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Slit-lamp Biomicroscopy (Handheld slit-lamp in younger children) or Examination
under illumination and magnification
1.Conjunctival congestion, circumciliary congestion
2.Anterior extraocular extension
3.Corneal horizontal diameter and clarity
4..Depth of anterior chamber, presence of tumor hypopyon
5.Iris neovascularization
6.Cataract
Fundus Evaluation
1. Bilateral fundus examination with 360 degree scleral depression
2.Tumor location, size, morphology, vascularity
3. Extent and location of subretinal fluid, subretinal seeds and vitreous seeds
b) Investigations:
Ultrasonography B-scan helps in confirming the clinical diagnosis when in doubt or if
the tumor is not directly visible because of media haze. CT-scan is reserved only to
rule out suspected extraocular extension and MRI to rule out optic nerve or
intracranial extension and pinealoblastoma. Fundus fluorescein angiography is rarely
performed. Bone marrow biopsy is performed to rule out systemic metastasis in
cases with clinical or histopathological risk factors and CSF cytology to rule out
intracranial extension or CNS metastasis.
Management:
A. Intraocular tumor, International Classification Group A to C, Unilateral or
Bilateral
1. Focal therapy (cryotherapy or transpupillary thermotherapy) alone for
smaller tumors (< 3mm diameter and height) located in visually noncrucial
areas
2. Standard 6 cycle chemoreduction and sequential aggressive focal therapy for
larger tumors and those located in visually crucial areas
3. Defer focal therapy until 6 cycles for tumors located in the macular and
juxtapapillary areas. Transpupillary thermotherapy or plaque brachytherapy
for residual tumor >6 cycles.
4. Focal therapy for small residual tumor, and plaque brachytherapy/external
beam radiotherapy (>12 months age) for large residual tumor if bilateral, and
enucleation if unilateral.
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