Retinoblastoma

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WILLS EYE HOSPITAL OCULAR ONCOLOGY SERVICE,840 WALNUT STREET SUITE 1440. PHILADELPHIA PA 19107,TELEPHONE 215 928 3105,FAX 215 928 1140. FOR MORE INFORMATION ON RETINOBLASTOMA VISIT,WWW FIGHTEYECANCER COM. TO READ THE STORIES OF OTHER RETINOBLASTOMA PATIENTS VISIT. WWW EYECANCERHEROES COM, TO MAKE A DONATION TO HELP OTHERS WITH RETINOBLASTOMA VISIT. WWW ETRF ORG,DESIGNED BY TIKA MARIE SIBURT,1 What is Retinoblastoma 4.
2 My Child Has Retinoblastoma 5,A Examination 5,B Diagnosis 5. 3 Procedures Treatment 6,A Chemoreduction 7,B Thermotherapy 7. C Laser Photocoagulation 7,D Cryotherapy 7,E Plaque Radiotherapy 7. F External Beam Radiotherapy 8,G Intra Arterial Chemotherapy 8. H Intravitreal Chemotherapy 9,I Enucleation 9,4 The Statistics 10.
5 Ocular Prosthesis 12,6 Moving Forward 12,A Future Appointments 12. B Eye Protection 13,C You Are Not Alone 13,Retinoblastoma is. t o m a the most common,t i n o b las eye cancer in children. Re It is a life threatening,cancer of the retina within. the back of the eye Retino,blastoma is discovered in babies.
between the ages of 6 and 24 months,although it can be found at earlier or later. ages Retinoblastoma occurs in approximately 1 in 15 000. live births and it is estimated to affect approximately 250 to 300 chil. dren each year in the United States Worldwide about 5 000 children. develop retinoblastoma each year, Retinoblastoma occurs equally in boys and girls and it is seen in all. races Retinoblastoma affects only one eye in about 70 of patients. and it affects both eyes in 30 of patients In some instances there. is a family history of retinoblastoma in a parent or relative but in the. majority of cases there is no other member of the family affected. with the tumor, The cause of retinoblastoma is unknown It has not been related to. nutrition smoking drinking or any maternal problem during preg. nancy It is not related to environmental toxins It can develop in an. otherwise healthy child despite a normal birth and early development. Retinoblastoma develops from a single affected cell in the retina. and it is invisible at its inception Later a small white nodule is noted. in the retina but this is not visible to the,parents and it does not affect the child. at this point With time the tumor grows,into a larger white vascular tumor caus.
ing the child to lose vision painlessly,This can manifest in a drifting eye or an. eye with a white or glassy appearing,pupil At this point the tumor is visible. White tumor called Retinoblastoma,to the parents or grandparents and the. child is typically taken to the doctor Once a child is suspected of. C hi l d having retinoblas,M y t o m a toma a complete. i n o b las eye and systemic,examination is required.
The ophthalmologist who,suspects retinoblastoma typ. ically refers the child relatively,urgently within one or two days or. weeks to a retinoblastoma expert The reti, noblastoma specialist evaluates the child s medical. and family history examines parents and family members and. then specifically focuses on the child s eye examination The eyes. are checked with the pupils dilated The doctor wears a headlight. called an indirect ophthalmoscope and she he uses a focusing lens. to see all of the details in the back of the eye It is generally necessary. to restrain the child to steady the head and body so that a reliable. examination can be performed Numbing eye drops are placed on. the child s eyes for comfort The child s eyelids are held open by a. lid speculum and soft cotton applicators are used to rotate the eyes. gently to allow maximal visualization of the back of the eye This is. generally not painful to the child but the child might cry. The diagnosis of retinoblastoma depends primarily on findings seen. on eye examination On examination retinoblastoma has a classic. chalky white appearance with prominent blood vessels It is rarely. necessary to biopsy the tumor for confirmation,To better assess the tumor an exam. ination with sedation of the child in the,operating room called an examina.
tion under anesthesia or EUA is usually,necessary During the EUA a complete. eye examination with a detailed draw,ing of all tumors and related findings. A child with a white pupil is performed Other confirmatory tests. like photography ultrasonography and, fluorescein angiography are performed These tests assist in planning. the treatment strategy for the child Fluorescein angiography uses. an intravenous dye that discolors the child s urine to a green yellow. color for one day, A pediatrician or a pediatric oncologist performs the pediatric exam. ination The systemic examination is based on how the eyes appear. and what treatment is needed It might include Physical examination. CT and MRI of brain and orbits blood tests bone marrow analysis. hearing and kidney tests, If on chemotherapy the hearing and brain tests are important The.
brain CT and MRI are usually performed twice yearly until age 5 years. These tests are done to evaluate for possible spread of the tumor as. well as other related cancers, If treatment is necessary it is usually performed while the child is under. anesthesia Later the doctors discuss the findings and treatment with. the family When the child recovers the eyes might appear somewhat. swollen and red for about 2 or 3 weeks If the child is in the midst of. treatment appointments are about 1 month apart If the child is. stable then appointments are usually 2 to 6 months apart Lifelong. all children with retinoblastoma should have an eye examination. once or twice yearly, The goals of treatment of a child with retinoblastoma are threefold. To save the child s life,To save at least one eye,To protect the vision. There are several ways to treat retinoblastoma depending on the. size location and multiplicity of tumors as well as the child s age. status of the opposite eye and systemic condition The treatment. methods include Chemoreduction thermotherapy laser photo. coagulation cryotherapy plaque radiotherapy external beam. radiotherapy and enucleation These are often used in combination. Chemoreduction is a method of using intravenous chemotherapy. to a reduce retinoblastoma to a small size so that the residual tumors. can be eradicated with focal treatment methods like thermotherapy. or cryotherapy Chemoreduction is used in nearly all children with. bilateral retinoblastoma and about 25 of children with unilateral. retinoblastoma This technique involves delivery of intravenous che. motherapy each month for 6 months The delivery takes about 2 days. of each month and is usually performed in Philadelphia at Children s. Hospital of Philadelphia Each chemotherapy session is coupled with. an examination under anesthesia so that treatment of the tumor scars. can be done the same day, Thermotherapy is a laser method in which the residual tumor is. heated for 5 to 15 minutes to a temperature that kills the cells It is. focal so that the surrounding tissue is unaffected It typically leaves no. external scars on the eye but some children might show an irregular. pupil after treatment, Laser Photocoagulation is a method to treat a small retinoblas.
toma by closing the blood vessels to the tumor with heat This causes. no external scars on the eye but leaves a small scar on the retina. Cryotherapy involves the use of a probe the size of a pencil placed. on the eye to deliver a focal freeze for about 1 minute through the. wall of the eye into a tumor inside the eye Surrounding tissue is usually. unaffected by this treatment It typically leaves no external scars on. the eye but the eye is often swollen for 2 or 3 weeks. Plaque Radiotherapy is a method of giving focal x ray treatment. to a small part of the eye by using a piece of metal called a plaque. The plaque has implanted radiation and it is sutured temporarily. onto the eye directly over the retinoblastoma The child stays in. Procedures,and Treatment,the hospital for several days usually 3 to 7. days while the radiation plaque is in place,The radiation dose to the retinoblastoma. is quite focal and limited to the eye itself,with little radiation elsewhere to the body. After the correct dose is given the plaque,is removed in the operating room and. the child is discharged Eye drops will be,prescribed to be used 3 times a day for.
3 weeks The eye heals well over a few,weeks Long term concerns include vision. loss from cataract or retinal swelling We,have not seen radiation related second. Radiation Plaque,cancers following plaque treatment. External Beam Radiotherapy is a method of x ray treatment. to the entire eye by a radiation machine This is available at only. a few experienced centers worldwide It takes about 4 weeks. Monday through Friday in which small doses of radiation are. given to achieve the final dose External beam radiotherapy can. cause the eye to feel irritated dry look red and the patient might. lose the eyelashes temporarily This generally resolves but the child. is also at risk for long term problems like vision loss from cataract. or retinal swelling There is also a risk for radiation related second. cancers especially in children with bilateral retinoblastoma. Intra Arterial Chemotherapy IAC was introduced in Japan. and modernized in the United States It is a novel method of deliv. ering chemotherapy directly into the eye A fine catheter is passed. into the femoral artery at the groin upwards through the heart and. then into the ophthalmic artery of the eye Chemotherapy is then. injected into the eye under sterile conditions in the operating room. This technique limits systemic exposure and allows infusion of more. potent chemotherapeutic agents Intra arterial chemotherapy. can be given primarily in patients with unilateral retinoblastoma or. secondarily when tumor recurs after chemoreduction or previous. intra arterial chemotherapy It is given monthly for approximately. 2 3 months Although very effective there is still a small risk of vision. loss intra operative stroke bone marrow toxicity and ocular vascular. toxicity Long term effects on vision are also unknown at this point. but current studies have shown it maintains normal retinal anatomy. after course completion, Intravitreal Chemotherapy is a method of delivering chemother. apy into the vitreous cavity Normally the vitreous is the jelly portion. of the eye that comprises the greatest volume of the eye and main. tains its form It is located in front of the retina and behind the lens. The vitreous is normally avascular thus penetration of chemotherapy. delivered through arteries is limited The indications of intravitreal. chemotherapy are limited to eyes with seeding of small retinoblas. toma cells into the vitreous when other methods of chemotherapy. fail to control proliferation of these seeds It is a highly potent and. targeted delivery of high dose chemotherapy directly into the vitreous. through an intraocular injection This limits penetration of drug into. the systemic vasculature as well as periocular structures It is given. weekly or bi weekly for approximately 5 6 times and is effective in. almost all cases This is always performed in the operating room with. utmost caution to prevent seeding of retinoblastoma cells outside. the eye through the needle tract Cryotherapy is always performed. during needle withdrawal to sterilize the needle tract Possible com. plications are infection bleeding and retinal detachment all of. which are serious potentially blinding conditions but thankfully are. rare occurring in less than 1 of all injections,Enucleation is a method of re.
moving the entire eyeball The,eyelids and muscles of the eye. remain This is used for eyes that,have large tumors or eyes that. have developed painful glauco,ma All other methods of treat. ment are considered before,advising enucleation but some. patients have life threatening,Example of an ocular prosthesis.
large tumors that necessitate, enucleation Following enucleation an implant is placed in the. empty orbit and allowed to heal for 1 or 2 months Then an artificial. eye prosthesis is made to match the remaining eye The cosmetic. appearance is generally outstanding with a natural appearance. and comfortable fit as well as some movement of the eye Protective. lenses made of polycarbonate are advised to be worn at all times in. the form of glasses during the day or goggles during activities or sports. Recommended reading for vision in one eye is a book entitled. A Singular View The Art Of Seeing with One Eye by Frank Brady. available online at www amazon com, Fortunately most children with retinoblastoma survive and lead a. good long and productive life In the United States nearly 98 of. children survive Not so in less advanced countries where about 50. of children die from tumor spread Long term ocular and pediatric. examinations are advised for the child, The prognosis of a child with retinoblastoma depends on several. factors most importantly the results of pathology Children with retino. blastoma that has invaded into the optic nerve choroid sclera orbit. or anterior chamber require chemotherapy as they are at greatest. risk for metastasis spread and death Those without invasion do not. Retinoblastoma occurs in approximately 1 in 15 000 live births and it is estimated to affect approximately 250 to 300 chil dren each year in the United States Worldwide about 5 000 children develop retinoblastoma each year Retinoblastoma occurs equally in boys and girls and it is seen in all races Retinoblastoma affects only one eye in about 70 of patients and it affects both eyes in

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