10/16/2009 Ophthalmic Anomalies in the Pediatric Patient Does my child see? Mar kTk T. Dun bar, ODO.D., FAAOF.A.A.O. Director of Optometric Services Optometric Residency Supervisor Bascom Palmer Eye Institute University of Miami, Miller School of Medicine Miami, Florida Does my child see? Visual Acuity How well does a Relatively poor in the 1st months to yrs neonate/infant of life see? Adult acuity not attained until 1-11/2 How do we yrs determine vision Well below the standard for legal in an infant or blindness neonate? Development Birth History Turn over by 2-3 months Birth Weight Sit-up by 5-6 months Full-term vs Premature Reach for an object by 4 months What kind of delivery Complications Play with objects in hand by 6 months ¾ During pregnancy How well does the baby respond to ¾ During delivery other stimuli (touch, sound)? Hypoxia Is the baby floppy or hypotonic? Bleeding 1 10/16/2009 Family History Visual Acuity Night blindness Does the baby Color vision fixate while eating? High myopia Do the eyes follow the parent’s face? Nystagmus Does the baby Cataracts respond to light? CNS disorders Does the baby reach for objects Good Vision Bad Vision Parents will tell Parents unsure if you the baby sees baby sees well Stares at bright light Will smile at a face Nystagmus Will follow the fact of a parent Hand waving Eye poking Will fixate while eating Disinterested in the environment Failure to smile Visual Acuity Fix and Follow Fixate within days Fixate and Follow of birth Central Steady and Follow by 6 weeks Maintained (CSM) Babies loose target OKN (Optokinetic after 5-10° nystagmus) ¾ Pursuit movement not well established Familiar figures ¾ Watch for micro- ¾ Allen Figures saccadic eye movements 2 10/16/2009 Teller Acuity Cards Electrodiagnostic Testing Preferential Looking ERG Rotation of the Infant OKN “Response to spin” Answers the Assesses the vestibulo-ocular response question “Does Tests the ability to generate a saccadic my child see?” eye movements Motor response used to assess a Slow drift of the eyes in the direction sensory function of the spin Check Fast phase, jerk nystagmus in the monocularly and opposite direction vertical Rotation of the Infant Two observations made: ¾ Does the child develop a nystagmus in response to vestibulo-oculi stimuli ¾ What is the time interval that the baby dampens the nystagmus when swinging stopped 3 10/16/2009 Rotation of the Infant Alignment of EOM’s Sighted child will visually inhibit the Cover test induced nystagmus in 3-5 seconds Hirshberg ¾ Nl05Normal 0.5 mm Blind child cannot visually inhibit the nasal nystagmus and it may continue for 15- Krinsky 20 seconds ¾ Neutralizing the corneal light reflex with prism Pupils Nystagmus Extremely important – Never lie! Rhythmic oscillation of the eyes Dim illumination Sign of poor vision Check size, direct and consensual ¾ Un til proven o therwi se Check for “APD” Will mimic focal neurologic disease Paradoxical pupil ¾ Constriction in dim illumination, dilation in bright illumination Nystagmus Nystagmus Afferent visual Cataracts pathway disease Corneal opacities Congenital (1:10) High Rx errors Focal neurologic Foveal hypoplasia disease (CNS Albinism disorders) Aniridia 4 10/16/2009 Congenital Motor Nystagmus Nystagmus Bilateral macular scar Benign condition (Toxoplasmosis) Present at birth (or shortly after) Leber’s Pendular or jerk CSNB Symmetric ROP ON Hypoplasia Horizontal Achromatopsia Horizontal on up-gaze Dampens on convergence Congenital Motor Paradoxical Pupil Nystagmus Latent component Pupil constricts in darkness Null point Dilates in with bright light Head turn Seen with: Near visual acuity usually better ¾ Leber’s ¾ CSNB ¾ ON Hypoplasia Neuro-Imaging Not Neuro-Imaging Necessary Mandatory Poor vision Poor vision Acquired nystagmus Acquired nystagmus Sluggish pupil Brisk pupil response Normal appearing fundus Normal appearing fundus Paradoxical pupil No paradoxical pupil 5 10/16/2009 Nystagmus Nystagmus Good case history Afferent Visual Pathway Disease Characteristics ¾ 20/200 Vision Variability ¾ If you can superimpose an OKN overtop of their nystagmus, visual prognosis is Symmetry excellent Null point Children can be mainstreamed into Head turn regular schools Latent component 5 Month Old Work Up? Suspected blindness or poor vision Only observation? Nystagmus noted at 6 wks of age Neuro-imaging? Sluggish pupil Electrodiagnostic testing? Cycloplegic retinoscopy + 5.00 No family Hx of nystagmus Normal appearing fundus Your Move Leber’s Congenital 5 Month Old Amaurosis Additional Information Rod cone dystrophy Present at birth or shortly after Photophobia 10-15% of kids in schools for the blind ERG performed Poor vision ¾ Depressed in both photopic and scotopic states Nystagmus or roving eye movements Poor pupil response: Paradoxical pupil Moderate Hyperopia Autosomal recessive 6 10/16/2009 Leber’s Congenital Diagnostic Criteria Amaurosis Fundus Leber’s Congenital Amaurosis May appear normal Diagnosis of exclusion Attenuated vessels Visual dysfunction since birth 10% bilateral macular dystrophy Abnormal ERG 10% peripheral RPE changes Nystagmus or roving eye movement Optic atrophy Moderate – high hyperopia Extinguished ERG Electrodiagnostics 6 Month Old Female When to do in children: No fix or follow Nystagmus or poor vision from birth No nystagmus ¾ Not due to obvious afferent visual pathway Brisk pupils – No afferent pupil defect conditions No paradoxical pupil Overt, but nondiagnostic macular lesion Absent OKN Generalized retinal degeneration Normal fundus exam present or suspected What are we missing? Decreased VA of unknown cause 6 Month Old Female Cortical Blindness Case History Loss of vision stemming from injury to the geniculostriate pathway Full term pregnancy Hypoxic insult to the posterior Cardiac surgery at 4 months pathway, occlusion of the post Cardiac arrest cerebral arteries Cannot see upon awakening 7 10/16/2009 Cortical Blindness Cortical Blindness Generalized hypotension Positive history Cardiac surgery No visual response Birth aphyxia No Nystagmus Hypotensive crisis Absent OKN Hydrocephalus Intact pupil response Metabolic derangements No paradoxical pupil Normal fundus exam Cortical Blindness Cortical Blindness CNS Defects Radiologic findings Mental retardation Diffuse atrophy of the occipital cortex Cerebral palsy Bi-occipital lobe infarction Seizure disorder Periventricular leukomalacia Hydrocephaly or microcephaly Parieto-occipital “watershed” infarction Cortical Blindness Cortical Blindness Prognosis Work-up Transient or permanent MRI Complete restoration of VA rare CT 50% may show significant improvement ERG Recovery is slow – months to years NLP -> LP -> Color -> Form perception-> Pediatric neurological work-up All will recover some vision No tests accurate in prediction recovery 8 10/16/2009 The Wet Watery Eye The Wet Watery Eye Nasal lacrimal duct Discharge obstruction Injection or redness (NLDO) Swe lling Viral conjunctivitis Corneal involvement Herpetic keratitis Does the eye feel hot Congenital Systemic involvement developmental anomalies Nasal Lacrimal Duct Obstruction (NLDO) Blockage at the lower end of the nasal lacrimal duct 6% of babies Failure of canalization of the epithelial cells ¾ At the valve of Hasner Chronic epiphora, mucopurulent discharge NLDO 80% spontaneously open by 6 months Probing after 6 months Advise parents to massage lacrimal sac ¾ Massage up to express mucous, then down to increase hydrostatic pressure May need antibiotic for 2° infection ¾ Zymar, Polytrim 9 10/16/2009 Blepharoconjunctivitis in Children Lorena 8 ½ yo Female Rosacea: “Acne Rosacea” 3 yr history of chronic “blepharitis” Last Dr rxed Bacitracin, Polytrim X 3 wks A chronic acneiform skin disorder affecting cheeks, chin, nose, forhead, Had been on Cefaclor, Max ung, Ocuflox and eye Medical Hx unremarkable, twin Etiology: Poorly understood ¾ Sister did not have the same eye sx VA: 20/15 OU Ant Seg: L > R ¾ Mild PEE RE and SEI RE – Photos LE Dermatologic Findings Ocular Rosacea Findings Meibomian gland Dz Axial facial erythema/hypere ¾ Foamy tears mia Recurrent chalazia Chronic blepharitis Telangiectasis ¾ Staph blepharo- conjunctivitis Papules ¾ Lid margin telangiectasia Pustules Papillae, follicules Sebaceous gland Hyperemia hypertrophy Rhinophyma 10 10/16/2009 Ocular Rosacea Findings Rosacea Keratitis Represents more significant clinical Corneal problem vascularization Cutaneous rosacea: Sterile corneal ¾ 5-30% cornea l invo lvemen t infiltrates Ocular rosacea: Corneal ulceration ¾ 75-85% corneal involvement Corneal perforation Inferior cornea usual site Episcleritis Characteristic “spade-shaped” infiltrates Scleritis Iritis Ocular Rosacea in Ocular Rosacea in Children Children Erzurum SA, Feder RS, Greenwald MJ Rybojad BE, Deplus S, Morel P. Ann Arch of Ophthal 1993 Dermatol Venereol 1996 ¾ 3 Cases of Rosacea keratitis between 10- 10 yo girl with red painful eye X 6mo 12 yo ¾ Dxed with episcleritis ¾ Characteristic dermatologic findings Erythematous papular and pustular ¾ All had ocular Sx > 6 mo duration eruption mid face X 1 mo ¾ 2 bilateral, 1 unilateral Txed with oral antibiotics and ¾ Tx oral TCN and/or Doxycycline erythromycin Patient Characteristics N = 20 Bilaterality 74% (usually asymmetric) Evaluation and treatment of Mean age of onset 6.3 yrs (range: 6 mos-17