Shindler Kenneth S, Liu Grant T, Womer Richard B
Scheie Eye Institute, Department of Ophthalmology, University of Pennsylvania, Philadelphia 19104, USA.
Am J Ophthalmol. 2005 Aug;140(2):236-41. doi: 10.1016/j.ajo.2005.02.054.
Nasopharyngeal rhabdomyosarcoma may present with a variety of ophthalmic symptoms. Direct extension of the tumor into the orbital apex can lead to ophthalmoplegia and loss of vision. The prognosis for recovery of vision and ocular motility in patients with an orbital apex syndrome due to nasopharyngeal rhabdomyosarcoma is examined.
Retrospective case series.
Six eyes from four patients with nasopharyngeal rhabdomyosarcoma who presented to the Children';s Hospital of Philadelphia with a clinical orbital apex syndrome were identified. Complete ophthalmic examination, including visual acuity and extraocular motility at the time of presentation, was reviewed. Tumor extension into the orbital apex was confirmed radiographically. Follow-up ophthalmic evaluations were reviewed for all patients with an average follow-up of 5.5 +/- 3.1 years (range 1 to 8 years).
Six eyes of four patients had limited ocular ductions along with marked loss of vision at presentation. All patients were treated with chemotherapy and radiation, with reduction of tumor mass. Ocular motility recovered in all patients, occurring by an average of 2.2 +/- 1.8 months after initiation of therapy. Four of six eyes had little or no recovery of visual acuity detected at long-term follow-up.
Patients with ocular motor deficits in orbital apex syndromes caused by extension of nasopharyngeal rhabdomyosarcoma have an excellent prognosis for recovery after treatment of the tumor. The long-term prognosis for visual recovery, however, is poor.
鼻咽横纹肌肉瘤可能会出现多种眼部症状。肿瘤直接蔓延至眶尖可导致眼球运动麻痹和视力丧失。本文对因鼻咽横纹肌肉瘤导致眶尖综合征患者的视力及眼球运动恢复预后进行了研究。
回顾性病例系列研究。
确定了4例因鼻咽横纹肌肉瘤就诊于费城儿童医院且患有临床眶尖综合征患者的6只眼。回顾了完整的眼科检查，包括就诊时的视力和眼外肌运动情况。通过影像学检查确认肿瘤蔓延至眶尖。对所有患者进行了随访眼科评估，平均随访时间为5.5±3.1年（范围1至8年）。
4例患者的6只眼在就诊时眼外肌运动受限且视力显著丧失。所有患者均接受了化疗和放疗，肿瘤体积减小。所有患者的眼球运动均得以恢复，平均在治疗开始后2.2±1.8个月出现。在长期随访中，6只眼中有4只眼的视力几乎没有恢复或未恢复。
因鼻咽横纹肌肉瘤蔓延导致眶尖综合征出现眼球运动障碍的患者，在肿瘤治疗后恢复预后良好。然而，视力恢复的长期预后较差。
