眼眶神经纤维瘤病Ⅰ型相关斜视1例  

Orbital neurofibromatosis typeⅠassociated strabismus a case report

作　　者：张裕琳 周荣妹 赵晨 韦严 ZHANG Yu lin;ZHOU Rong mei;ZHAO Chen;WEI Yan(Department of Ophthalmology,Eye&ENT Hospital,Fudan University,Shanghai 200031,China)

机构地区：[1]复旦大学附属眼耳鼻喉科医院眼科,上海200031

出　　处：《中国眼耳鼻喉科杂志》2025年第S1期34-37,共4页Chinese Journal of Ophthalmology and Otorhinolaryngology

摘　　要：27岁女性,因“发现左眼偏斜10余年”就诊。眼位检查:左眼内斜视合并下斜视,眼球运动左眼外转、上转不过中线。眼部检查视力:右眼1.0,左眼CF/15 cm。左眼上睑轻度肿胀,皮肤条索状瘢痕,上睑下垂。左眼颞侧球结膜见瘢痕,角膜明,前房常深,虹膜可见多个黄白色小结节,瞳孔欠圆,对光反应迟钝,人工晶状体位正,后囊混,眼底模糊窥不清。右眼前节及眼底未见明显异常。躯体查体:左侧额部见结节样隆起肿物,颈部见密集分布的粟粒状大小褐色斑点,手臂处见牛奶咖啡斑、结节样肿物。眼眶影像学回报:左侧蝶骨缺损,眼眶颞上方肿物,伴外直肌增粗浸润。初步诊断:神经纤维瘤病Ⅰ型相关斜视。收治入院后行斜视矫正术,术后患者外观改善满意。讨论主要围绕斜视及眼球运动障碍的病因、手术方案的具体制定展开。讨论体会:Ⅰ型神经纤维瘤易侵袭眼眶、眼外肌,且常合并蝶骨缺损,导致非共同性斜视及眼球运动障碍。外科手术是目前的主要治疗方法,瘤体血供丰富且无包膜,需谨慎选取手术方案,改善患者眼位并减少并发症的出现。A 27-year-old female presented with“left eye deviation for over 10 years”.Eye movement examination revealed esotropia and hypotropia in the left eye with limited abduction and upward movement that did not cross the midline.Physical examination:the visual acuity was 20/20 in the right eye(OD)and counting fingers(CF)at 15 cm in the left eye(OS).Mild edema and cord-like scarring was noted on the left upper eyelid.Slit-lamp examination showed no significant abnormalities in the right eye,but a temporal bulbar conjunctival scar in the left eye,with clear cornea,normally deep anterior chamber,multiple small yellowishwhite nodules in the iris,irregularly shaped pupil with delayed light reflex.The Intraocular lens(IOL)was in position with posterior capsule opacity and the fundus was blurry.Furhter examination revealed nodular bulge on the left forehead,densely distributed milia-sized brown spots on the neck,café-au-lait spots,and nodular masses on the arms.Orbital MRI showed a left sphenoid bone defect and an orbital supratemporal mass with thickening and infiltration of the lateral rectus muscle.The initial diagnosis was neurofibromatosis type I associated strabismus.Strabismus surgery was performed after admission,and the patient’s appearance was satisfactory postoperatively.The discussion mainly focused on the etiology of strabismus and surgical plans.Neurofibromatosis Type I was prone to invade the orbit and extraocular muscles,and often accompanied by sphenoid bone defects,leading to non-comitant strabismus and ocular motility disorders.Currently,surgical intervention is the main treatment.Given the rich vascularity and lack of encapsulation of the tumor,meticulous surgical plan was essential to improve the patient’s ocular alignment and minimize complications.

关 键 词：神经纤维瘤病Ⅰ型 斜视 眼眶 手术 影像学 

分 类 号：R73[医药卫生—肿瘤]