Visual outcomes in children with bilateral retinoblastoma

Retinoblastoma is the most common primary intraocular tumor of childhood. Although studies have explored trends in retinoblastoma management and prognosis, few have addressed visual outcome.

A retrospective chart review was performed on children in whom bilateral retinoblastoma was diagnosed at New York Hospital-Cornell Medical College. A total of 74 children were included in the study. All children underwent radiation to the eyes that were studied. Fundus drawings done at the time of diagnosis were evaluated to determine the location of tumors on presentation and the Reese-Ellsworth classification. Visual outcome was classified into 3 groups: group 1 represented visual acuity of 20/20 to 20/40; group 2, 20/50 to 20/400, and group 3, <20/400.

A total of 74 children were studied. Forty-six (62%) underwent enucleation of 1 eye. The visual acuity of the remaining 102 eyes was divided into groups 1, 2, and 3. Fifty-eight percent of these eyes were in group 1, 31% in group 2, and 9% in group 3. Two percent underwent subsequent enucleation after treatment. The tumors were analyzed on the basis of Reese-Ellsworth classification, location, size, and distance from the macula. Excluding Reese-Ellsworth group VB, there was no correlation between Reese-Ellsworth classification and final visual outcome. Tumors involving the macula were in 7 (78%) of 9 eyes with poor visual outcome (group 3), and 16 (33%) of 48 eyes with excellent vision (group 1) had macular tumors. Paradoxically, 2 (22%) of 9 eyes in group 3 did not have tumors involving the macula.

Children with retinoblastoma now have an excellent prognosis for life. Although correlated with tumor location, visual outcome is not always easily predicted on the basis of the initial presentation. Final acuity is excellent in most cases but may be influenced by multiple factors that must be considered when caring for these children and families.