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DESCRIPTION: A malignant and life-threatening intraocular tumor which appears in children; 66% appear before age 3, however, rare cases have been reported at almost every age. About 30% are bilateral. It usually is unnoticed until it has progressed to a point of producing a white pupil (unless it has caused a strabismus and is diagnosed earlier, since blind eyes in children will often turn inward). Generally, the earlier the tumor is discovered, the better the chance to treat it and prevent its spread through the optic nerve and orbital tissues. About 94% of these tumors are due to mutations, but survivors will pass the mutated gene on about 50% of the time.

TREATMENT: Enucleation is the treatment of choice when the tumor is large; radiotherapy and/or chemotherapy are other possibilities. Occasionally, cryotherapy or photocoagulation are effective. When retinoblastoma is bilateral, enucleation is done on the worst eye first, with radiotherapy attempted on the other eye. If no improvement is observed, enucleation is done on the second eye.


IMPLICATIONS: Since enucleation is an immediate and necessary treatment of retinoblastoma, these children will be blind early in life. Although educational programming will involve tactile and auditory techniques, there will often be good spatial orientation because of the early presence of some degree of vision. (Congenitally totally blind children do not have the benefit of this early spatial orientation.) There is some evidence that children with retinoblastoma have better tactile discrimination than other totally blind children, and many of them are above average in intelligence.

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