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Why worry about strabismus?

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Why worry about strabismus?. [1,8]. Initial Presentation. Most (75%) PARENTS detect abnormality. http://shainazhope.com/. Retinoblastoma. Autosomal dominant (RB1) with incomplete penetrance Increased risk other cancers Only 5-10% of cases have family history. Retinoblastoma. Age - PowerPoint PPT Presentation
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Retinoblastoma: Significance to Ambulatory Pediatrics

Why worry about strabismus?2

[1,8]Vitreous Hemorrhage (dark reflex)Hypopyon (layering of WBCs in anterior chamber)3Initial PresentationMost (75%) PARENTS detect abnormality

http://shainazhope.com/4RetinoblastomaAutosomal dominant (RB1) with incomplete penetranceIncreased risk other cancersOnly 5-10% of cases have family history

Up to 500 new cases annually in US

5RetinoblastomaAge90% younger than 5 years at diagnosisAverage of 18 monthsRare cases in adults [1]Case reports document ocular mass in fetal U/S [10]

6Morbidity/ Mortality5 year survival 93%If untreated: Metastatic spread within 6 months50% 1yr survivalWith early detection, among most curable childhood cancerLeukocoria is actually a late findingOcular survival 9% at 5 years

7Red Reflex

http://www.daisyseyecancerfund.org8

Patient: parents lap, calm, look directly at lightExaminer:Lens at zero, ophthalmoscope close to eyeArms length away, view parents eyes firstView simultaneously, then individually (Bruckner)Uncooperative child:View from several feet away, may try otoscope with magnifying lens removed [3,4].

9[9]

10

11Red ReflexScreens all aspects of optical pathwayTear film, cornea, aqueous and vitreous humor, iris, lens, retina.Over 30 Diseases: Cataracts, glaucoma, retinoblastoma, systemic disease [3].

DDx: Over 30 conditions:Cataract, vitreous hemorrhage, retinal detachment, ROP, ocular toxocariasis, toxoplasmosis, JRA, orbital cellulitis, Intraocular tumors or leukemia with ocular involvement, hemangioma, Coats dz [6].

12White Reflex White Pupil

tapetum lucidum"bright tapestry"13White Reflex

14

PCP first noted presenting signs in only 5%Delayed PCP referral 21% (3.75 months)PCP reassured parents of normalcy or made wrong diagnosisFalse reassurance with normal red reflex15

[11]16AAP Policy (Dec 2008)Document Red Reflex:In newborn nurseryALL routine health supervision visitsPrevious Recs: within 2months; 6 months; 1,3,5,10yrsRefer to ophtho:All high risk patientsFam Hx: RB, infantile cataracts, retinal dysplasia, glaucomaBased on parental observation aloneAbnormal red reflex (urgent)Contact ophthalmologist directlyDocument confirmation of proper followup [3]

17Whats your Dx

Retinoblastoma18

PseudostrabismusStrabismus1- Pseudostrabismus2- Strabismus19

www.kidsretina.blogspot.comPediatric retina blog

Persistent Fetal Vasculature20

Neuroblastoma with Raccoon Eyes21

Congenital CataractsCataracts assoc c maternal DM, galactosemia, ROP, genetic including DS, Infxn: rubella, cmv, toxo, HSV, VZV, syphilis/ radiation, steroid use22

Congenital GlaucomaCong Glaucoma: Glaucoma term used to indicate damage to optic nerve with visual field loss caused by elevated pressure within eye. Classic triad tearing, photophobia, blepharospasm ( eyelid squeezing) seen in 30%. Other signs corneal edema, corneal and ocular enlargement, conjunctival injection.Assoc w/ aniridia, port-wine stain, NF, ROP, cong cataracts23

Normal Retina24Take Home PointsScreen in the nursery and at every checkup!Take parental concerns seriously and refer!Do not trust a normal red reflex if concerns are present.Call ophthalmologist directly, especially if abnormal exam.Close followup of high risk patients.25

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