Disorder of the Optic Nerve Head Flashcards
Name and Signs/Symptoms of this conditions
Pseudopapilodema - commonly caused by drusen - there is no ONH swelling.
Signs/Symptoms: abnormal blood vessel configuration, sharp peripapillary RNFL, peripapillary retina not elevated (only disc), , spontaneous venous pulsations and no exudates
What are the differentials for Pseudopapilloedema?
Buried disc drusen
Tilted disc
Small, crowded disc
Name and signs/symptoms of this condition
Papilloedema
Swelling (bilateral) ONH due to raised intracranial pressure - usually bilateral but can be asymmetric
Early: Mild disc hyperaemia with preservation of the physiological cup. Indistinct disc margins & RNFL striations. Spontaneous Venous pulsation is absent (may not be significant because it is also absent in about 20% of the population). DDx: hyperopia + buried drusen.
Established (acute): Normal or reduced VA. Severe disc hyperaemia, moderate disc elevation, indistinct margins, absence of physiological cup. Venous engorgement, peripapillary flame haemorrhages, cotton wool spots. Circumferential retinal folds (Paton lines) develop, especially temporally (Enlarged blind spot
Chronic: Disc elevation without haemorrhages or CWS Telangiectasia, optico-ciliary shunts and drusen-like crystalline deposits may be present A macular star is common.
Atrophic: Secondary optic atrophy: VA severely impaired; optic discs are white, slightly elevated, few blood vessels, indistinct margin
Name, description and sign/symptoms of this condition
Drusen - Common, often familial. They are benign but can cause visual field defects and may need monitoring for driving purposes.
Signs / Symptoms: Occasionally associated with ocular and systemic disorders (e.g., disc haemorrhages or Alagille syndrome) Superficial drusen can be found with FAF
What are the differential diagnoses for Papilloedema?
Established acute: malignant hypertension
What are the differential diagnosises for drusen?
Buried disc drusen
Name description and sign/symptoms of this condition
Name: small crowded disc
Description: anatomical variation caused by a normal amount of axons coursing through a small scleral foramen (disc).
Signs/Symptoms: Asymptomatic. Indistinct disc margins and no or minimal apparent cup. OCT: Pseudo ‘lumpy - bumpy’ contour of the subretinal hypo reflective space due to blood vessel shadowing artefact (*). Intact adjacent retinal architecture (arrows). No Visible cup.
What are the differential diagnoses for small crowded disc?
Pseudopapilloedema
Name, description and signs/symptoms of this condition
Name: tilted disc
Description: Characterised by a disparity between the maximum and minimum elevations of the surface of the disc. Can be congenital or acquired usually associated with myopic and astigmatic refractive error
Signs/Symptoms: Asymptomatic except when associated with myopic maculopathy. Blurred, elevated disc margins temporal/inferonasal peripapillary atrophy. OCT: elevation would be more pronounced
Name, Description and Signs/Symptoms of this condition (top images only)
Name: Buried Disc Drusen
Description: Similar to Drusen - ONH drusen are calcified deposits which may appear at the surface or buried within the disc. They may be caused by a combination of disturbance of metabolism in the axons, abnormal vasculature, and a small scleral canal.
Signs/Symptoms:
Usually asymptomatic but can cause chronic peripheral vision loss. Rarely transient visual obscuration. Clinical appearance: elevated disc with irregular margins. Superficial drusen are yellow and reflective.
FAF/B - scan: hyper - autoflurescent areas of FAF. Hyper - echoic on B - scan (arrow)
OCT: “Lumpy bumpy” subretinal hypo reflective space which may correspond with the contour of drusenoid deposits (*). Cysts with hyper - reflective walls (arrow).
What are the differentials for Buried Disc Drusen?
Psuedopapilloedema
Name this condition?
Central Retinal Vein Occlusion
Signs and Symptoms of CRVO
Mild hyperaemic disc swelling. Venous engorgement (dilated and tortuous) widespread retinal haemorrhages (flame, dot, intraretinal), hard exudates and variable CWS (cotton wool spot). Peripheral VFD or potentially central loss due to macular oedema from ischaemia. OCT and FA useful in diagnosis
What is GCA?
Giant Cell Arteritis - GCA is a granulomatous necrotizing arteritis with a prediction for the major aortic branches and the superficial temporal (STA), ophthalmic, posterior ciliary and proximal vertebral arteries
Signs and Symptoms of GCA?
Scalp tenderness, jaw claudication, optic neuropathy (AAION), temporal arteritis (Thickened, tender, inflamed nodular arteries), arterial pulsation, ocular motor palsies. Investigation: Biopsy (Gold standard)
Description of this condition
Non - artertic Anterior Ischaemic Optic Neuropathy - Infarction of the optic nerve head not associated with GCA (caused by lack of optic nerve perfusion or embolic disease. Caused by occlusion of short posterior ciliary arteries resulting in partial or total infarction of optic nerve head.
Name and Describe this condition
AAION - Artertic Anterior Ischaemic Optic Neuropathy
Infarction of the optic nerve head caused by giant-cell arteritis. Painless and profound vision loss (permanent)
What are the signs and symptoms of NAION?
Signs: diffuse or sectoral hyperaemic disc swelling often associated with small peripapillary splinter haemorrhages RAPD, unilateral visual field defect
What is Leber Hereditary Optic neuropathy?
Inherited form of vision loss. Although this condition usually begins in a person’s teens or twenties, rare cases may appear in early childhood or later in adulthood. For unknown reasons, males are affected much more often than females.
Signs/Symptoms for leber hereditary optic neuropathy
Typically, in males - third decade. Occasionally in females - any age. Initially unilateral visual loss. Fellow eye involved within 2 months
Bilateral optic atrophy
Signs: Disc hyperaemia and dilated capillaries (telangiectatic microangiopathy) Vascular tortuosity, swelling of peripapillary nerve fibre layer, Subsequent bilateral optic atrophy
Name and description of this condition
Maligant (accelerated) hypertension: Hypertension associated with rapidly progressive end organ damage, variable retinal hypertensive vascular changes
Signs/Symptoms of Diabetic Papillopathy
Uncommon, unclear aetiology typically affects young diabetic patients. Swelling with surface telangiectasia
Signs and Symptoms of Compressive thyroid optic neuropathy, compromised venous drainage can result in bilateral disc swelling in patients with cavernous sinus thrombosis or direct carotid - cavernous fistula
What is optic neuritis?
Typical optic neuritis associated with MS causes vision loss over hours to days, with the nadir approximately 1 week after onset. Vision loss may be subtle or profound. Usually, unilateral rarely bilateral. Age typically 18 to 45 years. Retro-orbital pain, especially with eye movement. Acquired loss of colour vision. Reduced perception of light intensity. May have other focal neurologic symptoms (e.g., weakness, numbness, tingling in extremities). May have antecedent flulike viral syndrome. Occasionally altered perception of moving objects (Pulfrich phenomenon) or a worsening of symptoms with exercise or increase in body temperature (Uhthoff sign). More recently, immune-mediated disorders of the CNS, such as neuromyelitis Optica spectrum disorder (NMOSD), have been described as causes of atypical optic neuritis. Atypical features include advanced patient age, severe vision loss with poor recovery, and simultaneous or rapidly sequential bilateral optic neuritis.
Signs/Symptoms of Optic Neuritis
Critical. Relative afferent pupillary defect in unilateral or asymmetric cases; decreased colour vision; central, cecocentral, or arcuate visual field defects.
Other. Swollen disc (in one-third of patients) usually without Peripapillary haemorrhages (papillitis most commonly seen in children and young adults) or a normal disc (in two-thirds of patients. retrobulbar optic neuritis more common in adults). Posterior vitreous cells possible.
Differentials of Optic Neuritis
Ischemic Optic neuropathy, acute papilledema, severe systemic hypertension, Orbital tumour compressing the optic nerve
Treatment Optic Neuritis: Steriod within 14days of decreased vision
What is retrobulbar neuritis?
Intraocular form of optic neuritis that may be associated with a variety of infectious and non-infectious. conditions. Most common type in adults. Associated with MS patient sees nothing doctor sees nothing.
Optic disc appears normal. Hyperaemic disc swelling, associated with peripapillary haemorrhages. Demyelination most common, sinus related (ethmoiditis)
What is papillitis Optic Neuritis?
Hyperaemia and oedema of optic disc. Associated with peripapillary flamed shaped haemorrhages. Inflammatory cells seen in posterior vitreous. Most common type of optic neuritis in children
ONH swelling, hyperaemia and oedema of the optic disc, may be with peripapillary flamed – shaped haemorrhages (common in children). Demyelination uncommon
What is Neuroretinitis - Optic Neuritis?
Combination of optic neuritis and macular inflammation. Cat scratch fever responsible for 60% of cases. About 25% of cases are idiopathic. Other causes include Syphillis Lyme disease, mumps and leptospirosis
Symptoms: ONH swelling and inflammation of the macula star painless unilateral visual impairment, vision gradually worsens over a week
Signs: Papillitis with peripapillary oedema, macular star appears as disc swelling settles, macular star resolves with a return to normal or near normal visual acuity over 6 – 12 months
What is Papilo Phlebitis Optic neuritis?
Optic disc vasculitis: uncommon condition that may be a variant of CRVO although it affects younger healthy individuals and has a better prognosis
Signs: uncommon condition that may be a variant of CRVO although it affects younger healthy individuals and has a better prognosis
Fill in this table
Disease
Associated Signs/Symptoms
Laterality
Visual Acuity
Optic Neuritis
Unilateral
Headache, nausea, pulsatile tinnitus
Rarely affected
Psuedopapilloedema
Asymptomatic
Arteritic anterior ischemic optic neuropathy
Unilateral
Sudden severe vision loss
Painless loss of vision
Sudden less sever vision loss
Compressive optic neuropathy
Unilateral
Unilateral
Sudden loss of vision - variable magnitude
Disease
Associated Signs/Symptoms
Laterality
Visual Acuity
Optic Neuritis
Pain especially with eye movements, RAPD
Unilateral
Sudden loss of vision - variable magnitude
Papilledema
Headache, nausea, pulsatile tinnitus
Bilateral, can be asymmetric
Rarely affected
Psuedopapilloedema
Asymptomatic
Variable
Rarely affected
Arteritic anterior ischemic optic neuropathy
Headache, pain while chewing, scalp tenderness
Unilateral
Sudden severe vision loss
Non-arteritic AION
Painless loss of vision
Unilateral
Sudden less sever vision loss
Compressive optic neuropathy
Proptosis, limited EOM movement
Unilateral
Gradual loss of vision
Infiltrative optic neuropathy
History of tumours, cancer, autoimmune, infection
Unilateral
Sudden loss of vision - variable magnitude
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Compare Primary Optic Atrophy and Secondary Optic atrophy
What is Primary Optic Atrophy?
Atrophy of the ON without swelling of the ONH caused by lesions on the visual pathway between the ONH (retrolaminar) and the LGN. Lesion anterior to the optic chiasm result in unilateral optic atrophy.
What are the signs of Primary Optic Atrophy?
White, flat disc with a clearly delineated outline, Reduction in the number of small vessels on the disc, Attenuation of the peripapillary vessels, Thinning of the retinal nerve fibre layer (red free filter; OCT)
TEMPORAL PALLOR of optic nerve head may indicate atrophy of papillomacular bundle. classically seen following demyelinating optic neuritis (MS).
BAND ATROPHY is a similar phenomenon caused by involvement of fibres entering the optic disc nasally and temporally. occurs in lesions of optic chiasm or tract and gives nasal as well as temporal pallor
What is the cause of Secondary Optic Atrophy?
long standing swelling of the ONH,
Causes: chronic papilloedema, Anterior Ischaemic Optic Neuropathy (AION)
Papillitis
What are the signs of Secondary Optic Atrophy?
White or dirty grey slightly raised disc with poorly delineated margins
○ Obscuration of lamina cribrosa.
○ Peripapillary retinochoroidal folds temporal to disc (Paton lines)
○ Reduction in number of small vessels on the disc
Surrounding ‘water marks’
What is causes Optic atrophy?
Caused by disease or trauma of inner retina or its blood supply. obvious on fundus examination
What are the signs of Consecutive optic atrophy?
Vascular attenuation or sheathing
- Old occlusion of the central retinal artery or ophthalmic artery
- Retinitis pigmentosa
- Toxic optic neuropathy
- Old vasculitis as in Bechet disease
Macular lesions giving rise to temporal disc pallor
- Toxoplasmosis
- Cone dystrophy
Chorioretinal atrophy
- Degenerative myopia
- Extensive Panretinal photocoagulation
Diffuse retinal necrosis
- Cytomegalovirus retinitis
- Acute retinal necrosis
Progressive outer retinal necrosis
Give a specific example where FAF would have a diagnostic purpose
Superficial drusen can be found with FAF - they will appear as hyperfluorescent spots below the RPE
Give a specific example where FA would have a diagnostic purpose
In Central Retinal Vein Occlusion (CRVO) FA can be useful in assessing the risk of neovascularisation proportional to degree of capillary non perfusion
What is red disease? Give an example of where missing red disease could affect your clinical work up
Red disease: when a patient’s data falls into the lowest 1% of the reference database (OCT scan colour will be red) but the patient doesn’t actually have glaucoma
Red disease can be the difference between diagnosing glaucoma and ocular hypertension.
Name 3 OCT scans and give an example of where they could be indicated
- RNFL scan - when RNFL thinning is suspected or a wedge defect
- Ganglion Cell scan - assessement of the innermost retinal layers
- Macular thickness scan - essential in assessing glaucomatous damage
