Paediatric Ocular Pathology- Retina - Alistair

Question 1

What is the most common cause of visual blindness in children?

Answer 1

Cerebral visual impairment

Question 2

What is the most important electrophysiology test?

Answer 2

ERG

Question 3

What is the minimum age an EOG can be performed on and why?

Answer 3

7 years old because requires a lot of patient co operation

Question 4

What are three ophthalmic tests that can be done to assess the retina?

Answer 4

• OCT
• Electrophysiology
• Ultrasound

Question 5

What test should be the last thing you do at the end of your paeds examination?

Answer 5

Ophthalmoscopy

Question 6

How do you assess the retina and optic nerve in detail? And why do we do this?

Answer 6

Using OCT and electrophysiology (ERG, EOG, VEP) because retinal signs and optic nerve disorders can be very subtle but also co-occur

Question 7

What is used to conduct an ERG?

Answer 7

Skin or conjunctival electrode

Question 8

What are the two frequencies an ERG is done at ?

Answer 8

2 & 30 Hz

Question 9

What is presented with a VEP?

Answer 9

A flash or a pattern which tells you the intactness of the visual pathway - limited

Question 10

What eye condition is normally associated with an absence of septum pellucidum (the middle of the brain that splits the two hemispheres)?

Answer 10

Optic nerve hypoplasia

Question 11

Why are two frequencies used in an ERG?

Answer 11

2 Hz are for rods assessment
30 Hz are cone assessment

Question 12

What do the patients of the two right ERGS have wrong with them and how do you know that ?

Answer 12

They have no cones, at 30 Hz there was no response

Question 13

What classifies whether a baby is premature and how many people get affected by this?

Answer 13

Born before 37 weeks, affects 5-8%

Question 14

Does vision start to develop from the preterm date (that is if the baby is premature) or the due date?

Answer 14

Due date

Question 15

What are some signs of a preterm baby’s retina?

Answer 15

Not fully vascularised and thin vessels

Question 16

What week of gestation do blood vessels come out the optic disc?

Answer 16

12-13 weeks

Question 17

At what point of gestation is the retina fully vascularised?

Answer 17

Full term time

Question 18

With pre term babies, as their retinas are not fully vascularised, where do their retinas get blood supply from?

Answer 18

Choroid

Question 19

What are the two phases of retinopathy of prematurity (ROP)?

Answer 19

Acute phase and sequelae

Question 20

How long does the acute phase last?

Answer 20

Only occurs until a few weeks until after birth
*we dont need to know details about what this is

Question 21

What can severe ROP cause?

Answer 21

Blindness but can be prevented with timely screening

Question 22

Can mild ROP resolve without major sequelae?

Answer 22

Yes :) just like anisha and hardeep

Question 23

Where on the Retina does ROP develop?

Answer 23

At the junction between vascularised and non-vascularised retina

Question 24

How many stages are there of ROP?

Answer 24

5

Question 25

Which stages are classified into mild ROP?

Answer 25

Stage 1 and 2

Question 26

What does stage 1 look like?

Answer 26

White line within the retina is formed

Question 27

What is stage 2?

Answer 27

White line becomes a ridge

Question 28

What is stage 3?

Answer 28

Ridge with extra retinal fibrovascular proliferation (similar to DM - has CWS and neovasc of bv coming out of the retina into the vitreous)

Question 29

What stage is this?

Answer 29

3

Question 30

What changes in regard to the BVs from mild ROP to stage 3 ROP?

Answer 30

Bv become thick and tortuous

Question 31

If ROP is left untreated what might the child get?

Answer 31

An RD

Question 32

What is stage 4 ROP?

Answer 32

Partial RD

Question 33

What is stage 5?

Answer 33

Total RD

Question 34

What is stage 5?

Answer 34

Total RD

Question 35

Why is the risk for blindness greater in India than here?

Answer 35

They have a greater prevalence of pre term babies

Question 36

Why is the screening programme for preterm babies tight?

Answer 36

There is a narrow window for treating babies with ROP (you have to catch the baby at exactly the right time)

Question 37

What is treatment for severe acute ROP?

Answer 37

Cryotherapy, laser and AntiVEGF

Question 38

What is cryotherapy?

Answer 38

Freezing the retina where the tear has occurred

Question 39

What are patients who were born premature likely to have who have had NO or mild ROP?

Answer 39

Slightly reduced va and contrast

Question 40

What acuity is a patient who has severe ROP likely to have?

Answer 40

Ranges from normal to blind

Question 41

What acuity is a patient who has severe ROP likely to have?

Answer 41

Ranges from normal to blind

Question 41

What acuity is a patient who has severe ROP likely to have?

Answer 41

Ranges from normal to blind

Question 42

What % of preterm babies will have strabismus?

Answer 42

20% (it’s only 3% in full term babies!)

Question 43

If a baby had severe ROP what is the likelihood of having strabismus? (%)

Answer 43

>60%

Question 44

What does prematurity do to the eye?

Answer 44

Cornea develops abnormally so they have a mild degree of keratoconous and myopia

Question 45

What is a px who has had severe ROP likely to have?

Answer 45

Very high myopia (16-20D)

Question 46

What is this ? + at what stages does it occur?

Answer 46

Dragged disc - this is temporal retina is pulling or dragging of the disc and vessels Stage 3 or after

Question 47

What are causes of a dragged disc?

Answer 47

- ROP - Exudative vitreoretinopathies - Norrie’s disease - Congenital fold *all of which are rare diseases

Question 48

What is this? And what is that white stuff?

Answer 48

End stage ROP, px likely to be blind, retrofibroplasia is the white stuff

Question 49

In baby what is the ONLY cause if a sub-conjunctival haemorrhage?

Answer 49

Trauma

Question 50

If you see a retinal haemorrhage like this, what are you thinking could have happened to the baby?

Answer 50

Shaken baby syndrome

Question 51

What is this disease?

Answer 51

Coat’s disease

Question 52

What are some features of Coat’s disease?

Answer 52

- M>F - Unilateral - Retinal telangiectasia (massive exudates and RD) - Systemic association In the lecture just says know these vascular diseases exists, so down worry if you do not know this

Question 53

What is this disease and some features of the disease?

Answer 53

Von Hippel Lindau syndrome Bilateral, both sexes, tumours of the brain and retina

Question 54

What is this disease ?

Answer 54

Sickle cell retinopathy

Question 55

What people is sickle cell more prevalent in?

Answer 55

African or Mediterranean origin

Question 56

What are some signs of the Retina with sickle cell retinopathy?

Answer 56

Peripheral occlusion of BV, neovasc, could regress or cause RD

Question 57

What are five names of infections of the Retina?

Answer 57

- Toxoplasmosis - Cytomegalovirus - TB - Rubella - Toxocara

Question 58

What does this px have?

Answer 58

Toxoplasmosis

Question 59

What type of infection is toxoplasmosis?

Answer 59

Protozoa

Question 60

How can you get toxoplasmosis?

Answer 60

Eating raw meat or eating food that has been contaminated by cat faeces

Question 61

What else might someone with toxoplasmosis have?

Answer 61

Lesions to the brain, cataract, uveitis

Question 62

What is toxocara?

Answer 62

Worm infection from a cat or dog

Question 63

What is similar to toxoplasmosis?

Answer 63

Toxocara however, toxocara only occurs in one eye

Question 64

What is this ?

Answer 64

Toxocara

Question 65

Is rubella rare in this country?

Answer 65

Yes because we have all be vaccinated

Question 66

What problems can someone who has rubella have?

Answer 66

Heart defects, deafness, mental retardation, small eyes, pigmented retinopathy

Question 67

What does this px have and describe the fundus?

Answer 67

Speckled pigment giving a mottled appearance (rubella- salt and pepper appearance)

Question 68

What is someone with cmv likely to also have?

Answer 68

HIV

Question 69

What does CMV stand for ?

Answer 69

Cytomegalovirus

Question 70

What does this px have?

Answer 70

CMV

Question 71

What ophthalmic issues is a px with CMV likely to have?

Answer 71

Microphthalmos, cataract, keratitis, optic atrophy

Question 72

What are some stationary retinal dystrophies?

Answer 72

- Congenital night-blindness - Achromatopsia

Question 73

What does someone with congenital night blindness have a disorder of?

Answer 73

Rods (associated with myopia)

Question 74

What are five signs of someone with achromatopsia will have?

Answer 74

- Reduced vision - Photosensitivity - Nystagmus - High hyperopia - Absent flicker on an ERG - Normal looking retina

Question 75

What is a disease of a child with a progressive rod-cone retinal dystrophy? (There are three)

Answer 75

- Leber’s congenital amaurosis - Retinitis pigmentosa - Choroideremia

Question 76

What signs would a child with Leber’s congenital amaurosis have?

Answer 76

Reduced vas Nystagmus High hyperopia Normal Retina ( can later become pigmentary) Flat ERG

Question 77

If a child comes in with high hyperopia, nystagmus, reduced vas and sensitive to light, what two conditions should you be thinking of ?

Answer 77

Achromtopsia and Leber’s congenital amaurosis

Question 78

What are four inherited macular dystrophies?

Answer 78

- Stargardt’s disease - X-linked retinoschisis -Best’s disease - Pattern dystrophy

Question 79

As the signs are very subtle, what are the patients often labelled as?

Answer 79

Hysterical

Question 80

What does leucocoria mean?

Answer 80

White pupil

Question 81

Name some diseases with leucocoria

Answer 81

- Retinoblastoma - Cataract - ROP - Developmental anomalies such as a Coloboma - Coat’s disease

Question 82

What is the most common malignant ocular tumour of childhood?

Answer 82

Retinoblastoma

Question 83

Is a retinoblastoma likely to be bilateral or unilateral?

Answer 83

Unilateral

Question 84

What are three presentations of someone with retinoblastoma?

Answer 84

Leucocoria, strab, glaucoma

Question 85

What is treatment of retinoblastoma (it is a tumour remember)?

Answer 85

Chemotherapy

Question 86

What does ROP mean?

Answer 86

Retinopathy Of Prematurity

Question 87

Which stages are considered severe ROP?

Answer 87

Stage 3-5; they require treatment