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Retinoblastoma is a cancer of the eye. It is rare, occurring in 1/18,000 children. In the majority of cases, only one eye is involved, and the condition is not hereditary. Unilateral (one side) and bilateral (both eyes involved) hereditary cases make up about 40% of cases.
This cancer typically is found when the doctor notices a white pupillary light reflex, known as leukocoria, on a routine physical exam, or perhaps the child's parents note that the red reflex is absent in one eye on a flash photograph. Strabismus or eye deviation is commonly the earliest sign noticed by the parents.
Diagnosis is made by an ophthalmologist during a detailed examinatinon of the internal eye structures under general anesthesia. Other tests can include ultrasound and CT examination of the eye.
Advances in chemotherapy have led to improvement in salvaging vision without removal of the eye, but of course removal is sometimes the only sensible treatment. Cure rates can reach 90% for this tumor.