ABSTRACT.Purpose: To determine when patients with a family history of retinoblastoma and previously normal eye exam develop intraocular disease, and where the new retinoblastoma tumors occur. Methods: A retrospective chart review of retinoblastoma patients. Results: Sixty-two percent of the first eyes (eyes having a previously normal examination) were diagnosed with retinoblastoma by 6 months of age, 90% by 12 months and 100% by 28 months. For the second eye, 27% were identified by 6 months, 64% by 12 months, 91% by 30 months and 100% by 44 months. The younger the age at initial diagnosis of retinoblastoma, the greater the likelihood that tumors will initially be found in the posterior pole. Macular tumors were diagnosed very early (mean 4 months) and once a retinoblastoma focus had appeared in one eye no new tumors developed in the macula of either eye. Conclusion: The timing, location, and number of new retinoblastoma tumors follows a predictable pattern.

In the only other series that has been similarly analysed Coleman et al (New York) reported that tumour thickness was not a significant predictor of percentage reduction in height. However, they showed that ultrasonically derived spectral parameters did correlate with regression.3 At six months they found that acoustic tissue type (ATT) B had a 29-5% reduction in height, while ATT E had an 11-4% reduction in height. At 18 months ATT B had a 53 9% reduction in height, while ATT E had a 26-4% reduction in height.

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Children diagnosed with retinoblastoma in the first year of life present with differences in laterality, stage, signs, symptoms, and respond differently to treatments when compared to older children. Of those children diagnosed in the first year of life (between 1958 and 1983 inclusive), 280 were bilateral and 121 were unilateral, the most common stage of diagnosis (for unilateral and bilateral) was Group V, and children were affected equally by sex. Children examined in the first three months of life were more often seen because of a positive family history of retinoblastoma, rather than leukocoria. Children who were treated in the first year of life frequently develop second nonocular tumors because they harbor the germinal mutation and receive radiation.