Trilateral Retinoblastoma-A Case Report of Reconstruction

Abstract

Trilateral retinoblastoma is a rare malignancy of childhood with high mortality. We reported such a case with delayed treatment. An 18-month old female baby presented with left eye proptosis and right eye leukocoria. Retinoblastoma has been informed to her parents at the age 2 months; however, her family refused further managements. Orbit CT showed bilateral retinoblastoma with extraocular extension in left orbit. MRI of brain revealed a tumor mass in pineal gland. Her right eye was enulcleated with unwrapped hydroxyapetite implantation (size 20 mm) and the left orbit was exenterated. Pathologic examination confirmed a case of retinoblastoma. On the right eye, the optic nerve was not involved and the tumor was confined in the eyeball. On the left eye, the tumor cells extended out into the optic nerve and orbital tissue. Four months after exenteration when the bone surface has been covered with granulation tissue, hydrogel implant (Osmed(superscript TM) Hydrogel Orbit Expanders) was inserted in her left orbital socket without any wrapping or sutures. The hydrogel implant gradually expanded in the orbit and prosthesis was placed. The patient underwent chemotherapy and radiotherapy to achieve a temporary remission. However, the patient deceased in a follow-up of 2 years due to tumor invasion into the brain and sepsis.