DIS - Optic Nerve II - Week 10 Flashcards by B H

What is megalopapilla, what is the lamina support like, and compared to normal, what is the risk of glaucoma like?

Congenitally large optic disc that has weak lamina support
Greater risk of glaucoma by 10x

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What kind of notch is often seen in megalopapilla?

Horizontal notch in the neuroretinal rim

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How should megalopapilla papients be treated as? Explain what this means (3).

Treat them as glaucoma patients
-visual fields
-IOP
-optic nerve head appearance photo

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How should individuals with tight scleral sheaths be treated as?

Ischaemia suspects

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Are blood vessel trifurcations an anomaly or normal?

Anomaly

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List three general kinds of anomalous blood vessel patterns to look out for. Are they common or rare? Are they reliable in isolation? Explain.

Branching trifurcations
Unusual insertions
Unusual tortuosity
Commonly normal, unreadable signs in isolation
-flag for close evaluation

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What is situs inversus and what two things is it often associated with?

It is a blood vessel distortion where there is a nasal detour of the temporal retinal vessels as they emerge from the disc
Associated with tilted discs and astigmatism

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What are congenital arterial loops? How may it affect vision? What can rarely happen (2)?

Blood vessel that leaves and returns to the same place
May give transient vision loss
Rarely gives occlusions and vitreous haemorrhages

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Can congenital venous shunts be acquired?

Yes in addition to congenital

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What occurs with congenital venous shunts and give an example of a shunt.

Blood takes an alternative path, often choroidal flow
-optociliary shunt, from the retina to the choroid at the optic nerve head

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How can acquired optociliary shunts occur? What can it be a possible re-establishment of?

Caused by chronic venous flow compromise (venous stasis) at the optic nerve head
Possibly formed by re-establishment of previously defunt/empty channels

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List four common causes of acquired optociliary shunts.

ON drusen
Vein occlusion
Problems with blood vessels around the optic nerve head (papillophlebitis)
Problems with optic nerve head (chronic papillodema, glaucoma, papillitis, optic nerve sheath meningioma)

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What happens to VA with optic nerve sheath meningioma?

Progressive vision loss from 6/6 to hand movement

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Is RAPD present with optic nerve sheath meningioma?

Yes, if its unilateral

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What happens to colour perception with optic nerve sheath meningioma?

Red saturation - dimmer in the impaired eye

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List four stages of optic nerve appearance with optic nerve sheath meningioma.

Early optic nerve head swelling
Late optic atrophy
Venous congestion
Optociliary shunts

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Does longstanding amblyopia ever get worse?

No, never

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What should suspected loops and shunts be differentiated from?

anastamoses

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How can congenital be differentiated from acquired optic loop/shunts?

Congenital - expect no VA loss or optic atrophy

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What should you suspect if you see an acquired optociliary shunt, vision loss, and optic atrophy?

Optic nerve sheath meningioma

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Who should you refer to if you suspect optic nerve sheath meningioma?

Neuroophthalmologist, ocular oncologist

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Is the growth of optic nerve sheath meningioma rapid or slow? How does its position affect the difficulty of treatment? Can it diffuse intracranially or is it confined to the orbital space?

Slow growth, but the location is critical as it impacts vision
-is difficult due to proximity to the optic nerve
Can also diffuse intracranially

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What three serial exams should be considered for a case of suspected optic nerve sheath meningioma?

Visual fields
Peripapillary RNFL with OCT
MRIs

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What are congenital macrovessels? What path do they take (3)?

Abnormal single vein
-either enter or cross foveal avascular zone
-or cross horizontal raphe

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What two things are congenital macrovessels rarely associated with?

Exudates and haemorrhages

What visual acuity do you expect with congenital macrovessels?

Normal

What is the cilioretinal artery derived from?

Short posterior ciliary artery

In what percentage of eyes would a cilioretinal artery be found? Describe where it is and what kind of appearance it has.

20% It is found emerging from the middle of the optic disc and runs horizontally toward the macula (90%), and is thin -has a hooked appearance

Is it a good thing to have a cilioretinal artery or does it present many problems? Explain.

Good to have -it supplies the macula, good to have, especially in cases of CRAO

How can a cilioretinal artery be distinguished from any other vessel with a similar appearance emerging from the middle of the optic nerve head?

It has a distinct hooked appearance

What should you do and look out for if you see a cilioretinal artery?

Mostly conservative management -photodocument for baseline and monitor for changes

What are pigmented peripapillary crescents? How are they different from scleral crescents? What makes deeper layer visible? What causes displaced pigment?

They are a pigmented crescent in the peripapillary region Scleral crescents are not pigmented Loss of overlying tissue makes deeper layers visible Mobilisation of the RPE gives displaced pigment

List 5 possible causes of peripapillary crescents. Describe the look for each.

Congenital - smooth pigment Ageing - degeneration, moth eaten look Myopia - tilted disc, stretching, ectasia Glaucoma - loss of tissue Trauma - pigment clumping

What is optic nerve staphyloma?

Weakness in the scleral development at the optic nerve

What changes can be seen at the optic nerve head with optic nerve staphyloma?

Posterior outpouching, with near normal optic nerve at the bottom of the pouch

Compare the appearance of optic nerve staphyloma to thinning/ectasia.

It looks similar, but the outpouching is not the same as thinning/ectasia

Is there risk of retinal detachment with optic nerve staphyloma as with coloboma? Explain.

Yes, but maybe not to the same extent

What changes can be seen surrounding the optic nerve in optic nerve staphyloma (2)?

Pigmentary and degenerative changes

Is vision affected in optic nerve staphyloma?

Yes, from 6/9.5 to hand movement

What three visual impairments are common with optic nerve staphyloma?

Strabismus Amblyopia Myopic astigmatism

What is the prevalence of tilted discs?

3.5%

What can usually be seen with tilted disc syndrome at the ONH (aside from tilting)?

Usually some ON hypoplasia

In what percentage of tilted disc syndrome cases is it bilateral?

80%

What happens to the nerve fibre layer with tilted disc syndrome?

Downstream thinning of the nerve fibre layer

Does tilted disc syndrome typically have normal or anomalous blood vessel insertion patterns?

Anomalous

List three possible reasons for visual field defects due to tilted disc syndrome?

Retinal ectasia Optic nerve hypoplasia Possible pituitary involvement

Why should visual fields always be assessed side-by-side?

For possible homonymity

What is generally the management for tilted disc syndrome?

Mostly conservative -photodocument

What should be done with the following if tilted disc syndrome is present: Refractive error Retinal thinning Unilateral vision loss

Refractive error - myopic astigmatism correction Retinal thinning - yearly review for potential rhegmatogenous retinal detachment Unilateral vision loss - consider amblyopia treatment

What type of retinal detachment is tilted disc syndrome a risk factor for?

Rhegmatogenous retinal detachment

What kind of visual field defect requires care in tilted disc syndrome? Explain what should be done.

Bilateral visual field defects -tilted discs may mimic bitemporal loss -best to refer

Is optic nerve drusen related to retinal drusen?

Noi

What is the cause of optic nerve drusen? Describe the pathophysiology (4).

Caused by slowing of axoplasmic flow Flower flow > axonal swelling at the lamina > rupture and extrusion of intracellular substances Mitochondrial calcifications follow to form drusen

What is the mode of inheritence for genetic optic nerve drusen?

Autosomal dominant

List two diseases that can cause optic nerve drusen.

Papilloedema Retinitis pigmentosa

Can optic nerve drusen be idiopathic or is the cause generally always known?

Can be idiopathic

What appearance does the cup have with optic nerve drusen?

Congested looking, small cup

What is the blood vessel pattern like with optic nerve drusen?

Anomalous

Where does optic nerve drusen usually first appear?

On the nasal disc margin

How does optic nerve drusen affect vision and visual fields?

Typically normal VA but VF losses can occur with age - arcuate losses

List three common peripapillary changes common with optic nerve drusen.

Optociliary shunts Deep choroidal haemorrhages RPE disruptions

Does buried optic nerve drusen tend to apepar in younger or older patients? What may it look like and what is this called?

Buried drusen in younger people May look like papilloedema -pseudopapillodema

Is buried optic nerve drusen easier or harder to find with a normal view? What is the recommendation here?

Harder -use autofluorescence, OCT, or ultrasound for subtle cases

Compare how papilloedema and ON drusen appear on OCT.

Papilloedema - smooth elevation Drusen - lumpy/bumpy

Compare how the cup appears with papilloedema and ON drusen.

Papilloedema - absent Drusen - present?

Compare the presence of anomalous blood vessels in papilloedema and ON drusen.

Papilloedma - absent Drusen - present?

Compare how the RNFL appears with papilloedema and ON drusen.

Papilloedema - dirty, not visible Drusen - clean, visible

Compare the presence of spontaneous venous pulsation with papilloedema and ON drusen.

Papilloedema - absent (digital?) Drusen - visible?

in what percentage of ON drusen cases is spontaneous venous pulsation thought to be present?

~20%

Compare the type of haemorrhages seen with papilloedema and ON drusen.

Papillodema - multiple flame Drusen - deep choroidal

Compare whether or not you would expect papilloedema and ON drusen to affect a patient's relatives.

Papilloedema - normal relatives Drusen - affected (according to autosomal dominant inheritence)

Compare whether or not headaches (and other neurologic pathology) are present with papilloedema and ON drusen.

Papillodema - present Drusen - absent

How should patients with ON drusen be managed (2)? What about if there is a visual field defect (2)?

Differentiate between buried ON drusen and true papillodema Monitor with age for ischaemic events If visual field defect present, monitor for progression -possibly more susceptible glaucomatous damage, consider IOP lowering treatment

What kind of disorders are phakomatoses?

Neurocutaneous disorders

What are phakomatoses characterised by?

Multiple tumours

Are phakomatoses malignant or benign? Explain.

Are or can become malignant

Why are phakomatoses of concern to optometrists?

Because all have ophthalmic tumours as the primary and is the most prominent clinical manifestationd in some affected individuals

What are hamartomas? Are they benign or malignant? Do these cells all descend from one single mutated cell? Explain

Overgrowths of local tissue that do not invade or spread -typically they are multiple aberrant cells, rather than having a single common aberrant cell

Define the base tissue for the following hamartomas: Haemangioma Neuroma Neurofibroma Astrocytoma Melanocytoma Choristoma

Haemangioma - blood vessels Neuroma - peripheral nerves Neurofibroma - nerve sheaths Astrocytoma - astrocytes Melanocytoma - melanocytes Choristoma - bone

What percentage of hamartoma is associated with other neoplasia? What is the significance of this?

15-25% -some of these can be malignant

What is a racemose angioma? Is it congenital or acquired?

Twisty, corkscrew, frilly, tortuous vessels -congenital

If a racemose angioma is local/regional, what should be noted and why?

Find where they end -could lead to a mass/tumour

List two possible outcomes if a racemose angioma ruptures.

Intraocular haemorrhage -death if haemorrhaging intracranially

What can be said of retinal haemangiomas if they are in isolation?

Possibly benign

What syndrome are retinal haemangiomas an indication for?

Von Hippel Lindau syndrome

At what age does Von Hippel Lindau syndrome manifest?

20 to 35 yoa

What three lesions may be seen with Von Hippel Lindau syndrome?

Retinal, spinal cord, and cerebellar lesions

What are the blood vessels like with Von Hippel Lindau syndrome?

Tortuous

Are exudates present with Von Hippel Lindau syndrome?

Yes

What percent of Von Hippel Lindau syndrome cases are bilateral?

50%

List two organs affected by Von Hippel Lindau syndrome other than the eye.

Pancreas Liver

List three cerebral complications of Von Hippel Lindau syndrome.

Epilepsy Psychiatric disorders Dementia

What does sturge-weber syndrome affect the development of?

Blood vessels

What is characteristic of sturge-weber syndrome?

Portwine stain -forehead -scalp -around the eye

What can be seen in abundance near the skin surface of the port-wine stain in sturge-weber syndrome?

Capillaries

What three tissues/areas of the bodies are involved with sturge-weber syndrome? Describe how.

Skin (port-wine stain) choroid (choroidal haemangioma) Central nervous system (siezures)

Describe the association between sturge-weber syndrome and glaucoma. What can be determined by the position of the port-wine stain?

Open angle glaucoma in 30-70% 60% glaucoma ipsilateral to the port-wine stain

What nerve do most cases of sturge-weber syndrome involve?

Ophthalmic division of the trigeminal nerve

How likely is sturge-weber syndrome with non-ophthalmic division involvement to give glaucoma compared to ophthalmic involvement?

Less likely

List four potential systemic complications of sturge-weber syndrome.

Cerebral angioma Epilepsy Hemiparesis Retardation

What can be said of choroidal haemangioma if it is isolated?

It is benign

Can choroidal haemangioma be part of sturge-weber syndrome?

It might

How does choroidal haemagiomas appear (colour and depth)?

Deep, orange/dark raised retinal region

What is common on the retinal surface of choroidal haemiangiomas?

Glial proliferation

What is common in the vitreous with choroidal haemangiomas?

White vitreal condensations

How do choroidal haemangiomas affect visual fields?

Causes local VF loss

List two risks associated with choroidal haemangiomas.

Retinal detachment Glaucoma

What disease can neurofibromatosis be seen in?

Von recklinhausens disease

What five ocular involvements can be seen in von recklinhausens disease?

Lumps (neurofibroma) in lids Iris (lisch nodule) Optic nerve head Retina Skin

What ocular condition can manifest with tuberous sclerosis?

Retinal astrocytoma

List the three types of retinal astrocytoma.

Flat - smooth, semitransparent Raised - calcified, multinodular Intermediate

Aside from the ocular manifestations, what is also commonly seen with tuberous sclerosis?

Angiofibromas (small red bumps on the face)

What is a melanocytoma? What may it form with age?

Pigmentary tumour of the optic nerve May form malignant melanoma with age

What is a potential complication neurofibromas and what is it a risk factor for?

Ptosis due to the weight Risk fo glaucoma

What is the management for all these lesions (2)?

Watch for stability VA and visual fields