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Ocular Disease 3 > Ocular Disease III: Exam 2: Lecture 12: Retinal Vascular Disease > Flashcards

Ocular Disease III: Exam 2: Lecture 12: Retinal Vascular Disease Flashcards

1
Q

Sickle Cell Disease

  1. What is it?
    a. Get decreased solubility of Hb when?
  2. 4 Types of this disease
  3. Most common Trait type in USA?
  4. Which Disease has the MOST % of Ocular Manifestations?
A
  1. Normal Hemoglobin A, but Mutant Hb S +/o HbC
    a. when Deoxygenated (SICKLING of RBC)
  2. a. Sickle Cell Trait (AS)
    b. Sickle Cell Anemia (SS)
    c. Sickle Cell Disease (SC)
    d. Sickle Cell Thalasemia (SThaI)
  3. Any Sickle Hb (10%)
    * Sickle Trait AS (8% of US population)
  4. SC (Sickle Cell Disease). Highest rate (1/3). Although this disease is very RARE! (0.2% in US), but 33% have RETINOPATHY
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2
Q

Ocular Findings: Sickle Cell Findings

  1. What is seen in Bulbar Conj?
    a. What else?
    c. Neo?
A
  1. Comma-shaped vessels in the Bulbar Conj
    a. Black Sunbursts (RPE Hyperplasia 2ndary to DEEP Retinal Vascular Occlusions)
    b. Salmon Patch Hemorrhages (Orange-pink-colored Intraretinal Heme)
    c. Peripheral NEO (SEAFAN RETINOPATHY) w/Vitreous Heme and Tractional RD!
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3
Q

Nonproliferative Sickle Cell Retinopathy *KNOW

  1. Heme Sequelae of occlusion occur in Periphery…
    * KNOW THAT IT’s OKAY BECAUSE THEY’RE NOT PROLIFERATIVE!
A
  1. a. Salmon Patch Heme
    b. Refractile deposits or Spots
    c. Black Sunburst Lesions
  • Arterial, Capillary Occlusive disease
  • Anastomosis and Remodeling Occur in Periphery
  • VA loss in early Dz is d/t occlusion of parafoveal capillaries and arterioles
  • CRAO
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4
Q

Proliferative Sickle Cell: KNOW!

  1. Stage 1
  2. Stage 2
  3. Stage 3
  4. Stage 4
  5. Stage 5
A
  1. Peripheral Retinal Arteriolar Occlusions
  2. Peripheral Arterio-Venous ANASTOMOSES
  3. NEO Fronds known as SEA FANS
  4. Vit Heme as Tractional Forces and Vitreous Collapse Tear Fragile NEO Membranes
  5. ADVANCED Disease. See SEVERE VITREOUS Traction and RD.
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5
Q

Sickle Cell Retinopathies: Retinal Vascular Occlusion

  1. Proliferative
    a. AV Anastomoses (Remodeling) –> leads to what?
    b. This can lead to what 2 things?

Resolution

  1. NonProliferative
    a. What happens?
    b. This leads to one of three things:
    i. Preretinal

ii. Intraretinal Salmon Patch
iii. Subretinal

A
  1. a. Seafan NEO
    b. Autoinfarction, or VIT HEME which will lead to RETINAL BREAKS –> Tractional and Rhegmatogenous RD
  2. a. Hemorrhage
    b. i. Vitreous Fibrosis
    ii. Refractile Spots
    iii. Black Sunburst
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6
Q

Management of Sickle Cell Retinopathy

  1. Goal is to do what?
  2. How often SHOULD THEY BE SEEN?
  3. If they’re Dx with Proliferative Disease: what should be done?
  4. Does Cryotherapy work?
    * If he has this condition, which is the Treatment of choice?
  5. **KNOW: If someone had DR, with no Macular Edema and NO CSME, what would they do for Tx?
  6. Patient comes in with something like this…(details…of Sickle Cell Retinopathy): You notice a Seafan going on, what is the Tx of CHOICE?
  7. What medication is given for Sickle Cell Tx?
A
  1. Reduce risk, prevent or Eliminate RETINAL NEO!
  2. TWICE A YEAR for an Ocular Exam with DFE (DR –> Yearly)
  3. FA–> PRP! (basically…NEO)
  4. NO. Not efficacious for SC Retinopathy and is Associated with HIGH COMPLICATION RATES!
    * CIRCLE PRP, NOT Cryotherapy!!!
  5. PRP. they would NEVER do an Anti-VEGF Tx!
  6. PRP!
  7. Hydroxyurea
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7
Q

Retinopathy of Prematurity

  1. AKA?
  2. Normal Retinal Vascularity proceeds from optic disc to periphery and is complete in nasal quadrants at 36 wks, temporal at 40 wks gestation
    a. What increase risk of development of ROP?
  3. Location determines prognosis
A
  1. Retrolental Fibroplasia
  2. a. High O2 concentration, Low birth wt, short gestation period
  3. Zones I-III
    * Staged retinopathy: stages 1-5
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8
Q

Retinopathy of Prematurity

  1. Zone I: includes what?
  2. Zone 2?
  3. Zone 3?
  4. Retinopathy of Prematurity
    a. Should do 2 DFE via BIO at 4-6 wks, then q1-2 wks until retina is FULLY Vascularized if what?
  5. Threshold Disease:
A
  1. Optic nerve and Macula
  2. Midperiphery
  3. Ora Serrata
  4. a. Birth wgt 5 clock hrs of Extraretinal NEO w/in Zone 1 or 2
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9
Q

ROP: Retin…of Prematurity **KNOW

  1. Stage 1
  2. Stage 2
  3. Stage 3
  4. Stage 4
  5. Stage 5
    * 6. One of the MOST IMPORTANT things to SAY TO THE KID?
A
  1. DISTINCT LINE b/w VASCULARIZED and AVASCULARIZED region of the retina
  2. Line in Stage 1 GAINS DEPTH and HEIGHT (more pronounced)
  3. Vessels extend beyond retina and into the Vitreous
  4. Partial RD (DRAGGED DISK)
  5. Complete RD
  6. TELL them NO PE. NO GYM. No BASEBALL. NO FAST APPROACHING OBJECTS!! (can save the kids their eyesight).
    * Put them in swimming instead. Goal is to PREVENT RD.
    * LOW VISION PATIENTS!
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10
Q

ROP: Associated Ocular Conditions…

A

Myopia w/Astig. Aniso. Strab. Amblyopia. Cataract. Glaucoma. RD. Angle Closure Glaucoma in 5th Decade.
Exudative Retinopathy Late Sequelae.

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11
Q

ROP: Major Tx?!

  1. 2 big things
A
  1. CRYOTHERAPY OR LASER PHOTOCOAGULATION!

* to avascular ant. retina in ROP w/THRESHOLD DISEASE decreases by half unfavorable outcomes

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12
Q

Vasculitis

  1. May accompany what?
  2. Early Clinical manifestations?
A
  1. Primary Inflammatory Disease

2. Perivascular infiltrates and Sheathing of Retinal VESSELS

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13
Q

Vasculitis

  1. Sheathing looks like what?
  2. Is it common in Connective tissue Disorders?
  3. What would you see with Connective Tissue Diseases?
A
  1. WHITEISH around VESSEL. *White cuff along the vessels is a perivenous lymphocytic infiltration
  2. NO! EXTREMELY RARE (like in lupus).
  3. several Bland occlusions that appear like CWS, Branch retinal artery occlusions, and Obliterative Vasculopathy
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14
Q

Primary Idiopathic Retinal Vasculitis: EALES Disease

  1. Mainly in which sex?
  2. What is it?
  3. Uni/Bi?
  4. Commonly u see what?
  5. Associated with what?
  6. Cause of EALES DISEASE?
  7. Clinically indistinguishable from what?
  8. Dx/Tx?
A
  1. MALES
  2. Obliterative Periphlebitis
  3. Peripheral Retina BILATERALLY
  4. Peripheral Retinal NEO with Resultant Vit. Heme
  5. Tuberculin Hypersensitivity may be present.
  6. IDIOPATHIC!!
  7. From Past retinal Vasculitis
  8. Look for UNDERLYING ETIOLOGY, PRP!!! (NEO)
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15
Q

Coats Disease

  1. What is it?
  2. Vascular Abnormalities..
  3. Evidence suggests what?
A
  1. Retinal Telangiectasia
  2. Venous dilation…microaneurysms, Fusiform Capillary Dilation, Exudative RD, Capillary Non-perfusion on RSFA
    * Wide range in variation in Presentation
  3. a Somatic Mutation of the NDP gene (Norrie Disease Protein)
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16
Q

5 STAGES of COATS DISEASE (KNOW FOR THE TEST)

  1. Stage 1
    a. Tx?
  2. Stage 2
    a. Tx
  3. Stage 3
    a. 3a
    b. 3b
  4. Stage 4
    a. Tx?
  5. Stage 5
    a. Tx?
  6. Stage 2 a
  7. Stage 2b.
A
  1. Telangiectasia Only
    a. Tx: Laser Therapy
    b. High chance to save vision
    c. Disease RARELY SEEN in THIS STAGE
    d. Onset can be as EARLY as 12 MONTHS!
  2. Telangiectasia and Exudate
    a. Laser Photocoagulation or CRYOtherapy
    b. Exudate limited to 1 Quadrant: Good chance of restoring VISION!
  3. Subretinal or Retinal Detachment
    a. Laser Therapy or Cryotherapy used still. (Subretinal fluid now involved…so LASER Tx NOT AS EFFECTIVE as CRYOTHERAPY!!…QUESTION for EXAM!)

b. Complete RD! CRYOTHERAPY MAY BE USED if RD is SHALLOW. or Surgery to Re-Attach the RETINA may be NEEDED if DETACHMENT is ADVANCED and POSTERIOR To the LENS.

  1. Total RD and Glaucoma
    a. Like NO CHANCE for RECOVERY of VISION. DAMAGE to RETINA IS SEVERE. like 66% lose an EYE (ENUCLEATION…and you remove it DUE TO THE PAIN from NEO..not due to vision (already gone))
    * Laser therapy would only be used to try to stop leakage and maybe prevent increase in eye pressure that can lead to ENUCLEATION
  2. TOTAL BLINDNESS and is IRREVERSIBLE.
    a. No Pain: Aggressive Tx is not needed. Talk to Ophthalmologist about Tx if any should be done.
  3. restoring vision is good. fovea isn’t involved yet.
  4. can restore vision if FOVEA IS NOT HEAVILY INVOLVED. (If FOVEA has DENSE YELLOW NODULE in the CENTER (“Yellow Eye”, then visual prognosis is NOT GOOD))
17
Q

Coats Disease

  1. Hereditary?
  2. Mainly seen in whom?
  3. Mono/Binocular?
  4. Greatest Progression occurs under age of what?
  5. Tx
A
  1. no
  2. 85% Males
  3. Monocular
  4. under age of 4
  5. PRP, CryTx, Retinal Sx
    * Recurrent

Px: POOR

18
Q

Retinal Telangectasis: 3 Subgroups

  1. Group 1
    a. Localized Variant of what?
    b. Tx?
  2. Group 2
    a. Who is it involved with? Thickening of RETINA esp what area?
    b. Tx?
  3. Group 3
    a. Tx?
A
  1. UNILATERAL Parafoveal Telangiectasis
    a. of Coats Disease
    b. PRP
    * **THINK COATS cuz COATS is MONOCULAR!! (Retinal Telangiectasia)
  2. Bilateral Parafoveal Telangiectasis
    a. Males or females. Profound Thickening of Retina, esp the TEMPORAL FOVEA.
    b. NO Tx.
  3. Bilateral Perifoveal Telangiectasis w/Retinal Capillary Obliteration
    a. No Tx
19
Q

Temporal (Giant Cell) Arteritis (GCA)

  1. What is it?
  2. Form of Vasculitis
  3. Urgent or EMERGENT?
  4. Cells that do what?
A
  1. Inflammatory Disease of LARGE to MEDIUM BV’s of the HEAD
  2. Sudden Occlusive Disease of Branches of EXTERNAL CAROTID
  3. EMERGENCY!
  4. NARROW the LUMEN of the ARTERY (Giant cells that do this)
20
Q

GCA: Patho

  1. Hyperplasia –>
  2. Narrowing –>
A
  1. Arterial Luminal

2. Ischemia

21
Q

GCA: Symptoms

  1. Secondary to Systemic Inflammation Nonspecific Symptoms
A
  1. Fever, Malaise, Fatigue, depression
22
Q

GCA: Neck-Up Symptoms

  1. What they feel.
  2. If it’s a SUDDEN ONSET HA Over 50…?
A

Neck PAIN

JAW Pain (CLAUDICATION!!)

TEMPORAL LOBE HA or Pain!

Vision changes; Blindness

  1. Then its probably THIS!
23
Q

GCA: Visual Symptoms

  1. What may be a presenting symptom?
  2. Uni or BI Visual Loss
  3. IF a PATIENT IS YOUNGER THAN 50 and they PRESENT with Jaw Claudification, is it GCA?
  4. Mean age it happens?
A
  1. Visual Loss…can be Sudden and Painless. Transient and Intermittent
  2. UNILATERAL VISUAL LOSS
  3. NO!!! (even if they’re 48)
  4. 72 years. DOES NOT HAPPEN IN YOUNG PEOPLE. (risk increases with AGE)
    * MORE in WOMEN. as well as NORTHERN EUROPEAN WHITES
24
Q

GCA: Un- or Under-Treated

  1. Uni/Bi blindness?
A
  1. Either…in up to 50% of patients
    * Occulomotor dysfunctions
    * Vertigo and Hearing Impairments
    * Uni/Bi Limb Bruits and Claudication
25
Q

GCA Evaluation

  1. 2 TESTS YOU NEED TO ORDER (question)
  2. 3 evaluations?
  3. Can you still have GCA and normal ESR?
    a. So what’s the best way to confirm it?
A
  1. ESR and CRP!!!
  2. Neuro eval; Temporal Artery Palpation; Complete Eye Eval
  3. YES. in up to 20% of patients
    a. Do a Biopsy of temporal Artery
    * Color Doppler of TA??
26
Q

Indications for TA Bx

  1. Patients have the following
    a. AGE? **HE will ask a question about a 48 yr old patient with these symptoms and if you would order a biopsy…ANSWER = NO!
A
  1. Jaw Claudication, Pale optic disc edema, fever, systemic symptoms other than HA.
    a. HAS to be OLDER THAN 50!
27
Q

CI of TA Bx

  1. Prolonged Tx with what?
  2. Relative CI, Negative Result Properly Performed Bx….
A
  1. > 30 days. with GLUCOCORTICOID THERAPY

2. Positive contralateral biopsy about 1%

28
Q

Tx for GCA

  1. Oral what?
    a. How much?
    b. Improvement of SYSTEMIC SYMPTOMS w/in how long?
  2. Tx LENGTH?
A
  1. STEROIDS
    a. 60 mg/day
    b. w/in 72 HOURS
  2. 1-2 YEARS
    * Pt Education: Re: COMPLICATIONS ASSOCIATED with LONG-TERM STEROID USE!!!! (severe condition…drug will help…but this drug SUCKS!)
29
Q

Phacomatoses

  1. All AUTOSOMINAL DOMINANT except which one?
  2. What is it?
A
  1. ATAXIA-TELANGIECTASIA

2. just strange growth. Wide range of penetrance and expressivity. High RATE of SPORADIC CASES

30
Q

Sturge-Weber

  1. Ipsilateral what?
  2. What is it?
A
  1. Port-wine stains and Ipsilateral Congenital Glaucoma

2. Encephalotrigeminal Angiomatosis

Ocular Disease 3 (11 decks)

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