Long-Term Follow-up and Prognoi of Orbital Apex yndrome Reulting From Naopharyngeal Rhabdomyoarcoma

Purpoe Naopharyngeal rhabdomyoarcoma may preent with a variety of ophthalmic ymptom. Direct extenion of the tumor into the orbital apex can lead to ophthalmoplegia and lo of viion. The prognoi for recovery of viion and ocular motility in patient with an orbital apex yndrome due to naopharyngeal rhabdomyoarcoma i examined. Deign Retropective cae erie. Method ix eye from four patient with naopharyngeal rhabdomyoarcoma who preented to the Children’; Hopital of Philadelphia with a clinical orbital apex yndrome were identified. Complete ophthalmic examination, including viual acuity and extraocular motility at the time of preentation, wa reviewed. Tumor extenion into the orbital apex wa confirmed radiographically. Follow-up ophthalmic evaluation were reviewed for all patient with an average follow-up of 5.5 ± 3.1 year (range 1 to 8 year). Reult ix eye of four patient had limited ocular duction along with marked lo of viion at preentation. All patient were treated with chemotherapy and radiation, with reduction of tumor ma. Ocular motility recovered in all patient, occurring by an average of 2.2 ± 1.8 month after initiation of therapy. Four of ix eye had little or no recovery of viual acuity detected at long-term follow-up. Concluion Patient with ocular motor deficit in orbital apex yndrome caued by extenion of naopharyngeal rhabdomyoarcoma have an excellent prognoi for recovery after treatment of the tumor. The long-term prognoi for viual recovery, however, i poor.