Paediatrics - Ocular Pathology Flashcards

1
Q

What is important to remember in children when it comes to pathology?

A

Children are not mini adults, they suffer from conditions differently. Key difference is that there is the lack of the subjective response so need to go off instincts more.

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2
Q

What are the most common childhood eye conditions?

A
  • Allergic eye conditions
    o Often with children this may be first reaction to the allergy – with adult they may report history of allergies
    o 3 types common in children:
     Vernal keratoconjunctivitis: cobblestone papillae (can get ptosis from this as they are so heavy), trantas dots (esp if afrocaribean origin irrespective of climate – inflammatory infiltrates round edge of cornea, symmetrical & round), stringy discharge, corneal involvement - needs referral in most cases, can start with sodium cromoglycate qds and cold compresses
     Seasonal allergic conjunctivitis: watery discharge, small papillae, lid chemosis, bilateral, rhinitis - avoid allergen, cold compresses, avoid rubbing, mast cell stabilisers and systemic antihistamines
     Acute allergic conjunctivitis: in response to allergen e.g. dust animal dander, pollen, type 1 reaction, sudden response, can be unilateral, lid chemosis, no papillae, no corneal involvement - should self-resolve in a few hours can provide lubricants for symptomatic relief. Often v swollen – different response to adults.
     Management:
  • Acute allergic conjunctivitis: need to be careful of ages the drugs are prepared for, some are from 1 month old, from 2 years or from 6 years etc – is it appropriate for that age of child to use it
  • Go non-pharmacological first – avoid allergen, cold compresses
  • If cold compress not enough then add in systemic antihistamine
  • If that isn’t enough then think mast cell stabiliser drops – takes 2-4 weeks before fully active – 4 times a day. Olopatadine (IP) – takes action much more quickly
  • VKC management: refer to ophthalmology, give lubricants for symptoms. Seen at ophthalmology, VKC in child is long term and so may be on steroid for long time and risk of high IOPs (then glaucoma) and cataract
  • Conjunctivitis
    o Often seen under age 5 as they like hugging each other and sharing germs
  • Chalazion/hordeolum
    o Chalazion may be there for few months – manage parents expectations more than anything
  • Cataract
    o Rare for us to see in children but prognosis bad if not treated
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3
Q

Describe management of allergy in children?

A
  • Need to be extremely careful with your treatment regime
  • Prescribe only within licensing laws
  • Systemic antihistamines:
    o loratidine/claritin licensed from age 2
    o piriton/chlorphenamine licensed from age 6
    o Sodium cromoglicate - no minimum age for generic (POM), but minimum age 6 for opticrom (P)!
  • Systemic antihistamines – stick to non drowsy if possible
  • Piriton is drowsy so avoid that
  • IP – sodium cromoglicate – no minimum age
  • Entry-level – minimum age of 6 for sodium cromoglicate
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4
Q

Describe management of conjunctivitis in children?

A
  • In babies, 2 types of infection should be suspected and when?
  • Chlamydia trachomatis incubation 5-14 days
  • Neisseria gonorrhoeae incubation 2-5 days
  • Management - Urgent referral to ophthalmologist due to risk of sight threatening sequelae and need for systemic treatment of child and parent(s)
    o Neonatal infections are not something we will see everyday – most of the time these will present within first 2 weeks so usually still under the care of the midwife.
    o Chlamydial – gradual onset. Does not respond to antibiotics.
    o Often newborn babies will get bacterial conjunctivitis – use antibiotics, if doesn’t clear up with this then know it is likely chlamydial
    o Gonorrhoeae – happens quickly, usually pronounced, often presents in hospital as mother and baby likely still there
    o Chances of seeing them in practice are rare
    o Bacterial infections are common in babies but up to the ophthalmologist to decide on the diagnosis as risk to sight
  • A patient presents with her mum with a 1 day history of a cold and gunky eye, both started yesterday, her mum feels her eye is more gunky and red today. Vision is unaffected. Cornea is unaffected. Patient is 2 ½ years old. Clinical appearance below. How would you manage this patient?
    o Will see bacterial conjunctivitis in children all the time
    o Chemosis quite striking in children – much more than adults – have preseptal cellulitis in the back of your mind – double check motility, visioin, pupils – can often turn into this from bacterial conj
    o Boil water then cool it down
    o Explain to parent that could spread to other eye – usually around day 5 things will start to get better
    o Public health guidance: children should go to nursery – but advise them to not share towel e.g. when washing hands
    o Only give antibiotic if clinically indicated – if px presents within first couple of days of the infection, reduces bacterial load, reduces the length of disease and reduces chance of it spreading it round the nursery. Nursery are usually keen for antibiotics to be given
    o Antibiotics – chloramphenicol ointment – put in 3 times a day, stays on eye longer – put in before they go to nursery, dinner time and before bed
    o Need to take into account their daily routine and how it sutis the family – less times a day means child doesn’t get drops as often and children don’t like drops
  • 2 common conjunctival infections in children:
    o Bacterial conjunctivitis (often gram positive organisms)
    o Adenoviral conjunctivitis
  • Keep eye on them
  • If corneal involvement then keep reviewing them in practice
  • Watery discharge
  • Check CMGs for advice on if children should stay home – changing
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5
Q

Describe management of chalazion/hordeolum in children?

A
  • Affects same glands but management different
  • Hordeolum – infection – happens more quickly, sore, red, tender
  • Chalazion – inflammatory – slow onset, granulomatous mass in meibomian gland, less red, not sore, harder
  • Hordeolum can get in gland of zeis (right at base of eyelashes) and moll (further back)
  • Chalazian can be in gland of zeis but more likely in meibomian gland in tarsum
  • Top image – in gland of zeis - eyelashes
  • Bottom image – in gland of moll – further back
  • Advise internal hordeolum may progress to chalazion
  • Bottom set of photo:
    o Top right – in tarsal plate
    o Tarsal plate in children is really thin and chalazian can break through
    o Shows difference in presentation between child and adult
    o More dramatic in child – pinker but will resolve
    o Counselling – this may be on going issu – minimize by doing lid hygiene
  • Management Chalazion
    o refer if not resolving, recurrent or if inducing astigmatism
    o most often self resolve, treat any associated blepharitis and advise hot compresses
  • Management Hordeolum
    o Normally hot compresses and lid hygiene
    o May prescribe topical antibiotics if copious mucopurulent discharge - be careful of patient age!
  • Only refer if recurrent and inducing astigmatism (can interrupt visual development – need referred and they may excise it)
  • Most chalazion are just annoying and take longer that you would like to disappear
  • Give them realistic expectations – bring them back in 1 month to check how it is getting on and making sure not inducing astigmatism – can grumble on for 3 months
  • Hordeolum – hot compress as blocked glands. Give antibiotics if lot of discharge – don’t have to do that very often
  • Both of these can have blepharitis
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6
Q

Describe cataract in children?

A
  • 1 in 3000 live births
  • Congenital cataracts normally spotted at new-born screening, however cataract can also develop through childhood
  • High risk of amblyopia with significant cataracts which develop before age 6-7, especially if unilateral
  • Often seen at newborn screening – but does not always develop in utero – so may be picked up at pre-school screening
  • Pxs at high risk of amblyopia
  • If under 6 – check media is clear
  • Even if small posterior subcaps – due to position – really high risk of developing amblyopia (especially if one eye)
  • Symptoms
    o Undiagnosed cataract may present with
    o Strabismus
    o Nystagmus
    o Clumsy behaviours
  • Signs
    o Cataract visible on retinoscopy
    o Childhood cataracts vary in appearance and have a different appearance to age related cataracts
  • Must do retinoscopy to see the cataract – really vital
  • Some childhood cataracts are not operated on if not affecting vision too much
  • Anterior polar cataract – usually develops in utero – not usually develop in adults
  • Most common type – hyaloid remnant – really extreme example. When babies are growing in womb, blood supply that supply eye in womb, this is the remnants of that. Often get a posterior remnant which looks like posterior sub caps – at nodal point and really disruptive to vision – most common to see a small dot but can be really disruptive to vision
  • Blue dot cataract, benign, not very central, not 100% opaque – not as disruptive to vision – centre of lens is quite clear so wouldn’t normally operate
  • Manage in secondary care due to high risk of amblyopia development
  • Child often heavily patched after surgery
  • Management
    o Urgent Referral if not previously diagnosed in an infant
    o If discharged from HES refractive correction
    o IOL then need multifocal lenses (but only in eyes with IOL if unilateral)
    o If cataract in situ then full refractive correction and assess accommodative function
    o Px may need bifocal – take out lens means lose accommodation just like adult
    o If cataract has been left then refract as normal as best you can
    o Px in 20s/30s may need vari in one eye and SV in other eye
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7
Q

What are the key signs indicating pathology in children?

A
  • White reflex: think retinoblastoma/cataract
  • Squint: ? underlying eye disease or nerve palsy  check squint is concomitant
  • Large eyes or foggy corneas: think glaucoma
  • Unhappy child with watery eyes: think glaucoma
  • Swollen eye with ↓ movement: cellulitis
  • Purulent conjunctivitis in an infant (v young baby): gonorrhoea/chlamydia
  • Glaucoma in children is less common – can see it but not common
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8
Q

What are the most common childhood posterior eye conditions?

A
  • Optic Disc Hypoplasia
  • Ocular Albinism
  • Foveal hypoplasia
  • Retinopathy of Prematurity
  • Autosomal dominant optic atrophy
    o Optic disc pit at risk of developing CSR as a late stage complication, work into your advice
    o Mention spontaneous venous pulsation as check for papilloedema
    o Everything in children is rare – children are generally healthy – all of them are so uncommon
    o LS has Seen the top 3 in practice
    o All rare  need to be aware that you are looking for normal and any deviation you need to work out what is going on
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9
Q

What are the most common causes of childhood blindness in USA?

A
    1. Cortical Visual Impairment
    1. Retinopathy of Prematurity
    1. Optic Nerve Hypoplasia
    1. Albinism
    1. Optic Atrophy
      o Cortical blindness 19% - Cortex hasn’t developed properly in utero
      o ROP 13% - if does occur it can have dire visual consequences
      o Optic nerve hypoplasia 7% - most commonly seen at back of eye in children – soome children are v affected by it and some arre not
      o Albinism 5%
      o Optic atrophy 5%
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10
Q

What are posterior eye conditions that a child may have?

A
  • Tilted and torsional optic discs
  • Optic disc hypoplasia
  • Optic disc coloboma
  • Optic disc drusen
  • Autosomal dominant Optic Atrophy
  • Papilloedema
  • Retinoblastoma
  • Retinal Dystrophies
  • Albinism
    o Tilted and torsional discs often have a stable visual field defect, 10% of population have stable vf defects which are physiological
    o Optic disc pit at risk of developing CSR as a late stage complication, work into your advice
    o Mention spontaneous venous pulsation as check for papilloedema
    o Top right – very tilted disc – doesn’t mean it is unhealthy
    o Papilloedema is a sign of other things – it is not a disease process in itself
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11
Q

Describe optic disc hypoplasia in child?

A
  • Diminished number of nerve fibres in the optic nerve
  • Relatively rare
  • Unilateral or bilateral
  • Linked to teratogenic agents
  • Agents which the mother is exposed to during pregnancy which can cause birth defects
  • Quinine, PCP(Phenylcyclidine), LSD, Cocaine, Heroin, Alcohol, Dilantin, Maternal diabetes
  • Numerous systemic associations
  • Septo-optic displasia
  • Developmental midline brain defects
  • Pituitary and hypothalamic defects
  • Ocular associations
  • Aniridia, VF loss, foveal hypoplasia, microphthalmos and AMBLYOPIA
    o PCP – aenasthetic, sedative agent
    o Dilantin - anti convolsuant used in eplipsy
    o Patients with hypoplastic discs tend to have smaller axial lengths and hence hyperopia but
    o Depends on severity but less nerve fibres in optic nerve then can affect quality of vision
    o A lot of it in West of Scotland – can be linked to alcohol, drugs etc
    o Can occur in isolation or with systemic associations
    o Reduced no. of nerve fibres  worse vision  likely amblyopia
    o Fovea also likely underdeveloped
  • Symptoms
    o If severe can present with roving eye movements nystagmus in infancy
    o If unilateral, or less severe may present with strabismus or decreased VA at visual screening
    o Mild cases may be asymptomatic
    o New strabismus  must check optic disc
  • Signs
    o VA from normal to LP
    o An RAPD if unilateral and severe and sluggish pupil responses bilaterally if both discs severely hypoplastic
    o Smaller than normal disc – measure using slit lamp if possible
    o A foveal to disc centre which is three or more times the disc diameter highly suggestive of hypoplasia – think it is smaller than normal
  • If you can fit it in then indicative of optic disc hyploplasia
  • Have in back of mind that optic disc may be smaller than normal
  • With strabismus, refer them to hospital
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12
Q

Describe optic disc coloboma in child?

A
  • Congenital, non progressive, unilateral or bilateral
  • In more pronounced cases may affect ability to meet visual field driving standard
  • Quite often vision is fine but may be significant VF defect and may impede ability to meet driving standard if bilateral – and they may need to declare to DVLA if it is severe
  • Consider automated perimetry to assess VFs earlier
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13
Q

Describe optic disc drusen in child and what is the management?

A
  • Often first noted in childhood
  • In younger children are often buried and may present as pseudopapilloedema
  • In early teens drusen often become more visible as pearly raised areas on the disc
  • Can cause visual field loss which may be progressive, but is rarely sight threatening
  • Can look like optic disc is raised
  • Note it so it can be monitored
  • Can see calcified appearance on the scan

Management:
* Assess automated visual fields for progression where possible
* OCT to assess/monitor RNFL and to assess drusen
* Refer if struggle to tell if optic disc drusen or papilloedema… may wish to discuss with opthalmology urgency of referral based on individual patient circumstances
* Really want to get it right – send images across to ophthalmology and they can decide if they want to see it and if the referral urgency
* No one will be annoyed if you refer if unsure

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14
Q

Describe papilloedema in child?

A
  • Symptoms/Signs
    o Headache, worse in the morning
    o Nausea and projectile vomiting
    o Diplopia due to 6th nerve palsy
    o Pulsatile tinitus
    o Swollen optic nerve
    o Visual obscuration lasting up to 30 seconds
    o Reduced VisionAbsence of spontaneous venous pulsation
  • Can be as result of many things – could be severe e.g. space occupying lesion
  • Need to get them referred to find out most likely cause
  • 6th NP path is longer – more easily affected by things
  • Spontaneous venous pulsation – if you can see that then can kind of rule out papilloedema – some people do not have it and they still may be healthy. Very unusual for papilloedema to have it
  • Differential diagnosis
    o Tilted discs
    o Disc drusen
    o Peripapillary myelinated nerve fibres
     Myelinated nerve fibres are usually in one area not usually the whole way round the disc
    o Crowded discs in hyperopic patients
  • No one will be annoyed if want to get this checked or not
  • Idiopathic Intracranial Hypertension:
    o The most widely utilized diagnostic criteria are the revised criteria proposed by Friedman et al. in 2013.A definitive diagnosis is established as the presence of:
    o Papilloedema
    o A normal neurological examination aside from 6th cranial nerve palsy
    o Normal CSF composition associated with an elevated opening pressure (>250 mm CSF in adults and > 280 mm CSF in children)
    o Neuroimaging (MRI typically, or CT if MRI is contraindicated) showing normal brain parenchyma and no evidence of mass or structural lesion, hydrocephalus, or meningeal enhancement
    o In the absence of papilloedema, a definitive diagnosis requires a 6th nerve palsy
    o In the absence of papilledema and a 6th nerve palsy, a suggestive diagnosis requires 3 of 4 neuroimaging findings
    o A diagnosis is deemed “probable” if all other criteria are met but no lumbar puncture is performed to determine opening pressure
  • IIH – less common in children
    o Emergency referral to ophthalmology
  • Management:
    o Emergency referral to ophthalmology
  • Bilateral & asymmetric
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15
Q

Describe autosomal dominant optic atrophy?

A
  • Hereditary disorder – most common hereditary optic neuropathy
  • Presentation in 1st and Second decade of life with progressive vision loss
  • Most patients maintain VA above 6/60
  • Colour vision disorders
  • VA loss is symmetrical, insidious, slow and gradual
  • Typically temporal wedge shaped atrophy but can be diffuse atrophy of the NRR
    o Look for symmetrical wedge shaped are of atrophy – where it is much paler, usually temporal. Look for gradual reduction in vision – if vision keeps getting worse into teenage years
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16
Q

Describe retinoblastoma in child?

A
  • Rare childhood condition, however it is the most common ocular childhood cancer. 1 in 18,000 live births
  • Can be unilateral (60%) or bilateral
  • Symptoms
    o White pupil
    o Strabismus
    o Associated painful red eye with secondary glaucoma
    (if left long enough)
  • Signs
    o Dome shaped white elevation(s)
    o White elevation of the retina which project into the vitreous
    o Subretinal mass(es) causing an overlying retinal detachment
  • Management - Urgent referral to ophthalmology – convo on call with ophthalmologist
  • Relatively rare – most common child ocular cancer
  • They need dealt with imminently – they can become life-threatening
  • If very raised retinal detachment, means something is behind it pushing it forward
17
Q

Describe retinal dystrophies in children?

A
  • Stargardt macular dystrophy - fundus appearance often out of proportion in early stages, colour vision defects, poor dark adaptation central VA will eventually drop sharply from 6/12 to 6/6-0 before stabilising
  • Best vitelliform macular dystrophy - loss of central vision as disease progresses though childhood/early teens
  • Retinitis pigmentosa - poor dark adaptation, notice peripheral vision deficits prior to any central visual loss, visual symptoms start later (if at all) in childhood)
  • Usher syndrome – associated hearing loss
  • Leber congenital amaurosis - severe vision loss from birth, fundus appearance may be confused with albinism - ERG confirms diagnosis
  • Bardet-Biedl syndrome – lots of non-ocular feature such as extra digits
  • Management
    o Referral to Opthalmology for definitive diagnosis
18
Q

Describe albinism in child & management?

A
  • OA – ocular albinism
  • OCA – oculocutaneous albinism
  • Signs
    o Foveal hypoplasia – lack of an observable foveal pit, or underdeveloped foveal pit
    o Poor VA
    o Translucent pinkish blue irides
    o Nystagmus - typically pendular and horizontal – because of reduced VA
    o Lack of fundus pigmentation with visbility of choroidal vessels
    o Abnormal VEP’s as fewer uncrossed fibres at chiasm
    o High RX – may have astig
    o Strabismus
    o Poor stereo
  • Albinism is a genetically determined, heterogeneous group of disorders of melanin synthesis in which either the eyes alone (ocular albinism) or the eyes, skin and hair (oculocutaneous albinism) may be affected
  • Tyronaise negative is full albinism positive is incomplete albino, va in tyronnairse negative often 6/6o with poor macular vascular arcades and foveal hypoplasia
  • Some pxs who have albinism may only have ocular symptoms – sometimes they have normal features elsewhere but their eye is paler
  • Management
    o Photochromatic lenses for everyday use
    o Sunglasses for summer
    o Advise use of hats with brim, especially in summer
    o Low vision aids
    o Referral to ophthalmology if previously undiagnosed
  • If have px who may struggles to fund photochromatic lenses – talk to ophthalmology, and they may be able to add it to the voucher but there is not code anymore (was taken off the voucher)
  • Some pxs can have really light funduses – so need firm diagnosis