Common Problems in Common Problems in PaediatricPaediatric

Dr. Lee Ming YuehDr. Lee Ming Yueh

Opthalmology DeptOpthalmology Dept

Penang Hospital Penang Hospital

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

Congenital CataractCongenital Cataract

Congenital cataractCongenital cataract

CausesCauses• metabolic disorder ie. Galactosemia, hyper/hypoglisemiametabolic disorder ie. Galactosemia, hyper/hypoglisemia• intrauterine infections ie. Rubella, Toxo, Varicella intrauterine infections ie. Rubella, Toxo, Varicella • hereditary AD, AR, X-linked (30%) hereditary AD, AR, X-linked (30%) • Chromosomal disorder ie. Down’s Chromosomal 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

Congenital CataractCongenital Cataract

Presenting SignsPresenting Signs• white pupils/leucocoriawhite pupils/leucocoria• Eccentric fixation/squintEccentric fixation/squint• roving eye movementroving eye movement

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 exposure Radiation exposure

Clinical Examination:Clinical Examination:

• Visual acuityVisual acuity – – fixation, forced preferential looking charts, hundreds fixation, 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)

Congenital cataractCongenital cataract

Lamellar

Cortical spoke-like

Coronary

Central pulverulent

• 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)

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,

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

• SurgerySurgeryLens aspiration and primary posterior capsulotomyLens aspiration and primary posterior capsulotomyImportant 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 months can 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

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

Soemmerring ring Soemmerring ring following congenital cataract surgeryfollowing congenital cataract surgery

Retinopathy of PrematurityRetinopathy of Prematurity

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 including Epidemic in developing countries including

Malaysia.Malaysia.

Retinopathy of prematurityRetinopathy of prematurity

• Proliferative retinopathy affecting pre-term & low Proliferative retinopathy affecting pre-term & low birth weight infants birth 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?

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

Clinical Practice Guideline (Malaysia)Clinical Practice Guideline (Malaysia)

• Birthweight < 1500gBirthweight < 1500g• Gestation < 32 weeksGestation < 32 weeks• Others -Others - as determined by neonatologist as determined by neonatologist

ie. Prolonged O2 therapy, very ill withie. Prolonged O2 therapy, very ill with

unstable clinical course unstable clinical course

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

Retinopathy of prematurityRetinopathy of prematurity

Plus Disease

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

Threshold DiseaseThreshold Disease

• Zone I or IIZone I or II• 5 5 contiguous clock hours or contiguous clock hours or 8 8 noncontiguous noncontiguous

clock hoursclock hours• Stage 3Stage 3• Plus diseasePlus disease

Threshold ROP is associated with 50% risk of Threshold ROP is associated with 50% risk of having VA 1/60 or worse without treatment.having VA 1/60 or worse without treatment.

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

SquintSquint

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 2 Ocular alignment should stabilize by age of 2

monthsmonths• Any misalignment after age of 3 months Any misalignment after age of 3 months

- refer to ophthalmologist- refer to ophthalmologist

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

SquintSquint

EsotropiaEsotropia

Exotropia

SquintSquint

ManagementManagement• proper assessment proper assessment

degree of deviationdegree of deviation• causes of squintcauses of squint• optical correctionoptical correction• surgical correctionsurgical correction

Refractive ErrorRefractive Error

Refractive ErrorRefractive Error

• Image is focused either Image 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 with vision usually improves with pinholepinhole

Refractive ErrorRefractive Error

Myopia

Hyperopia

Refractive errorRefractive error

• Vision or refraction should be screened at least Vision 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 are Generally contact lens and refractive surgery are

not advisable in childrennot advisable in children

Allergic conjunctivitisAllergic conjunctivitis

• Allergic rhinoconjunctivitisAllergic rhinoconjunctivitis• Vernal keratoconjunctivitisVernal keratoconjunctivitis

Allergic rhinoconjunctivitisAllergic rhinoconjunctivitis

• Hypersensitivity reaction Hypersensitivity reaction to specific airbone to specific airbone antigensantigens

• Frequently associated Frequently associated with nasal symptomswith nasal symptoms

• May be seasonal or May be seasonal or perennialperennial

Transient conjunctival oedema

Vernal keratoconjunctivitisVernal keratoconjunctivitis

• Frequently associated with Frequently associated with atopy- asthma, hay atopy- asthma, hay fever,dermatitisfever,dermatitis

• Recurrent, bilateralRecurrent, bilateral• Affecting children and young Affecting children and young

adultsadults• More common in males and More common in males and

warm climateswarm climates• Itching, mucoid discharge Itching, mucoid discharge

and lacrimationand lacrimation• Treatment –topical mast cell Treatment –topical mast cell

stabiliser and topical steroidsstabiliser and topical steroids

Giant papillae which give a cobblestone appearance

Trantas' dot are whitish dots seen at the limbus in some people with vernal keratoconjunctivitis during active phase of the disease

Ocular TumourOcular Tumour

Ocular TumoursOcular Tumours

• Can present as proptosis, squint or a white Can 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 glioma optic nerve glioma

• Rhabdomyosarcoma - most common primary Rhabdomyosarcoma - most common primary paediatric orbital malignancypaediatric orbital malignancy

• Metastases – neuroblastoma, leukemiaMetastases – neuroblastoma, leukemia

RetinoblastomaRetinoblastoma

•Leukocoria - 60%

• Strabismus - 20%•Secondary glaucoma

Anterior segment invasion

Orbital inflammation & invasion

RetinoblastomaRetinoblastoma

• Most common primary intraocular malignancy of Most 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

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)

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

Ocular infectionsOcular infections

Bacterial conjunctivitisPreseptal cellulitis

BlepharitisBlepharitis

• Chronic irritation worse in morningsChronic irritation worse in mornings

Scales around base of lashes Hyperaemia and telangiectasia of anterior lid margin

Corneal ulcerCorneal ulcer

Herpetic keratitis Bacterial keratitis

Ocular InfectionOcular Infection

Orbital cellulitisOrbital cellulitis • lid and conjunctival swelling, proptosis, lid and conjunctival swelling, proptosis,

restricted eye movement and tenderness restricted eye movement and tenderness over sinuses, RAPD positiveover sinuses, RAPD positive

• patient usually ill & febrilepatient usually ill & febrile

Ocular traumaOcular trauma

• Blunt or sharp injuriesBlunt or sharp injuries• hyphema, vitreous hemorrhage, commotio hyphema, vitreous hemorrhage, commotio

retinaeretinae• corneoscleral lacerationcorneoscleral laceration• lid lacerationlid laceration• ruptured globeruptured globe

Ocular traumaOcular trauma

• Toys can be a potential source of injuryToys can be a potential source of injury• hammock, cloth hanger, pencil, metal ruler, wire hammock, cloth hanger, pencil, metal ruler, wire

and etc are all dangerous items in children’s and etc are all dangerous items in children’s hand without supervisionhand without supervision

Traumatic cataract

Iridodialysis

Vossius ring

Traumatic mydriasisSympathetic Ophthalmia

Commotio retinae Choroidal rupture and haemorrhage

Traumatic optic neuropathy

Congenital Naso-lacrimal Duct Congenital Naso-lacrimal Duct ObstructionObstruction

Congenital Nasolacrimal Duct ObstructionCongenital Nasolacrimal Duct Obstruction

• At birth, the lower end of the nasolacrimal duct At 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 neonates No clinical significance in most neonates because it canalizes spontaneously soon after because it canalizes spontaneously soon after birth.birth.

Anatomy of Nasolacrimal Drainage SystemAnatomy of Nasolacrimal Drainage System

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.

Congenital Congenital Nasolacrimal Duct Nasolacrimal Duct obstruction obstruction complicated with complicated with acute dacryocystitisacute dacryocystitis

Management:Management:

• ConservativeConservative- Massage of the nasolacrimal duct increases the - Massage of the nasolacrimal duct increases the hydrostatic pressure and thereby rupture the hydrostatic 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

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