黏多糖贮积症继发青光眼1例  

A case of glaucoma secondary to mucopolysaccharidoses

作　　者：李梦玮 卢苇 孙兴怀 陈君毅 陈宇虹 LI Mengwei;LU Wei;SUN Xinghuai;CHEN Junyi;CHEN Yuhong(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5-8,共4页Chinese Journal of Ophthalmology and Otorhinolaryngology

摘　　要：23岁男性,因“检查发现双眼眼压高1年”至门诊就诊。患者最佳矫正视力:双眼0.4;眼压:右眼23 mmHg(1 mmHg=0.133 kPa),左眼22 mmHg。裂隙灯检查双眼角膜雾状混浊,角膜直径正常,前房周边浅,虹膜未见明显异常,瞳孔圆,直径3 mm,余眼内结构窥不清。眼部B超显示双眼各方位球壁明显增厚。眼轴:右眼19.86 mm,左眼19.85 mm。超声生物显微镜显示双眼各方位房角裂隙样窄开,各方位睫状上皮囊肿。同时,该患者身材矮小、短颈、粗颈。追问病史,患者曾诊断为黏多糖贮积症Ⅰ型。因此诊断为黏多糖贮积症继发性青光眼,用足量降眼压药物控制眼压尚平稳。数月后某一天患者左眼急性闭角型青光眼大发作,用药物及激光均无法控制眼压,遂行左眼小梁切除+巩膜床切开术。后右眼也行相同术式。眼压目前控制稳定。讨论体会:黏多糖贮积症继发青光眼的发病机制既有开角也有闭角因素,其临床评估和手术治疗有挑战,需要今后不断探索。A 23-year-old male presented with a history of high intraocular pressure(IOP)in both eyes for one year.His best corrected visual acuity was 0.4 in both eyes.The IOP was 23 mmHg(1 mmHg=0.133kPa)in the right eye(OD)and 22 mmHg in the left(OS).Slit lamp biomicroscopy revealed corneal clouding with normal corneal diameter,shallow peripheral anterior chamber,normal iris,and round pupil with a diameter of 3 mm.The other intraocular structures were not clearly visible.B-scan ultrasonography showed significant thickening of the ocular wall in all quadrants of both eyes.Axial length was 19.86 mm(OD)and 19.85 mm(OS).Ultrasound biomicroscopy showed slit-like narrow anterior chamber angle and ciliary epithelial cysts in all quadrants in both eyes.Meanwhile,the patient had short stature,a short and thick neck.Further medical history revealed this patient with a previous diagnosis of mucopolysaccharidosis type I.Therefore,he was diagnosed with secondary glaucoma due to mucopolysaccharidosis.His IOP was initially controlled with maximal antiglaucoma drugs.Several months later,the patient had an acute attack of angle-closure glaucoma in his left eye that was unresponsible to either medications or laser therapies,necessitating trabeculectomy combined with prophylactic sclerotomy on the left eye.The same procedure was later performed on the right eye.The IOP is currently well-controlled and stable.Discussion and experience:The pathogenesis of secondary glaucoma due to mucopolysaccharidosis involves both open-angle and angle-closure mechanisms.The clinical assessment and surgical treatment of this condition are challenging and require continuous exploration in the future.

关 键 词：青光眼 眼压 黏多糖贮积症 角膜混浊 小眼球 

分 类 号：R73[医药卫生—肿瘤]