Page 66 - April 2016 Life In Naples Magazine
P. 66

RETINOBLASTOMA

IN CHILDREN

by Víctor M.Villegas, M.D.                                                                                                           FIGURE 1. CHILD WITH RETINOBLASTOMA IN THE
                                                                                                                LEFT EYE AND A WHITE PUPIL REFLEX ON FLASH PHOTOGRAPHY.
R etinoblastoma is the most common
            intraocular malignancy in children.It affects   systemic side effects. Currently, Bascom Palmer Eye Institute is one of the
            1 in 15,000 live births. Early diagnosis is of  few hospitals in the world that is performing intra-arterial chemotherapy for
            critical importance because small tumors        retinoblastoma.The technique consists of catheterization of the internal carotid
have the best prognosis. Historically, leukocoria or        artery and the ophthalmic artery with direct infusion of chemotherapeutic
“white pupil” has been the most important sign as
seen in Figure 1. It may sometimes be noticed in a                                                 agents into the eye vasculature (Figure 2).
photograph taken with flash. Strabismus, nystagmus,                                                This technique allows the ocular oncologist
red eye, and loss of binocularity can all be presenting                                            to use different chemotherapeutic options
signs depending on the location of the tumor.                                                      and dosing strategies. Direct infusion of
                                                                                                   chemotherapies to the ophthalmic artery
   Therapies for retinoblastoma have dramatically                                                  also decreases the systemic side effects
advanced during the last 10 years. A significant                                                   experienced by the patient.
trend away from systemic chemotherapy to directly
ocular and intra-arterial chemotherapy is currently                                                    The significant tumoricidal effects
underway. Technological changes and strategies                                                     reported with intra-arterial chemotherapy
focus on local treatments due to decreased morbidity                                               generated enthusiasm to study intravitreal
to patients and excellent tumor response. New                                                      delivery for vitreous seeding. During the last
treatments are providing new hope to patients,                                          FIGURE 2 three years, Bascom Palmer Eye Institute has
especially to those with the most severe disease.           been on the forefront in adapting new intravitreal techniques that help control
                                                            vitreous seeding in patients that would have otherwise lost the eyeglobe. The
   Management of retinoblastoma tumors requires             combination of intra-arterial and intravitreal chemotherapy has allowed us to
a multidisciplinary approach that may include an            take care of patients in unprecedented ways. We are extremely excited about
ocular oncologist, pediatric oncologist, pediatric          the improvements made during the last decade and are looking forward to
ophthalmologist, pediatrician, interventional               developing new techniques that improve further the visual outcomes.
radiologist, and ocular pathologist. Individualized
treatment, considering factors such as the
International Classification of Retinoblastoma,
laterality, location of tumors, age of patient, family
history, and prior treatment must be considered.
Retinoblastoma treatment is aimed at child survival.
Globe salvage and preservation of vision are
secondary goals. Early diagnosis remains the most
crucial step in decreasing morbidity and mortality.

   Treatment of small tumors may only require
transpupillary thermotherapy. Laser treatments may
be repeated monthly until complete tumor regression
is documented. It is important to follow up patients
closely to monitor for recurrence. If recurrence is
present, adjuvant chemotherapy may be considered.

   New technologies have allowed physicians to
improve tumor regression rates while decreasing

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