Page 139 - NAME OF CONDITION: REFRACTIVE ERRORS
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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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