Common problems in paediatric ophthalmology. A lecture from Penang Medical College

1. Common Problems inCommon Problems in
PaediatricPaediatric
Dr. Lee Ming YuehDr. Lee Ming Yueh
Opthalmology DeptOpthalmology Dept
Penang HospitalPenang Hospital

2. Topics:Topics:
• Congenital cataractCongenital cataract
• Congenital glaucomaCongenital glaucoma
• Retinopathy of prematurity (ROP)Retinopathy of prematurity (ROP)
• SquintSquint
• Refractive errorRefractive error
• Allergic conjunctivitisAllergic conjunctivitis
• Ocular tumour ie. Retinoblastoma (RB)Ocular tumour ie. Retinoblastoma (RB)
• Ocular infectionsOcular infections
• Ocular traumaOcular trauma

3. Congenital CataractCongenital Cataract

4. Congenital cataractCongenital cataract
CausesCauses
• metabolic disorder ie. Galactosemia, hyper/hypoglisemiametabolic disorder ie. Galactosemia, hyper/hypoglisemia
• intrauterine infections ie. Rubella, Toxo, Varicellaintrauterine infections ie. Rubella, Toxo, Varicella
• hereditary AD, AR, X-linked (30%)hereditary AD, AR, X-linked (30%)
• Chromosomal disorder ie. Down’sChromosomal disorder ie. Down’s
• Syndromic disorder ie. Lowe’s , Nance-HoranSyndromic disorder ie. Lowe’s , Nance-Horan
• Ocular abnormality ie. Micropthalmos, aniridiaOcular abnormality ie. Micropthalmos, aniridia
• IdiopathicIdiopathic

5. Congenital CataractCongenital Cataract
Presenting SignsPresenting Signs
• white pupils/leucocoriawhite pupils/leucocoria
• Eccentric fixation/squintEccentric fixation/squint
• roving eye movementroving eye movement

6. History:History:
• Age of presentationAge of presentation
• Family history – consanguinityFamily history – consanguinity
• Birth history – trauma at birth?Birth history – trauma at birth?
• Maternal – DM? infection? eg. RubellaMaternal – DM? infection? eg. Rubella
• Drug exposure - steroid, chlorpromazineDrug exposure - steroid, chlorpromazine
• Radiation exposureRadiation exposure

7. Clinical Examination:Clinical Examination:
• Visual acuityVisual acuity
–– fixation, forced preferential looking charts, hundredsfixation, forced preferential looking charts, hundreds
& thousands test, Catford drum& thousands test, Catford drum
• LensLens
- Visually significant?- Visually significant?
- Type of cataract- Type of cataract
eg.eg. oil-droplet (galactosemia)oil-droplet (galactosemia)
multicolor flecks (hypothyroidism)multicolor flecks (hypothyroidism)

8. Congenital cataractCongenital cataract
Lamellar
Cortical spoke-
like
Coronary
Central pulverulent

9. • Associted Ocular anomaliesAssocited Ocular anomalies
- microphthalmos (rubella)- microphthalmos (rubella)
- mesenchymal dysgenesis, aniridia- mesenchymal dysgenesis, aniridia
- glaucoma (Rubella, Lowe’s)- glaucoma (Rubella, Lowe’s)
- uveitis (juvenile Rheumatoid Arthritis)- uveitis (juvenile Rheumatoid Arthritis)
• Associated systemic anomaliesAssociated systemic anomalies
- chromosomal eg.Down’s- chromosomal eg.Down’s
- skin rashes (atopic dermatitis)- skin rashes (atopic dermatitis)
- renal ds (Lowe’s, Alport’s)- renal ds (Lowe’s, Alport’s)

10. InvestigationsInvestigations
• Full blood countFull blood count
• Renal function testRenal function test
• Serum calciumSerum calcium
• Serology for rubella, toxoplasmosisSerology for rubella, toxoplasmosis
• GPUT & galactokinase activity in RBCGPUT & galactokinase activity in RBC
• UrineUrine
- reducing sugar, amino acid,- reducing sugar, amino acid,

11. Cataract Surgery in PediatricCataract Surgery in Pediatric
• Timing of SurgeryTiming of Surgery
Bilateral severeBilateral severe – 2-3 months, operate fellow eye in 1 week– 2-3 months, operate fellow eye in 1 week
Unilateral severeUnilateral severe – before 8 weeks– before 8 weeks
Bilateral or unilateral mildBilateral or unilateral mild – wait till child is older– wait till child is older
• SurgerySurgery
Lens aspiration and primary posterior capsulotomyLens aspiration and primary posterior capsulotomy
Important issue: to implant IOL or not?Important issue: to implant IOL or not?
- No lens implantation if < 2 year-oldNo lens implantation if < 2 year-old
can consider IOL implantation > 6 monthscan consider IOL implantation > 6 months
- IOL power selection. Aim for emmetropic? Hyperopic?- IOL power selection. Aim for emmetropic? Hyperopic?
- If no IOL implant >Aphakic correction by glasses/contact lens- If no IOL implant >Aphakic correction by glasses/contact lens

12. Problems in Congenital Cataract SurgeryProblems in Congenital Cataract Surgery
• Intraoperative problemsIntraoperative problems
-- risk of GA due to prematurity or asso systemic dsrisk of GA due to prematurity or asso systemic ds
- small eye, anatomical difference- small eye, anatomical difference
- low scleral rigidity, solid vitreous- low scleral rigidity, solid vitreous
-- elastic anterior capsuleelastic anterior capsule
• Postoperative problemsPostoperative problems
-- intense inflammationintense inflammation
- posterior capsule opacification- posterior capsule opacification
- IOL decentralization- IOL decentralization
- Frequent refractive assessment- Frequent refractive assessment

13. Soemmerring ringSoemmerring ring
following congenital cataract surgeryfollowing congenital cataract surgery

14. Retinopathy of PrematurityRetinopathy of Prematurity

15. Incidence & Health BurdenIncidence & Health Burden
• Baby less than 1250g > 65% have ROPBaby less than 1250g > 65% have ROP
• Baby less than 1000g > 80% have ROPBaby less than 1000g > 80% have ROP
• 6% needed treatment6% needed treatment
• Severe loss of visionSevere loss of vision
• Epidemic in developing countries includingEpidemic in developing countries including
Malaysia.Malaysia.

16. Retinopathy of prematurityRetinopathy of prematurity
• Proliferative retinopathy affecting pre-term & lowProliferative retinopathy affecting pre-term & low
birth weight infantsbirth weight infants
• Risk factorsRisk factors
- prematurity & low birth weight- prematurity & low birth weight
– exposure to prolonged oxygen therapyexposure to prolonged oxygen therapy
– intraventricular hemorrhageintraventricular hemorrhage
– VEGFVEGF
– LightLight??
– Vitamin E deficiency?Vitamin E deficiency?

17. Normal Retinal AngiogenesisNormal Retinal Angiogenesis
• Starts at 16 weeksStarts at 16 weeks
• Reaches nasal ora at 36 weeksReaches nasal ora at 36 weeks
• Complete vascularization at 40 weeksComplete vascularization at 40 weeks

18. Clinical Practice Guideline (Malaysia)Clinical Practice Guideline (Malaysia)
• Birthweight < 1500gBirthweight < 1500g
• Gestation < 32 weeksGestation < 32 weeks
• Others -Others - as determined by neonatologistas determined by neonatologist
ie. Prolonged O2 therapy, very ill withie. Prolonged O2 therapy, very ill with
unstable clinical courseunstable clinical course

19. Active ROPActive ROP
• LocationLocation – zone I. II, III– zone I. II, III
• ExtentExtent – number of clock hours– number of clock hours
• StageStage
Stage 1 - 5Stage 1 - 5
• Plus diseasePlus disease
- dilatation & tortuosity of veins- dilatation & tortuosity of veins
- vitreous haze- vitreous haze
- engorged iris vessels- engorged iris vessels
- poor pupil dilation- poor pupil dilation

21. Retinopathy of prematurityRetinopathy of prematurity

22. Plus Disease

23. Clinical Practice Guideline (Malaysia)Clinical Practice Guideline (Malaysia)
• First screeningFirst screening – 4-6 weeks post gestation– 4-6 weeks post gestation
• Follow-upFollow-up
- stage 0 : 2 – 3 weeks- stage 0 : 2 – 3 weeks
- stage 1 : 2 weeks- stage 1 : 2 weeks
- stage 2 : 1 week- stage 2 : 1 week
- stage 3 : 1 week or less- stage 3 : 1 week or less
• When do you stop follow up?When do you stop follow up?
- ROP regression- ROP regression
- Fully vascularised retina- Fully vascularised retina

24. Threshold DiseaseThreshold Disease
• Zone I or IIZone I or II
• 55 contiguous clock hours orcontiguous clock hours or 88 noncontiguousnoncontiguous
clock hoursclock hours
• Stage 3Stage 3
• Plus diseasePlus disease
Threshold ROP is associated with 50% risk ofThreshold ROP is associated with 50% risk of
having VA 1/60 or worse without treatment.having VA 1/60 or worse without treatment.

25. Management of ROPManagement of ROP
• CryotherapyCryotherapy
- to ablate retina anterior to ridge- to ablate retina anterior to ridge
- ‘- ‘Multicentre Cryotherapy for ROP studyMulticentre Cryotherapy for ROP study’’ arch opht 1996arch opht 1996
>50% ↓ in poor VA>50% ↓ in poor VA
>50% ↓ in poor fundus status>50% ↓ in poor fundus status
• Indirect laser photocoagulationIndirect laser photocoagulation
- less traumatic, better visual outcome- less traumatic, better visual outcome
• Vitreo-retinal surgery (For stage 4 disease)Vitreo-retinal surgery (For stage 4 disease)
• Intravitreal triamcinolone & anti-VEGFIntravitreal triamcinolone & anti-VEGF

26. SquintSquint

27. SquintSquint
• ““misalignment of the eyesmisalignment of the eyes””
- Esotropia, exotropia, hyper or hypotropia- Esotropia, exotropia, hyper or hypotropia
• Ocular alignment should stabilize by age of 2Ocular alignment should stabilize by age of 2
monthsmonths
• Any misalignment after age of 3 monthsAny misalignment after age of 3 months
- refer to ophthalmologist- refer to ophthalmologist

28. Hirschberg testHirschberg test
• Rough measure of deviationRough measure of deviation
• Note location of corneal light reflexNote location of corneal light reflex
• 1 mm = 71 mm = 7˚˚or 15or 15ΔΔ
Reflex at border of pupil=15˚ Reflex at limbus=45ْ

29. SquintSquint
EsotropiaEsotropia
Exotropia

30. SquintSquint
ManagementManagement
• proper assessmentproper assessment
degree of deviationdegree of deviation
• causes of squintcauses of squint
• optical correctionoptical correction
• surgical correctionsurgical correction

31. Refractive ErrorRefractive Error

32. Refractive ErrorRefractive Error
• Image is focused eitherImage is focused either
- in front > Myopia (short-sighted)- in front > Myopia (short-sighted)
- behind retina > Hyperopia (long-sighted)- behind retina > Hyperopia (long-sighted)
• AstigmatismAstigmatism
• vision usually improves withvision usually improves with pinholepinhole

33. Refractive ErrorRefractive Error
Myopia
Hyperopia

34. Refractive errorRefractive error
• Vision or refraction should be screened at leastVision or refraction should be screened at least
at age of 3 years and 6 yearsat age of 3 years and 6 years
• Can be treated by spectaclesCan be treated by spectacles
• Generally contact lens and refractive surgery areGenerally contact lens and refractive surgery are
not advisable in childrennot advisable in children

35. Allergic conjunctivitisAllergic conjunctivitis
• Allergic rhinoconjunctivitisAllergic rhinoconjunctivitis
• Vernal keratoconjunctivitisVernal keratoconjunctivitis

36. Allergic rhinoconjunctivitisAllergic rhinoconjunctivitis
• Hypersensitivity reactionHypersensitivity reaction
to specific airboneto specific airbone
antigensantigens
• Frequently associatedFrequently associated
with nasal symptomswith nasal symptoms
• May be seasonal orMay be seasonal or
perennialperennial
Transient conjunctival oedema

37. Vernal keratoconjunctivitisVernal keratoconjunctivitis
• Frequently associated withFrequently associated with
atopy- asthma, hayatopy- asthma, hay
fever,dermatitisfever,dermatitis
• Recurrent, bilateralRecurrent, bilateral
• Affecting children and youngAffecting children and young
adultsadults
• More common in males andMore common in males and
warm climateswarm climates
• Itching, mucoid dischargeItching, mucoid discharge
and lacrimationand lacrimation
• Treatment –topical mast cellTreatment –topical mast cell
stabiliser and topical steroidsstabiliser and topical steroids

38. Giant papillae
which give a
cobblestone
appearance
Trantas' dot are
whitish dots seen
at the limbus in
some people with
vernal
keratoconjunctivi
tis during active
phase of the
disease

39. Ocular TumourOcular Tumour

40. Ocular TumoursOcular Tumours
• Can present as proptosis, squint or a whiteCan present as proptosis, squint or a white
reflex (absence of red reflex on fundoscopy)reflex (absence of red reflex on fundoscopy)
or poor visionor poor vision
• Primary intraocular tumour -retinoblastoma,Primary intraocular tumour -retinoblastoma,
optic nerve gliomaoptic nerve glioma
• Rhabdomyosarcoma - most common primaryRhabdomyosarcoma - most common primary
paediatric orbital malignancypaediatric orbital malignancy
• Metastases – neuroblastoma, leukemiaMetastases – neuroblastoma, leukemia

41. RetinoblastomaRetinoblastoma
•Leukocoria - 60%
• Strabismus - 20%
•Secondary glaucoma
Anterior segment
invasion
Orbital inflammation & invasion

42. RetinoblastomaRetinoblastoma
• Most common primary intraocular malignancy ofMost common primary intraocular malignancy of
childhoodchildhood
• tumour of primitive photoreceptor cellstumour of primitive photoreceptor cells
• prevalence of 1 in 20,000prevalence of 1 in 20,000
• No sexual or racial predilectionNo sexual or racial predilection
• Presents before the age of 3 years (average 3 months)Presents before the age of 3 years (average 3 months)
• 40% hereditary, 60% non heritable40% hereditary, 60% non heritable
• Predisposing gene (RPE1) on 13q14Predisposing gene (RPE1) on 13q14
• cryotherapy & laser photocoagulation, enucleation,cryotherapy & laser photocoagulation, enucleation,
radiotherapy, chemotherapyradiotherapy, chemotherapy

43. Ocular infectionsOcular infections
Ophthalmia neonatorumOphthalmia neonatorum
• Conjunctivitis during neonatal periodConjunctivitis during neonatal period
• causescauses
– Gonococcal (2-4 days)Gonococcal (2-4 days)
– Staph., Haemophilus spp (4-5 days)Staph., Haemophilus spp (4-5 days)
– Herpes simplex (5-7 days)Herpes simplex (5-7 days)
– Chlamydia (5-14 days)Chlamydia (5-14 days)
– Chemical ie. Silver nitrate (within 24 hr)Chemical ie. Silver nitrate (within 24 hr)

44. Ocular InfectionOcular Infection
• ConjunctivitisConjunctivitis
• Preseptal cellulitisPreseptal cellulitis
– inflammed & swollen lid with tendernessinflammed & swollen lid with tenderness
– orbital contents not involvedorbital contents not involved
• BlepharitisBlepharitis
– lid margin inflammationlid margin inflammation
• Corneal ulcerCorneal ulcer

45. Ocular infectionsOcular infections
Bacterial conjunctivitis
Preseptal cellulitis

46. BlepharitisBlepharitis
• Chronic irritation worse in morningsChronic irritation worse in mornings
Scales around base of lashes Hyperaemia and telangiectasia
of anterior lid margin

47. Corneal ulcerCorneal ulcer
Herpetic keratitis Bacterial keratitis

48. Ocular InfectionOcular Infection
Orbital cellulitisOrbital cellulitis
• lid and conjunctival swelling, proptosis,lid and conjunctival swelling, proptosis,
restricted eye movement and tendernessrestricted eye movement and tenderness
over sinuses, RAPD positiveover sinuses, RAPD positive
• patient usually ill & febrilepatient usually ill & febrile

49. Ocular traumaOcular trauma
• Blunt or sharp injuriesBlunt or sharp injuries
• hyphema, vitreous hemorrhage, commotiohyphema, vitreous hemorrhage, commotio
retinaeretinae
• corneoscleral lacerationcorneoscleral laceration
• lid lacerationlid laceration
• ruptured globeruptured globe

50. Ocular traumaOcular trauma
• Toys can be a potential source of injuryToys can be a potential source of injury
• hammock, cloth hanger, pencil, metal ruler, wirehammock, cloth hanger, pencil, metal ruler, wire
and etc are all dangerous items in children’sand etc are all dangerous items in children’s
hand without supervisionhand without supervision

52. Traumatic cataract
Iridodialysis
Vossius ring
Traumatic mydriasis
Sympathetic Ophthalmia

53. Commotio retinae Choroidal rupture and haemorrhage
Traumatic optic neuropathy

54. Congenital Naso-lacrimal DuctCongenital Naso-lacrimal Duct
ObstructionObstruction

55. Congenital Nasolacrimal Duct ObstructionCongenital Nasolacrimal Duct Obstruction
• At birth, the lower end of the nasolacrimal ductAt birth, the lower end of the nasolacrimal duct
is frequently non-canalized n(valve of Hasner).is frequently non-canalized n(valve of Hasner).
• No clinical significance in most neonatesNo clinical significance in most neonates
because it canalizes spontaneously soon afterbecause it canalizes spontaneously soon after
birth.birth.

56. Anatomy of Nasolacrimal Drainage SystemAnatomy of Nasolacrimal Drainage System

57. Clinical Features:Clinical Features:
• Epiphora and matting of the lashesEpiphora and matting of the lashes
• Reflux of purulent material from the punctumReflux of purulent material from the punctum
• Acute dacryocystitis is uncommon.Acute dacryocystitis is uncommon.

59. CongenitalCongenital
Nasolacrimal DuctNasolacrimal Duct
obstructionobstruction
complicated withcomplicated with
acute dacryocystitisacute dacryocystitis

60. Management:Management:
• ConservativeConservative
- Massage of the nasolacrimal duct increases the- Massage of the nasolacrimal duct increases the
hydrostatic pressure and thereby rupture thehydrostatic pressure and thereby rupture the
membranous obstructionmembranous obstruction
- 10 mstokes 4x a day.- 10 mstokes 4x a day.
• SurgicalSurgical
- 95% spontaneous canalization- 95% spontaneous canalization
- Probing to overcome the obstructive membrane at- Probing to overcome the obstructive membrane at
Hasner’s valve.Hasner’s valve.
- Can be repeated 6 weeks after if no improvement.- Can be repeated 6 weeks after if no improvement.
- 90% cured by first probing and a further 6% by the- 90% cured by first probing and a further 6% by the
secondsecond

62. Thank You