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Optic Disc, Pallor and Atrophy Raymond G. Magauran, M.D. Oculoplastics Surgery Associates Danvers, Massachusetts Information Sources • • • • • • 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 • • • • • Axons die Remaining astrocytes are opaque Rearrange at right angles to entering light Surround blood vessels Reflect light Pathology of Pallor • • • • No astrocyte proliferation FA shows blood vessels still function (Hayreh, 1970) Wallerian (anterograde) degeneration (7 days) Anterograde degeneration (4 weeks) Optic Atrophy: Working backwards • • • • • • • • Retina/ganglion cell Nerve fiber layer Optic disc Optic Nerve Chiasm Optic Tract Lateral geniculate body Trans-synaptic degeneration Differential Dx: • • • • • • • • • 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 • • • • • • Patient’s opinion? PHI, PMH, PSH, FH, Soc HX Medications Exposures - via social history Traumatic events Review of systems: – – – – – 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 • • • • Retina Vasculature Periphery Optic Disc Optic Atrophy: Optic Disc • • • • • Tilted Color Cupping Edema High water marks • • • • • 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 – – – – • • • • 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 • • • • • 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 – – – – 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 • • • • • • • 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 • • • sharply circumscribed retrobulbar and intraconal contrast injection no enhancement – other well-circumscribed orbital tumors enhance (schwannoma, neurofibroma, hemangiopericytoma). Ultrasonography • • cystic high internal reflectivity