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Dr Jusuf Wijaya , SpM

FK - UKI
Cawang
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þ ë
: Dokter Umum , Vrije Universiteit Brussel
(Belgia)
þ ë : Dokter Spesialis Mata , Vrije Universiteit
Brussel (Belgia)
þ 200ë : Fellow Ilmu Bedah , Foundation Eye Care
Himalaya (Belanda ² Nepal)
þ 2002 : Fellow di bidang Glaukoma , Rotterdam
Eye Hospital (Belanda)
þ 200
: Adaptasi (penyesuaian) , Universitas Sam
Ratulangi (Manado)
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I . Retinopathy of Prematurity (ROP)
II. Retinoblastoma
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þ Retinopathy of prematurity (ROP) is a disease
that affects immature vasculature in the eyes of
babies.
premature babies.
þ It can be mild with no visual defects, or it may
become aggressive with new blood vessel
formation (neovascularization) and progress to
retinal detachment and blindness
blindness..
þ As smaller and younger babies are surviving, the
incidence of ROP has increased.
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þ It was first suggested that ROP was related to
the introduction of oxygen therapy into the
newborn nursery
þ Today, after oxygen therapy has been studied
and found not to be the single causative agent,
the factors that play a role in the pathogenesis of
ROP are still unknown.
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þ In a normally developing retina, blood vessels emanating from
the optic disk grow toward the peripheral retina during the
fourth month of gestation.
þ Typically, the nasal retina is completely vascularized by 36 weeks·
gestation, and the temporal retina is vascularized by
0 weeks·
gestation.
þ Retinopathy of prematurity (ROP), a condition that develops
mainly in premature infants, occurs when normal retinal
angiogenesis is not complete at the time of birth.
þ Histologically, it is characterized by replacement of the
neurosensory retina by fibrous tissue and immature blood
vessels..
vessels
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þ Risk Factors :
- Prematurity < 32 weeks
- Low birth weight < ë00 g
- Supplemental oxygen therapy
- Others : - Intraventricular hemorrhage
- Abruptio placentae
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þ j  Demarcation line:
line: a flat, white, thin line that visibly separates the avascular
retina anteriorly (toward the retinal periphery) from the vascularized retina
posteriorly.
þ j  Ridged demarcation line: line: the flat line from stage ë has grown in height,
width, and volume and has become a pink- pink-white ridge.
þ j  xtraretinal fibrovascular proliferation:
proliferation: proliferating tissue can be
continuous with the posterior aspect of the ridge; immediately posterior to the ridge,
but not apparently connected with it; or extending directly into the vitreous.
þ j  Subtotal retinal detachment:
detachment: dragging vessels and subtotal traction retinal
detachment can be seen.
þ j  Total retinal detachment:
detachment: funnel-
funnel-shaped retinal detachment. Anterior and
posterior portions appear open or narrowed on ultrasonographic scans.
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  The designation ´+µ is placed after the stage when dilated posterior
veins, tortuous retinal arteries, vitreous haze, and pupillary rigidity are observed. If
plus disease is observed in the posterior portion of the retina, patients must be
monitored closely, as there is high risk of ROP progressing rapidly to stage  within a
few days.

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Almost all ( 0%) of stage ë and 2 ROP regresses
spontaneously, and approximately 0% of stage 3
disease regresses spontaneously.
If the condition progresses substantially, however,
patients can be treated with either laser
photocoagulation or cryotherapy (transconjunctival
application of a cold probe to the eye surface) to reduce
growth of abnormal blood vessels in an attempt to
inhibit disease progression
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þ The most common primary malignant intraocular
tumor of childhood & the second most common
primary ocular malignancy of all age groups (choroidal
melanoma is more common)
þ ë in 20 000 live births
þ No sexual prediliction
þ Unilateral or bilateral
þ Average age at diagnosis is ë months & the vast
majority become clinically apparent prior to the age of
3 years
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- Leukocoria or ´amaurotic
´amaurotic cat·s eyeµ
eyeµ : A white
dot filling the pupil in flash photography or a
white spot in the pupil in ordinary illumination
- Strabismus

- Heterochromia : One eye being a different color

than the other
- Red, painful eyes
- Poor vision
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þ Inheritance
- Familial cases
Only in 6 % of cases (Autosomal dominant
but with high but incomplete penetrance)
RB gene (RBë
RBë)) maps to chromosome ë3 q ë

- Sporadic cases
Account for the remaining
%
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Originates from neuroretina (primitive cone cells)

2 main types of growth:

- Endophytic tumor (projects into vitreous cavity)
- Exophytic tumor (grows into subretinal space)

Homer Wright rosettes, Flexner Wintersteiner resettes

and fleurettes
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  . Rosette
   . Rosette from area of dysplastic retina in the
present case. Flexner
Flexner--Wintersteiner rosette from a case of
retinoblastoma. Short arrows, external limiting membrane; long
arrow in A, possible cone nuclei; asterisk, possible rod nucleus.
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ëst goal is to save life
2nd goal is to save eye
3rd goal is to maximise vision
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- Enucleation
- External beam radiotherapy
- Chemotherapy (eg. Chemoreduction, systemic
chemotherapy, subconjunctival
chemoreduction, intrathecal cytosine
arabinoside)
- Focal therapy (eg. Laser, cryotherapy,
radioactive plaque, thermotherapy)
- Orbital exenteration
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Prognosis is dependant on :
- Location
%  year survival if intraocular tumor
%  year survival with extraocular
extension
- Tumor size & grade
- Iris rubeosis
- Bilateral tumors (risk of second malignancy)
- Age of patient (older worse)
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