Pediatric Ophthalmology Dr.jusuf

Pediatric Pediatric OphthalmologyOphthalmology

Dr Jusuf Wijaya , SpMDr Jusuf Wijaya , SpM

FK - UKIFK - UKI

CawangCawang

Curriculum VitaeCurriculum Vitae 1994 : 1994 : Dokter UmumDokter Umum , Vrije Universiteit , Vrije Universiteit

Brussel Brussel (Belgia)(Belgia) 1999 : 1999 : Dokter Spesialis MataDokter Spesialis Mata , Vrije , Vrije

Universiteit Universiteit Brussel (Belgia)Brussel (Belgia) 2001 : 2001 : Fellow Ilmu BedahFellow Ilmu Bedah , Foundation Eye , Foundation Eye

Care Care Himalaya (Belanda – Nepal)Himalaya (Belanda – Nepal) 2002 : 2002 : Fellow di bidang GlaukomaFellow di bidang Glaukoma , ,

Rotterdam Rotterdam Eye Hospital (Belanda)Eye Hospital (Belanda) 2004 : 2004 : Adaptasi (penyesuaian)Adaptasi (penyesuaian) , Universitas , Universitas

Sam Sam Ratulangi (Manado)Ratulangi (Manado)

Pediatric OphthalmologyPediatric Ophthalmology

I .I . Retinopathy of Prematurity (ROP)Retinopathy of Prematurity (ROP)

II.II. RetinoblastomaRetinoblastoma

Retinopathy of Retinopathy of PrematurityPrematurity




Retinopathy of prematurity (ROP) is a Retinopathy of prematurity (ROP) is a disease that affects disease that affects immature vasculature immature vasculature in the eyes of premature babiesin the eyes of premature babies..

It can be mild with no visual defects, or it It can be mild with no visual defects, or it may become aggressive with new blood may become aggressive with new blood vessel formation (neovascularization) and vessel formation (neovascularization) and progress to progress to retinal detachmentretinal detachment and and blindnessblindness..

As smaller and younger babies are As smaller and younger babies are surviving, the incidence of ROP has surviving, the incidence of ROP has increased.increased.


It was first suggested that ROP was It was first suggested that ROP was related to the introduction of oxygen related to the introduction of oxygen therapy into the newborn nurserytherapy into the newborn nursery

Today, after oxygen therapy has Today, after oxygen therapy has been studied and found not to be the been studied and found not to be the single causative agent, the factors single causative agent, the factors that play a role in the pathogenesis that play a role in the pathogenesis of ROP are still unknown. of ROP are still unknown.


In a normally developing retina, blood vessels In a normally developing retina, blood vessels emanating from the optic disk grow toward the emanating from the optic disk grow toward the peripheral retina during the fourth month of peripheral retina during the fourth month of gestation.gestation.

Typically, the nasal retina is completely vascularized Typically, the nasal retina is completely vascularized by 36 weeks’ gestation, and the temporal retina is by 36 weeks’ gestation, and the temporal retina is vascularized by 40 weeks’ gestation.vascularized by 40 weeks’ gestation.

Retinopathy of prematurity (ROP), a condition that Retinopathy of prematurity (ROP), a condition that develops mainly in premature infants, occurs when develops mainly in premature infants, occurs when normal retinal angiogenesis is not complete at the normal retinal angiogenesis is not complete at the time of birth.time of birth.

Histologically, it is characterized byHistologically, it is characterized by replacement of replacement of the neurosensory retina by fibrous tissue and the neurosensory retina by fibrous tissue and immature blood vesselsimmature blood vessels. .


Risk FactorsRisk Factors : :

-- Prematurity Prematurity < 32 weeks< 32 weeks

-- Low birth weight Low birth weight < 1500 g< 1500 g

-- Supplemental Supplemental oxygen therapyoxygen therapy

- - Others Others : - Intraventricular : - Intraventricular hemorrhagehemorrhage

- Abruptio placentae- Abruptio placentae

Stages of ROPStages of ROP

Stage 1 Demarcation lineDemarcation line: a flat, white, thin line that visibly : a flat, white, thin line that visibly separates the avascular retina anteriorly (toward the retinal separates the avascular retina anteriorly (toward the retinal periphery) from the vascularized retina posteriorly. periphery) from the vascularized retina posteriorly.

Stage2 Ridged demarcation lineRidged demarcation line: the flat line from stage 1 has : the flat line from stage 1 has grown in height, width, and volume and has become a pink-grown in height, width, and volume and has become a pink-white ridge. white ridge.

Stage 3 EExtraretinal fibrovascular proliferationxtraretinal fibrovascular proliferation: proliferating : proliferating tissue can be continuous with the posterior aspect of the ridge; tissue can be continuous with the posterior aspect of the ridge; immediately posterior to the ridge, but not apparently connected immediately posterior to the ridge, but not apparently connected with it; or extending directly into the vitreous. with it; or extending directly into the vitreous.

Stage 4 Subtotal retinal detachmentSubtotal retinal detachment: dragging vessels and : dragging vessels and subtotal traction retinal detachment can be seen.subtotal traction retinal detachment can be seen.

Stage 5 Total retinal detachmentTotal retinal detachment: funnel-shaped retinal : funnel-shaped retinal detachment. Anterior and posterior portions appear open or detachment. Anterior and posterior portions appear open or narrowed on ultrasonographic scans. narrowed on ultrasonographic scans.

Plus disease The designation “+” is placed after the stage The designation “+” is placed after the stage when dilated posterior veins, tortuous retinal arteries, vitreous when dilated posterior veins, tortuous retinal arteries, vitreous haze, and pupillary rigidity are observed. If plus disease is haze, and pupillary rigidity are observed. If plus disease is observed in the posterior portion of the retina, patients must be observed in the posterior portion of the retina, patients must be monitored closely, as there is high risk of ROP progressing monitored closely, as there is high risk of ROP progressing rapidly to stage 5 within a few days.4rapidly to stage 5 within a few days.4

Prognosis of ROPPrognosis of ROP

Almost all (90%) of stage 1 and 2 ROP Almost all (90%) of stage 1 and 2 ROP regresses spontaneously, and approximately regresses spontaneously, and approximately 50% of stage 3 disease regresses 50% of stage 3 disease regresses spontaneously.spontaneously.

If the condition progresses substantially, If the condition progresses substantially, however, patients can be treated with either however, patients can be treated with either laser laser photocoagulationphotocoagulation or or cryotherapycryotherapy (transconjunctival application of a cold probe (transconjunctival application of a cold probe to the eye surface) to reduce growth of to the eye surface) to reduce growth of abnormal blood vessels in an attempt to abnormal blood vessels in an attempt to inhibit disease progressioninhibit disease progression

RetinoblastomaRetinoblastoma



RetinoblastomaRetinoblastoma The most common primary malignant The most common primary malignant

intraocular tumor of childhoodintraocular tumor of childhood & the & the second most common primary ocular second most common primary ocular malignancy of all age groups (choroidal malignancy of all age groups (choroidal melanoma is more common)melanoma is more common)

1 in 20 000 live births1 in 20 000 live births No sexual predilictionNo sexual prediliction Unilateral or bilateralUnilateral or bilateral Average age at diagnosis is 18 months & Average age at diagnosis is 18 months &

the vast majority become clinically the vast majority become clinically apparent prior to the age of 3 yearsapparent prior to the age of 3 years

Signs of retinoblastomaSigns of retinoblastoma

- LeukocoriaLeukocoria or “ or “amaurotic cat’s eyeamaurotic cat’s eye” : ” : A white dot filling the pupil in flash A white dot filling the pupil in flash photography or a white spot in the photography or a white spot in the pupil in ordinary illuminationpupil in ordinary illumination

- StrabismusStrabismus

- - HeterochromiaHeterochromia : One eye being a : One eye being a different color than the other different color than the other

- - Red, painful eyesRed, painful eyes

- - Poor visionPoor vision


InheritanceInheritance

- - Familial casesFamilial cases

Only in 6 % of cases (Autosomal Only in 6 % of cases (Autosomal dominant dominant but with high but but with high but incomplete penetrance)incomplete penetrance)

RB geneRB gene ( (RB1RB1) maps to chromosome ) maps to chromosome 13 q 1413 q 14

- - Sporadic casesSporadic cases

Account for the remaining 94 %Account for the remaining 94 %

Pathology of Pathology of RetinoblastomaRetinoblastoma

Originates from neuroretina (primitive cone Originates from neuroretina (primitive cone cells)cells)

2 main types of growth:2 main types of growth:

- - Endophytic tumorEndophytic tumor (projects into vitreous (projects into vitreous cavity)cavity)

- - Exophytic tumorExophytic tumor (grows into subretinal (grows into subretinal space)space)

Homer Wright rosettes, Flexner Wintersteiner Homer Wright rosettes, Flexner Wintersteiner resettes and fleurettesresettes and fleurettes

Comparison of dysplastic rosettes to the neoplastic Comparison of dysplastic rosettes to the neoplastic rosette of retinoblastomarosette of retinoblastoma. . aa) ) Rosette from area of Rosette from area of dysplastic retina in the present case.dysplastic retina in the present case. b b) ) Flexner-Flexner-Wintersteiner rosetteWintersteiner rosette from a case of retinoblastoma. from a case of retinoblastoma. Short arrows, external limiting membrane; long arrow in Short arrows, external limiting membrane; long arrow in A, possible cone nuclei; asterisk, possible rod nucleus.A, possible cone nuclei; asterisk, possible rod nucleus.

Aims of management of Aims of management of RetinoblastomaRetinoblastoma

11stst goal is to save life goal is to save life

22ndnd goal is to save eye goal is to save eye

33rdrd goal is to maximise vision goal is to maximise vision

Treatment MethodsTreatment Methods

- EnucleationEnucleation- External beam radiotherapyExternal beam radiotherapy- Chemotherapy Chemotherapy (eg. Chemoreduction, (eg. Chemoreduction,

systemic systemic chemotherapy, chemotherapy, subconjunctival subconjunctival chemoreduction, chemoreduction, intrathecal cytosine intrathecal cytosine arabinoside)arabinoside)

- Focal therapyFocal therapy (eg. Laser, cryotherapy, (eg. Laser, cryotherapy, radioactive plaque, radioactive plaque,

thermotherapy)thermotherapy)- Orbital exenterationOrbital exenteration

PrognosisPrognosis

Prognosis is dependant on :Prognosis is dependant on :- - LocationLocation

95% 5 year survival if intraocular tumor95% 5 year survival if intraocular tumor5% 5 year survival with extraocular 5% 5 year survival with extraocular

extensionextension- - Tumor size & gradeTumor size & grade- - Iris rubeosisIris rubeosis- - Bilateral tumorsBilateral tumors (risk of second malignancy) (risk of second malignancy)- - Age of patientAge of patient (older worse) (older worse)



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Pediatric Ophthalmology Dr.jusuf