08013a9d-dc65-474f-bcf9-3d3f603d4231.pptx

It's is a disorder of eye i.e ratinoblastoma

1. Md, Azizul Islam

2. Presentation Lay Out

> We should able the workup of RB patient proceed
> Statistics of Retinoblastoma

> Clinical Evaluation of RB

> Signs & Symptoms of Retinoblastoma

> The differential diagnosis of white pupil

> Retinoblastoma classified

> Treatment regimen of RB

3. Healthy eye Retinoblastoma
Per Alien =

Foves
| central

4. Statistics of Retinoblastoma

The most important thing to learn from these statistics is that bilateral
disease is genetic, meaning that each off spring has a 50% chance of having
the disease. Unilateral retinoblastoma is most likely a somatic mutation
occurring in the eye and is not hereditary. However it must be understood
that 15% of unilateral cases can be hereditary.

> 85% sporadic

> 15% familial

> 60% unilateral

> 40% bilateral

> 85% of unilateral have somatic mutations, 15% have germinal
> 90% of bilateral have germinal mutations, 10% somatic

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6. Momalratinal —— Petimalcollal Aatimblastona
cell fit

7. Types Of RB/Growth Patterns

Endophytic:

» Tumor grows into vitreous cavity

+ yellow white mass

> Progressively fills vitreous cavity & vitreous seeds occur
> Retinal vessels not seen on tumor surface.

Exophytic :

+ Tumor grows towards sclera

> Solid R.D usually occurs

> Retinal vessels seen on tumor surface

Diffuse infiltrating tumor:
+ Tumor diffusely involves retina causing placoid thickness

9. Differential Diagnosis of RB

The several conditions listed below can mimic different presentations of
retinoblastoma.

>» Coats Disease

» Persistent Fetal Vasculature (PFV or PHPV)
» Toxocariasis and other infections

+ Medulloblastoma

r Stage 5 Retinopathy of Prematurity

r Cataract

> Astrocytic hamartoma (Tuberous sclerosis)
> Pars planitis / endophthalmitis

» Convergent or divergent strabismus

> Pseudohypopyon

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10. Differential Diagnosis of RB

> Coats Disease: 90% unilateral, 6:9 8:1,
Congenital peripheral retinal telangectasias that
develop exudative retinal detachments.

> Persistent Fetal Vasculature: i
Persistent hyperplastic primary vitreous or failure of the fetal
vasculature.

> Astrocytic Hamartoma:

Systemic associations, Idiopathic

Tuberous sclerosis: Adenoma sebaceum, ash leaf spots,
ceizurec Naurmfihromatneie 1

11. Differential Diagnosis of RB

> Toxocarisis: Toxocarisis due to a round worm larva
is contracted from exposure to a dog, usually a house pet.

> Medulloepithelioma: Congenital tumor of Non Pigmented
Ciliary Epithelium.

can affect CB, retina, ON (intraocular and orbit)

First decade of life, but can present in adults

> Retinopathy of prematurity. is associated with low-birth-
weight infants who receive supplemental oxygen

in the period immediately after birth, and it involves damage to

the retinal tissue and may lead to retinal detachment.

;

12. Murphree Classification of RB

+ Group A’ Small tumors away from foveola and small peripheral lesions.
> — Tumors <3 mm in greatest dimension confined to the retina and Located
at least 3 mm from the foveola and 1.5 mm from the optic disc.
«+ Group 8: All remaining tumors confined to the retina.
+ Allother tumors confined to the retina and not in group A
> Subretinal fluid (without subretinal seeding) < 3 mm from the base of the tumor.

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Group C: Local subretinal fluid or vitreous seeding.
Subretinal fluid alone >3 mm and < 6 mm from the tumor.
Vitreous or subretinal seeding < 3 mm from the tumor.

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<=

Group D: Diffuse subretinal fluid or seeding
Subretinal fluid > 6 mm from the tumor.
Vitreous or subretinal seeding > 3 mm from the tumor.

Group E: presence of any one or more of these poor prognosis features.
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13. International Classification of RB

An international classification deals more with survivability.

+ Stage: (0) «Patients treated conservatively (subject to pre-surgical ophthalmologic
classifications)

* Stage: (I) Eye enucleated, completely resected histologically
+ Stage: (Il) :- Eye enucleated, microscopic residual tumor in optic nerve.

+ Stage: (Ill) ‘Regional extension
> — A Overt Orbital Disease
» B. Preauricular or cervical lymph node extension

+ Stage: (IV) :- Metastatic disease

> À Hematogenous metastasis
+ DN | et

14. Examination

Anything causing a white pupil reflex as the only sign suggesting the presence of
retinoblastoma. parental concern about a white pupil deserves a retinal examination with the
indirect ophthalmoscope.

Work-up of patient with retinoblastoma starts with a history, eye exam (including complete
retina exam with dilated pupil using indirect ophthalmoscope) and then:

> — Fluorescein angiography

> — Ultrasonography ( B-Scan)

» — Neuroimaging: CT and MRI

> Thorough general physical examination
Followed by (if indicated):

> — DNA testing in peripheral and in tumor cells
> Spinal tap

+ Bone marrow aspirate/bionsy

15. Diagnosis of Retinoblastoma

> The red reflex: checking for a normal reddish-
orange reflection from the eye's retina with an | =
ophthalmoscope or retinoscope from about 30cm LY à
or 1 foot, usually done in a dimly lit or dark room. = == e
> In most cases the diagnosis of retinoblastoma is | }
made with the indirect ophthalmoscope and = E
confirmed by finding calcium on the ultra sound or NN
CT scan, or a mass on a MRI. a) Y
> > 90% diagnosed by ophthalmoscopy, ——
ultrasonography and computed tomography, Gt =

16. Treatment of Retinoblastoma

All of these methods can be useful in the treatment of retinoblastoma.
The order depends on the unique findings in each patient.

> External beam irradiation/Radiotherapy
> Systemic chemotherapy

> Periocular chemotherapy

> Radioactive plaque

> Transpupillary thermotherapy

> Photocoagulation

> Cryopexy

> Selective intra-arterial chemotherapy

> Enucleation

17. Treatment of Retinoblastoma

> Small lomours: (<3mm wide x 2mm thick) - Laser
photocoagulation - Transpupillary thermotherapy - Cryotherapy

> Medium Tumours, (lémm wide x 6mm thick) - Brachytherapy -
Primary Chemotherapy (CEV) - External beam radiotherapy

> Large Tumours:- Chemotherapy followed by local treatment -
Enucleation

> Extraocular Extension: - Adjuvant chemotherapy - External beam
radiotherapy » Metastatic disease - Chemotherapy

18. Radiation Therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types

of radiation to kill cancer cells or keep them from growing. There are two types of radiation

therapy:

> External-beam radiation therapy. uses a machine outside the body to send radiation
toward the area of the body with cancer,

> Intensity-modulated raalation therapy. (therapy that uses a computer to make
pictures of the size and shape of the tumor

> Proton-beam radiation therapy: Proton-beam therapy is a type of high-energy,
external radiation therapy.

> Internal radiation therapy: uses a radioactive substance sealed in needles, seeds, wires,
or catheters that are placed directly into or near the cancer.
> Plaque radiotherapy; Radioactive seeds are attached to one side of a disk, called a

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20. ENUCLEATION With Ol

+ Enucleation remains an important therapeutic option for this disease.

> Children who have unilateral advanced intraocular disease.

» Enucleation is sometimes recommended for both eyes in children who have bilateral
far-advanced disease not amenable to any eye-preserving therapy and for the more
severely affected eye in markedly asymmetrical bilateral cases,

> If enucleation is performed, the ophthalmic surgeon should attempt to obtain a
long section of the optic nerve during surgery.

+ Orbital Implant:

> Promotes orbital growth

> Provides better cosmetic

+ Enhances prosthesis motility

> Non integrated (PMMA/ silicon)
> Myoconjunctival technique

21. >

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N References

Kanski's Clinical Ophthalmology,7th Edition by Jack Kanski

Lewis R (March 19, 2013). "Some Aggressive Retinoblastomas Lack RB1 Mutations’. Medscape
Online. Archived from the original on September 19, 2017.

Lohmann DR, Gallie BL (2010). “Retinoblastoma”. GeneReviews. Seattle, WA: University of
Washington. PMID 20301625

How to test for the red reflex in a child, Community Eye Health 2014: 27(86): 36.
Picture + Vedio : Google + Me

22. THANKS
ALL