Optic Nerves that Pale in Comparison
Edward Chu, OD, FAAO
Abstract:
Optic nerve pallor is a sign of insult or damage to the nerve fiber layer commonly from trauma,
ischemia, infection, inflammation, or a space occupying lesion. This exam finding warrants a proper
investigation and potentially a work-up to determine the underlying cause.
Learning Objectives
1. Review management of optic nerve pallor through case presentations.
2. Review important case history questions, proper ancillary testing, and ocular findings that
may reveal underlying cause of optic nerve pallor
3. Review role of labs and imaging in diagnosing and when it is appropriate to order them
I.
Optic Nerve Characteristics
A. Normal color
-Result from small vessels on disk and nerve fiber layer
B. Pallor
-Sign of irreversible damage to normal optic nerve
-Difffuse or sectoral atrophy
-Unilateral or bilateral
-Pallor in excess of cupping
-Visual acuity, visual field, color vision level correlated with degree of atrophy
C. Ancillary Testing
-Best corrected visual acuity
-Pupils
-Visual Fields
-Color Testing
-Nerve fiber layer analysis
D. Differential Diagnosis
-Pseudophakia Pallor
-Trauma Related: Purtscher’s retinopathy, Traumatic Optic Neuropathy
-Toxic Optic Neuropathy (Nutritional, Medication)
-Vascular: Non-Arteritic Ischemic Optic Neuropathy, Arteritic AION
-Infectious/Inflammatory: Optic Neuritis
-Optic nerve compression from tumor (Pituitary Adenoma, ON glioma, ON sheath
meningioma, dolichoectatic carotid atery)
-Foster-Kennedy, Pseudo-Foster Kennedy
-Glaucoma, Disk Drusen
-Congenital defect: Optic Nerve Hypoplasia
II. Anterior Ischemic Optic Neuropathy
A. Characteristics
-Acute unilateral vision loss, typically noticed in the morning ~80%
-Temporary hypoperfusion or nonperfusion of anterior optic nerve circulation
-Predisposing risk factors: systemic: HTN, nocturnal hypotension, diabetes,
hyperlipidemia, atherosclerosis
-Optic Nerve Head: absent or small cup, location of watershed zones
-Precipitating risk factors, “last straw”: Nocturnal Hypotension, ED medication
-Difference vs. Arteritic AION
B. Management
-Disk at risk?
-Baseline visual field
-Discontinue erectile dysfunction medication
-Consider switching BP medications from PM to AM if no contraindication
-ESR, CRP, Platelets?
III. Traumatic Optic Neuropathy
A. Characteristics
-Visual loss usually instantaneous
-Visual acuity usually significant reduced to 20/400 or less
-Permanent vision loss in ~50%
-H/O loss of consciousness is common
-Indirect injury more common: Contrecoup Impact
B. Management
-Confirm history of trauma and immediate vision loss
-Corticosteroids, Optic Canal Decompression, Observation all had similar visual outcome
IV. Segmental Optic Nerve Hypoplasia: Topless Optic Disk Syndrome
A. Characteristics
-Segmental optic nerve hypoplasia: Superior disk pallor, sectoral scleral halo, relative
superior entry of central retinal artery, superior NFL loss with inferior field defect
-Blind spot oriented visual field defects
-Young patients, asymptomatic for field loss
-Women > Men
-Bilateral > Unilateral
-Visual acuity 20/20
-Normal color vision, pupil response
B. Management
-Patient Education: Link to poor maternal diabetic control (Type I DM and gestational),
shorter gestation time, low birth weight
-Recommend siblings have eye exam with screening visual field
-Monitor with visual field every year
- Avoid misdiagnosis
-Avoid unnecessary medical testing
V. Nutritional Optic Neuropathy
A. Characteristics
-Painless, bilateral vision loss
-Central or cecocentral scotoma (papillomacular bundle)
-Bilateral nerve pallor/atrophy, temporal
-Associated with alcohol, tobacco, methanol, tuberculosis medications, epilepsy
medications (Vigabatrin), Disulfiram
-Prisoners of War
B. Management
-MRI to rule out tumor
-Visual Field, Color Vision, OCT
-B1, B2, B6, B9, B12, Folate, Cysteine
VI. Chiasmal Syndrome
A. Characteristics
-Related to trauma
-Young males, motor vehicle accidents, skull fractures
-Direct tearing, contusion hemorrhage, contusion necrosis of optic chiasm
-Bow Tie Atrophy
-Compared to pituitary adenoma
B. Management
-Visual field: common to have either bitemporal defect or total field loss one eye and
temporal hemianopia in fellow eye
-MRI
-30% go on to develop Diabetes due to endocrine damage
VII. Optic Nerve Drusen
A. Characteristics
-superior of buried hyaline material, nasal > temporal
-75% bilateral
-congenital
-small cupping
B. Management
-B Scan for confirmation
-Baseline visual field, OCT
-VF defects most common with superficial drusen and increasing age
-Consider IOP lowering medication with ocular hypertension in presence of visual field
defect
VIII. Retinal Arterial Occlusion
A. Characteristics
-Pallor 2 months after occlusion, diffuse for CRAO, sectoral for BRAO
-Cilioretinal collaterals (CRAO)
-Sclerosed vessels
-Artery attenuation
-Macular RPE changes
B. Management
-Carotid Ultrasound
-EKG
IX. Optic Neuritis
A. Characteristics
-Multiple Sclerosis most common cause
-Autoimmune disease
-Adult women, people who live in high latitude
-Under 50 years old
-PAIN with vision loss, dyschromatopsia
B. Management
-IV methylprednisolone
-Interferon Beta-1a,b
-H/O Autoimmune disease (Sarcoidosis, Lupus)
Bibliography
Auw-Haedrich C, Staubach F, Witschel H. Optic Disk Drusen. Survey of Ophthalmology 47: 515-532,
2002.
DeWitt CA, Johnson LN, Schloenleber DB, et al. Visual Function in Patients with Optic Nerve Pallor (Optic
Atrophy). Journal of the National Medical Association 2003; 95: 394-397.
Foroozan R. Chiasmal Syndromes. Curr Opin Ophthalmol 14: 325-331. 2003.
Greenfield DS, Siatkowski M, Glaser JS, et al. The Cupped Disc: Who Needs Neuroimaging?
Ophthalmology 1998: 105: 1866-874
Grippo TM, Shihadeh WA, Schargus M, et al. Optic Nerve Head Drusen and VF Loss in Normotensive and
Hypertensive Eyes. J Glaucoma 2008; 17: 100-104.
Hassan A, Compton J, Sandhu A. Traumatic Chiasmal Syndrome: a series of 19 cases. Clincical and Exp
Ophthalmology (2002) 30, 273-280.
Hayreh SS. Management of NA-AION. Graefes Arch Clin Exp Ophthalmol (2009) 247: 1595-1600.
Hayreh SS, Podhajsky PA, Zimmerman MB, BRAO: Natural History of Visual Outcome. Ophthalmology
2009; 116: 1188-1194.
Hayreh SS, Zimmerman MB. CRAO: Visual Outcome. Am J Ophthalmology 2005; 140: 376-391.
Hayreh SS, Zimmerman MB, NAION: Natural History of Visual Outcome. Ophthalmology 2008; 115: 298305.
Kale N. Management of Optic Neuritis as a Clinically First Event of Multiple Sclerosis. Curr Opin
Ophthalmol 2012 Nov; 23 (6): 472-6.
Kim R, Hoyt W, Lessell S, et al. Superior Segmental Optic Hypoplasia A Sign Of Maternal Diabetes. Arch
Ophthalmol 1989; 107: 1312-1315.
Laundau K, Bajka JD, Kirschschlager B. Topless Optic Disks in Children of Mothers with Type I DM. Am J
Ophthalmol 1998; 125: 605-611.
Levin LA, Beck RW, Joseph MP, et al. The Treatment of Traumatic Optic Neuropathy: The International
ON Trauma Study. Ophthalmology 1999; 106: 1268-1277.
Orssaud C, Roche O, Dufier JL. Nutritional Optic Neuropathies. Journal of the Neurological Sciences 262
(2007) 158-164.
Mejico L, Miller N, Dong LM. Clinical features associated with lesions other than Pituitary Adenoma in
Patients with an Optic Chiasmal Syndrome. Am J Ophthalmol 2004; 137: 908-913.
Sarkies N. Traumatic Optic Neuropathy. Eye (2004) 18, 1122-1125.
Steinsapir K. Traumatic Optic Neuropathy. Current Opinion in Ophthalmology 1999, 10: 340-342.
Steinsapir K, Goldberg R. Traumatic Optic Neuropathy. Surv Ophthalmol 38: 487-518.
Smiddy WE, Green WR. Nutitional Amblyopia: Histopathologic study with retrospective clinical
correlation. Graefes Arch Clin Exp Ophthalmol (1987) 225: 321-324.
Vidyapeeth B. Optic Neuritis, its Differential Diagnosis and Management. The Open Ophthalmology
Journal, 2012, 6, 65-72.
Woon C, Tang RA, Pardo G. Nutrition and Optic Nerve Disease. Seminar in Ophthalmology, Vol 10, No 3,
1995: 195-202