Transcript OCULOPLASTICS AND NEURO

Optic Disc,
Pallor and Atrophy
Raymond G. Magauran, M.D.
Oculoplastics Surgery Associates
Danvers, Massachusetts
Information Sources
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Spalton
Spoor
Rootman
Walsh & Hoyt
MEEI/DJO
mrcophth.com
Optic Atrophy
“Optic atrophy is not a disease. It is a
morphologic sequela of disease - any
disease - that causes damage to
ganglion cells and axons of the optic
nerve.”
Walsh & Hoyt’s Clinical Neuro-Ophthalmology, 5th Edition
Why is the normal
optic nerve pink?
• Axons surrounded by glial columns
– carry blood vessels -> red
• Nerve fibers are translucent
• Light rays are carried like fiberoptics
– rays that escape assume the pink color of the
surrounding columns
Quigley and Anderson , Am J Ophthalmol 83:709-717, 1977a
When is the normal
optic nerve pale?
• Nerve fibers are translucent
• Surrounded by glial supports
• Temporal side more pale
– fewer BVs, crescents, shape of cup
• Physiologic cupping:
– central pallor from cribriform plate
– direct light is reflected leading to
yellow gray appearance
Pathology of Pallor
Pink Nerve -> Axonal damage -> Pallor
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Axons die
Remaining astrocytes are opaque
Rearrange at right angles to entering light
Surround blood vessels
Reflect light
Pathology of Pallor
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No astrocyte proliferation
FA shows blood vessels still function (Hayreh, 1970)
Wallerian (anterograde) degeneration (7 days)
Anterograde degeneration (4 weeks)
Optic Atrophy:
Working backwards
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Retina/ganglion cell
Nerve fiber layer
Optic disc
Optic Nerve
Chiasm
Optic Tract
Lateral geniculate body
Trans-synaptic degeneration
Differential Dx:
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Congenital/genetic
Vascular
Traumatic
Infectious
Toxic
Metabolic
Autoimmune
Neoplastic
Ocular
Optic Atrophy Work-up
• History
• Physical
• Testing
The insult is more than 8 weeks old:
in general, this is not an emergency.
Optic Atrophy: History
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Patient’s opinion?
PHI, PMH, PSH, FH, Soc HX
Medications
Exposures - via social history
Traumatic events
Review of systems:
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pituitary
CAD/Atherosclerosis
headaches,migraines
neuro, taste/smell
sleep apnea, nocturnal hypotension?
Optic Atrophy: History
• Positive history:
– helps refine the differential
• Completely negative:
– little help with differential
• examine for more clues
Optic Pallor: Examination
• External exam
– retraction/ptosis, palpation/retropulsion
• Cranial nerves
• Strabismus
• Slit lamp
– conj, cornea, a/c, lenses
– gonio -> narrow, recession, etc.
• Pupil exam
– injury, synechia, TI defects, PIs?
Optic Atrophy: Posterior Segment
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Retina
Vasculature
Periphery
Optic Disc
Optic Atrophy: Optic Disc
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Tilted
Color
Cupping
Edema
High water marks
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Disc margin
Vasculature
Shunt vessels
Drusen
Pits
Optic Atrophy: Cupping
Non-glaucomatous
• Cupping - rim intact
• Non-cupped areas: pale
Glaucomatous
• Cupping - rim lost
• Non-cupped areas: pink
• VF c/w pallor
• VF not c/w cupping
• VF c/w cupping
• VF can be ok w/ early
cupping
Optic Atrophy: Testing
• Automated Perimetry
– Learning curve?
• Does the OA look serious?
– Eg. 0.5 vs 0.9
• What do you do now?
incongruous bitemporal visual field defect
that respects the vertical midline
Optic Atrophy : More tests?
“be judicious or you will break the bank”
• H/P may point to a diagnosis
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sudden/rapid visual loss
h/o MS, sarcoid, unusual headaches, etc
+ FH
angle closure
Poor vision
Shunt vessels
Orbital signs
Progressive VF decline without glaucoma
Optic Atrophy: Neuro-imaging
• CT - regular, spiral CT
– BUN/CR if on metformin
• MRI/A
– Head and/or orbit
– Fat suppression
– Contrast is non-iodinated
Optic Atrophy:
Additional testing
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Ultrasound - A/B scan
Fluorescein angiogram
Carotid dopplers
Carotid/CNS angiogram
Cardiac ECHO/TEE
Optic Neuropathy Lab tests
• CBC, ESR, CRP
• TFTs including TSH
• Autoimmune
– Lupus anticoag (including ANAs), ANCA, RF, ACE,
serum lysozyme
• Clotting Problems
– Anti-cardiolipin Abs, Protein S, resistance to act
Protein C, anti-thrombin III, Factor V Leiden
• Infections:
– RPR/FTA-ABS, Lyme
Optic Atrophy: more tests
• MRV if MR negative
• Consider LP (lat decubitus OP for PTC)
• Extras
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PPD, anergy testing
mDNA for LHON
anti-optic nerve Ab
CAR-Ab + CT Chest for lung ca (small cell)
Axial CT Scan
Normal T1 noncontrast MR without fat suppression
Axial noncontrast T1-weighted MRI
Axial noncontrast T1-weighted MRI
Axial postgadolinium T1-weighted MRI with fat saturation
Coronal postgadolinium T1-weighted MRI with fat saturation
Axial postgadolinium T1-weighted MRI with fat saturation
Axial postgadolinium T1-weighted MRI
Coronal T1-weighted MRI post gadolinium
Axial noncontrast T1-weighted MRI
Axial T2-weighted MRI in a 46-year-old man
demonstrates a mass in the LGN of the thalamus
MRI/MRV: Superior sagittal sinus thrombosis
Boston Life Sciences' Central Nervous
System Program Identifies
Optic Nerve Regeneration Pathway
Glaucoma Added to the Company's Spinal
Cord Injury and Stroke Drug Development
January 21, 2000-Boston, MA-Boston Life
Sciences, Inc. (NASDAQ: BLSI)
http://www.bostonlifesciences.com/news27.htm
NA-AION
Arteritic-AION
Optic Nerve: Shunt Vessels
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Old CRVO
Meningioma
Optic Nerve glioma
Chronic papilledema
Glaucoma
Idiopathic
Congenital
Optic Pit
NA-AION
Astrocytic Hamartoma
High Myopia
Bergmeister's papillae
Disc Drusen
Tilted Disc
Hypoplastic disc
Shunt vessels post HRVO
Myelinated Nerves
Disc edema
PHPV
Optic Disc,
Pallor and Atrophy
Raymond G. Magauran, M.D.
Oculoplastics Surgery Associates
Cavernous
Hemangiomas
CT/MR
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sharply circumscribed
retrobulbar and intraconal
contrast injection no enhancement
– other well-circumscribed orbital tumors
enhance (schwannoma, neurofibroma,
hemangiopericytoma).
Ultrasonography
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cystic
high internal reflectivity