange pigment
● Lack of overlying drusen
● Juxtapapillary location (<3 mm from optic disc)
● Basal dimension larger than 6 mm
● Low to medium internal reflectivity on A scan
● Absence of halo (circular band of depigmentation)
123
What are the risk factors for developing intraoperative choroidal hemorrhage?
● Systemic hypertension
● Tachycardia
● Obesity
● High myopia (axial length >26.5 mm)
● Anticoagulation
● Glaucoma
● Advanced age
● Chronic ocular inflammation
124
What are the indications for retinopathy of prematurity (ROP) screening?
● Birth weight 1500 g or less
● Gestational age 30 weeks or less
● Require supplemental oxygen
125
What is the etiology and examples of ciliopathy conditions?
Etiology
● Mutation in a gene needed for ciliary function affects multiple organs
Exampes
● Usher syndrome: retinitis pigmentosa plus congenital sensorineural hearing loss
● Bardet-Biedl syndrome: pigmentary retinopathy, bull’s-eye maculopathy, obesity, polydactyly, hypogonadism, renal disease, cognitive disability
● Joubert syndrome: retinal dystrophy, cerebellar malformation, hypotonic, renal disease
● Jeune syndrome: cystic kidney disease and asphyxiating thoracic dystrophy
126
What are the vitreous findings in primary intraocular lymphoma?
● Vitreous: Atypical lymphocytes, high IL-10 to IL-6 ratio (not diagnostic), immunohistochemistry with lambda or kappa light chains , flow cytometry with monoclonality
● Vitreous biopsy negative in 1/3 cases, if high suspicion then repeat it
127
What are the serologies used to test for syphilis and what are their interpretations?
● RPR: Indicator of active syphilitic infection
● FTA-ABS and MHA-TP: Confirms patient had the infection. Does not correlate with active disease. Results may be positive for a lifetime
128
What are the causes of cherry-red spot?
● Tay-Sach’s disease (deficient hexosaminidase A)
● Niemann-Pick disease (deficient sphingomyelinase)
● Quinine
● Central retinal artery occlusion
129
What do the different waves on an ERG represent?
● A wave = outer retina (photoreceptors)
● B wave = inner retina (Muller and bipolar cells)
● Oscillatory potentials → response from inner retina
130
What is protanopia?
● Missing L-cone (long-wavelength)
● 1% of males
● Red-green blindness
131
What is deuteranopia?
● Missing M-cone (medium wavelength)
● 1% of males
● Red-green blindness
132
What is tritanopia?
● Missing S-cone (short-wavelength)
● Autosomal dominant
● 0.001% population
● Blue-yellow blindness
133
What are the differences between RPEH (RPE hamartoma) and CHRPE (congenital hypertrophy of RPE)?
RPEH
● Seen in Gardner’s syndrome (autosomal dominant)
● Result of hyperplasia
● Bilateral
● More haphazard
● Irregular and jagged borders
CHRPE
● Solitary well demarcated, flat, darkly pigmented
● Non hereditary and sporadic
134
What are the risk factors for retinoblastoma metastasis and poor survival?
● Optic nerve invasion → most common route for extension out of the eye, increases risk of CNS metastasis by direct access or along nerve or seeding subarachnoid space
● Massive choroidal invasion → risk of hematogenous spread
● Direct extraocular extension
● Anterior chamber involvement
135
What are the clinical findings in leukemic retinopathy?
● Intraretinal and subhyaloid hemorrhages
● Hard exudates
● CWS
● White centered retinal hemorrhages (pseudo-Roth spots)
● Leukemic infiltrates → yellow-white deposits in the retina and subretinal space
● Perivascular leukemic infiltrates produce gray-white streaks
● Clinical findings are a result of anemia, hyperviscosity and thrombocytopenia
● Vitreous involvement is rare
● Diagnostic vitrectomy can be performed for diagnosis
136
What is the presentation, work up, and treatment for leukemic infiltration of the optic nerve?
Presentation
● Ophthalmic emergency
● Severe vision loss and optic nerve edema
Work up
● Systemic imaging, CNS assessment including lumbar puncture with cytology, bone marrow evaluation → necessary to confirm diagnosis
Treatment
● Urgent external beam radiation of optic nerves plus systemic and intrathecal chemotherapy
137
What is a melanocytoma?
● Benign
● Magnocelluar nevi
● Location: Eccentric over optic nerve head, iris, ciliary body, choroid
● Description: Large, polyhedron-shaped nevus cells that have small nuclei and abundant cytoplasm filled with large melanin granules
● Clinical findings: APD and visual field defects
138
What is the mechanism of action of aflibercept (Eylea)?
● Also known as VEGF Trap
● Soluble protein that acts as a VEGF receptor decoy
○ Nonspecific VEGF receptor analog
○ Binds both VEGF and placental like growth factor
139
What is the mechanism of action of banibizumab (Lucentis) and bevacizumab (Avastin)?
● Bind to all isoforms of VEGF and nonspecific inhibitors of VEGF
140
What is the mechanism of action of pegaptanib (Macugen)?
Aptamer, binds to VEGF isoform 165
141
What are the systemic associations with asteroid hyalosis?
● Diabetes
● Hypertension
142
How does optical coherence tomography (OCT) function?
● Uses interference of broadband or tunable coherent light to generate optical sections of retina and cornea
● Optical analogue to ultrasound imaging → uses infrared light instead of sound
143
What is the definition of clinically significant macular edema (CSME) based on the Early Treatment Diabetic Retinopathy Study?
● At least one of the following:
○ Retinal thickening within 500 μm of macular center
○ Hard exudates within 500 μm of macular center, with adjacent retinal thickening
○ One or more disc diameters of retinal thickening, part of which is within 500 μm of the macular center
144
What are the etiologies of endogenous endophthalmitis and their prevalence?
● North America: 40% of endogenous endophthalmitis are due to bacterial endocarditis secondary to staph or strep
● Asia: 60% of endogenous endophthalmitis are due to liver abscess secondary to Klebsiella pneumoniae
● Other cases -urinary tract infection, intravenous drug use, indwelling catheter
145
What are the causes of acute retinal necrosis?
● VZV (most common)
● HSV-1
● HSV-2
● CMV (rare)
● Syphilis (treponema pallidum)
146
Which areas of the vitreous are most firmly attached?
● Vitreous base
● Optic disc margin
● Major blood vessels
● Edges of lattice degeneration
● Chorioretinal scars
147
What are the different sickle cell variants and their respective risks of retinopathy?
● Sickle cell hemoglobin C (SC) → 33%
● Sickle cell thalassemia (SThal) → 14%
● Sickle cell homozygous (SS) → 3%
● Sickle cell trait (AS) → rare
148
What is von Hippel Lindau disease?
Genetics
● Tumor suppressor gene mutation
● Chromosome 3
● Autosomal dominant
Another name
● Cerebello retinal angiomatosis
Associations:
● Retinal capillary hemangioblastomas
● Angiomatous lesions of CNS
● Cerebellar hemangioblastoma
● Pancreatic cysts
● Renal cell carcinoma
● Pheochromocytoma
Primary cause of death
● Cerebellar hemangioblastomas
● Renal cell carcinoma
149
What were the conclusions of the United Kingdom Prospective Diabetes study (UKPDS)?
Intensive glycemic control in type 2 diabetes reduced rate of retinopathy, nephropathy, and neuropathy
150
What were the conclusions of the Diabetes Control and Complications Trial (DCCT)?
Reduction in complication rates in type 1 diabetes under strict glycemic control
151
What is the description of circumscribed choroidal hemangioma on ultrasound?
● Highly reflective mass
● Uniform high internal spikes
152
What are the indications for pneumatic retinopexy?
● Confidence that all retinal breaks have been identified
● Retinal breaks are confined to superior 8 clock-hours
● Multiple breaks within 1-2 clock hours
● Absence of proliferative vitreoretinopathy grade CP or CA
● Cooperative patient who can maintain proper positioning
● Clear media
153
What are the stages of macular hole?
● Stage 0: vitreomacular adhesion
● Stage 1A: foveal pseudocysts with foveal traction
● Stage 1B: outer breaks in fovea
● Stage 2: early full-thickness holes, <400 μm in diameter
● Stage 3: full thickness, at least 400 μm diameter
● Stage 4: full thickness holes, at least 400 μm diameter, complete PVD
154
What is the presentation of intraocular iron toxicity?
● Subacute
● Causes siderosis
● Can lead to vision loss
155
What is the presentation of intraocular zinc and aluminum toxicity?
Minimal inflammation and foreign body becomes encapsulated
156
What are the functions of the RPE?
● Absorption of light
● Phagocytosis of rod and cone outer segments
● Participates in retinal and polyunsaturated fatty acid metabolism
● Forms the outer blood-ocular barrier (in vitamin A metabolism, RPE generates 11-cis-retinaldehyde)
● Maintenance of subretinal space
● Heals and forms scar tissue
157
What is lattice retinal degeneration?
● Area of inner retinal atrophy
● Loss of internal limiting membrane
● Adherent vitreous at edges of lesion
● Overlying pocket of liquified vitreous
● Sclerosis of remaining retinal vessels
● Seen in 10% of population
● Present in 20%-40% of rhegmatogenous retinal detachment
● Radial perivascular lattice degeneration -more common in Stickler syndrome
158
What is cobblestone or paving stone?
● Scalloped area of retinal atrophy
● Dense adhesions of inner retina to Bruch membrane
● Retinal detachment typically do not occur in this area
159
What is the treatment for choroidal hemangioma?
● Circumscribed choroidal hemangioma → Photodynamic therapy (PDT)
● Diffuse choroidal hemangioma → external beam radiotherapy
160
What are combined retinal-retinal pigment epithelial hamartomas?
● Rare
● Seen in children
● Association with neurofibromatosis-2, incontinentia pigmenti, X-linked retinoschisis, facial hemangioma
● Pathology: proliferation of RPE cells, dysplastic glia, and retinal blood vessels
161
What are asteroid bodies?
● Composed of calcium
● Stain positive with alcian blue and other stains for neutral fats, phospholipids, and calcium
● They stain metachromatically and birefringent with polarized light
162
What are the causes of purtscher-like retinopathy?
● Renal failure
● Long bone fracture
● Acute pancreatitis, pancreatic adenocarcinoma
● Orthopedic surgery
● Valsalva maneuver or weight lifting
● Lymphoproliferative disorders
● Bone marrow transplantation
● Pre-eclampsia or childbirth
● Connective tissue disorder
● Barotrauma
● Steroid injections in and around orbit or nasal passages
● Retrobulbar anesthesia
● Hemolytic uremic syndrome
● Cryoglobulinemia
● Shaken baby syndrome
163
What is the duration of various agents used for retinal tamponade in retinal detachment treatment?
● Air: 24 hours
● Sulfur hexafluoride gas (SF6): 2 to 3 weeks
● Perfluoropropane gas (C3F8): 2 months
● Perfluoropropane liquid (PFO): heavier than water, used temporarily intraoperatively to stabilize retinal detachment but not left in the eye
● Silicone oil: permanent, has to be surgically removed
164
What is the risk of retinal tear with posterior vitreous detachment (PVD)?
● All acute PVD’s: 7-18% risk of retinal tear
● PVD plus vitreous hemorrhage: 50-70% risk of retinal tear
● Pigment granules in anterior vitreous plus PVD: 7-fold increase in retinal tear
○ PVD’s start as perifoveal detachment and then spread circumferentially
165
What is a safe dose of hydroxychloroquine?
● 5 mg per kg per day or less
○ Real body weight; not ideal body weight
166
What is a safe dose of chloroquine?
● 2.3 mg per kg per day or less
○ Real body weight; not ideal body weight
167
What are the risks of radiation retinopathy?
● Typically occurs after 30-35 Gy of radiation
○ Sometimes even after 15 Gy
● Usually manifests after 18 months following treatment
○ May develop earlier with plaque brachytherapy
168
What are the risks of radiation optic neuropathy?
● Partly dose-related, more likely to occur with >5,000 cGy (>50 Gy)
● Occurs approximately 18 months after treatment
● MRI will show enhancement of optic nerves
169
What are ocular findings in quinine toxicity?
● Acute severe vision loss → can lead to permanent blindness
● Retinal ganglion toxicity (cherry red spot, mimicking central retinal artery occlusion) with ganglion cell layer thickening and hyperreflectivity → leads to diffuse inner retinal atrophy, optic atrophy, and retinal vascular attenuation
● Electronegative ERG
170
What are ocular findings in methanol toxicity?
● Acute blindness
● Acute transient optic head edema and macular edema → leads to optic atrophy
● Electronegative ERG
171
What are the ocular findings in thioridazine and phenothiazine toxicity?
Chlorpromazine
● Abnormal pigmentation of eyelids, interpalpebral conjunctiva, cornea, anterior lens capsule
● Posterior subcapsular cataracts
● Pigmentary retinopathy is rare
Thioridazine
● Toxicity rare at dose of 800 mg/day or lower
● Severe pigmentary retinopathy within few weeks or months of initiating medication
● Initially: blurred vision, coarse RPE stippling in posterior pole → progresses to patchy nummular atrophy of RPE and choriocapillaris → late stage -visual field loss and nyctalopia clinically mistaken for choroideremia or Bietti crystalline corneoretinal dystrophy
● Typically patients are not monitored because toxicity is rare on standard doses
172
What are the ocular findings in taxanes and niacin toxicity?
Taxanes
● Class of microtubule inhibitors → paclitaxel and docetaxel → chemotherapeutics
● Cystoid macular edema → visible on exam and OCT mac but does not leak on fluorescein angiogram
Niacin
● Cholesterol-lowering agent
● Causes cystoidmacular edema→ does not leak on fluorescein angiogram
● Initially central vision impaired → full recovery after discontinuation of drug and resolution of macular edema
173
What are the ocular findings in glitazones rosiglitazone and pioglitazone toxicity?
● Oral hypoglycemics used for diabetes mellitus treatment
● Associated with development or exacerbation of macular edema
174
What are the ocular findings in deferoxamine toxicity?
● Iron-chelating agent
● Reticular or vitelliform RPE changes in macula
○ Macular edema secondary to RPE pump failure
175
What is the prevalence of diabetic retinopathy over time after diagnosis of type 1 diabetes mellitus?
● After 7 years: 50%
● After 15 years: 90%
● After 20 years: 99%
176
What is the pathophysiology of diabetic retinopathy?
● VEGF upregulation
● Retinal capillary basement membrane thickening
● Increased platelet adhesion
● Retinal capillary pericyte loss
177
What are the indications for prophylactic treatment of retinal breaks?
● Acute symptomatic dialysis: treat promptly
● Acute symptomatic horseshoe tear: treat promptly
● Acute asymptomatic operculated hole: consider treatment
● Asymptomatic atrophic round hole: usually observed without treatment
● Asymptomatic dialysis: no consensus guidelines, but consider treatment
● Asymptomatic horseshoe tear (no subretinal fluid): can be observed without treatment
● Asymptomatic lattice degeneration with or without holes (no subretinal fluid): usually does not require treatment
● Eyes with lattice degeneration, atrophic holes, or asymptomatic retinal tear where the fellow eye has had retinal detachment: no consensus guidelines, but consider treatment
178
What is the differential diagnosis for leukocoria?
● Persistent fetal vasculature
● Retinoblastoma
● Retinopathy of prematurity
● Endophthalmitis
● Dysplasia of retina
● Inflammatory cycliticmembrane
● Congenital cataract
● Coats disease
● Coloboma of choroid or optic disc
● Toxocariasis
● Incontinentia pigmenti
● Norrie disease
● Familial exudative vitreal retinopathy
179
What is the Susac triad?
● Encephalopathy
● Hearing loss
● Retinal artery branch occlusions (“boxcar” segmentation)
180
What is the diagnosis criteria for acute retinal necrosis (ARN)?
● One or more foci of retinal necrosis with discrete borders in the peripheral retina
● Rapid progression in the absence of antiviral therapy
● Circumferential spread
● Occlusive vasculopathy with arteriolar involvement
● Prominent vitritis
● Optic neuropathy or atrophy
● Scleritis
● Pain
181
What are the layers of the retina in order from the inner to outer retina?
● Internal limiting membrane
● Nerve fiber layer (axons of ganglion)
● Ganglion cell layer
● Inner plexiform player
● Inner nuclear layers
● Middle limiting membrane
● Outer plexiform layer
● Outer nuclear layer (nuclei of photoreceptors)
● External limiting membrane
● Rod and cone inner and outer segments
182
What is the differential diagnosis for central serous chorioretinopathy?
● Age-related macular degeneration
● Optic pits
● Idiopathic polypoidal choroidal vasculopathy
● Idiopathic uveal effusion syndrome
● Vogt-Koyanagi-Harada
● Uveal effusion syndrome
● Pachychoroid pigment epitheliopathy
● Pachychoroid neovasculopathy
183
What is the differential diagnosis for bone spicules?
● Retinitis pigmentosa
● Trauma
● Past inflammation
● Syphilis
● Leber congenital amaurosis
● Measles retinopathy
● Progressive cone-rod dystrophy
● Kearns-Sayre syndrome
184
Which VEGF isoform is the most dominant in neovascular AMD?
VEGF-A 165
185
What is the risk of retinal detachment in untreated, asymptomatic lattice degeneration?
• The risk of retinal detachment in untreated, asymptomatic lattice degeneration is 1%
186
What risk factors warrant prophylactic laser for lattice degeneration?
• High myopia
• History of retinal detachment in the fellow eye
• Flat tears within the lattice
• Aphakia
187
What are the differences between ultrasound biomicroscopy (UBM), B scan, and anterior segment-OCT?
UBM
● High-frequency ultrasonography
● Water-bath immersion technique
● Shorter wavelength compared to B scan
● Depth of tissue penetration is 5 mm and resolution of 35 to 70 μm
○ Limited penetration through sclera
● Detailed visualization of iris, ciliary body, and ciliary process
● Clinical use: iris cysts/ciliary body melanomas, white-to-white, sulcus-to-sulcus, angle width measurement, allows anterior and posterior chamber structures to be visualized through opaque media
Anterior segment-OCT
● Higher resolution and high definition
○ Based on emission and reflection of light
○ Fine resolution of images, 5-10 μm
● Minimal scleral penetration
● Measures depth, width and angle of anterior chamber
● Limited ability to provide images of ciliary body and adjacent structures
● Clinical use: Delineation of the layers of cornea, anterior chamber, and iris, true corneal power
B scan
● High frequency sound
● Penetrates the sclera well
● Elucidate whether iris masses, ciliary body lesions, retinal/choroidal/ciliary body detachments, vitreous opacity, posterior pole tumor or staphyloma, foreign body, optic disc drusen are present
● Clinical use: if mediate opacities preclude fundus evaluation
