Ocular Disease: Lecture 2: Orbital Vascular Anomaly Flashcards

1
Q
  1. Carotid Cavernous Fistula: 2 types?
A
  1. Direct and Indirect
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2
Q

Carotid Cavernous Fistula (1)

  1. Define
  2. Arterialization of Venous BF
    a. What happens with Venous pressure
    b. Arterial BF?
    c. What happens to Arterial Pressure? Why?
A
  1. A/V fistula b/w the ICA and the Cavernous Sinus
  2. a. Increases. You get Retrograde flow of arterial blood into Orbit via Ophthalmic Veins
    b. Decreased Arterial BF to Cranial Nerves
    c. Decreases. It’s kind of like a leaky pipe…decreased arterial BF due to decreased arterial pressure
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3
Q

Carotid Cavernous Fistula

  1. Etiology (2)?
  2. Hemodynamics (2)?
  3. Anatomy (2)?
A
  1. Spontaneous or Traumatic
  2. High (ICA) or Low
  3. Direct or Indirect (High Volume, High BF)
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4
Q

Direct Carotid Cavernous Fistula

  1. % of cases?
  2. High Flow shunts: Why?
  3. Caused by what 2 things?
A
  1. 50% of all cases
  2. Blood flows DIRECTLY from intrcavernous portion of ICA to cavernous sinus via WALL DEFECT
  3. Trauma (75%) or Spontaneous (25%)-generally lower flow and Less symptomatic.
    * Generally, spontaneous are Burst aneurysms (usually middle Age Women who are Hypertensive)
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5
Q

Direct Carotid Cavernous Fistula (2)

  1. Clinical Triad?
  2. Ipsilateral to insult or bilateral/contralateral?
A
  1. a. Pulsatile Proptosis (associated w/Bruit and ALLEVIATED w/Ipsilateral Carotid Compression)
    b. Conjunctival Chemosis (Swollen, can’t close Eyelids)
    c. “Whooshing” noise (supposedly happens w/Cavernous sinus fistula)
  2. Usually Ipsilateral to Insult, but can also be Bilateral or Contralateral.
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6
Q

Direct Carotid Cavernous Fistula (3)

Other Signs

  1. What happens to Epibulbar?
  2. Type of Chemosis?
  3. Ptosis?
  4. IOP?
  5. What can happen to the Anterior Segment?
  6. What is seen 60-70% of the time?
A
  1. Severe Epibulbar Injection (blood has no where to go. Backs up and we see a LOT of vascular injection)
  2. Hemorrhagic Chemosis
  3. Possible Ptosis
  4. Elevated IOP
  5. can have Anterior Segment Ischemia
  6. Ophthalmoplegia. (6th nerve is most common and you can get Engorgement of EOMs)
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7
Q

Direct Carotid Cavernous Fistula (4)

  1. Vision: Immediate Loss due to what?
  2. Vision: Delayed loss can lead to what 5 things?
  3. What palsy is seen?
A
  1. due to ON damage at time of injury

2. Anterior segment Ischemia, CRV occlusion, Exposure keratopathy, Ischemic Optic Neuropathy, and 2ndary Glaucoma

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8
Q

Direct Carotid Cavernous Fistula (5)

Testing

  1. CT and MRI: to see what?
  2. What is the definitive test?
A
  1. Engorgement of Superior Ophthalmic Vein

2. CT and MRI Angiography

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9
Q

Direct Carotid Cavernous Fistula (6)

  1. When is treatment necessary?
  2. What can they do to treat it?
A
  1. only if spontaneous closure doesn’t happen

2. Endovascular Embolization: Coils or Balloons.

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10
Q

Indirect Carotid Cavernous Fistula (Dural Shunt) (1)

  1. Communication with what exactly?
  2. Blood flow level?
  3. Causes? (2/3)
A
  1. w/Meningeal Branch of the ICA or ECA, or both
  2. Low flow
  3. Congenital Malformations

and

Spontaneous Rupture (HTN Patients (constipation) or caused by Minor trauma or Valsalva)

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11
Q

Indirect Carotid Cavernous Fistula (Dural Shunt) (2)

  1. How does it present? (2)
  2. What is exaggerated?
  3. IOP level?
A
  1. Gradual onset; Epibulbar injection WITH or W/O Chemosis (can be unilateral or bilateral)
  2. Ocular pulsation (noted on tonometry)
  3. Elevated IOP
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12
Q

Indirect Carotid Cavernous Fistula (Dural Shunt) (3)

  1. Epibulbar Vessels: What happens to them?
  2. Proptosis possible?
  3. Ophthalmoplegia?
  4. Fundus?
A
  1. Corsckrew Epibulbar Vessels (Late sign)
  2. Mild proptosis possible
  3. 6th nerve and EOM Congestion
  4. can be normal or may demonstrate venous dilation
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13
Q

Indirect Carotid Cavernous Fistula (Dural Shunt) (4)

  1. Treatment?
A
  1. Similar to DIRECT, but us must use a TRANS VENOUS METHOD
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14
Q

Thyrotoxicosis (Hyperthyroidism) (1)

  1. What is it?
  2. What does it lead to?
  3. 2 things that cause it?
  4. Female:male ratio?
  5. Can be associated with what disorders?
A
  1. Over production of thyroid hormones
  2. Elevation of BMR
  3. a. Graves (85% of hyperthyroidism; AI)
    b. Toxic nodular goiter and Thyroiditis
  4. 10:1
  5. with other AI disorders
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15
Q

Thyrotoxicosis (Hyperthyroidism) (2): Presentation

  1. What age?
  2. Signs overall? (8)
A
  1. 3rd to 4th decade
  2. Heat intolerance, Irritability, Increased bowel movements, Nervousness, Palpitations, Sweating, Weakness and fatigue, and Weight loss
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16
Q

Thyrotoxicosis (Hyperthyroidism)
(3)

  1. External Signs? (8)
A
  1. Alopecia
  2. Finger clubbing and plummers nail
  3. Hand Tremor
  4. Pretibial Myxedema
  5. Reddening of the palms
  6. Thyroid Enlargement
  7. Vitiligo
  8. Warm, sweaty skin
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17
Q

Thyrotoxicosis (Hyperthyroidism) (4)

  1. Cardiovascular signs? )(4)
A
  1. Atrial Fibrillation
  2. High output heart failure
  3. Premature ventricular beat
  4. Sinus tachycardia
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18
Q

Thyroid Eye Disease (Ophthalmopathy) (1)

  1. When can it occur?
  2. Is it dependent upon anything systemic?
  3. Major risk factor?
  4. More likely in whom?
A
  1. Anytime
  2. No. Independent of Systemic sx/sn
  3. Smoking (increased cigarettes/day = increased risk for TED)
  4. 5x’s more likely in women
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19
Q

Thyroid Eye Disease (Ophthalmopathy) (2)

  1. Pathogenesis
    a. Cause?
    b. What does it do to EOMs?
    c. Leads to what?
    d. What EOM is most common?
A
  1. a. AI reaction (rx)

b. Inflammation of EOMs (can enlarge up to 8x’s normal size)
c. Fibrosis
d. IR, then MR, SR and finally LR

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20
Q

Thyroid Eye Disease (Ophthalmopathy) (3)

  1. Clinical manifestations? (5)
A
  1. a. Lid retraction
    b. Optic Neuropathy
    c. Proptosis
    d. Restrictive Myopathy
    e. Soft tissue involvement
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21
Q

Thyroid Eye Disease (Ophthalmopathy) (4)

  1. Stages of TED and what happens in each?
A
  1. Congestive (Inflammatory)
    a. Red painful eyes
    b. Remits after 3 years
    c. about 10% develop long term complications
  2. Fibrotic Stage
    a. White and Quiet
    b. Painless Motility Defect
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22
Q

Thyroid Eye Disease (Ophthalmopathy) (5): Soft Tissue Involvement

  1. 3 Signs?
  2. 5 Symptoms?
A
  1. a. Epibulbar Hyperemia
    b. Periorbital Swelling (Chemosis, Prolapse of fat into eyelid)
  2. a. Discomfort behind eyes
    b. Dryness
    c. Grittiness
    d. Lacrimation
    e. Photophobia

**Sausage like rolls (Prolapse of fat into eyelid)

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23
Q

Thyroid Eye Disease (Ophthalmopathy) (6): Soft Tissue Involvement

  1. 4 Treatments?
A
  1. a. Lubricants
    b. Lid Taping
    c. Head Elevation while sleeping

d. Topical Anti-Inflammatories (Cyclosporin, NSAIDS, and Steroids)

24
Q

Thyroid Eye Disease (Ophthalmopathy) (7): Lid Retraction

  1. Occurs in what % of patients with Graves?
  2. Theories behind how this happens (3)?
A
  1. 50%
  2. a. Fibrotic contracture of levator
    b. Humorally induced overaction of Muller muscle
    c. Secondary over action of LPS complex
25
Q

Thyroid Eye Disease (Ophthalmopathy) (8): Lid Retraction

  1. Normal Lid Position?
  2. When do we suspect it?
A
  1. a. Superior Lid Margin 2mm below the Limbus
    b. Inferior Lid margin AT LIMBUS
  2. a. Lid margin AT or ABOVE Limbus Superiorly (SCLERAL SHOW)
    b. Lid margin BELOW LIMBUS INFERIORLY
26
Q

Thyroid Eye Disease (Ophthalmopathy) (9): Lid Retraction

  1. Lid Signs?
  2. Kocher Sign?
  3. Von Graefe Sign?
A
  1. Dalrymple Sign (Lid retraction in primary Gaze)
  2. Frightened appearance
  3. Lid Lag on down gaze

**He kind of made fun of the names and signs

27
Q

Thyroid Eye Disease (Ophthalmopathy) (10): Lid Retraction

  1. Management with MILD RETRACTION?
  2. with STABLE RETRACTION?
A
  1. No treatment
  2. a. Surgery; ONLY after addressing any proptosis and/or Strabismus
    b. *Orbital decompression, Strabismus surgery, Eyelid Surgery
28
Q

Thyroid Eye Disease (Ophthalmopathy) (11): Lid Retraction

  1. 3 Different types of Lid Retraction Surgeries.
A
  1. a. Muellerotomy (Severe case might need recession of Levator Aponeurosis too) (Loss of mueller’s effect)
    b. Recession of Lower Lid Retractors
    c. Botox (Levator and mueller…but this is very TEMPORARY)
29
Q

Thyroid Eye Disease (Ophthalmopathy) (12): Proptosis

  1. How is it in nature?
  2. Unilateral or Bilateral?
  3. Symmetric or Asymmetric?
  4. Is it Permanent?
  5. What can it compromise? Leads to what?
A
  1. Axial in nature
  2. can be either
  3. can be either
  4. Often permanent
  5. Lid closure. (exposure keratopathy and risk for infection)
30
Q

Thyroid Eye Disease (Ophthalmopathy) (13): Proptosis

Management

  1. What 2 types of steroids can be used and what does it do?
A
  1. a. Oral Prenisolone (60-80 mg/day)
    b. Taper it off w/in a few days when symptoms start to improve
  2. IV Methylpredisolone .5g in solution given over 30 minutes
    a. This is used usually only for ACUTE Compressive Optic Neuropathy
31
Q

Thyroid Eye Disease (Ophthalmopathy) (14): Proptosis

  1. Radiotherapy
  2. What combination of treatment?
A
  1. In addition to or in place of steroids, and response usually occurs w/in 6 weeks
  2. Steroid + Radiation + immunosuppressive (Probably not doing this anymore)
32
Q

Thyroid Eye Disease (Ophthalmopathy) (15): Proptosis

Management

  1. What can be done surgically for the Proptosis?
A
  1. Surgical decompression will increase Orbital Volume (removing bony walls or Orbital Fat)
    a. 1 wall (deep lateral decompression)
    b. 2 wall (balance: medial/lateral decompression)
    c. 3 wall (includes orbital floor)
    d. 4 wall (includes orbital roof)
    * He’s never seen a 3 or 4 wall decompression
33
Q

Thyroid Eye Disease (Ophthalmopathy) (16): Proptosis

Management

  1. 1 Wall
    a. amt of reduction
    b. risk for what?
  2. 2 wall
    a. reduction amt?
    b. risk?
  3. 3 wall
    a. amt?
    b. risk for what 2 things?
  4. 4 wall
    a. done when?
A
  1. a. 4-5 mm reduction
    b. lower risk for post-op diplopia
  2. a. greater reduction
    b. risk for post-op diplopia
  3. a. 6-10 mm reduction
    b. can cause HYPOGLOBUS , high risk of infraorbital nerve damage and High risk of diplopia
  4. a. reserved for very severe proptosis
34
Q

Thyroid Eye Disease (Ophthalmopathy) (17): Restrictive Myopathy

  1. Occurs in what % of TED patients?
  2. Is it permanent?
  3. Restriction is what in nature at the start?
  4. What happens to IOP with Upgaze?
A
  1. 30-50%
  2. Yes, it can be.
  3. Inflammatory in nature
  4. It INCREASES. 2nday to ocular compression by Fibrotic Inferior Rectus Muscle
35
Q

Thyroid Eye Disease (Ophthalmopathy) (18): Restrictive Myopathy

  • Other types of Restrictions in order of Frequency
    1. Elevation
    2. Abduction
    3. Depression
    4. Adduction
A
  1. Fibrotic IR (mimics SR palsy)
  2. Fibrotic MR (mimics 6th nerve palsy)
  3. Fibrotic SR
  4. Fibrotic LR
36
Q

Thyroid Eye Disease (Ophthalmopathy) (19): Restrictive Myopathy

Treatment

  1. Surgical
  2. Goal?
  3. Technique?
A
  1. Diplopia in primary/reading gaze. Disease has subsided, and 6 months stability in Angle
  2. Binocular Single vision in Primary Gaze and Reading positions
  3. Recession (NOT RESECTION) of INFERIOR and/or Medial Recti
37
Q

Thyroid Eye Disease (Ophthalmopathy) (20): Optic Neuropathy

  1. Is it common?
  2. What does it do and result in? (5)
A
  1. No. Pretty uncommon
  2. ON compression; ON Blood Supply compression; Happens at ORBITAL APEX; can occur in PRESENCE OR ABSENCE of Proptosis, and SEVERE VISION LOSS
38
Q

Thyroid Eye Disease (Ophthalmopathy) (21): Optic Neuropathy

  1. 5 Signs/Symptoms?
  2. What about the ONH Appearance?
A
  1. a. Decreased VA
    b. RAPD
    c. Color Desaturation
    d. Perceived Dimness
    e. VF Defects (central or paracentral and Arcuate)
  2. Usually normal, but MAY be SWOLLEN or ATROPHIC
    * Decreased VA usually means we are too late. Patients will say room looks dimmer.
39
Q

Thyroid Eye Disease (Ophthalmopathy) (22): Optic Neuropathy

  1. Treatment? (2)
A
  1. Systemic Steroids (Immediately: 100-200 mg/day)

2. Orbital Decompression

40
Q

Thyroid Eye Disease (Ophthalmopathy) (23): Management

  1. Exposure? (2)
  2. Eyelid Retraction (1)?
  3. Diplopia? (3)
  4. Optic Neuropathy? (3)
A
  1. a. Lubrication
    b. Lid Taping/moisture chamber
  2. Surgical Intervention (w/stable interval)
  3. a. Oral Steroids (Controversial)
    b. Fresnel Prism
    c. Strabismus Surgery (Stable interval)
  4. a. Immediate Oral Steroids (100 mg pq qd 1-2 weeks)
    b. Radiation (controversial)
    c. Orbital Decompression
41
Q

Orbital Lesions and Tumors

  1. 4 Benign Pediatric Tumors?
A
  1. a. Capillary Hemangioma
    b. Dermoid Cyst
    c. Lymphangioma
    d. Optic Nerve Glioma
42
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Dermoid Cyst

  1. Lined with what?
  2. What’s it filled with?
  3. Where can it be?
A
  1. with a “Skin Like” Epithelium
  2. Sweat glands, sebaceous glands and hair follicles
  3. can be Superficial or Deep
43
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Superficial Dermoid Cyst

  1. When is it present?
  2. Nodule looks like what and its size?
  3. Location?
  4. Treatment?
A
  1. In Infancy
  2. Firm, Round, Smooth and painless nodule. 1-2 cm in diameter and it’s MOBILE
  3. Superior Temporal or Superior Nasal
  4. Excision
    * Very Benign
44
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Deep Dermoid Cyst

  1. When do we see them? (age)
  2. Signs?
  3. Treatment?
A
  1. Adolescent or Adult
  2. Proptosis and/or Dystopia
  3. Complete Excision
45
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Lymphangioma (1)

  1. Cause?
  2. When do we see it?
  3. Signs? (4)
A
  1. Vascular Malformation
  2. Early Childhood
  3. a. Can hemorrhage w/in the cyst (Chocolate Cysts)
    b. Generally filled w/Lymphatic Fluid
    c. Slow progressive Proptosis IF POSTERIOR
    d. Soft BLUISH Masses in Upper Nasal Quadrant
46
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Lymphangioma (2)

  1. Complications?
  2. Treatment? (1)
A
  1. Severe Proptosis (secondary to hemorrhage of posterior lesion)
  2. a. Drainage
    b. Complete Excision not possible.
47
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Capillary Hemangioma (1)

  1. Frequency?
  2. More common in whom?
  3. Presentation variation?
  4. AKA?
A
  1. Most Common BENIGN tumor of Orbit and Periorbit in CHILDREN (usually present in 1st month)
  2. in FEMALES than males
  3. Huge variation
  4. Strawberry Nevus
48
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Capillary Hemangioma (2): signs

  1. Superficial (cutaneous)
  2. Preseptal
  3. Deep Orbital
  4. May involve what?
A
  1. Bright Red Lesion
  2. Dark Blue or Purple; Superior
  3. Unilateral Proptosis; No Skin Discoloration; Imaging needed
  4. Palpebral Conjunctiva.
    * May present with any combination of these things
49
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Capillary Hemangioma (3)

  1. Growth rate?
  2. Resolution?
  3. Treatment if…?
A
  1. Rapid growth for 3-6 months
  2. Slow resolution. 30% by age 3 and 70% by age 7
  3. Amblyopia, Optic Nerve Compression, Exposure Keratopathy, Severe Cosmetic Concerns, Necrosis or Infection
50
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Capillary Hemangioma (4)

  1. Treatment? (2)?
A
  1. a. Laser: close superficial BVs
    b. Steroid Injections: superficial or Preseptal (may see regression in 1-2 weeks)…lots of complications possible. (skin depigmentation, necrosis, bleeding, fat atrophy, adrenal suppression)
51
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Capillary Hemangioma (5): treamtent 2

  1. 3 other treatments?
A
  1. Systemic Steroids (large orbital Lesions)
  2. Systemic Beta Blocker
  3. Local Resection
52
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Optic Nerve Glioma

  1. Growth rate?
  2. Comes from what?
  3. Associated with what?
  4. Commonality of Malignant Gliomas?
A
  1. Slow
  2. Pilocytic Astrocytoma
  3. with Neurofibromatosis 1
  4. They’re VERY RARE (usually occur in adults, not children)
53
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Optic Nerve Glioma (2)

  1. When does it tend to present?
  2. What happens?
A
  1. W/in 1st Decade
  2. a. Slow progressive Vision loss
    b. Proptosis (follows VA’s)
    c. Swollen or Atrophic ONH
    d. Optociliary collaterals and Central Retinal Vein occlusion possible
    e. Can spread to chiasm and hypothalamus
54
Q

Orbital Lesions and Tumors: Benign Pediatric Tumors: Optic Nerve Glioma (3)

  1. Imaging
  2. Treatment (3) and when?
  3. Prognosis?
A
  1. CT and MRI useful
  2. a. Monitor: (no growth, good vision, no cosmetic concerns)
    b. Excision if Large or complications
    c. Radiotherapy and chemotherapy if intracranial extension
  3. a. Depends on course of the lesions
    b. Slow or no growth is a GOOD SIGN
    c. Intracranial extension can be life threatening
55
Q

An optimist sees an opportunity in every calamity; a pessimist sees a calamity in every opportunity.

A