Dr Casey Ung. Ophthalmologist Dunedin Hospital Eye Clinic Marinoto Clinic & Specialists Dunedin

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1 Dr Casey Ung Ophthalmologist Dunedin Hospital Eye Clinic Marinoto Clinic & Specialists Dunedin 8:30-9:25 WS #140: Paediatric Ophthalmology 9:35-10:30 WS #150: Paediatric Ophthalmology (Repeated)

2 Paediatric Ophthalmology Casey Ung GPCME Conference 2017 Horncastle Arena Christchurch

3 Examination in paediatrics The examination has to be tailored for the development of the child Cooperation and understanding of the parents/caregivers is essential Sometimes a repeat appointment has to be made should an examination be unable to be completed

4 Equipment that I use

5 Equipment that I use (cont.)

6 Equipment that I use (cont.)

7 Preferential looking

8 Equipment that I use (cont.)

9 The direct ophthalmoscope

10 The direct ophthalmoscope An extremely versatile and essential item of ophthalmic equipment; Checking the pupil reflexes Detect ocular misalignment (observing the corneal light reflexes) Media opacities and leukocoria A preliminary assessment of the angle Blue light filter for checking fluorescein uptake Fundoscopy

11 The reflex

12 Examination in paediatrics (continued) Paediatric ophthalmology has its challenges and rewards During my training and fellowship, it was always stressed that one had to engage the child s attention: 1 toy, 1 look Also; NEVER SHOW FEAR!

13 Corneal light reflections (reflexes)

14 BRÜCKNER REFLEX Comparison of fundus red reflexes when viewed simultaneously (at arms length) through direct ophthalmoscope Good for detecting small angle squints and amblyopia Sensitivity 86% and specificity 68% for detecting amblyopia risk factors In children with amblyopia, 95% sensitive

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16 Cover testing

17 A selection of Paediatric Ophthalmology Amblyopia Strabismus/Squint

18 Visual Development Requires clear images and aligned eyes during early childhood The two eyes compete with one another from birth to make connections to the visual cortex Vision at birth 6/480, 6/36 at 6 mos, 6/9 at age 3 Fusion and stereopsis develop at about 4 mos of age (coincident with foveal maturation) Can occur only up to age 8 years

19 Amblyopia Amblyopia: Poor vision due to abnormal visual experience in early life Prevalence is about 3% Prevalence is decreased when screening and early intervention are instituted (supported by RCTs) Prevalence is higher in medically underserved populations

20

21 Amblyopia The management consists of trying to give the amblyopic eye more use, exercising it. Can be done through patching the good eye or penalising it with Atropine eye drops (both work equally as well according to the PEDIG studies) Amblyopia treatment continues until age 7-9yo, but be aware of reverse amblyopia

22 STRABISMUS (SQUINT) DEFINITION: Misalignment of the Eyes TERMINOLOGY: Eso- Convergent Exo- Divergent -Phoria: a Latent Squint (held in place by fusion) -Tropia: a Constant Squint SQUINT AMBLYOPIA REFRACTIVE ERRORS

23 SQUINT TYPES Esotropia: A constant convergent squint (crossed eyes) Infantile Esotropia Acquired Esotropia Exotropia: A constant divergent squint Intermittent Constant Others IV N palsy III N palsy Vertical squints

24 Infantile Esotropia Not truly congenital Onset usually between four and six months of age Typically have moderate to large angle of squint Treatment is surgical, anytime after six months Early surgery = better chance for binocularity

25 Accommodative Esotropia

26 Exotropias

27 DETECTION OF SQUINT External Appearance Asymmetry of Corneal Light Reflexes (Hirschberg Test) Cover Test Bruckner Test Amblyopia

28 Corneal light reflexes

29 Epiphora A common scenario where there is congenital NasoLacrimal Duct Obstruction (NLDO) Critical Features: Watery eyes, since birth Can intermittently be mucopurulent, although no conjunctivitis (more colonisation rather than infection) Can spontaneously resolve (80-90% in 1 st year) hence normally do Probe and Syringe after 1 year old

30 Blockage of the Nasolacrimal Duct

31

32 Dangerous epiphora Dacryocoele: Congenital blockage whereby there is no drainage and develops a collection which can become infected Needs urgent Probe + Syringe

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34

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36 Dangerous epiphora (cont.) Congenital glaucoma: Epiphora Photophobia Blepharospasm Corneal clouding Buphthalmos

37 Infantile Glaucoma Affects 1/8-10,000 children (M East 1/2,500) Primary congenital glaucoma usually sporadic, 80%>recessive, rarely dominant 60% present by 6/12, 80% by 1 2/3 male, 2/3 bilateral GLC3 gene-2p21, 1p36

38 Blepharitis, Styes and Chalazia Quite a common condition and blepharitis is much more common in Trisomy 21

39 Ophthalmia neonatorum Any severe conjunctivitis in the newborn period Bacterial - N Gonorrhoeae Chlamydia - Ps aeruginosa - Staph aureus - Strep pneumoniae - H influenzae Viral - HSV type II (and I) Chemical

40 Investigation Conjunctival swabs (culture/gram stain Conjunctival scrapings (Chlamydia PCR) Immunofluorescent studies (HSV/Chlamydia)

41 Infected or Not?

42 Orbital cellulitis Potentially life-threatening infection of the tissues behind the orbital septum More common in children but any age may be affected Critical features: Unilateral, tender, red periorbital and lid oedema Proptosis Painful ophthalmoplegia Optic nerve dysfunction (RAPD, decreased vision) Child is sick!

43 Orbital cellulitis

44 Orbital cellulitis Typically associated with sinus disease however may be due to extension of preseptal cellulitis, local spread (dacryocystitis, dental abscess), haematogenous or post-traumatic/surgical Complications: Ocular: endophthalmitis, CRA/V occlusion, optic neuropathy Intracranial (rare): meningitis, abscess, cavernous sinus thrombosis Subperiosteal/Orbital abscess

45 Orbital cellulitis

46 Before and After

47 LEUKOCORIA (WHITE PUPIL) Commonest cause - Congenital cataract Must always exclude Retinoblastoma

48 CONGENITAL CATARACTS Hereditary Metabolic Infectious Systemic Disease

49

50 Paediatric Cataract (cont.) Estimates of 130,000 to 200,000 with cataract Can be a huge economic impact, because they require extra care and attention At present, there is no medical/non-surgical treatment for cataract

51 Principles of Treatment Clearing the visual axis to allow visual development In the 1970 s, treatment delayed until (at least) 6 months of age. More surgeries are now being performed at earlier and earlier ages (some within the first 2 weeks of life) Visual rehabilitation included: Aphakic spectacles Aphakic C/Ls, and more recently Secondary IOLs Primary IOLs

52 Principles of Treatment (cont.) Anaesthetic risks have to be balanced against the impact on child s visual development Bilateral cataracts (especially if symmetrical) typically operated on within a week or two of each other. Unilateral cataracts bear discussion

53 Aetiology of Paediatric Cataract Younger children Genetic aberrations Metabolic disorders Prematurity Intrauterine infections Older children Trauma Drug-induced Radiotherapy Laser therapy (ROP)

54 Unilateral or Bilateral Bilateral cataracts more likely to suggest systemic / hereditary / infective cause: Down Syndrome, Trisomy 13 or 15, Alport Sd, Lowe Sd TORCH, Varicella, Rubella Ocular abnormalities: Aniridia, AntSeg dysgenesis Sd Toxic: Steroids, radiotherapy Unilateral cataracts tend to be sporadic or traumatic : PHPV, AntSeg dysgenesis Traumatic Possibly Rubella or masked bilateral cataract

55

56

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58 Retinoblastoma Malignant eye cancer 1/18,000 live births Often results in loss of eye if tumour growth can not be controlled Many tumours now treatable with laser

59 Retinoblastoma (cont.) Recessive Gene Two Hit Hypothesis Dominant Inheritance

60 RETINOBLASTOMA Multiple tumours in 1 eye Bilateral involvement Germinal Mutation One tumour in 1 eye Somatic Mutation

61 Nystagmus and wobbly eyes Used to be categorised as Congenital Motor or Congenital Sensory Nystagmus However, now simply called Infantile Nystagmus syndrome

62 Nystagmus (cont.) The nystagmus syndromes that we see can be associated with ocular or systemic causes. Ocular causes: typically associated with poor vision, with VA less than 4/24 or 6/36; e.g. Ocular/Oculocutaneous Albinism, untreated congenital cataracts, optic nerve hypoplasia Isolated Idiopathic Nystagmus Syndrome ( Congenital Motor Nystagmus ) is a nystagmus syndrome not associated with systemic or ocular abnormalities. In contrast to above may have reasonable vision

63 Child with Poor Vision Acute intervention (PLVC) Mobility Low Vision Aids Counselling for Parents Medicolegal issues Whole of life cost $1m

64 Thank you for your attention! Any questions?

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