Retinoblastoma
Most common intraocular tumor of childhood 1 in every 18,000 births Up to 500 new cases diagnosed each year in United States Diagnosed usually at birth or within few years of life Less than 10% have family history of retinoblastoma Risk of death in untreated cases nearly 100% Early diagnosis and treatment of small tumors lead to best outcomes
White (cat’s eye) pupil , also called leukocoria, if tumor large and near back of lensSmaller tumors visible only on ophthalmoscopy Many young children present with poor vision or misaligned eyes (strabismus )
Leukocoria also caused by many other vitreoretinal disorders, usually separable from retinoblastoma by ophthalmoscopy, ultrasound, optical coherence tomography, CT, MRI
Check for leukocoria and strabismus in babies and screen for subnormal vision in young children Refer suspicious cases within weeks
Cryo, laser, radiation, and chemotherapy provide 30% to 90% cure depending on pre-treatment extent of tumor Enucleation is performed for very large tumors or eyes with very poor sight Genetic counseling is critical as transmissibility is complex issue
