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    Morning DiscussionMorning Discussion

    LeukokoriaLeukokoria

    Naila Izzati

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    - Leukokoria (white pupil) can be defined byabnormal pupilary light reflex that usuallyresulted from intraocular abnormality.

    - Itis more commonly seenin children. One-half ofthe cases is caused byRetinoblastoma. Therefore routine screeningshould be done in children < 2 years old.

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    RETINOBLASTOMARETINOBLASTOMA

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    y Primary eye tumor that commonly affectedchildren.

    y Itis a rapidly developing cancer whichdevelops inthe cells of retina.

    y Inthe developed world, Rb has one ofthe

    best cure rates ofall childhood cancers (95-98%), with more thannine out of every tensufferers survivinginto adulthood.Retinoblastomais a very treatable cancer.

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    y Retinoblastomais caused by a mutationin

    agene controlling cell division, causingcells to grow out of controland becomecancerous.

    y In most ofthe cases the mutationispresentin several family members.

    y The cancer generally affects children

    under the age of 6. Itis most commonlydiagnosed in childrenaged 1 - 2 years.

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    Endophytic growthy E

    ndophytic growth occurs whenthe tumorbreaks throughthe internallimitingmembrane and has an ophthalmicappearance ofawhite-to-cream massshowing either no surface vessels or small

    irregular tumor vessels

    Exophytic growthy Exophytic growth occurs inthe subretinal

    space. This growth patternis oftenassociated with subretinal fluidaccumulationand retinal detachment.

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    SymptomsSymptoms

    y One or both eyes may be affected.They pupil may appear white or have white spots.

    y Other symptoms caninclude:y Crossed eyesy Double visiony Eyes that do notalign

    y Eye painand rednessy Poor visiony Differing iris colors in each eyey Ifthe cancer has spread, bone painand other

    symptoms may occur.

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    Strabismus+leukokoriaStrabismus+leukokoria

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    funduskopyfunduskopy

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    TreatmentT

    reatment

    y Treatment options depend uponthe size and locationofthe tumor.

    y Smalltumors may be treated by laser surgery orcryotherapy.

    y Radiationis used for bothlocaltumor and for largertumors.

    y Chemotherapy may be needed ifthe tumor hasspread beyond the eye.

    y The eye may need to be removed (a procedure calledeucleation)ifthe tumor does not respond to other

    treatments. In some cases, it may be the firsttreatment.

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    PrognosisPrognosis

    y Ifthe cancer has not spread beyond the

    eye, almostall patients can be cured. Acure, however, may require aggressivetreatmentand even removal ofthe eye inorder to be successful.

    y Ifthe cancer has spread beyond the eye,the likelihood ofa cure is lower anddepends onhowthe tumor has spread.

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    CONGENITALCONGENITAL

    CATARACTCATARACT

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    y A congenital cataract is clouding ofthelens ofthe eye, thatis presentat birth.

    y The number of people bornwith cataractsis low. In most patients, no specific cause

    can be found.P

    ossible causes ofcongenital cataracts include the following:

    y Chondrodysplasia syndrome

    y Congenital rubella

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    SymptomsSymptoms

    y Cloudiness ofthe lens thatlooks like awhitespotinan otherwise normally dark pupil --

    often obvious at birthwithout special viewingequipment

    y Failure ofaninfantto show visualawareness

    ofthe world around him or her (if cataractspresentin both eyes)

    y Nystagmus (unusual rapid eye movements)

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    TreatmentTreatment

    y In some cases, congenital cataracts are mild anddo notaffect vision, and these cases require no

    treatment.

    y Moderate to severe cataracts thataffect visionwill require cataract removal surgery, followed byplacement ofanartificialintraocular lens (IOL).Patchingto force the child to use the weaker eye

    may be required to preventamblyopia.

    y Treatment for any underlying disorder may alsobe needed.

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    RETINAL DYSPLASIARETINAL DYSPLASIA

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    y Retinal dysplasiais a retinal malformationwhichis characterized by Rosette formation,

    retinal foldingand retinal detachment

    y Often diagnosed in children below 1 y.opresentingwith congenital pink or white

    retrolental membrane ina microcephalic eye,witha shallowanterior chamber andelongated ciliary processes.

    y Bilateralinvolvement may be associated with

    Norrie disease, Patau and Edwardsyndromes.

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    OCULAROCULAR

    TOXOCARIASISTOXOCARIASIS

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    y Toxocariasis is caused by infestationwithToxocara canis, a commonintestinal

    roundworm of dogs and cats.

    y Ingestion of soil or food contaminated withova shed inthe faeces

    y Affectliver, lungs, skin, brainand eyes->OLM

    y Death ofthe larvainitiate inflmmatoryreaction -> usually unilateral

    y Treatment for OLM:Peri-ocular or systemiccorticosteroid injection,Vitrektomy.

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    y Ocular larva migrans Clinical manifestation:x Reduce visionx Leukocoria

    x Strabismus

    x Paininthe eye

    x Endophtalmitis

    x Uveitis

    x Vitreus abses

    x Optic neuritis

    Rarely found

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    RETINOPATY OFRETINOPATY OF

    PREMATURITYPREMATURITY

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    y Retinopati proliferatif

    y Preterm baby terpapar oksigen jenuh

    konsentrasi tinggiy History of prematur, berat lahir rendah

    y Retina : tidak ada pembuluh darah sampai usia gestasi 4 bulan

    Pembuluh darah berkembang dari diskus optikus ke

    perifer Mencapai perifer bagian nasal usia gestasi 8 bulan

    Mencapai perifer bagian temporal 1 bulan setelahkelahiran

    Vaskularisasi retina bagian temporal yangtidak komplit rentan terhadap kerusakan yang disebabkan olehoksigen

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    Screening : Born < 36 weeks Oxygen exposure

    Body weight < 1500gram

    Treatmenty Ablasio retinaimatur avaskuler

    Crioterapy

    Laser photocoagulationy Scleral Buckling

    With or without vitrektomi pars plana For traction retinalablation Improvementin visionnot quite statisfactory

    y Vit E

    Protect spindle cell from free radical

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    PHPV (persistentPHPV (persistenthyperplastichyperplastic primaryprimary

    vitreous)vitreous)

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    y Kegagalan regresi vitreus primery Sangat menyerupai retinoblastomay

    Untuk membedakannya dilakukan CT scan: tidak terdapat kalsifikasi dan terdapathipoplastik bola mata pada mata yangterkena

    y Hasil CT scan : infiltrasi jaringan lunak pada badan kaca

    jaringan lunak retrolental ketiadaan kalsifikasi abnormal dalam orbita Microphthalmus retrohyaloid layered blood hipervaskularisasi badan kaca.

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    Tipe :

    y PHPV anterior Mikroftalmus 90% unilateral Retrolental mass, elongated cilliary process melibatkan kapsul posterior katarak

    Tatalaksana :x Pengangkatan retrolental mass, cataractous lens

    y PHPV posterior Jarang white dense opaque membrane, prominent non-

    opaque retinal fold keluar dari diskus optikus keretina perifer Terbanyak : inferior

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    THANK YOUTHANK YOU

    Refferences

    y Fredrick DR. Special subjects of pediatric interest. In: Vaughan D,

    Asbury T, Riordan-EvaP, editors. General ophthalmology. 15th ed.Stanford: Prentice HallInternational. 1999. pp.336-8.y Alex V. Levin MD, MHSc, FRCSC Department of Ophthalmology

    The Hospital for Sick Children University of Torontoy Jack J. Kanski, Clinical Ophthalmology: A systematic approach,

    5th Edition.y Smirniotopoulos JG, Bargallo N, Mafee MF. Differential diagnosis

    ofleukokoria: radiologic-pathologic correlation. RadioGraphics1994; 14(9): 1059-79.y Harper RA, Shock JP. Lens. In: Riordan-EvaP, Whitcher JP,

    editors. Vaughan & Asburys General Ophthalmology. 2004.Boston: McGrawHill. p:174-7