Retinoblastoma Presenting as Strabismus and Leukocoria

A 22-month-old otherwise healthy girl presented for evaluation of an inturned left eye of 3 months duration. She had been followed approximately every 6 months via red-reflex evaluation by her pediatrician, who noted a dramatic change over a 4-month period. External examination revealed a left esotropia with leukocoria (Figure 1). Ophthalmologic examination revealed a normal right eye and total retinal detachment in a tumor-filled left eye (Figure 2). Magnetic resonance imaging showed a large heterogeneous mass filling the left globe with extension into the left optic nerve head, but no metastases or secondary lesions. Further questioning disclosed that the child’s mother had bilateral retinoblastoma requiring enucleation of both eyes 38 years earlier at 4 months of age.

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• Figure 1 

  External examination showing esotropia and leukocoria of the left eye.

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• Figure 2 

  Close-up examination showing total retinal detachment in an eye filled with white tumor.