Ⅱ型眼眶神经纤维瘤病的整复治疗

Surgical management of type Ⅱ orbital

目的探讨Ⅱ型眼眶神经纤维瘤的临床特点及手术治疗方法。方法对2001年1月至2006年10月在上海第九人民医院手术治疗的16例（16只眼）Ⅱ型眼眶神经纤维瘤患者的临床资料和随访结果进行回顾性分析。结果16例均有不同程度的眶颞部肿瘤、眶内肿瘤、眼睑和眼眶畸形。所有病例均行肿瘤次全切除联合一期眼睑整复术和眼眶重建术。术后随访6—12个月，未见肿瘤复发，6例术后视力得到提高，10例视力保持不变。所有病例行眼睑整复术后外形良好，内外眦角基本对称，上睑缘弧度可，但5例因上睑下垂复发再次手术，术后外形得到改善。所有病例眼眶重建术后眼眶外形和眼球突出度与健侧眼基本对称，眼球运动明显改善。所有病例均未出现严重并发症。结论Ⅱ型眼眶神经纤维瘤病患者通过肿瘤次全切除联合一期眼睑和眼眶整复手术可以获得功能和外观上的共同改善。

Objective To study the clinical manifestations and surgical treatment of Type Ⅱ orbital neurofibromatosis. Methods From Jan, 2001, to Oct, 2006, clinical data of 16 cases with type Ⅱ orbital neurofibromatosis were retrospectively analyzed in the Department of Ophthalmology, Ninth People＇ s Hospital, Medical School of Shanghai Jiao Tong University. Results All patients had orbito-temparal or intra-orbital neurofibroroatosis, combined with periorbital deformities. After partial tumor resection, the orbital reconstruction and blepharoplasty were performed in the same stage. The patients were followed up for 6-12 months with no relapse. The vision was improved in 6 cases and kept the same in 10 cases. The cosmetic results were satisfactory in all cases. 5 cases were re-operated because of relapse of blepharoptosis. Conclusions The function and appearance can be markedly improved after one-stage partial tumor resection, orbital reconstruction and blepharoplasty in patients with typeⅡ orbital neurofibromatosis.