Paediatric Ophthalmology

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Margaret Dawson
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1 Paediatric Ophthalmology Erika Maka MD SEMMELWEIS UNIVERSITY, FACULTY OF MEDICINE DEPARTMENT OF OPHTHALMOLOGY, BUDAPEST DIRECTOR: PROF. DR. ZOLTÁN ZSOLT NAGY

2 Why? When? How?.?.?.?. without problem - Screening with problem - Examination

4 Inspection Visual Acuity Cover test Examination (sometimes EUA: Examination Under Anaesthesia)

5 Tear Discharge Red eye Leukocoria Anisocoria Diplopia Trauma

6 Brückner test

7 Ophthalmoscopy / Funduscopy Direct: an upright, or unreversed, image of approximately 15 times magnification. Red reflex Brückner test Indirect: an inverted, or reversed, direct image of 2 to 5 times magnification Binocular Slit lamp

8 Developmental disorders Inflammations Tumours Trauma

9 CNLDO Congenital Nasolacrimal Duct Obstruction Epiphora No pain Discharge Sticky eyes No red eye Management Conservative massaging of the ducts may be enough because of the high rate of spontaneous resolution regular cleaning of any discharge from the eyelids and nose Use topical antibiotics for any episodes of associated conjunctivitis Surgical 6 months - 1 year of age: probing (Occasionally a dacryocystorhinostomy procedure is required)

10 Neonatal conjunctivitis Eyelids are usually swollen. Red eye: conjunctival injection & oedema Discharge: Purulent Watery Sanguinary Time 3-5 days: Neisseria (!) 7-14 days: Chlamydia (!)

11 Congenital glaucoma Buphthalmos Photophobia Pain Cloudy cornea Maka E. et al.

12 Aniridia Bilateral Nystagm Visual impairment Photophobia Glaucoma With or without: Wilms tumour (nephroblastoma) Urogenital anomalies Mental retadation WAGR

13 Congenital cataract uni-, bilateralis Presentation: Leukocoria Strabismus Nystagmus Amblyopia (deprivation)

14 Brückner test: check the red reflex Newborn before admission, 1, 3, 6 and 12 month of age and If the parents recognise abnormality + cover test

15 Morphology Cataracta totalis Early intervention!!! Cataracta polaris anterior Indication for surgery: >3mm Cataracta subcapsularis anterior Uveitis Trauma Irradiation Atopic dermatitis Alport syndrome Cataracta stellata /suturalis Cataracta nuclearis oil drop : Galactosaemia (+C. subcapsularis posterior, C. nuclearis, C.corticalis) Cataracta nuclearis, lamellaris et coronaria Indication for surgery: >3mm Period without inflammation Systemic and local perioperative treatment

16 Morphology Cataracta membranous Cataracta subcapsularis posterior Steroid induced Irradiation Cataracta punctata corticalis (coerulea) Blue dots Syndrome: Down PFV (persistent foetal vasculature)

17 Paediatrics Intrauterin infection? (prenatal histotory, microcephalia, growth retardation, cardiology?, hearing problem?) TORCHS (toxoplasmosis, rubeola, herpes, syphilis) Bilateral cataract galactosemia?, TORCHS? galactokinase deficiency? Glaucoma? Hypotonia? Developmental delay? Lowe syndrome Hypocalcaemia, mannosidosis, glucose?, hyperferritinaemia

18 Therapy Without surgery: Peripheral opacities < 3mm Check the refractive power! (astigmatism? ) Glasses? Amblyopia? Occlusion

19 Therapy - surgery At time! (critical periode! in visual development) unilateral: 4-6 week Bilateral: 6-8 week

20 EUA: Examination Under Anaesthesia AL: axial length of the eye K: Refractive power of the cornea (keratometry) IOP White-To-White corneal diameter

21 Amblyopia treatment Unilateral: (4 months of age 4 hours) After 8 months of age half of the day Bilateral

22 Check-up subconjunctival steroid + IOP? Removement of the sutures 4-6 weeks Refractive error? IOP? RE?, IOP?, PCO?

23 Retinoblastoma Malignant tumour 1: / live birth Different prognosis? Health care? Early or late diagnosis? Available treatment? Cultural differences

24 Genetics RB1 13q14 27 exon mrns nucleophosphoprotein (prb) (regulation of cell cycle) 26 intron (point mutation - deletion)

25 Genetics Two mutation (both RB1 allel) (Knudson model) First: germinal or somatic Second: somatic (retina) Heritable RB In 90-95% of cases bilateral Age of onset: usually less than 1 year In % of cases positive family history (familiar) High risk: pineal gland tumours, secondary osteosarcoma, melanoma 50% risk of passing on an RB1 mutation to an offspring Nonheritable form Unilateral Later diagnosis

26 Presenting features Number of patients Abramson et al. USA Main presenting signs(%) Leukocoria (56), Strabismus (24) Poor visison (8) Wallach et al. Switzerland Leukocoria (48), Strabismus (20) Zhao et al. China Leukocoria (73) Chang et al. Taiwan Leukocoria (71), Red eye (18) Strabismus (14) Ali et al. Sudan Enlarged eye (56) Leukocoria(32) Owoeye et al. Nigeria Proptosis (85) Saiju et al. Nepal Boubacar et al. Mali Leukocoria (43) Extraocular mass (33) Proptosis (55) Leukocoria (38), Strabismus (6) Buphthalmus (2)

27 MR - Diagnosis Optic nerve? Chorioidea? Corpus pineale?

28 Treatment Oncoteam Goals: Save the life Save the eye Preserve as much vision as possible Minimize the risk of late sequelae from treatment Local therapy: Transpupillary thermotherapy, Cryotherapy Chemotherapy: intravitreal, intraarterial, systemic Radiation therapy: plaque radiation therapy (brachytherapy), external beam radiation therapy (EBRT) Surgical therapy: enucleation, exenteration

29 ROP= Retinopathy of prematurity 3 2,5 2 Increasing survival; sub-optimal neonatal care; low coverage with ROP programs Gilbert, The Lancet The third epidemic of blindness due to ROP. 1,5 1 0,5 High mortality amongst preterm infants 0 High Income Eastern Europe/ Central Asia Latin America and Caribbean East and S.East Asia and Pacific North Africa and Middle East South Asia Sub-Saharan Africa

30 Control of blindness due to ROP Primary prevention reduce preterm birth antenatal steroids appropriate resuscitation techniques very good neonatal care Secondary prevention diagnosis by ophthalmic examination timely treatment and follow up Tertiary prevention sight cannot be restored once lost

31 Vasculogenesis & angiogenesis JT Flynn, T Chan-Ling: Retinopathy of Premaurity: Two Distinct Mechanism That Underlie Zone 1 and Zone 2 Disease. Am J Ophthalmol 2006;142:46-59 RF Gariano: Special features of human retinal angiogenesis. Eye 2010;24:

Pathogenesis 1 st phase: Hyperoxia VEGF vasoobliteration regression of capillaries 2 nd phase: Hypoxia VEGF vasoproliferation A Hellstrom és mtsai: Low IGF-1 suppresses VEGF-survival signaling in

32 Pathogenesis 1 st phase: Hyperoxia VEGF vasoobliteration regression of capillaries 2 nd phase: Hypoxia VEGF vasoproliferation A Hellstrom és mtsai: Low IGF-1 suppresses VEGF-survival signaling in retinal endothelial cells: direct correlation with clinical retinopathy of prematurity. Proc Natl Acad USA 2001;98: LEH Smith: Pathogenesis of retinopathy of prematurity. Seminars in Neonatology 2003;8: J Chen, LEH Smith: Retinopathy of prematurity. Angiogenesis 2007;10:

33 The gold standard of the examination dilation of the pupils : tropicamid 0.5% & phenylephrin 2.5% local anaesthesia: oxybuprocaine 0.4% sterile wire lid speculum & muscle hook indirect binocular ophthalmoscopy (IBO) lenses (20, 28, 40 D)

Timing of first eye examination based on gestational age at birth Gestational age at birth Age at initial examination (w) (w) Postmenstrual Chronologic 22 30-31 9 23 30-31 8 24

34 Timing of first eye examination based on gestational age at birth Gestational age at birth Age at initial examination (w) (w) Postmenstrual Chronologic Screening Examination of Premature Infants for Retinopathy of Prematurity, Pediatrics 2006;117:

Arch Ophthalmol 2005,123:991-999 (The International

35 Arch Ophthalmol 2005,123: (The International Classification of Retinopathy of Prematurity Revisited)

36 Type 1 ROP Type 2 ROP Zona I Zona II any Stage + Stage 3 - Stage 2 + Stage 3 + Zona I Zona II ROP1- ROP2- treatment ROP3- observation

37 Laser photocoagulation Dilation of the pupils : tropicamid 0.5% & phenylephrin 2.5% Sterile wire lid speculum & muscle hook Lenses (20, 28, 40 D) Arteficial tears drops Portable laser with an indirect binocular ophthalmoscope NICU or operating theater Under general anaesthesia

38 VEGF Development of retina (vasculogenesis, regulation of permeability) Nervous system Peripheral (neurotrophic) Central (neuroprotection) Lung (pneumotrophic) Nature Medicine 2002;8: Nature Neuroscience 2011;14:

39 Anti-VEGF treatment for ROP: BE CAUTIOUS! Anti-VEGF therapy may be indicated in selected severe cases after careful explanation to parents no alternative available vitreous hemorrhage failure of adequate laser in randomized clinical trials with careful long term follow-up

40 ROP story doesn t end with acute ROP At 5-7 days? Plus disease? Stage 3 Supplementary treatment? (laser/vitrectomy)? Vitreous haemorrhages Refractive errors Myopia (MOP) Astigmatism Amblyopia Strabismus Nystagmus Glaucoma (GOP) Late retinal detachment PVL cerebral visual impairment Important! to follow these children

41 Time in childhood Early diagnosis Early treatment (treatment at time) Vision QoL throughout the life

KPA PFIZER EDUCATION GRANT

KPA PFIZER EDUCATION GRANT What every Paediatrician needs to know in Paediatric Ophthalmology Dr. Njambi Ombaba Paediatricians knowledge in ophthalmology Outline Visual development in a child Amblyopia