Optic Atrophy

In station 5 you may be asked to assess a patient with deteriorating vision in one or both eyes

History (3 mins)

  • Was the change in vision sudden/gradual/subacute
  • Is it painful or painless
  • Are 1 or both eyes affected
  • Is it getting worse
  • Is it intermittent ie. coming and going or constant
  • Is the problem blurring (near/far) vs distortion vs bits missing (centre/peripheral, top/bottom,) vs double
  • Is colour vision affected
  • Since when did the problem start
  • Does it matter whether it is day or night
  • Treatment so far
  • PMH eye problems, Dx, Fx, Sx

Examine (3mins)

  • VA (reduced or normal)
  • VF (central scotoma/peripheral field loss if pituitary tumour)
  • Pupils (normal/consensual light response only/+/-RAPD). Don’t miss an ARP (tertiary syphilis)
  • Fundo (Pale clearly demarcated OD). Don’t miss disc cupping of glaucoma, retinitis pigmentosa, CRAO (central retinal artery occlusion). Is the problem uni or bilateral.
  • Eye movements- don’t miss an INO!
  • Colour vision ( may be reduced, may have red colour desaturation)
  • Test for cerebellar signs if young
  • Feel temporal artery if patient is >50yo and don’t miss a temporal artery biopsy scar
  • Any hearing aid/bossed skull? (Pagets)
  • Do a quick neuro examination

Return to history after seeing a pale OD (if time allows) to ask about associated symptoms which might point towards an underlying cause:

  • Headache, vomiting, seizures (compressive)
  • In a young patient, pain on eye movement, numbness, unsteadiness or weakness suggests demyelination
  • CVS RFs: History of diabetes, hypercholesterolaemia and hypertension is common in patients with non-arteritic anterior optic neuropathy
  • Any history of increased eye pressures? (glaucoma)
  • In an older patient temporal pain, jaw pain, scalp tenderness, weight loss, fatigue and myalgia is suggestive of (arteritic ischaemic optic neuropathy due to GCA)
  • Drug history: drugs can be toxic to the optic nerve (e.g. ethambutol, amiodarone, alcohol, methotrexate, ciclosporin)
  • Fx (hereditary causes of optic neuropathies)
  • Any PMH of Infections or cancer

ICE and EXPLANATION (2 mins)

I can see by looking in the back of your eye that the nerve that supplies the eye has been damaged.  There are lots of causes for this. I need to do further tests before we can say for definite what has caused this. This will involve blood tests and a brain scan. I will refer you to a specialist eye doctor.

 

VIVA:

Optic atrophy (also termed optic neuropathy) is the loss of some or all of the nerve fibres in the optic nerve. It is an important sign of advanced optic nerve disease and is frequently seen in visual loss. Optic neuritis is the most common cause of optic nerve disease in younger adults, while ischemic optic neuropathy is the most common aetiology in older patients

Causes of a pale disc:

  1. Demyelination leading to optic neuritis
  2. Ischaemic Optic Neuropathy (non arteritic ie. Atherosclerotic vs arteritic ie. GCA)
  3. Compression
    1. Optic Nerve Tumour eg. Glioma, meningioma, metastasis
    2. Thyroid Eye Disease
    3. Granuloma
    4. Pituitary tumour/craniopharyngioma
    5. Aneurysm
    6. Pagets disease of bone
  4. Chronic glaucoma
  5. Retinal disease eg. CRAO/CRVO

Less common…

  1. Hereditary eg. Lebers optic neuropathy, Friedrichs Ataxia, DIDMOAD, Retinitis Pigmentosa
  2. Inflammatory
    1. Infective eg. Syphilis, TB, lyme, HIV, CMV, toxo
    2. Noninfective eg. Sarcoid, wegeners, SLE, behcets
  3. Nutritional eg. B12 def, B1 def, folate def
  4. Toxic eg. Tobacco, alcohol, methanol, ethambutol, isoniazid, cyanide, lead, arsenic, vincristine, ciclosporin
  5. Trauma

Investigations:

Optic atrophy is diagnosed on fundoscopy and may be confirmed with optical coherence tomography (a quick and painless imaging technique that can be performed in the outpatient clinic)

Further investigation may then be required to assess function, such as formal visual field and colour testing.

Further tests depend on the most likely cause….

If MS or compressive aetiology suspected: MRI brain and spine, LP, Visual Evoked Potentials. Start steroids!

If ION suspected: stroke type management eg. test glucose and lipids, BP, dopplers

If GCA suspected: ESR, CRP, PV, temporal artery biopsy

If Inflammatory cause suspected: ESR, syphilis, ANA, ANCA, Ca, ACE etc.

 

When thinking of the most likely cause think of:

  1. Is the patient old or young? Optic neuritis is the most common cause of optic nerve disease in younger adults, while ischemic optic neuropathy is the most common aetiology in older patients
  2. Speed of onset: rapid onset suggests demyelination, inflammation, ischaemia or trauma. More gradual onset suggests compressive, toxic/nutritional and hereditary causes
  3. Uni/bilateral. Bilateral- demyelination, chiasmal compression, glaucoma, inherited, nutritional, toxic. Unilateral- demyelination, compression, ION, retinal, trauma

Management = treat the cause! Eg. Remove pit tumours, for ION manage CVS risk, for optic neuritis give steroids, replace b12 if deficient.

 

Written by Sarah Kennedy

Resources used include those on the references section of this webpage and also:

https://patient.info/doctor/Optic-Atrophy

https://www.uptodate.com/contents/optic-neuropathies?source=search_result&search=optic%20atrophy&selectedTitle=1~120