Stargardt disease in a patient with retinoblastoma.

Retinoblastoma is the most common primary ocular malignancy of young children, with an incidence of 1:17000 to 1:34000 new births. Retinoblastoma is bilateral in about 30% of cases. Although new modalities offer an enhanced chance of eye salvage, in bilateral disease, management often leads to enucleation of the more-involved eye, with more conservative treatment of the better eye. Subsequently, the child is watched carefully for recurrence and for any evidence of central nervous system involvement or other malignancies. The retinoblastoma gene (Rb) is located in the 14 band of the q, or long arm, of chromosome 13. The Rb gene causes cancer when its protein product is absent or dysfunctional. We describe a child with bilateral retinoblastoma after enucleation ofthemore-involvedeyeat13months of age, who was diagnosed as having Stargardtdisease in thepreservedeye at age10years.Thesignificantpoints wewish tohighlight are thedifficulty indiagnosingStargardtdiseasebecause of subtle retinal findings, and the opportunity this case affords for investigatingearlypreclinicalstagesofStargardtdisease.Toourknowledge,there is only 1 previously reported case of these2disorders in thesamepatient.