The development of young children with retinoblastoma.

OBJECTIVES: To assess the health and development of children with retinoblastoma (RB), or cancer of the retina, and to determine if they are at greater risk for developmental delays than normal children. Specific aims were to determine if type of RB (unilateral vs bilateral), family history, and number of treatment types affected mental and motor development.
DESIGN: Descriptive study based on medical record review and pediatric, psychological, and visual evaluations.
SETTING: Major referral center for patients with RB and early intervention program in a voluntary urban hospital. SUBJECTS AND METHODS: Fifty-four children younger than 41 months with RB who attend an ophthalmology oncology clinic were recruited for study. Measures included demographic variables such as social class and race/ethnicity, and medical factors such as age at diagnosis (<18 months vs >18 months), type of RB (unilateral or bilateral), family history of RB, and number and types of treatments. All children received a pediatric examination that assessed physical growth and health; a behavioral test of visual acuity using Teller acuity cards; and the Bayley Scales of Infant Development II, a standardized test of mental and motor development. Children found to have delays were referred to intervention services to treat their specific areas of weakness.
RESULTS: Three quarters of the children had had 1 eye enucleated; 51 of 54 had normal vision in at least 1 eye, and the other 3 had partial vision in 1 eye. Except for the RB, 46 children were largely normal in growth and health, and 8 had medical diagnoses that were unrelated to RB or its treatment. The average mental and motor development scores were in the normal range (91.4 +/- 16.3, and 91.1 +/- 13.4) and not significantly lower than the normal population. Twenty-six children were referred for early intervention services, and 21 of 26 were referred for services to improve their visuomotor coordination. Demographic variables were not associated with medical variables or outcome. Children with bilateral RB, in which both eyes are affected, performed significantly less well in motor development, received many more types of treatments, and were more likely to be referred for visuomotor therapy than children with unilateral RB.
CONCLUSIONS: Children with RB generally function normally in terms of physical health and mental and motor development. However, they are more likely to show delays in visuomotor integration. Early developmental evaluations may improve the visuomotor development of children with visual impairment due to RB.