13. PD IV - Paediatric Ocular and Systemic Conditions

Question 1

What are common reasons for paediatric consultations?

(15 points)

Answer 1

1. Fails maternal health nurse vision screening
2. Fails school screening
3. Paediatrician/GP referral
4. Systemic condition with ocular complications
5. Educational psychologist/teacher referral
6. Allied Health referral
7. Routine checkup/pro-active parents
8. Vision concerns
9. Eye turning
10. Learning concerns
11. Behavioural issues
12. Family history concerns
13. Sore, red eye
14. Trauma
15. Second or third opinion

Question 2

Paediatric Ocular Conditions
Condition, common?, who is best to treat?

..., ... common, ...
..., ... common, ...
..., ..., ...
..., ..., ...
... and ... eye conditions, ..., ...

Answer 2

Paediatric Ocular Conditions
Condition, common?, who is best to treat?

Refractive error, most common, optometrist
Accom-Verg dysfunction, second most common, optometrist
Strabismus, not noted, either optometrist/ophthalmologist
Amblyopia, not noted, either optometrist/ophthalmologist
Anterior and Posterior eye conditions, uncommon, either optometrist/ophthalmologist

Question 3

What are typical management options for children?

(7 points)

Answer 3

1. Spectacle/Contact correction
2. Medical therapy
3. Vision therapy
4. Referral coordination and follow-up
5. Monitor and review
6. Reassurance and routine review
7. Professional advice and recommendations

Question 4

Management of refractive error in children up to 3 years

Hyperopia: >...D in any meridian
Myopia: >...D in any meridian
Astigmatism: >...D at ... or ... or >...D at ...
Anisometropia: >...D

Answer 4

Management of refractive error in children up to 3 years

Hyperopia: >+3.50D in any meridian
Myopia: >-3.00D in any meridian
Astigmatism: >1.50D at 90 or 180 or >1.00D at oblique axis
Anisometropia: >1.50D

Question 5

Guidelines for Management of Refractive Error
Age, refractive error, How much to prescribe

1-4, >...D, ...
4-5, >...D, ...
5+, >...D, ...
5+, ... myopia, ... and ... if ...
3.5+, >...D anisometropia, ...
4+, >...D astigmatism, ...

Answer 5

Guidelines for Management of Refractive Error
Age, refractive error, How much to prescribe

1-4, >+3.50D, 1D less
4-5, >+2.50D, 1D less
5+, >+1.50D, full
5+, any myopia, full and near add if eso
3.5+, >1.00D anisometropia, full
4+, >1.50D astigmatism, full

Question 6

Retinoblastoma
Definition: ...

Occurs in ... years and presents as ... on Bruckner’s. Occurs in 1 in ... births in ... and .... It’s ... and ... and requires ....

Answer 6

Retinoblastoma
Definition: malignant tumour of embyronic retinal cells

Occurs in first 2 years and presents as leukocoria on Bruckner’s. Occurs in 1 in 15,000 births in US and Northern America. It’s very serious and life-threatening and requires immediate referral.

Question 7

Congenital cataracts

Results in ... or ... red reflex, ..., and may present similar to .... Treated with ... and maybe ....

Answer 7

Congenital cataracts

Results in limited or no red reflex, poor sight, and may present similar to leukocoria. Treated with surgical lens extraction and maybe IOL insertion.

Question 8

Ectopia Lentis

Otherwise known as ... or ....
May be associated with ... and ....

Answer 8

Ectopia Lentis

Otherwise known as displaced lens or dislocated lens.
May be associated with Marfan Syndrome and other genetic syndromes.

Question 9

Aniridia

Kids are born with ..., often comes with ..., ..., ..., and is often ....

Answer 9

Aniridia

Kids are born with no iris, often comes with cataracts, poor sight, nystagmus, and is often bilateral.

Question 10

Microphthalmos

... globe with marked ... and .... These eyes usually have ....

Answer 10

Microphthalmos

Small globe with marked hyperopia and macular hypoplasia. These eyes usually have not much vision.

Question 11

Optic Nerve Hypoplasia

... optic nerve and may/may not have .... It is typically laterality, the size of the ... is ..., and it is often associated with .... The distance from disc margin to macula is usually >...DD.

Answer 11

Optic Nerve Hypoplasia

Under-developed optic nerve and may/may not have reduced vision. It is typically bilateral, the size of the disc is small, and it is often associated with gestational diabetes. The distance from disc margin to macula is usually >2DD.

Question 12

Retinopathy of Prematurity

Incomplete ... leads to ..., can result in ... and .... Plus disease (stages I to IV) based on ... and .... Parents may forget during history; need to ask ....

Prematurity of ... weeks, birth weight below ...kg, and/or getting ... at birth. The last point triggers ADE in retina and ...; kids often require ... at birth.

Answer 12

Retinopathy of Prematurity

Incomplete retinal vessel growth leads to vascular disruption, can result in detachment and vessel tortuosity. Plus disease (stages I to IV) based on neovascularisation and haemorrhages. Parents may forget during history; need to ask Were there any issues at birth?.

Prematurity of <30 weeks, birth weight below 1.5kg, and/or getting supplemental oxygen at birth. The last point triggers ADE in retina and retinal vessel growth; kids often require laser treatment at birth.

Question 13

Papilloedema - comanaged with ophthalmology

Usually *laterality with ... and ... of the disc. Results from ... due to ..., ..., .....

Answer 13

Papilloedema - comanaged with ophthalmology

Usually bilateral with vascular tortuosity and swelling of the disc. Results from raised intracranial pressure due to brain tumours, hydrocephalous, acute disseminated encephalomyelitis.

Question 14

Optic nerve head drusen

Usually laterality but can be laterality. Drusen is ... and results in a ... appearance. The cause is unknown, but may be associated with ....

Answer 14

Optic nerve head drusen

Usually unilateral but can be bilateral. Drusen is acellular calcified deposits and results in a lumpy, bumpy appearance. The cause is unknown, but may be associated with high hyperopia.

Question 15

Toxoplasmosis

Infection carried from .... Big signs are ... which is generally ... and an ... that has a ... at the posterior pole and macula.

Two forms:
static lesion/... - ... that is ...
progressive lesion/... - ... with a ... appearance

Answer 15

Toxoplasmosis

Infection carried from cat faeces. Big signs are posterior uveitis which is generally self-limiting and an atrophic scar that has a round, pigmented edge at the posterior pole and macula.

Two forms:
static lesion/inactive - chorioretinitis that is pigmented
progressive lesion/active - active inflammation with a foggy appearance

Question 16

Toxocariasis

Infection carried from .... Big signs are ... and a .... Common in ...-... year olds.

Answer 16

Toxocariasis

Infection carried from dog faeces. Big signs are posterior uveitis and a worm-like parasite infection living in retina. Common in 4-8 year olds.

Question 17

Fundus flavimaculatus
Otherwise known as ....

Most common form of ... in children; involves ... and is laterality. Follows an ... inheritance pattern and is quite rare, 1 in ... children. Loss of ... in ... or first ... years of life.

Answer 17

Fundus flavimaculatus
Otherwise known as Stargardt Disease.

Most common form of macular degeneration in children; involves progressive loss of central vision and is laterality. Follows an autosomal recessive inheritance pattern and is quite rare, 1 in 10,000 children. Loss of central vision in school age or first 20 years of life.

Question 18

Congenital/Childhood glaucoma - presentation

Cornea looks ..., ..., and .... Affects ...-... year olds and generally won’t be seen after the higher age .... Other symptoms: ..., ..., ..., and .... Affects ... more than .... Typically laterality but very ...; if laterality, ... is ....

Answer 18

Congenital/Childhood glaucoma - presentation

Cornea looks enlarged, swollen, and hazy. Affects 0-3 year olds and generally won’t be seen after the higher age untreated. Other symptoms: photophobia, excessive tearing, blepharospasm, and elevated IOP. Affects males more than females. Typically bilateral but very rare; if unilateral, one eye is larger.

Question 19

Glaucoma - definition and management

Definition: IOP > ...mmHg, ...or ..., ... and ..., ..., and ....

Management: ..., ..., typically ..., ... are temporary, and high risk of ..., ..., and ....

Answer 19

Glaucoma - definition and management

Definition: IOP > 21mmHg, disc cupping or asymmetry, corneal oedema and enlarged diameter, myopic shift, and visual field defect.

Management: immediate referral, exam under general anaesthetic, typically surgical treatment, drops are temporary, and high risk of impaired visual development, myopia, and amblyopia.

Question 20

Factors that place children at risk of visual impairment or reduced visual functioning

(9 points)

Answer 20

• Prematurity
• Low birth weight
• Oxygen at birth
• Infection of mother during pregnancy
• Difficult or assisted labour
• High refractive error
• Strabismus
• Anisometropia
• Known or suspected CNS dysfunction developmental delay, cerebral palsy, seizures, or hydrocephalus

Question 21

Down syndrome

Genetic condition, extra .... From conception with no ... or ... pattern and affects 1 in ... babies worldwide. Ocularly manifests as: ..., ..., ..., sensitivity to ... reduced ..., and ... and/or ....

Answer 21

Down syndrome

Genetic condition, extra chromosome 21. From conception with no social or ethnic pattern and affects 1 in 700 babies worldwide. Ocularly manifests as: strabismus, amblyopia, high refractive error, sensitivity to medication, reduced accommodation function, and vision impairment and/or cataract.

Question 22

Cerebral palsy

..., ... of ... and ... caused by damage to ... (... thought to be the cause of ...% of cases).
In Australia, 1 child is born with CP every ... hours and is the ... in Australia. Risk is greater in babies with .... Ocularly manifests as ..., ..., ..., and ....

Answer 22

Cerebral palsy

Permanent, non-progressive disorder of movement and posture caused by damage to developing brain (pre-natal trauma thought to be the cause of 75% of cases).
In Australia, 1 child is born with CP every 18 hours and is the most common disability in Australia. Risk is greater in babies with low birth weight. Ocularly manifests as visual impairment, high refractive error, strabismus, and cataract.

Question 23

Cerebral Palsy - Cortical/Cerebral Visual Impairment (CVI)

A deficit of visual function caused by ... to or ... of ...; ... to the LGN. Affects the ..., ..., and/or .... Common complication of cerebral palsy and affects ...-...% of children and has ... on VA. Can see ... with ....

Answer 23

Cerebral Palsy - Cortical/Cerebral Visual Impairment (CVI)

A deficit of visual function caused by damage to or malfunctioning of retro-geniculate visual pathways; posterior to the LGN. Affects the optic radiations, occipital cortex, and/or visual associative areas. Common complication of cerebral palsy and affects 60-70% of children and has variable effect on VA. Can see lesions with CT scans.

Question 24

Autism Spectrum Disorder

Lifelong ...; results in ..., ..., ... and ... interests and behaviours, and .... Effects of autism can be ... with .... Ocularly manifests as avoidance of ..., ..., ..., ..., ..., ..., ..., and ..., but ... is usually very well developed.

Answer 24

Autism Spectrum Disorder

Lifelong developmental difference; results in difficulty with social interaction, impaired communication, restricted and repetitive interests and behaviours, and sensory sensitivities. Effects of autism can be minimised with early intervention programs. Ocularly manifests as avoidance of eye contact, excessive blinking, strabismus, amblyopia, anisometropia, visual motor and sequencing problems, accommodation dysfunction, and astigmatism, but visual processing is usually very well developed.

Question 25

Juvenile Idiopathic Arthritis

An ... - ... and affects 1 in ... children in Australia. ... is required, looking for ..., ..., and .... Ocularly manifests as ... (...% overall and ...% in ...), ... laterality ... ..., and is ... as children don’t present with .... Complications include ..., ..., ..., and ....

Monitor every ~... months until puberty, ~... months until adulthood, and ~... year afterwards. If a child has ... without any other causes, send to ....

Answer 25

Juvenile Idiopathic Arthritis

An autoimmune condition - inflammation of joints and affects 1 in 1000 children in Australia. Blood work up is required, looking for ANA ESR, CRP, and HLA B27. Ocularly manifests as uveitis (8% overall and 20% in ANA+), asymptomatic bilateral non-granulomatous iridocyclitis, and is insidious as children don’t present with pain. Complications include cataract, glaucoma, band keratopathy, and phthisis bulbi.

Monitor every ~3 months until puberty, ~6 months until adulthood, and ~1 year afterwards. If a child has uveitis without any other causes, send to GP.

Question 26

Learning disabilities

... problems over represented in these groups. May impact ... so must test .... Can present as ..., ..., ..., ..., and ....

... and ... are common.

Answer 26

Learning disabilities

Visual efficiency problems over represented in these groups. May impact learning potential so must test visual efficiency. Can present as intellectual delay, language delay, motor delay, auditory processing delay, and visual processing delay.

Dyslexia and attention deficit hyperactivity disorder are common.

Question 27

Excessive blinking in children

Very common ... but is mostly ... and presents frequently in .... Around ...% of children between ...-... years have benign tics. Facial muscle spasms are usually ... and ... and improve with time and cause .... Some do go on to have ... in later life and should be ... if symptomatic. Excessive blinking is not necessarily associated with ... or ... disorders but must be ... to ensure any ... is uncovered and managed by .... Generally only need to ... and ... back to GP if you or parents are still concerned.

Answer 27

Excessive blinking in children

Very common presenting complaint but is mostly benign and presents frequently in pre-school years. Around 12% of children between 6-12 years have benign tics. Facial muscle spasms are usually benign and transient and improve with time and cause little functional impairment. Some do go on to have blepharospasm in later life and should be referred if symptomatic. Excessive blinking is not necessarily associated with ocular or systemic disorders but must be examined to ensure any possible ocular cause is uncovered and managed by way of exclusion. Generally only need to reassure patients and parents and report and refer back to GP if you or parents are still concerned.

Question 28

Psychogenic vision loss/Streff Syndrome
Otherwise known as ... or ....

Unexplained vision loss which is laterality at ... and/or ... with .... Able to pass some ... that should not be able to pass if .... No evidence of any ..., ... or ... causes.

Typically presents in children ...-... years, in ... more than ..., and there’s a coexisting .... Tricky to diagnose; needs to rule out any ... or ... cause such as ....

Answer 28

Psychogenic vision loss/Streff Syndrome
Otherwise known as functional vision loss or hysterical amblyopia.

Unexplained vision loss which is bilateral at distance and/or near with psychogenic origin. Able to pass some psychophysical tests that should not be able to pass if true visual loss. No evidence of any pathology, refractive or amblyogenic causes.

Typically presents in children 7-12 years, in females more than males, and there’s a coexisting emotional disturbance. Tricky to diagnose; needs to rule out any sinister or pathological cause such as Stargardt disease.