Paediatric Ophthalmology Lectures Flashcards
(388 cards)
1
Q
Which key questions form a paediatric ophthalmology history?
A
Presenting complaint Age of onset, duration Uni/bilateral? Constant/intermittent? Discharge (watery/pus) Pain/photophobia/vision loss Affected contacts
2
Q
What birth history questions should be asked in paediatric ophthalmology?
A
Trauma?
Prematurity? (ROP)
C-section?
3
Q
How can photographs taken by parents aid a paediatric ophthalmology history?
A
Documenting changes in squint
4
Q
What are the main causes of ophthalmia neonatorum?
A
Chlamydia trachomatis
N. gonorrhoeae
S. aureus, Strep pneumoniae, Haemophilus, Pseudomonas
Herpes simplex virus
5
Q
How does gonococcal neonatal conjunctivitis present? (Note: Same for other bacteria but 2-5 days instead)
A
1-3d of birth:
Severe purulent discharge, lid oedema, chemosis, keratitis
6
Q
How does chlamydial neonatal conjunctivitis present?
A
4-28d of birth:
Mucopurulent discharge, papillae, pre-septal cellulitis
7
Q
How do you investigate and treat ophthalmia neonatorum?
A
MC&S: Prewet swab or conjunctival scrapings
Gonococcal: Cefotaxime IM
Chlamydial: Erythromycin
Other: Chloramphenicol (gram +ve) or tobramycin (gram -ve)
8
Q
What are the signs and symptoms of conjunctivitis?
A
Irritation (pain if cornea involvement) Conjunctival hyperaemia (max in fornix) Associated discharge
9
Q
What are the signs of bacterial conjunctivitis?
A
Bilateral mucopurulent discharge, papillary reaction, ‘glued eyes’, no itching
10
Q
How is bacterial conjunctivitis treated?
A
Topical Abx: Chloramphenicol
11
Q
How is viral conjunctivitis treated?
A
Infection control, spread prevention
12
Q
What are the signs and symptoms of allergic conjunctivitis?
A
Itchy, watery discharge, lid oedema, chemosis, mild papillary hypertrophy,
13
Q
How is allergic conjunctivitis treated?
A
Limit allergen exposure
Flare-ups: Cold compress, artificial tears, topical NSAID’s, corticosteroids, antihistamines, mast cell stabilisers
14
Q
What are the features of vernal keratoconjunctivitis?
A
80% atopic history, 5-15 years onset
Itchy, thick mucous discharge, cobblestone papillae, shield ulcers, keratitis
15
Q
How is vernal keratoconjunctivitis treated?
A
Steroids, subtarsal injection
16
Q
How does microbial keratitis present?
A
Painful red eye, mucopurulent discharge, photophobia, blurred vision
Epithelial defect, infiltrate, hypopyon
17
Q
How is microbial keratitis treated?
A
Controls ulcer swab
Topical Abx: Ofloxacin
18
Q
How are corneal abrasions treated?
A
Fluorescein drops to visualise
Topical anaesthesia, chloramphenicol, oral analgesia, pad
19
Q
What is blepharitis?
A
Meibomian gland (produces lipid layer of tears) infection in the lid margin, chalazia may be present
20
Q
How is blepharitis treated?
A
Cold compress, lid hygiene
21
Q
What is the difference between a chalazion and a stye/hordeolum?
A
Chalazia: Usually non-painful lumps from clogged oil gland deep in eyelid
Styes: Painful lumps near lid margin due to infection
22
Q
How does herpetic eye disease present and how is it treated?
A
Epithelial defect (dendritic ulcers seen via fluorescein drops) 3% aciclovir 5x daily, weekly review
23
Q
What is assessed when investigating a corneal foreign body?
A
Mechanism and depth of injury, intraocular pressure check
24
Q
What test is used to assess full thickness corneal laceration?
A
Seidel’s test via fluorescein drops
25
How do you treat corneal foreign body?
Remove if trained
| Chloramphenicol
26
What is hyphaema?
Blood in the anterior chamber
27
What types of ocular chemical injuries can occur?
Acid, alkali, thermal, UV
28
How are ocular chemical injuries investigated?
```
Intraocular pressure check, visual acuity, slit-lamp examination
Lid eversion (foreign bodies?)
```
29
How are ocular chemical injuries treated?
Irrigation (check pH), Abx, cycloplegia, vitamin C
30
How does juvenile idiopathic arthritis present in the eye?
```
Anterior uveitis (no symptoms/floaters/less VA)
Keratic precipitates, cataract, glaucoma, vitritis, CMO, IOP change, posterior synechiae
```
31
What could a hazy cornea indicate in juvenile idiopathic arthritis?
Pankeratopathy, CMO
| Vitreous cells in cornea
32
How is juvenile idiopathic arthritis treated?
Steroids, cycloplegia, methotrexate, anti-TNF agents
33
What is the main cause of endophthalmitis?
Recent eye surgery
34
How does endophthalmitis present?
Painful red eye, rapid visual loss, hypopyon, vitritis, hazy cornea
35
What pathology causes watering eyes and how is it treated?
Nasolacrimal duct blockage
| Massage/compress (persistent: syringing/probing)
36
How does pre-septal cellulitis present and what causes it?
Painful swollen lid, fever, malaise
| Staph, strep
37
How does orbital cellulitis present?
Sinusitis, facial cellulitis, dacryocystitis, dental abscess
| Swollen painful lids, proptosis, less eye movements, optic nerve dysfunction
38
What are the complications of orbital cellulitis?
Meningitis, cavernous sinus thrombosis, orbital/cerebral abscesses
39
What are the differentials of leucocoria?
Retinoblastoma, cataract, toxocariasis, Coat's disease, ROP, RD
40
What is pathogenesis of retinoblastoma?
13q14 deletion: 90% sporadic uni/bilateral primitive photoreceptor cell tumour
41
How is retinoblastoma treated?
External-beam radiation, plaque radiotherapy, cryotherapy, chemotherapy, enucleation
42
What features are seen in optic disk swelling?
Ill-defined disk margins, tortuous retinal vessels, cotton wool spots, haemorrhages
No symptoms or nausea /photophobia
43
How is optic disk swelling investigated?
OCT (drusen?), AF
44
What can ptosis and congenital cataracts cause?
Amblyopia
45
How are congenital cataracts treated?
Atropine, patching
46
In which embryological week does the retina become completely vascularised?
Week 36
47
What causes retinopathy of prematurity?
Angiogenesis following retinal ischaemia due to incomplete vascularisation
48
What were the main paediatric amblyopia trials?
ATS (PEDIG), ROTAS, MOTAS
49
What did ATOM stand for in the ATOM trial?
Atropine for the Treatment Of childhood Myopia
50
Which were the main ROP trials?
CRYO-ROP, ET-ROP, LIGHT-ROP, STOP-ROP, BEAT-ROP, PHOTO-ROP, HOPE-ROP, ROP1
51
What were the main paediatric cataract studies?
IATS, IOLu2
52
What were the results of the IOLu2 study?
Uni/bilateral IOL implant effects on cataract negligible
| Glaucoma risk when cohort >4 y/o
53
What were the main paediatric exotropia studies?
CITS, Newcastle Control Score
54
What did ATS stand for and how many studies have been published?
Amblyopia Treatment Studies
| 17 (#18 being reviewed)
55
How did ATS classify amblyopia according to BCVA?
Mild to moderate: >20/80 in amblyopic eye
| Severe: 20/100-20/400 in amblyopic eye
56
What is occlusion therapy to treat amblyopia?
Patching of the non-amblyopic eye to stimulate the amblyopic eye
57
What is penalisation therapy to treat amblyopia?
Atropine drops to blur vision in non-amblyopic eye to stimulate the amblyopic eye
58
What did the "Prescribed patching regimens for severe amblyopia" study reveal?
6-hour daily patching increased VA as much as full-time patching
59
What were the daily patching recommendations following the severe amblyopia study?
Mild: 2 hours daily
Moderate: 4 hours daily
Severe: 6 hours daily
60
What were the treatment criteria for the "Atropine vs patching for moderate amblyopia" study?
Patching group: >6-hours daily
| Atropine group: 1 drop daily
61
What were the results of the "Atropine vs patching for moderate amblyopia" study?
Mean VA improvement of 3 lines (20/60 to 20/30)
| Daily vs weekend atropine: Similar results after 17 weeks
62
When is the critical period for amblyopia treatment?
First 7 years of life
63
What amblyopia treatment may improve vision in those aged 7-12?
2-6 hours daily patching
| Near activities, atropine
64
What amblyopia treatment may improve vision in those aged 13-17?
2-6 hours daily patching
| Near activities
65
How effective are 2 hours daily patching and near activities to treat amblyopia in those aged 3-7?
Not at all according to the respective trial
66
What was the inclusion criteria for the refractive correction anisometropic amblyopia study?
No prior treatment
VA 20/40-20/250
Occlusion + spectacles
67
When is the glasses adaptation period?
16-18 weeks
68
What were the results of the refractive correction anisometropic amblyopia study?
77% >2 lines VA gain
60% >3 lines VA gain
Glasses alone work not occlusion therapy
69
What conclusions could be drawn from the amblyopia studies?
Moderate amblyopia: Occlusion vs atropine, 2 hrs vs 6 hrs daily patching, weekend vs daily patching all equally effective
Severe amblyopia: Part-time vs full-time patching equally effective
70
What studies examined amblyopia occlusion treatment compliance rates? How so? What were the results?
MOTAS, ROTAS
Occlusion dose monitor
<50% compliance rate, parents respond to child's discomfort
71
What is the global incidence of RD in >6D myopia?
3%
72
What did ATOM1 investigate?
Effect of 1 daily 1% atropine drop in 1 eye over 2 years to treat myopia
73
What were the results of the ATOM1 study?
77% reduction in myopic progression
| Increased myopia progression following treatment cessation
74
What did ATOM2 investigate?
Effect of 0.5% vs 0.1% vs 0.01% atropine over 12 months to treat myopia
75
What were the results of the ATOM2 study?
All doses: Similar effects
0. 5%: Increased post-cessation myopia progression
0. 01%: Less pupil dilation and accommodation loss
76
What was done in the 5-year ATOM2 extension trial?
Those with post-cessation myopia progression were restarted on atropine
77
What were the results of the 5-year ATOM2 extension trial?
Myopia progression and axial elongation change lowest in 0.01% atropine group
78
What does CRYO-ROP stand for? What were the results of this study?
Cryotherapy for ROP
Introduced threshold ROP in staging
50% had visual loss and RD despite treatment
79
What does ET-ROP stand for? What were the results of this study?
Early treatment for ROP
Introduced pre-threshold ROP
Poor visual and structural outcomes reduced by 5.5% and 6.6% respectively
80
What do HOPE-ROP and STOP-ROP stand for? What were the results of these studies?
High O2 percentage in ROP
Supplementary O2 therapy in ROP
Inconclusive results
81
What does LIGHT-ROP stand for? What were the results of this study?
Ambient light in ROP
| No effect on ROP progression
82
What did the PHOTO-ROP study investigate?
Digital fundus imaging vs indirect ophthalmoscopy in ROP screening
83
What were the results of the PHOTO-ROP study?
Digital fundus imaging sensitivity, specificity, accuracy depends on image quality
Immediate review <24 hours recommended
84
What did the BEAT-ROP study investigate?
Bevacizumab vs laser to treat stage 3+ ROP
85
What were the results of the BEAT-ROP study?
ROP recurrence: 4% with Avastin, 22% with laser
| Zone I/II ds benefit with Avastin
86
Which was the lowest effective Avastin dose in treating ROP?
0.031mg
87
What is the inheritance pattern of metabolic disorders affecting the eye?
Generally autosomal recessive
| X-linked recessive: Fabry’s disease, ornithine transcarbamylase deficiency
88
Which lysosomal storage disorders affect the eye?
```
Fabry’s disease
Cystinosis
Mucopolysaccharidoses
Neuronal ceroid lipofuscinosis
Neumann-Pick disease
Gaucher’s type 3
```
89
Which metabolic disorders have unique eye findings?
Fabry's disease
Mucopolysaccharidoses
Cystinosis
90
Which metabolic disorders have unique eye findings?
Fabry's disease
Mucopolysaccharidoses
Cystinosis
91
In which metabolic disorders are the eyes affected late in the disease course?
```
Mucopolysaccharidoses
Propionic acidaemia
LCHAD deficiency
Refsum's
Homocystinuria
```
92
In which metabolic disorders are the eyes affected late in the disease course?
Propionic acidaemia
| LCHAD deficiency
93
Which metabolic disorders have no effect on vision?
Fabry's disease
| Wilson's disease
94
Which metabolic disorders present with vision loss?
Batten's disease
| Gyrate atrophy
95
Which metabolic disorders affect eye movement?
Gauchers type 3
| Niemann-Pick type C
96
Which metabolic disorder presents with transient vision loss?
Ornithine transcarbamylase deficiency
97
What causes Fabry’s disease?
α-galactosidase A deficiency
98
What are the systemic signs of Fabry's disease?
Acroparesthesia
Angiokeratoma corporis diffusum
Neuropathy, renal failure
Hypertrophic cardiomyopathy, stroke
99
What are the ocular manifestations of Fabry's disease?
Corneal verticillata
Conjunctival + retinal vessel tortuosity
Retinal vascular occlusion, cataracts
100
What term is used to describe corneal verticillata? What causes them? Do they affect vision?
Vortex keratopathy (‘whorls’)
Fabry’s disease, amiodarone
No effect on vision
101
What fundoscopy signs are seen in Fabry's disease? What causes these?
Venous dilation, arterial narrowing, AV-nicking
| Cytoplasmic inclusions in endothelial cells and pericytes
102
What is the pathogenesis of corneal verticillata? How are they visualised? How are amiodarone deposits different?
Corneal epithelial basal layer intracellular inclusions
Slit-lamp, confocal scanning laser microscopy
Amiodarone deposits more reflective, different size
103
What conjunctival signs are seen in Fabry’s disease?
Venous dilation/tortuosity
Sludged blood, microaneurysms
Chronic chemosis
Lymphangiectasia
104
How do cataracts appear in Fabry's disease?
Bilateral wedge shape
| Along suture lines from posterior pole (Spoke-like)
105
What ocular symptoms occur following strokes in Fabry's disease?
Homonymous hemianopia (37%)
106
How is Fabry's disease treated?
ERT: Fabrazyme, replagal
107
What causes cystinosis? What is the pathophysiology?
CTNS gene mutation (cystinosin)
| Cystine buildup in kidneys, bone marrow, pancreas, muscles, brain and eye
108
How does cystinosis present systemically? Which syndrome is it linked to?
Renal failure, anaemia, short stature
| Fanconi’s syndrome
109
How does cystinosis present in the eye?
Crystals: Cornea, conjunctiva, retina (later erosions and NV)
Photophobia
110
How is cystinosis treated?
Topical cysteamine (0.44%)
111
What are the main types of mucopolysaccharidoses?
I: Hurler, Hurler-Scheie, Scheie
II: Hunter, IV: Morquio
VI: Maroteaux-Lamy
112
Which bodily systems do MPS affect?
Skeletal
Cardiovascular
Neurological
Eyes
113
Which MPS types involve corneal clouding, photophobia and exposure keratopathy? How are these treated?
```
I, IV, VI, VII
Corneal transplantation (PK, DALK)
```
114
What anterior segment changes occur in MPS?
Thicker peripheral cornea
Variable central cornea thickness
Altered corneal hysteresis
Thicker iris, narrower angle
115
What optic nerve changes occur in MPS?
```
Thicker sclera, greater optic nerve diameter, risk of high ICP damage
Optic atrophy (severe visual loss)
```
116
How does glaucoma present in MPS?
Raised IOP, optic disc GAG deposition, corneal opacity, poor dilation, visual fields affected
117
Retinopathy occurs in which types of MPS? How does it present?
I, II, III
Nyctalopia, peripheral field loss
II: Choroidal folds, maculopathy, RD
118
Which genes are affected in neuronal ceroid lipofuscinosis?
CLN1-10
CLN10: Congenital, 11p15, Cathepsin D
CLN1: Infantile, 1p32, Palmitoyl thioesterase
CLN3: Batten’s, 16p12
119
What is gyrate atrophy? What causes it?
Amino acid metabolism disorder
| Ornithine aminotransferase deficiency
120
How does gyrate atrophy present?
Progressive vision loss, myopia
| Cataracts, chorio-retinal degeneration
121
What lifestyle factor can slow gyrate atrophy progression?
Arginine restricted diet
122
What causes propionic and methylmalonic acidaemias?
Branched chain amino acid catabolism defects
123
How do propionic/methylmalonic acidaemias present?
Vomiting, drowsiness, acidosis, hyperammonaemia, late severe visual loss (optic neuropathy)
124
How are propionic/methylmalonic acidaemias treated?
Dietary protein restriction
125
Which protein is inadequately metabolised in homocystinuria? Where is this condition more prevalent?
Methionine
| Ireland (1 in 52000)
126
How does homocystinuria present systemically?
Development delay, seizures, PE, DVT
| Tall, long limbs, fair, blue iris
127
How does homocystinuria present in the eyes?
90%: Ectopia lentis
| Myopia, cataract, RD, RAO, glaucoma, anaesthesia risky
128
What is galactosaemia? What causes it?
Carbohydrate metabolism disorder
| Galactokinase deficiency
129
How does galactosaemia present? How can it be stabilised?
Jaundice, poor feeding, oil-droplet cataract (progress to lamellar)
Hepatosplenomegaly
Dietary control
130
Which sterol metabolism disorders affect the eye?
Cerebrotendinous xanthomatosis
| Smith-Lemli-Opitz syndrome
131
What causes cerebrotendinous xanthomatosis? How does it present?
Sterol 27-hydroxylase gene mutation (CYP27A1)
Diarrhoea, ataxia, seizures
Cataracts, optic neuropathy
Tendon xanthomas, atherosclerosis
132
How is cerebrotendinous xanthomatosis treated?
Chenodeoxycholic acid, statins
133
What causes Smith-Lemli-Opitz syndrome? How does it present?
Raised 7-dehydrocholesterol
Microcephaly, polydactyly, genital/heart/gut malformations
Cataract, ptosis, strabismus, optic atrophy
134
Which process involves LCHAD?
Mitochondrial fatty acid beta-oxidation
135
How does LCHAD deficiency present?
Hypoglycaemia, hypotonia, seizures, cholestatic liver
Chorioretinal breakdown, myopia
Visual loss, photophobia, nyctalopia
136
What types of Niemann-Pick disease are there? What causes them?
A, B: Sphingomyelinase deficiency
| C, D: Brain sphingolipid buildup
137
Which epidemiological group is most affected by Niemann-Pick disease types A and B?
Ashkenazi Jewish population
138
How do Niemann-Pick disease types A and B present?
A: Neurological degeneration
B: Hepatosplenomegaly
Cherry red spot, pre-orbital fullness, macular granular deposits
139
How does Niemann-Pick disease type C present?
NPC 1 or 2 gene mutation
Liver disease, splenomegaly, seizures, ataxia, dystonia
Vertical supranuclear gaze palsy
140
How is Niemann-Pick disease type C treated?
Miglustat
141
What causes Gaucher’s disease type 3?
Glucocerebrosidase gene mutation
142
How does Gaucher’s disease type 3 present? How is it treated?
Oculomotor apraxia (defective horizontal saccades), intermittent strabismus, vertical gaze palsies
Failure to thrive, hepatosplenomegaly
ERT: Recombinant glucocerebrosidase
143
What is ornithine transcarbamylase deficiency?
| How does it present?
Urea cycle disorder
Bilateral vision loss, encephalopathy, headaches
Sluggish pupils, nystagmus, flat VEP’s
144
What are the UK risk factors for Retinopathy of Prematurity?
<32 weeks gestational age, <1501g birth weight, supplemental O2, M>F, Caucasians, mechanical ventilation
145
Which aggressive form of ROP progresses rapidly?
AP-ROP
146
What are the UK risk factors for Retinopathy of Prematurity?
<32 weeks gestational age
| <1501g birth weight
147
What percentage of premature babies are affected by ROP?
20%
148
How is ROP detected?
Clinical examination
| No signs/symptoms
149
What retinal development occurs at 16 weeks gestation?
Blood vessels grow out from the optic nerve
150
Which cells form retinal blood vessels? In which gestational weeks do they grow then form vessels?
Spindle cells
Grow: 16 weeks
Form vessels: 29 weeks
151
What environmental factors affect the immature retina?
Systemic O2 levels
| VEGF levels
152
Which cells form retinal blood vessels? In which gestational weeks do they form then develop?
Spindle cells
Grow at 16 weeks
Form vessels at 29 weeks
153
What term is used for the state of the retina in early foetal development?
Physiological hypoxic state
154
What promotes early foetal vasculogenesis
Increased retinal thickeness thus increased local metabolic demand
155
What is the pathophysiology of ROP?
Premature neonate exposed to higher O2 levels, incomplete retinal vessel development, scarring, RD/blindness
156
What is the pathophysiology of ROP?
Premature neonate exposed to higher O2 levels, incomplete retinal vessel development, scarring, RD/blindness
157
What factors cause abnormal retinal vessel development?
Low IGF-1 (O2)
High VEGF (O2)
Genetics
Poor early weight gain
158
How does supplemental O2 affect ROP progression?
Retinal vasoconstriction
Vascular closure, endothelial loss
Proliferating endothelial nodules (popcorn) canalise through ILM
159
Which genes are linked to ROP?
```
FEVR similar phenotype
Norrie disease (NDP) gene
```
160
How is ROP classified?
Location (Zones 1-3), extent (clock hours), severity (0-5), plus disease, AP-ROP
161
How are zones 1-3 defined in ROP?
1: 2x optic disc diameter
2: Circumferential from nasal aura
3: Remainder of temporal region
162
How is stage 0 defined in ROP?
Immature retina
163
How is stage 1 defined in ROP?
Demarcation line of vascularisation
164
How is stage 2 defined in ROP?
Fibrovascular ridge (late: popcorn)
165
When does a double demarcation line form?
Stage 2/3 ROP regressing: Retinal vascularisation passes the ridge then recurs
166
When does a double demarcation line form?
Stage 2/3 ROP regresses: Retinal vascularisation passes the ridge then recurs
167
How are stages 4a and 4b defined in ROP?
Partial retinal detachment (4a: Fovea-sparing, 4b: Non-sparing)
168
How is stage 5 defined in ROP?
Complete retinal detachment (funnel-shaped), retrolental fibroplasia
169
What is seen in Plus disease in ROP?
Posterior retinal vessel abnormal dilatation and tortuosity, iris rigidity
170
How is Pre-Plus disease defined in ROP?
Insufficiently severe Plus disease in 2+ quadrants
171
What causes Plus disease in ROP?
Periphery shunting through ridge tissue
172
What vascular sign of aggressive ROP normally disappears after birth?
Tunica vasculosa lentis
| Capillary network over posterior/lateral lens
173
What stage of ROP warrants immediate treatment?
Stage 3 with 2+ quadrants of Plus disease
174
What is seen in AP-ROP? What can it cause?
No demarcation line, zone 1, can lead to RD
175
What are the stages of prematurity in ROP screening? When must patients first be seen for each?
<32 weeks PMA: Term
27-32 weeks: 30-31 weeks PMA
<27 weeks (extreme): 32-35 weeks PMA
176
How is ROP treated?
Mild ROP: Resolves itself
Laser, anti-VEGF
RD surgery
177
How is ROP treated?
Mild ROP: Resolves itself
| Laser, anti-VEGF
178
What are the post laser sequelae for ROP?
Myopia, strabismus, amblyopia, glaucoma, retinal vessel dragging
179
What forms of RD surgery are done for ROP?
Vitrectomy (oil/gas or scleral buckle)
| Endoscopic VR surgery
180
When can imaging be useful in paediatric ophthalmology?
Documenting, diagnosis, telemedicine, education, research, teaching, screening, EUA alternative
181
What are the imaging challenges in paediatric ophthalmology?
Cooperation, attention, fatigue, positioning, photophobia, disabilities
182
How are imaging challenges in paediatric ophthalmology overcome?
Quick, mobile devices, adjustable light intensity, good communication, parental support, prioritise, positioning. EUA
183
When is external photography useful in paediatric ophthalmology?
Strabismus surgery outcome, disease progression, treatment analysis
184
When is external photography useful in paediatric ophthalmology?
Recording surgical outcomes (strabismus)
| Disease progression, treatment analysis
185
What are the benefits of external photography in paediatric ophthalmology?
Readily available, easy, fast, cheap, non-contact, no cooperation needed, familiar, low light intensity
186
What are the drawbacks of external photography in paediatric ophthalmology?
Flash causes shadow rings, limited, low magnification
187
When is slit lamp photography used in paediatric ophthalmology?
Magnified, detailed or slit beam anterior segment images (cystinosis, MPS)
188
What are the benefits of slit lamp photography in paediatric ophthalmology?
Video: Movement tolerated, recorded (Siedel's test)
Flash: Brief, moderate light intensity, sharp, less movement artefact
189
What are the drawbacks of slit lamp photography in paediatric ophthalmology?
Video: Intense light
Flash: Expensive, training, good cooperation needed
190
How is corneal tomography done in paediatric ophthalmology? Which principle is used?
Placido-based systems (Pentacam)
| Scheimpflug
191
What does Pentacam measure?
Corneal thickness (pachymetry)
192
When is Pentacam useful in paediatric ophthalmology?
Keratoconus, corneal/lens thickness, anterior chamber depth, some refractive surgeries
193
What are the benefits and drawbacks of Pentacam use?
Pros: Fast, easy, non-contact, low light intensity
Cons: Cooperation needed
194
What system is used for handheld digital fundus cameras?
RetCam
195
How are digital fundus images captured?
White light ring on fundus, reflected light captured on charge coupled device
196
What types of handheld digital fundus imaging are there?
```
Direct contact (video)
Anterior segment (goniophotography)
```
197
What ocular structure does goniophotography highlight?
Iridocorneal angle
198
When is digital fundus imaging used?
Documenting examination findings
| Telemedicine
199
What are the benefits and drawbacks of digital fundus imaging?
Pros: Both A/P segments seen, awake
Cons: Expensive, difficult to use, slow, cooperation needed
200
How are anterior segment OCT images formed? What domains may be used?
Interferometry
| Time domain, Fourier/spectral domain (swept source)
201
When is anterior segment OCT used in paediatric ophthalmology?
Corneal issues, placing IOL's/glaucoma draining tubes, corneal thickness, anterior chamber angle check
202
What are the benefits and drawbacks of anterior segment OCT in paediatric ophthalmology?
Pros: Non-contact, fast, easy, light tolerated, flexible, eye tracking functions
Cons: Cooperation needed, expensive
203
What are the forms of posterior segment imaging?
OCT, OPTOS/SLO, FFA, B-scan, RetCam
204
What does OPTOS allow in terms of imaging?
Ultra-wide fundus imaging: 200 degrees field of view
205
When is OPTOS used in paediatric ophthalmology?
Complex posterior uveitis, peripheral retinal disease
206
What are the benefits and drawbacks of OPTOS in paediatric ophthalmology?
Pros: Non-contact, fast, undilated, high resolution
Cons: Cooperation needed, noise, not true colour
207
How is fundus fluorescein angiography carried out?
Rapid fundus images following IV sodium fluorescein using fundus camera or SLO
208
When is fundus fluorescien angiography useful in paediatric ophthalmology?
Assessing retina/choroid vasculature (Coat's disease, CNV, uveitis)
209
What risks are associated with fundus fluorescein angiography?
Anaphylaxis, injection risks
210
Where is light focused in emmetropic eyes?
Directly onto the retina
211
Where is light focused in hypermetropic eyes?
Behind the retina
212
Where is light focused in myopic eyes?
In front of the retina
213
Which spherical lenses correct myopia?
Concave (minus) lenses
214
Which spherical lenses correct hypermetropia?
Convex (plus) lenses
215
What is astigmatism?
Deviation from spherical curvature, rays do not meet at common focus, distorted vision, cylindrical lenses needed
216
What occurs in accommodation?
Crystalline lens flat when distant focus, thickens for near objects
217
What is presbyopia?
Accommodation loss with age
218
What is aphakia?
Absence of the eye's crystalline lens
219
What % of neonates and 6-8y/o's have spherical refraction of 2 and 1 respectively?
22%
| 35%
220
What refraction type do most neonates have?
Hypermetropic (<3.00), decreases with age, 17mm
221
How is a child's prescription checked objectively?
Trial frame worn, room lights dimmed, distance fixation target (Thompson), retinoscopy determines lens power
222
How is a child's prescription checked subjectively? Which minimum age does it work best with?
Offer +/- lenses (push the +): Cross cyl to check astigmatic axis and power, 7+ y/o
223
When is cycloplegic refraction needed?
For those whom it is difficult to obtain accurate subjective measurements from
224
What agent is used for cycloplegic refraction?
Cyclopentolate 1%
225
What are the benefits and drawbacks of cycloplegic refraction?
Pros: No accommodation,
easy cooperation
Cons: Invasive, acidic irritation, blurred vision for hours
226
What are the benefits of refractive correction?
Preventing amblyopia/strabismus
| Providing clear, binocular vision
227
When is refractive correction prescribed for children of any age?
Extreme refractive errors
Strabismus/amblyopia
Anisometropia
228
How is insignificant refractive error managed above and below 2 years old?
<2y/o: Monitor only
| >2y/o: Monitor if refractive error not decreasing
229
How is significant refractive error defined in those >2 years old?
>+3.00D hypermetropia (>+2.00 if no binocular issues), +2.50D astigmatism
230
What can cause paediatric aphakia?
Congenital cataract, trauma, lens subluxation (Marfan’s, ectopia lentis)
231
What is the corneal radius and axial length of eyes on average at birth?
Corneal radius: 6.9mm
| Axial length: 17mm (21mm at 6/12 months)
232
What prescription are aphakic paediatric spectacles?
+25DS to +15DS in 1st year of life
233
What are the benefits and drawbacks of aphakic paediatric spectacles?
Pros: No infection risk, well-tolerated
Cons: Challenging if unilateral, expensive, easily break, poor cosmesis
234
What are the benefits and drawbacks of paediatric contact lenses?
Pros: Light, easy upon insertion better unilaterally, good cosmesis
Cons: Infection/abrasion risk, difficulty inserting, easily taken out
235
Which paediatric aphakic lenses are currently in use?
Silicone hydrogel lenses (Ultravision)
236
What are benefits and drawbacks of silicone hydrogel paediatric contact lenses?
Pros: Flexible, stable, less corneal hypoxia risk
Cons: Red/sticky eyes, handling, lens loss, de-centering, compliance
237
What is the aftercare involved with paediatric contact lenses?
Addressing issues, compliance, lens fit/centration, over-refraction, gross corneal examination (Burton or slit-lamp)
238
What refractive correction is prescribed for aphakic infants?
Until mobile/toddling: +2.00D overcorrection (CL’s/glasses)
Toddlers: +1.00D overcorrection
Preschool: Distance correction with CL’s/bifocals, add +3.00D for near work
239
How many individuals worldwide suffer from childhood blindness? How many in the UK aged under 18?
1.4 million globally
| UK visual loss <18y/o: 28000
240
What is included with Low Vision Assessment Clinic appointment letters?
Parent/guardian questionnaire covering education, school, support, QTVI hours, font size, etc.
241
What is involved in Low Vision Assessment histories?
Who supports child, health concerns, mobility/orientation, TV/reading/playing issues, interaction with others
242
What low vision aids are available in schools?
Larger font, sit at front of class, sloping boards, contrast/lighting, magnifiers (Fresnel)
243
What does QTVI stand for?
Qualified Teacher of the Visualy Impaired
244
How is distance visual acuity assessed?
Monocular (worse eye first) then binocularly start at easier distance
Appropriate test: LogMAR/Snellen/Lea symbols
245
How is near visual acuity assessed?
Binocular: Symbols/words/sentences with M or N notation
246
Why is assessing only visual acuity insufficient? What visual factors should also be considered?
Determining effect on daily tasks difficult
| Contrast sensitivity, colour vision, visual fields
247
How are contrast sensitivity and colour vision tested in children?
Contrast: Hiding Heidi
Colour: Panel 16 Quantitative Colour Vision test (adult: D15)
248
Which conditions can reduce contrast sensitivity?
Visual pathway issues, cataract, glaucoma, optic atrophy, macular/retinal dystrophies, amblyopia
249
Which techniques are used for visual field tests? Which strategies are used for central and peripheral scotomas
Goldmann techniques
Central: Optimal reading
Peripheral: Enhancement
250
Which near vision/reading and distance magnifiers are available?
Near: Bar/Brightfield, handheld, stand, illuminated
Distance: Binoculars
251
What does P-EVES stand for in terms of low vision aids?
Portable Electronic Vision Enhancement
252
How is strabismus defined? What impairment may it highlight?
Misalignment of the eyes, visual axes are not simultaneously directed at the object in one or all directions
Loss of binocular vision
253
What are the Worth's classification grades of binocular vision?
I: Simultaneous macular perception, II: Fusion, III: Stereopsis
254
What are the horizontal forms of ocular deviation?
Esotropia (inward), exotropia (outward)
255
What are the vertical forms of ocular deviation?
Hypertropia (upward), hypotropia (downward)
256
What affects the extent of misalignment in comitant squints?
Accommodation
257
What factors determine squint classification?
Deviation direction, duration, comitancy, refraction, accommodative effort, fixation distance, strabismus surgery, visual status
258
What ocular consequences can squints cause?
Diplopia, suppression, amblyopia, psycho-social issues (e.g. bullying)
259
What occurs in suppression due to squint?
Brain ignores image from deviating eye, common in <7y/o's
260
How is amblyopia defined?
Reduced visual acuity in the absence of physical eye abnormalities
261
How is amblyopia treated?
Patching good eye, atropine, near objects
262
What is the difference between a 'tropia' and a 'phoria'?
A 'tropia' occurs when both eyes are uncovered, a 'phoria' occurs for covered eye(s)
263
What is nystagmus?
Involuntary movement of the eyes
264
What types of nystagmus are there?
Manifest (always)
Latent (covered)
Manifest-latent
265
How should manifest-latent nystagmus be treated?
Atropine
266
When does infantile esotropia occur? At which angle and refraction?
<6 months old
Moderate angle
Normal refraction
267
What is cross fixation?
Fixation between eyes switches when object moves between L and R visual fields
268
How are cover tests carried out?
Cover fixing eye, other eye visual axis straightens
269
If there is a right hypertropia what tropia is usually seen in the left eye?
Hypotropia?
270
What is Dissociated Vertical Deviation? Which tropia is most common in this?
When only one eye moves during a cover test in vertical squints
95% hypertropia
271
What are the two types of accommodative esotropia?
Refractive
| Non-refractive
272
When do children develop the ability to accommodate?
>1 years old
273
Which other tropia is refractive accommodative esotropia linked with? How is it treated?
Hypermetropia
| Spectacles (bifocals if convergence excess)
274
What eye movement issue is seen in non-refractive accommodative esotropia?
Convergence excess (high AC/A ratio)
275
When does exotropia usually present? What refraction and angle is seen?
Adulthood
Normal refraction
Large angle
276
What affects intermittent exotropia?
Distance, concentration, bright light (loss of fusion)
277
How are macular stress tests carried out?
Shine bright light in patient's eye then test visual acuity
278
When does sensory exotropia occur?
Drops in visual acuity (e.g. cataract)
279
In paralytic strabismus, what ocular issues do CN III, IV and VI palsies cause?
III: Ptosis, mydriasis, exotropia, hypotropia
IV: Hypertropia
VI: Esotropia
280
What is another term for incomitant esotropia?
Brown's syndrome
281
What can cause incomitant esotropia?
CN VI palsy, Duanes syndrome, medial orbital floor fracture, myasthenia gravis, myositis, thyroid myopathy
282
What is Duane's syndrome
Abnormal extraocular muscle co-contraction
283
How is incomitant esotropia managed?
Examination, refraction, ocular deviation stability, surgery (medial recti), 2-3 botox injections
284
Which are the key laws of eye movement?
Sherrington's (reciprocal innervation)
| Hering's (equal innervation)
285
What tests are carried out during strabismus surgery?
Forced duction/resistance test under local or general anaesthesia for passive eye movement
286
What is the manoeuvre which tests CN VI function? How is it carried out?
Doll's head manoeuvre
| Move head to one side, eye should remain fixed
287
How is hypermetropic esotropia treated?
Spectacles, variable patching
288
What are the terms used for pathological and non-pathological DNA variants?
Pathological: Mutation
| Non-pathological: Polymorphism
289
How are DNA variants scaled?
Benign, uncertain, pathogenic
290
What types of DNA changes are there?
Substitutions (SNP's)
Insertions/deletions
Splice site changes
Structural/chromosomal
291
Which inheritance types involve a single gene or multiple components?
Mendelian
| Multifactorial
292
What are the 3 main types of Mendelian inheritance?
Autosomal
X-linked
Mitochondrial
293
Which tables are used to calculate inheritance patterns?
Punnett squares
294
What are the types of autosomal inheritance? How many alleles can lead to disease and what are the inheritance chances for each?
Dominant: 1 allele needed, 50% chance
Recessive: Both alleles needed, 25% chance
295
In X-linked inheritance which sex displays the disease phenotype?
Males
296
X-linked recessive case: Affected dad, unaffected mum. What are the son/daughter phenotypes?
Unaffected sons
| Carrier daughters
297
X-linked recessive case: Unaffected dad, carrier mum. What are the son/daughter phenotypes?
50% affected sons
| 50% carrier daughters
298
Mitchondrial inheritance is from which parent? Which sex does it affect?
Always from mother
| Both sexes affected
299
What is penetrance?
The extent to which those with the disease genotype express the phenotype
300
What is consanguinity? What risk to offspring is there? What inheritance pattern is it linked with?
Parents being related as second cousins or closer, genetic mutation risk, autosomal recessive
301
Which inheritance pattern is suggested by 1 parent affected, 50% offspring affected?
Autosomal dominant
302
Which inheritance pattern is suggested by unaffected parents, rarely affected offspring?
Autosomal recessive
303
Which inheritance pattern is suggested by severely affected males, no male-to-male transmission?
X-linked recessive
304
Which inheritance pattern is suggested by no male-to-male transmission, affected mothers producing affected/carrier offspring?
Mitochondrial
305
What are the typical inheritance patterns of simplex or sporadic cases?
Usually autosomal recessive, dominant if less family history or de novo, males: X-linked?
306
What % of childhood visual impairment is accounted for by inherited eye disease?
33%
307
What types of inherited eye disease are seen?
Structural globe (6%), anterior segment (11%), retinal (14%), albinism (4%)
308
Which 3 inherited structural globe abnormalities are seen in the eye?
Anophthalmia
Microphthalmia
Coloboma
309
What horizontal corneal diameter indicates microphthalmia? How is it investigated?
<1cm
| Ultrasound
310
What is a coloboma? What causes it?
Absence of an ocular tissue in the eye
| Optic fissure non-closure in week 6 of development
311
What is investigated in a MAC (micro/anophthalmia/coloboma) assessment?
Corneal diameter
USS, CT/MRI
Cryptophthalmia (fused eyelids)
312
What are 6 anterior segment developmental abnormalities?
```
Posterior embryotoxon
Axenfield-Rieger syndrome
Peter’s anomaly
Aniridia
Paediatric glaucoma
Congenital cataracts
```
313
What abnormalities often occur alongside iris malformation?
Dental, navel abnormalities
314
What causes posterior embryotoxon?
Descemet's membrane ends anteriorly (10-15%)
315
What makes up Axenfeld-Rieger syndrome?
Axenfeld anomaly: Anterior iris strands
| Rieger anomaly: Iris hypoplasia
316
What is Peter's anomaly?
Central corneal opacity, irido/lenticulocorneal adhesions
317
What are the features of aniridia?
Iris/fovea hypoplasia, nystagmus, peripheral corneal opacity
318
Which tumour is linked to aniridia? How is it investigated?
Wilm's tumour
| Abdominal ultrasound
319
What causes paediatric glaucoma?
Primary: Trabeculodysgenesis
Secondary: Post-cataract surgery
320
What features are seen in paediatric glaucoma?
Lacrimation, blepharospasm, photophobia
| Corneal opacities
321
What % of congenital cataracts are bilateral and isolated?
66% bilateral, 55% isolated
322
What commonly causes unilateral congenital cataracts?
Hyperplastic primary vitreous
323
When are congenital cataracts referred?
Infection, biochemical or metabolic disorders
324
What cause of congenital cataracts warrants genetic testing?
Cancer (70% diagnosis if bilateral)
325
What congenital cataracts occur in the lens of the eye?
Nuclear (yellow/brown), lamellar/zonular
| external to lens nucleus
326
What type of cataract occurs in the posterior lens? What can it cause? How is it treated?
Polar cataract
Amblyopia risk
Vitreo-retinal surgery
327
What causes oil-droplet cataracts?
Galactosaemia
328
How do cortical cataracts appear?
White wedge opacities extending spoke-like from lens periphery to centre
329
Which structures do inherited retinal diseases often affect? What are the two main types?
Rods, cones, RPE
Stationary (dysfunction)
Progressive (dystrophy)
330
How do stationary and progressive inherited retinal disorders differ in onset, fundus and extraocular appearance?
Stationary: Congenital, normal fundus, rarely extraocular
Progressive: Variable age, abnormal fundus, some extraocular
331
What is affected in stationary cone>rod disorders? What condition does it cause?
Colour vision
| Achromatopsia
332
What are the main progressive inherited retinal disorders?
Stargardt disease (AR)
Retinitis pigmentosa
Leber's congenital amaurosis, SECORD, Best disease, gyrate atrophy
333
How does Stargardt disease present?
Bullseye appearance around macula, hyper-AF flecks, VA drop
334
How does Stargardt disease present?
Bullseye appearance around macula, hyperAF flecks, decreased VA
335
What is retinitis pigmentosa? How does it present?
Rod-cone dystrophy, peripheral RPE dysfunction
| Early life nyctalopia
336
In which inherited retinal disorders are both rods and cones affected? How do they present?
LCA, SECORD, Best disease, gyrate atrophy
| Severe VA drop, nystagmus, initial normal fundus
337
What is albinism?
Non-degenerative disorder of melanin production/transport
338
How does albinism present in the eye?
Nystagmus, VA drop, iris/retina hypopigmentation, foveal hypoplasia
339
In which day of gestation does eye development begin? By which week are the main structures formed?
Day 22
| Week 5
340
By which week of gestation are precursors of retinal cells present and final ocular structures formed?
Week 8
341
Which ocular structures are formed in month 4 of gestation?
Retinal vessels
Iris, ciliary body
Canal of Schlemm
342
From months 5-8 of gestation what ocular developments occur?
5: Eyelids separate
6: Nasolacrimal duct forms
7: Optic nerve myelination
8: Anterior chamber forms
343
Which years are described as the critical period of vision maturation?
5-7 years old
344
Which years are described as the critical period of vision maturation?
5-7 years
345
By which age can pupils react to light?
From birth
346
By what age can pupils react to light?
From birth
347
By 6 months old how developed is light sensitivity compared to adult levels? By which age is it fully developed?
2/3rd of adult levels
| 12-14 years old
348
By which age ranges is central vision good enough to (a.) react to faces, (b.) distinguish objects, (c.) shapes, (d.) drawn images?
(a. ) 4-6 months old
(b. ) 2-3 months old
(c. ) 7-10 months old
(d. ) 2-3 years old
349
When does colour perception begin and then fully develop? Which colours are recognised first?
2-6 months old
4-5 years old
Red, blue then green
350
How much narrower is field of view at pre-school age than adulthood? By which age is at adult levels?
10%
| School age
351
How much larger are blind spots at school age than adulthood from 1m away?
2-3cm larger
352
Which is the last visual function to develop? What does it allow perception of?
Binocular vision
| Allows 3D perception
353
At which ages are (a.) 2D, (b.) depth through touch, (c.) direction, (d.) distance, (e.) stereopsis perceived?
(a. ) 2 months old
(b. ) 3 months old
(c. ) 4 months old (grasp reflex)
(d. ) 6 months old (crawling)
(e. ) 2-7 years old
354
What are the 3 requirements for stereopsis (ability to see in 3D)?
Simultaneous perception
Sensory fusion
Motor fusion
355
What stereopsis tests are there?
Wirt (Titmus)
Lang Stereo Test
Frisby
TNO
356
How might a child's 3D vision not develop?
Squint before 6 months old, eyes cannot straight
357
When is the critical age range to test children's vision?
Birth-7.5 years old
358
How would you test visual acuity in babies/infants?
Preferential looking
Fixing and following (<1 month)
Objection to occlusion (>3 months)
Picking up fine objects (>6 months)
359
How is fixing and following tested?
Bright light/toy, 33-50cm away, neither or 1 eye occluded
360
What are the main preferential looking tests used to test visual acuity in babies/infants?
Keeler
Teller (1974)
Cardiff (Woodhouse, 1994)
361
What is observed in fixing and following?
Position, persistence, steadiness, speed
362
Which preferential looking tests involves black and white stripes on (a.) circular, (b.) square grating patterns?
(a. ) Keeler acuity cards
| (b. ) Teller acuity cards
363
Which preferential looking test involves vanishing optotypes on grey cards tested from 50cm or 1m away?
Cardiff acuity cards
364
What are the benefits of preferential looking tests and Cardiff cards?
Detects gross amblyopia
| Monitor treatment progress for e.g. cataracts, strabismus
365
What are the limitations of preferential looking tests and Cardiff cards?
Overestimates vision, interest/fatigue/looking speed vary, guessing (repeating helps), observer bias
366
How do you test fine stimulus response in babies?
Hundreds and thousands test
367
What is the Kay picture test (1983)?
Visual acuity test for those aged 2-3, 3-4 objects per page at each acuity level from 6m away
368
How would you test visual acuity in children >2 years old?
Kay picture test
| Cardiff acuity cards
369
How would you test visual acuity in children >3/4 years old?
Kay picture test
Sheridan-Gardiner test
LogMAR
Keeler logMAR
370
Which visual acuity test is aimed at amblyopic children?
Keeler logMAR
371
Which visual acuity test for those aged 2-5 involves matching single optotypes from 3m or 6m away?
HOVT or Sheridan-Gardiner test
372
Which variant of the Sheridan-Gardiner test uses multiple optotypes?
Sonksen-Silver logMAR test
373
How would you test visual acuity in children >5 years old?
Snellen chart
375
What are the test age ranges for (a.) fixing and following, (b.) preferential looking, (c.) Cardiff cards?
(a. ) 2-3 months old
(b. ) 2 months-1 year old
(c. ) 1-2.5 years old
376
What are the test age ranges for (a.) Crowded Kays, (b.) Keeler LogMAR (crowded), (c.) LogMAR/Snellen’s?
(a. ) 2.5-3.5/4 years old
(b. ) 4-5/6 years old
(c. ) >5-6 years old
377
What shoud be considered in suspected non-accidental head injuries?
Not necessarily abuse?
Medicolegal issues
Child protection
378
What are the most common abuse-related eye injuries?
Subluxation of the lens
| Orbital fractures
379
How might abuse-related eye injuries present?
Bruising (e.g. near ears), behaviour change (encephalopathy?), emesis, squint
380
How might brainstem injury present?
Sun-setting: Eyelids retract, down-facing pupils, superior sclera visible
381
What types of retinal haemorrhages are there?
Pre-retinal (under fovea), flame (retinal nerve fibre layer), dot and blot (deep retinal)
382
Non-accidental head injury and birth are the only causes of what?
Multiple retinal haemorrhages without disease or severe trauma
383
What can cause retinal haemorrhages?
High ICP
Post-partum hypoxia
Newborn haemolytic disease (vitamin K deficiency)
Coagulation disorders
384
Which studies suggested retinal haemorrhages are insufficient to diagnose non-accidental head injuries?
Geddes III
| CONI study
385
What ocular features suggest brain injury or optic nerve damage?
Unreactive pupils, RAPD
386
How are paediatric head injuries assessed in ophthalmology?
Pre: MRI (haemorrhages or fractures)
| RetCam, direct ophthalmoscopy after eye drops instilled
387
How does Shaken Baby Syndrome present?
Hypoxia
Low venous pressure
Vitreous shearing, traction, retinal haemorrhages (flame)
388
Which courts address suspected non-accidental head injury cases?
City County Court
389
What occurs in suspected non-accidental head injury court proceedings? How long do these last?
Single joint expert witness (or treating clinician) assesses trier of fact
26-week track
