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History
History
General examination
Vitals : PR 85/min, RR 16/min
Oral Hygiene N
Systemic examination:
Abdomen - No organomegaly, no ascites
CVS NAD,
Respiratory system - NAD
Mild nasal discharge +
Ocular Examination
R
Visual
acuity
Eye balls
RDS + , no
nystagmus
Normal
Movement N
s
Palpebral
aperture
Slight prominence N
of eye ball
Ocular examination
Eye lids
NAD
NAD
Cornea
NAD
NAD
Sclera
NAD
NAD
A.C
No cells,flare
No cells; flare
Iris
Brown,no
nodules
Brown,no
nodules
Pupil
3mm,round,cent 3mm,round,cent
ral,nosyn,C+,D+ ral,C+D+
Lens
Clear
Clear
I.O.P
DTN
DTN
Fundus
Whitish
reflex,total
Rd,a mass
lesion seen
Red glow,C:d.
.3:1,NRRN,superiorly
located mass
seen
Investigations
Hb 13.5gm%
Urine protein, sugar nil
USG R - Soft tissue intraocular mass
height 7.6mm, base 10.6mm with total
exudative RD. The mass shows presence of
calcification
USG L Superiorly located intraocular
mass with calcification. Height 5mm, base
6.3mm. No RD+
Investigations
CT scan R-shows soft tissue density filling
posterior segment with multiple
calcifications, optic nerve extension absent
N brain parenchyma
CT scan L a small soft tissue posterior
chamber mass present superiorly ,with
presence of calcification . Mass appear
localised, no involvement of optic nerve or
CNS seen
Differential Diagnosis
RETINOBLASTOMA
PHPV
ROP
COATS DISEASE
COLOBOMA OF CHOROID /DISC
CONGENITAL CATARACT
TOXOCARA GRANULOMA
RETINAL DYSPLASIA
Clinical Significance
Retinoblastoma (RB) is most common
intraocular malignancy in children
Untreated it is uniformly fatal
Confused and misdiagnosed with other
conditions simulating it
It is the only cause which can prove fatal
Any child presenting with leucocoria should
be considered as RB unless proved
otherwise
Retinoblastoma
Incidence 1:18000- 1:20,000
No sex predeliction
Peak age 18 months
Familial tumours present earlier
Inheritance 60% non hereditary{85% U/L}
40%germline mutation
Inheritance
Unaffected parent with 1 affected child 5%
risk
With 2 or more affected child 50% risk
Survivor of hereditary RB has 50% risk of
having a child with RB.
Pathogenesis
Classification
RPC - IRCH
1. Or more lesion <4D.D,<4m.m height
1 or more lesion 4-6D.D,4-8m.mheight
1 or more lesion >6D.D > 8m.m height
Extraocular extension to orbit
Distant metastasis
Investigations
History
Family History
Clinical examination[ EUA]
USG - Estimate presence, dimension,
associated RD
CT- confirm presence, detect calcification,
estimate dimension, invasion of orbit, gross
involvement of optic nerve, CNS extension
Investigations
MRI is not routinely required ,only for
better delineation of optic nerve involvment
and cns soft tissue involvment
MRI useful in differentiating Rb. From
simulating lesions
Metastatic workup not routinely required
unless signs of spread present
Metastatic workup includes CSF , bone
marrow examination,LFT ,bone scans
Treatment
Enucleation
Indication ->1/2 globe involved, ant.
Segment involvment, total R.D , absence of
useful vision, glaucoma
Removal of entire eyeball intact along with
a long stump of optic nerve
Meticulous surgical technique
Enucleation
Enucleation
CHEMOTHERAPY
Indications
Focal lesions involving macula and optic nerve
Along with cryo or laser if lesion not totally
regressed or presence of significant vitreous
seedings
Metastatic disease,extra ocular spread
High risk cases with more chance of metastasis
Neoadjuvant chemotherapy for advanced
stages as primary modality
CHEMOTHERAPY
Regimens
4 week regimen :
Week 0 Carboplatin 560mg/m2, Etoposide
200mg/m2 I.V.,
Week 3 Vincristine1.5 mg/m2,
cyclophosphamide150mg/m2 oral for 7 days
VAC regimen :
Vincristine 1.5mg/m2, Cyclophosphamide
150mg/m2; Adriamycin25 mg/m2 all given on day
1 repeated every 3 weeks
CHEMOTHERAPY
Side effects
Cyclophosphamide bone marrow toxicity,
cystitis, case report of bladder carcinoma
Carboplatin nausea , vomiting ,renal
toxicity
Vincristine- vesicant,neurologic,marrow,
Adriamycin- cardiac toxicity
PHOTOCOAGULATION
Direct treatment of tumour with
argon/diode/laser
Destroy blood vessels supplying tumour
Small size, posteriorly located tumours
250-300 mw ,400-600msec
CRYOTHERAPY
Lesion anterior to equator,confined to
sensory retina, <2.5mm diameter, <1mm
thickness
3 freeze thaw cycles
Side effects : retinal hole , R.D, SRF,
Vitreous hemorrhage
NEW MODALITIES
GENE replacement therapy
Phase 1 clinical trial of gene therapy
altered adeno virus with thymidine kinase
with ganciclovir injected in tumour cells ;
ONYX_O15
Discovery of vitamin D receptors in Rb.has
lead to use of 1 alpha hydroxy D2 and other
analogues ,increase p53 expression
[2002]Wisconsin ,USA