Objective: To report the clinical features and surgical treatment of orbitotemporal neurofibromatosis. Methods: It was a retrospective case series study. The clinical records of 24 patients who were treated for orbitotemporal neurofibromatosis between April 2007 and July 2017 at Beijing Tongren Hospital, Capital Medical University were analyzed. Data collected included sex, age, laterality, periorbital deformities, surgical treatment, follow-up time, complication and recurrence. Results: Sixteen males and 8 females were included. Age at surgery was (15±7) years (4-30 years). All patients were unilaterally involved. Twenty-three patients (96%) had upper lid involvement and ptosis. Nine patients (38%) had lower lid involvement. Fourteen patients (58%) had lateral canthal disinsertion. Three patients (13%) had brow involvement, 10 patients (42%) had conjunctival involvement and 2 patients (8%) had lacrimal gland infiltration. All patients had tumor debulking procedure. Twenty-two patients (92%) had correction of ptosis. Fourteen patients (58%) required lid reconstruction and lateral canthus reattachment surgery. Three patients (13%) had correction of brow ptosis.One patient (4%) had skull and orbit reconstruction. The median follow-up time was 3.5 (1.0-10.0) years. All patients had improved appearance. Ptosis recurred in 6 patients, but were corrected with surgery. On the last follow-up, 7 patients were free of ptosis. In 14 patients, mild ptosis was noted, but the upper lid did not cover the pupil. In 2 patients the pupil was half covered. Only in 1 patient who had not received ptosis correction surgery the pupil was covered completely. Conclusions: The periorbital deformities of orbitotemporal neurofibromatosis include upper eyelid infiltration with ptosis, lateral canthal disinsertion and infiltration of lower eyelid, brow, conjunctiva and lacrimal gland. The appearance of patients with orbitotemporal neurofibromatosis can be significantly improved through oculoplastic surgery. (Chin J Ophthalmol, 2019, 55: 828-833).
目的： 探讨眶颞部神经纤维瘤病的临床特征和眼睑畸形整复方法及效果。 方法： 回顾性系列病例研究。收集2007年4月至2017年7月在首都医科大学附属北京同仁医院北京同仁眼科中心接受肿瘤切除和畸形整复手术治疗的24例眶颞部神经纤维瘤病患者的病历及随访资料，观察总结性别、年龄、眶周组织受累情况、手术方式、随访时间、并发症和复发情况，并对典型病例进行分析。 结果： 24例患者中男性16例，女性8例；手术时年龄（15±7）岁（4~30岁）。24例患者全部为单侧受累，其中23例（96%）上睑受累伴下垂，9例（38%）下睑受累，14例（58%）外眦韧带离断，3例（13%）眉受累，10例（42%）结膜受累，2例（8%）泪腺受累。24例患者均接受肿瘤切除术，其中22例（92%）联合上睑下垂矫正术，14例（58%）联合眼睑重建及外眦成形术，3例（13%）联合眉下垂矫正术，1例（4%）联合颅骨及眼眶重建术。术后随访时间中位数为3.5年（1.0~10.0年）。所有患者术后眼睑外形获得明显改善。6例上睑下垂复发，再次手术后得到改善。末次随访时，24例患者中7例无上睑下垂；14例有轻度上睑下垂，但瞳孔区不被遮挡；2例上睑下垂遮挡1/2瞳孔；1例尚未行上睑下垂矫正术者上睑下垂完全遮挡瞳孔区。 结论： 眶颞部神经纤维瘤病的眶周畸形包括上睑受累伴上睑下垂、外眦韧带离断，下睑、眉、结膜及泪腺受累，通过切除肿瘤及行各种眼睑畸形的整复手术，患者面部外观可明显改善。（中华眼科杂志，2019，55：828-833）.
Keywords: Eyelids; Neurofibromatoses; Orbital neoplasms; Reconstructive surgical procedures.