1第一部分SECTION1

A60-year-oldmanpresentedtoourclinicwitha6-monthhistoryofprogressivevisuallossinthelefteye.Twelvemonthsprior,heexperiencedanacutelefthemianopiasecondarytoastroke.Hewasanticoagulatedandfullyrecovered.Sixmonthsfollowingthestroke,hebegantonoticeblurredvisioninhislefteye.Therewasnoformalophthalmicexaminationperformedatthattime,butaplasticsurgeonbelievedthataleftmedialuppereyelidmasslesionwascausingdistortionofhisvision,andproceededtoexcisionbiopsy.HistopathologyrevealedRosai-Dorfmandisease(RDD).Postoperatively,thepatient’svisuallosscontinuedtoworsenandhewasreferredtoourclinic.

患者60岁男性,以“进行性左眼视力丧失6个月”为主诉就诊。12个月前,患者因脑卒中突发左侧偏盲,随后接受了抗凝治疗并完全康复。卒中后6个月,患者渐感左眼视物模糊。但患者当时没有进行正式的眼科检查,而整形外科医生认为其视物模糊是由左上眼睑内侧的肿块引起,并进行切除手术和活检。组织病理学检查提示为罗道病(Rosai-Dorfmandisease,RDD)。但术后患者的视力丧失继续恶化,遂转诊至我们诊所。

Onexamination,visualacuitywas6/6ontherightand6/12ontheleft.ThepatientcorrectlyidentifiedallIshiharacolorplateswiththerighteye,andonlythetestplateandoneotherplatewiththelefteye.Therewasalsoaleftrelativeafferentpupillarydefect.Funduscopyrevealedsubtleleftopticdiscpallor.Orbitalexaminationwasnormal.Humphreyvisualfieldofthelefteyeshowedsuperiorandinferiorarcuatedefectsandanormalrightvisualfield(figure,A).Neuroimagingrevealedadural-based,intenselyenhancingmassintheleftanteriorcranialfossa,whichhadbeendocumentedasasmallincidentalmeningioma6monthsearlier(figure,BandC).

体格检查发现:右眼视力为6/6,左眼视力为6/12;石原氏色盲测验检查提示右眼能正确识别所有色图,而左眼只能识别一块测试图和其他另一块色图;左侧相对性传入性瞳孔障碍;眼底检查可见左侧视乳头轻微苍白;眼眶检查正常;汉弗莱视野检查可见右眼视野正常,左眼上下弓形缺陷(图,A)。神经影像检查显示左侧前颅窝硬脑膜见一高信号肿块,6个月前报告为小的偶然发现的脑膜瘤(图,B和C)。

Questionsforconsideration:

1.Whatisthedifferentialdiagnosis?

2.Whatistheappropriatenextstepindiagnosticevaluation?

问题思考:

1.鉴别诊断有哪些?

2.下一步该采取哪些检查以助于诊断?

2SECTION2第二部分

Thedifferentialdiagnosesforagrowingintracraniallesionthatmaymimicmeningiomaincludelymphoma,metastaticcarcinoma,Langerhanscellhistiocytosis,neurosarcoidosis,granulomatousdiseases,andneurofibromatosistype1.1Sincethepatient’svisionwasthreatenedandatissuediagnosiswasrequiredfordefinitivediagnosis,hewasreferredtoaneurosurgeonforurgentdebulkingsurgeryviaendoscopictranssphenoidalapproach.Giventhepatient’shistoryofRDDandtheexpandinglesion,intracranialRDDwassuspected.Intraoperatively,thesurgeonnotedseveredistortionoftheleftopticnervewithinferomedial







































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