Amblyopia Flashcards
Aged 3 years.
Fundus and media normal. Refractive error RE: +0.25 LE +0.50 Not prescribed
Possible inward turning of left eye noticed since a baby.
VA R: 0.0 (6/6) L: 0.8 (6/38)
CT N & D Sl LET
OM Full
Fixation Ophthalmoscope RE Central LE Nasal, parafoveolar, unsteady
- Strabismic amblyopia
- Fundus and media check confirms no pathology
- Fixation ophthalmoscopy of right (better eye) assesses cooperation
- Fixation is adjacent to foveola, on side of retina towards nose
Aged 6 years.
Referred after routine visit to optometrist
Fundus and media normal. Refractive error RE: +6.00 LE +1.50
Glasses prescribed: R: +5.00 L: +0.50 (kids tend to overaccommodate so we may reduce their prescription slightly to allow them to relax into the glasses)
Has worn glasses for 10 days
VA with gls R: 1.00 (6/60) L: -0.1 (6/4.8)
CT with gls N & D No deviation
4ΔPRT R central suppression (foveal suppression, eye doesn’t move)
Frisby 600 secs of arc
- Anisometropic amblyopia
- Refractive adaptation should be allowed prior to start of occlusion
- If no improvement order full prescription as accommodation may be reduced in an amblyopic eye and this patient is undercorrected
Aged 8 years.
Has attended Eye clinic and Orthoptics since 6 months old
Had left unilateral congenital cataract at birth. Surgery to remove this at 4 months of age with intraocular lens (that is a lens placed within the eye).
Glasses prescribed: R: +1.00 L: +1.50/-0.75@180
VA with gls R: -0.1 (6/4.8) L: 0.6 (6/…)
CT with gls N & D sl LET
Bag gls Left suppression
Fixation Ophthalmolscope RE Central LE Central, unsteady
- Stimulus deprivation amblyopia
- Strabismic amblyopia
- Visual acuity with pinhole checks that reduction not due to need for change in refractive correction
- Care with occlusion to ensure intractable diplopia does not occur
What is the definition of Amblyopia?
A unilateral or bilateral decrease of visual acuity caused by pattern vision deprivation or abnormal binocular interaction for which no causes can be detected on physical examination of the eye, and which in appropriate cases is treatable by
therapeutic measures
Reduced visual acuity which is not the result of any current pathology and which cannot immediately be improved by the correction of the refractive error.
What’s the prevalence of amblyopia like?
Hashemi et al (2018) conducted a global & regional estimate of prevalence of amblyopia with a systematic review & found the pooled prevalence to be 1.75%. Europe reported a higher prevalence (3.67%) than Africa (0.51%). The most common cause of amblyopia was anisometropia (61.64%).
Overall estimated to occur in between 2 and 4% of children
Large studies estimate between 1.6% Kvanstrom et al (2001) and 3.6% (Williams et al 2001)
Higher in medically underserved / lower socioeconomic classes (Williams et al, 2008). Populations may encounter barriers to accessing healthcare (Ahmed et al, 2011)
What do we need to develop for visual acuity?
Normal visual experience necessary
Rods and cones synapse with ganglion cells in retina
Parvocellular (X) system needs
stable, well focused image (central vision)
Lateral geniculate body – relay station
Cells in visual cortex
What might interfere with normal visual input?
- Born with cataracts
- Mutation of the FOXL2 gene – BPES (blepharophimosis, ptosis, epicanthus inversus, and telecanthus)
- Coloboma (abnormal development of the eye)
- Retinopathy of premature
- Fetal alcohol syndrome
- Maternal Measles/maternal general health
Strabismus - Ptosis
- Bleed in the eye
- Children are born hypermetropic as the eye isn’t big enough for the refractive power of the eye +2.0DS or +3.0DS and emmetrisation occurs to become emmetropic over time. If born myopic it would affect their vision or if one eye is more hypermetropic than the other
When in Amblyopia are we more likely to see an improvement?
Amblyopia develops during the critical period when impaired input affects neural plasticity.
Critical Period (7-8yo)
Deprivation results in loss of function
Sensitive Period (up to 12yo)
Improvement possible
What happens to ocular dominance columns during the critical period?
Ocular-dominance columns are not fully wired at birth, but take shape during the first months of life.
If one eye is not used during this critical period, neurons in the ocular dominance column that should receive visual information from the unused eye do not develop normally, and instead become wired to the normal eye as trying to make the most of the information coming into it. Approx 90% will go to the seeing eye = lack of cortical processing of the amblyopic eye.
Ocular dominance columns representing the eye that is not used waste away. Once the critical period ends, sight is permanently impaired.
What is a key attribute of amblyopia?
The crowding phenomenon in which a line of letters or symbols of the same size on a test type are identified less easily than single optotypes
How does amblyopia affect fixation?
Fixation preference i.e. in CT the fixing eye may quickly return to fixation and find it harder to take up fixation with the amblyopic eye.
May use a pseudofovea, parafoveal or paramacular fixation point in amblyopia.
What are the fixation patterns and strabismus in Amblyopia?
In strabismic amblyopia unilateral constant deviation expected
Aid in comparing the VISION in one eye to the other, when more accurate tests not possible. Consider:
- Rate of fixation
- Accuracy of fixation
- Ability to hold fixation ?through blink or version - Can see if there’s a strong fixation preference by how quickly they can fixate with one eye = have reasonable vision in this eye for accurate fixation. Do they hold fixation when the occluder is taken away? Can see if they hold it through a blink. Does a child tolerate the occlusion of one eye but not the other?
- Objection to covering one eye
Have cross-fixation:
Esotropia but prefer to look with right eye when looking to the left and prefer the left eye when looking to the right
What is uniocular fixation?
The point on the retina which is used for fixation when fellow eye is occluded
Use the graticule (circle on the ophthalmoscope) to test
What is central fixation?
The reception of the image of the fixation object by the fovea, the fixation object lying in the principal visual direction.
Central fixation may be steady or unsteady (unsteady = worse vision)
Fixation is more unstable in amblyopic eyes than in fellow eyes and control eyes (Chung et al, 2015)
What is Eccentric fixation?
A uniocular condition in which there is fixation of an object by a point other than the fovea.
This point adopts the principal visual direction.
The degree of eccentric fixation is defined by its distance from the fovea in degrees.
What is wandering fixation?
A uniocular condition in which the fovea has lost its functional superiority and no one retinal element is used for fixation.
Worse fixation of all
What is fixation like in amblyopia?
VA is reduced if fixation is not central or if unsteady
Area of retina used dictates potential VA but amblyopia superimposed on this may reduce VA further
Where amblyopia is present, uniocular fixation may be assessed and sometimes fixation can get back on the fovea
What are the types of amblyopia?
Strabismic
Anisometropic
Stimulus Deprivation
Ametropic
Meridional
Idiopathic
Toxic
Reverse/Occlusion
Psychogenic
Which type(s) of Amblyopia is/are usually bilateral?
Ametropic
Meridional
Idiopathic
Toxic
What is Refractive Amblyopia?
Anisometropic or Ametropic Amblyopia
Uncorrected refractive errors are considered the most common cause of amblyopia. There are two main types of refractive amblyopia. Refractive errors have to be high enough to prohibit a clear retinal image at any distance. Patients with 3D of myopic anisometropia or more, 1.5D to 2D of astigmatic anisometropia and only 1D of hyperopic anisometropia are considered at risk for developing refractive amblyopia.
What is Anisometropic Amblyopia?
Refers to unilateral amblyopia caused by a distinct refractive error of each eye.
Anisometropic amblyopia is likely in the presence of 1.0–1.5 D or more anisohyperopia, 2.0 D or more anisoastigmatism, and 3.0–4.0 D or more anisomyopia.
What is Ametropic amblyopia?
Aka bilateral or isoametropic amblyopia
Due to bilateral high refractive error (ametropic amblyopia) result from large, approximately equal, uncorrected refractive error in both eyes of a young child.
Presence of 5.0–6.0 D or more of myopia, 4.0–5.0 D or more of hyperopia or 2.0–3.0 D or more of astigmatism
Isoametropic amblyopia is caused by image blur due to a high amount of bilateral ametropia. Lower values of ametropia can also result in anisometropic amblyopia—in addition to blur, the difference in refractive error can cause abnormal binocular vision and suppression. Can occur bilaterally
What is Ametropic amblyopia also known as?
Bilateral or isoametropic amblyopia
What is Meridional amblyopia?
Caused by significant astigmatism. Tends to be bilateral.
What is Strabismic amblyopia?
Results from suppression of the deviating eye. Constant strabismus leads to more severe amblyopia than intermittent strabismus. It is important to note that the size of the deviation is not related to the development of amblyopia, nor is the size of the deviation related to the severity of amblyopia, and even a very small-angle, constant, unilateral strabismus can cause strabismic amblyopia.
What is Deprivation amblyopia?
Amblyopia can be caused by form deprivation. Least common type but is typically the most severe form of amblyopia and develops when the visual axis is obstructed such as from ptosis, cornea opacities, cataracts, vitreous haemorrhage among others. Deprivation amblyopia is caused by a physical obstruction along the line of sight, which prevents a well focused, high contrast image on the retina.
What is Reverse Amblyopia?
Also known as occlusion amblyopia
Result of penalisation of the sound eye with patching or atropine during amblyopia treatment of the original amblyopic eye. Affects visual acuity but also binocularity, contrast sensitivity, grating acuity and central vs. eccentric fixation.
What is occlusion amblyopia also known as?
Reverse Amblyopia
What is Idiopathic amblyopia?
When there’s no known cause for the amblyopia (idiopathic)
What is psychogenic amblyopia?
Untapped potential so under the umbrella of amblyopia even though it’s not a true amblyopia
Most common in the teenage population of bilateral amblyopia.
Called functional visual loss or Streff syndrome, visual conversion reaction and hysterical amblyopia.
Often have multiple symptoms and reduced academic achievement.
Linked to visual or emotional stress and the prognosis is usually good. Visual acuity measures are variable, kinetic visual fields may spiral and an accommodative/vergence disorder is often present.
They may concentrate hard and read very slowly. It’s often emotional/ psychogenic.
What is Organic amblyopia?
In the strict sense of the definition of organic amblyopia, no lesion can be detectable and the amblyopia is irreversible. However, this term is used for conditions in which the reason for reduced visual acuity has been identified such as:
Achromatopsia - congenital cone deficiency.
Albinism - lack of pigment, translucent cornea, abnormal decussation of nerve fibres and associated with nystagmus
Macular degeneration.
Nystagmus.
Also the term may be used where the reduction in vision is reversible. i.e:
Tobacco amblyopia.
Other toxins.
Causes of truly organic amblyopia have been suggested but disputed:
Transient retinal haemorrhages at birth - follow up of series showed no detriment to visual acuity.
Malorientation of retinal receptors - unconfirmed.
What do you do if the amblyopia doesn’t respond to treatment?
A characteristic sign of amblyopia is that it responds to treatment but if it doesn’t it requires further investigation.
If not responding to treatment this will be organic amblyopia or a pathology that isn’t amblyopia.
However, Meridional can be slow to show improvements and need to ensure that their meridian is correct in their glasses. If there’s no improvement within 9 months this is where worry should occur. Find in anisometropia this can improve quickly with glasses alone and then can be pushed further with atropine/patching but no improvement we need to investigate the reason for this; need to ensure they’re following the correct treatment plan (that the kids and parents are saying the same thing also).
What is Toxic Amblyopia? & what are the types?
Toxic -
VA loss due to absorption of toxic agents such as ethambutol, cyanide, ibuprofen. Usually reversible (arsenic poisoning not reversible)
Tobacco amblyopia –
Toxins of tobacco constrict retinal blood vessels. Optic nerve very sensitive to tobacco – can cause optic neuritis (swelling of optic nerve).
Nutritional amblyopia -
Caused by deficit of vitamin B12. Often seen in alcoholics & with extreme diets. Complete recovery is possible with improved diet – but if deprivation is too prolonged VA damage permanent.
Alcohol amblyopia –
Painless bilateral loss of vision. Toxic effects of alcohol cause optic neuropathy. Alcohol depletes the body of nutrients – often associated with B1 deficiency
How do we investigate and diagnose amblyopia?
- Case History
- Fundus and media check
- Refraction
- Visual acuity
Crowded if possible
Interpret grating acuity with care
May include pinhole - Contrast sensitivity
Strabismic & anisometropic amblyopias may have mkd losses of CS especially at higher
spatial frequencies - Cover test – fixation pattern
- 10Δ prism test
- Uniocular fixation
- Neutral density filter test
Eyes with strabismic amblyopia may VA with
neutral density filters less than fellow
eye/other amblyopias
An initial examination should include VA, fixation, refractive error, sensory and motor fusion, stereoacuity, accommodation, ocular motility and a thorough ocular health evaluation. Amblyopic patients often perform poorly on crowded VA charts compared to their single-letter/line scores. On follow-up examination you must avoid obtaining an artificially high or low VA measurement. VA should be done before and after cycloplegia.
Which eye is more commonly affected in amblyopia?
Anisometropic amblyopia, with or without strabismus, occurs more often in left eyes than right eyes
59% left amblyopes among 2635 participants.
Strabismic amblyopia 50% left eye, 50% right eye
What is the aim for management of amblyopia?
To achieve and maintain maximum visual acuity
How do we manage amblyopia?
- Remove any cause for stimulus deprivaton and correct refractive error. Allow period of time (up to 18 weeks) for refractive adaptation
- Choose and agree a regimen of occlusion / atropine penalisation therapy
- Discontinue occlusion / penalisation therapy when maximum acuity is achieved and observe for stabilisation of acuity
- Go back to (2) if VA is not maintained
What must we consider in the treatment of amblyopia?
Critical / sensitive periods – what is the patient’s age?
Correction of refractive error
The refractive adaptation period (to see if glasses improve it alone or if further treatment is needed)
Further treatment
What is occlusion?
Reduction or prevention of visual stimulation by the embarrassment of vision. This may be full time or part time.