양안 안구적출술을 받은 망막모세포종 환아들의 임상경과

The Clinical Courses of Retinoblastoma Patients Who Underwent Bilateral Enucleation

Purpose: To investigate the courses leading to bilateral enucleation in bilateral retinoblastoma patients. Methods: Medical records of 5 bilateral retinoblastoma patients who underwent bilateral enucleation were reviewed for patient information, history, change of tumor state during the treatment and the cause of bilateral enucleation. Results: Out of 48 bilateral retinoblastoma patients, both eyes were saved in 6 children, 1 eye was saved in 37 children, and the remaining 5 children lost both eyes. All patients who underwent bilateral enucleation were female and had no family history of retinoblastoma. At diagnosis, 3 children were 4 months old and the remaining 2 patients were 1 year and 1.5 years old each. Out of 10 eyes, 8 eyes were in Reese Ellsworth group V and the remaining 2 eyes were in group III. The initial treatment was enucleation of 1 eye followed by chemotherapy in 3 patients, and chemotherapy alone in 2 patients. Additional treatment included laser photocoagulation, cryotherapy, external beam radiation therapy and proton beam irradiation. Vitreous seeding, development of new tumors, and increase in tumor size despite of intensive, conservative treatment resulted in second enucleation. Conclusion: Most patients who underwent bilateral enucleation were 1 year old or younger at diagnosis of retinoblastoma. All patients had a progressed disease status at presentation and showed poor response to intensive, conservative treatment.