Peads - Anterior Segment - Alistair Flashcards

1
Q

what is a coloboma?

A

failure of fetal fissure to close (at around 4-5 weeks)

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2
Q

What anterior segment developmental anomalies are there?

A
  1. Coloboma
  2. Albinism
  3. Aniridia
  4. Glaucoma
  5. Anterior seg dysgenesis

Many of these are associated with glaucoma.

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3
Q

What are examples of anterior dysgenesis?

A
  1. Posterior embryotoxon
  2. Axenfeld-rieger syndrome
  3. Sclerocornea
  4. Peters anomaly : corneal opacity and sticks to the iris
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4
Q

What defect causes Rieger’s anomaly?

A

Defect of the neural crest

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5
Q

a child with Reiger’s is at a high risk of what disease?

A

glaucoma

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6
Q

What are the signs of Rieger’s anomaly?

A
  1. Facial formation problems
  2. Iris abnormalities - strand of iris stuck to cornea + posterior embryotoxon, iris stroma hypoplasia (multiple pupils)
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7
Q

What are signs of peters anomaly?

A

Strands of the iris which will stick to corneal opacity (cataract)

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8
Q

How do we measure IOP in children?

A

iCare.

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9
Q

What is a albinism neuroectodermal defect?

A

disorder of the neuroectoderm
The two layers behind the iris.

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10
Q

what is the colour of the iris in albinism?

A

any colour- translucent

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11
Q

Where does the colour of the iris come from?

A

iris stroma
This is why in Horner’s syndrome you get heterochromia as the pigment cells have been effected.

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12
Q

What other signs do albinism px have?

A
  1. Fovea hypoplasia
  2. Strabismus
  3. complete crossing of fibres at the chiasm (decussation)
  4. Nystagmus
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13
Q

What is aniridia and how does it come about?

A

complete or partial loss of iris
Non genetic : Signs of kidney tumour !!
Genetic : dominant gene

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14
Q

What are px with aniridia risk of? (3)

A
  1. Glaucoma (50%)
  2. Ectopia lentis (lens is displaced downwards)
  3. Keratopathy : superficial neovas (progressive)
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15
Q

What are the consequences of anterior segment development anomalies?

A
  1. Vision
  2. Glaucoma
  3. Cataract
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16
Q

What are the non specific consequences of reduced vision?

A

strab and nystagmus

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17
Q

Why may the cornea or the eyeball be a small size?

A
  1. Due to maldevelopment
  2. Shrinkage following an infection or trauma
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18
Q

What might the cornea or the eyeball be big size?

A
  1. Maldevelopment
  2. Glaucoma (primary or secondary type)
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19
Q

what is the normal cornea diameter??

A

11.5 (+ or - 0.5mm)

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20
Q

What cause light sensitivity?

A
  • Pigmentation : being blonde / albinism
  • Anterior segment disorders
  • Retinal conditions (achromatopsia and retinitis pigmentosa)
  • CNS : meningitis and migraine
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21
Q

why do anterior segment disorders cause photosensitivity in a child?

A

light scatter

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22
Q

What is photosensitivity a first sign for?

A

Meningitis

23
Q

What is buphthalmos?

A

This is when the eyeball is bigger than normal, can be caused by glaucoma

24
Q

What are the two classifications of glaucoma?

A

Primary
Secondary

25
Q

What causes primary glaucoma?

A
  1. Infantile / congenital
  2. Juvenile
26
Q

What causes secondary glaucoma?

A
  1. Anterior segment dysgenesis
  2. Ocular disease and its treatment
  3. Inflammatory causes such as uveitis
  4. Chromosomal disorders such as downs syndrome
  5. Other : there is loads
27
Q

What are the signs of infantile / congenital glaucoma?

A

There are breaks in the descement’s membrane due to forceps injury’s

28
Q

Is infantile glaucoma bilateral or unilateral?

A

It can be both

29
Q

What are the symptoms for infantile glaucoma?

A

photophobia

30
Q

signs of infantile primary glaucoma?

A
  1. Red eye
  2. Enlarged cornea
  3. Corneal clouding
    CAN BE V DIFFICULT TO SPOT
31
Q

How do we treat infantile primary glaucoma?

A
  • Surgically for the glaucoma
  • treat refractive error
  • Amblyopia therapy
32
Q

ocular associations of cataract? (specific)

A

anterior segment dysgenesis

33
Q

ocular associations of cataract? (non-specific)

A

microphthalmos
nystagmus/strabismus –> due to low vision

34
Q

What causes cataract in paeds?

A

sporadic or inherited

35
Q

What are systemic associations on cataract?

A
  1. Intrauterine infections ; toxoplasmosis / RUBELLA
  2. Metabolic : DIABETES and galactosemia
  3. Dermatological : atopy. SKIN PROBLEMS
  4. Drugs / treatment : steroids
  5. Chromosomal : downs
  6. Renal
  7. Musculoskeletal
  8. CNS and hearing
  9. Birthweight
36
Q

Why do you get cataract with atopy(dermatological diseases)?

A

The intraocular lens is ectodermal (LIKE the skin)

37
Q

What is amblyopia caused by?

A

Obstacle to the visual development. Especially one eye

38
Q

Does cyls causes amblyopia in babies? why/why not?

A

NO - they are going through emmetropisation

39
Q

How do we manage unilateral cataract?

A
  1. Operate before 9-10 weeks for function
  2. Patching post operative
  3. Optical correction post operative
40
Q

why are IOLs in infancy a controversial opinion?

A

risk of glaucoma is v high

41
Q

How do we manage bilateral cat?

A
  1. Do surgery when opacity is interfering with vision
  2. post op refractive error correction (CLs, glasses)
42
Q

What complications can occur due to bilateral surgery?

A
  • Amblyopia
  • Glaucoma
  • Capsular opacification
  • Infections
43
Q

What is ectopia lentis?

A

This is a lens that is not the correct place

44
Q

What are the ocular associations for ectopia lentis?

A
  • Anterior segment dysgensis
  • glaucoma
  • surgical trauma
45
Q

What are the systemic assiocations for ectopia lentis?

A
  1. MARFANS syndrome - connective tissue disorder
  2. Homocystinuria - cardiac abnormalities
  3. Weill-marchesani - short stubby fingers
46
Q

How can we diagnose ectopia lens?

A
  1. unstable refraction
  2. Iridodensis (wobbly iris)
  3. Stretched zonules (Direction can indicate th aetiology)
47
Q

What is anterior uveitis in childhood associated with?

A
  1. Juvenile arthritis ; px is normally asymptomatic, so they need to be screened
  2. Idiopathic
  3. Sarcoidosis - inflammatory disease
  4. TB
  5. Behcet’s disease - Blood Vessels inflammatory disease
48
Q

is anterior uveitis in childhood common?

A

no

49
Q

Why does the pupil margins become irregular?

A

It is stuck to the lens (posterior synechiae)

50
Q

What is white pupils?

A

leukocoria

51
Q

leukocoria is indicative of….

A

Retinoblastoma

52
Q

why do anterior segment disorders cause photosensitivity in a child?

A

light scatter

53
Q

causes of leukocoria

A

retinoblastoma
coats
ROP
toxocara + toxoplasmosis
AND MORE