CAJ | 학술논문

目的探讨McCune-Albright综合征(MAS)的眼部临床特点.方法系列病例研究.分析从2008年1月至2011年6月就诊北京协和医院眼科的6例MAS患者最佳矫正视力、视野、色觉、视网膜神经纤维层厚度、视觉诱发电位、眼底、视神经管CT扫描,多科综合治疗方法及随访结果.结果 6例MAS患者中男性3例,女性3例,就诊年龄在4～ 43岁(中位数16岁).视神经管CT扫描发现6例(12只眼)MAS患者视神经管有不同程度的缩窄,其中全周缩窄3例(6只眼)、部分缩窄3例(6只眼),视神经管全周缩窄中有2例(3只眼)发现有视神经病变,其余9只眼均未发现有视神经病变.有4例(8只眼)MAS患者视神经被不同程度拉长.结论 MAS患者视神经管有不同程度的缩窄,部分患者出现视神经病变,应当在临床上受到高度关注.
목적 탐토McCune-Albright종합정(MAS)적안부림상특점.방법 계렬병례연구.분석종2008년1월지2011년6월취진북경협화의원안과적6례MAS환자최가교정시력、시야、색각、시망막신경섬유층후도、시각유발전위、안저、시신경관CT소묘,다과종합치료방법급수방결과.결과 6례MAS환자중남성3례,녀성3례,취진년령재4～ 43세(중위수16세).시신경관CT소묘발현6례(12지안)MAS환자시신경관유불동정도적축착,기중전주축착3례(6지안)、부분축착3례(6지안),시신경관전주축착중유2례(3지안)발현유시신경병변,기여9지안균미발현유시신경병변.유4례(8지안)MAS환자시신경피불동정도랍장.결론 MAS환자시신경관유불동정도적축착,부분환자출현시신경병변,응당재림상상수도고도관주.
Objective To present the ophthalmological characteristics of McCune-Albright syndrome (MAS).Methods Case series study.Best corrected visual acuity (BCVA),visual field,color vision,retinal nerve fiber layer (RNFL) thickness,visual evoked potentials (VEP),fundus examination,computed tomography (CT) imaging of the optic canal,follow up results of multidisciplinary comprehensive treatments were analyzed.Results Of the 6 patients,three were male and three were female.Their age were between 4 to 43 years old (median,16 years old ). Based on CT imaging of the optic canal,encasement of the optic canal was found in all 6 patients (12 eyes ); three patients (6 eyes ) had circumferential encasement of the optic canal ; the remaining 3 patients ( 6 eyes ) had partial encasement of the optic canal. Two patients (3 eyes ) with circumferential encasement of the optic canal had optic neuropathy versus none in the remaining 9 eyes with circumferential or partial optic canal encasement.In addition,lengthening of the optic nerve was found in all 4 patients ( 8 eyes).Conclusion Optic canal encasement and lengthening of the optic nerve are common in MAS patients,which is worthy of attention in clinical practice.