Case39-:An18-Year-OldManwithDiplopiaandProptosisoftheleftEye
左眼复视和突出的18岁男孩一例
PresentationofCase
An18-year-oldmanwasevaluatedatthishospitalbecauseofdiplopiaandproptosisofthelefteye.
18岁男孩，因左眼复视和突出入院。
Thepatienthadbeenwelluntilapproximately33monthsbeforethisevaluation,wheneyepainandperiorbitalswellingdevelopedontherightside.Thepainwasconstant,andtheuseofophthalmicketotifenfumaratedidnotproviderelief.Ontheeighthdayofillness,thepatientwasseenintheophthalmologyclinicatanotherhospital.Examinationrevealedvisualacuityof20/25intherighteyeand20/30inthelefteye,alongwith3mmofproptosisoftherighteyeandswelling,erythema,andptosisoftherighteyelid.Ophthalmicfluorometholoneandoraldoxycyclinewereprescribed.
患者既往体健，在进行检查前33个月一直处于健康状态，直到右侧出现眼部疼痛和眼眶周围肿胀。疼痛是持续的，使用眼用富马酸酮替芬无缓解。在发病第八天，医院的眼科就诊，右眼视力20/25,左眼视力20/30，右眼突出3mm,右眼睑肿胀、红斑、上脸下垂。处方为眼科氟甲基羟酮和口服强力霉素。
Fivedayslater,thepatientreturnedtotheophthalmologyclinicandreporteda1-dayhistoryoffuzzyvisionintherighteye.Hehadpersistentproptosis,ptosis,andeyelidswelling.Onexaminationoftherighteye,visualacuitywas20/50andtherewasanewsuperiorvisual-fielddefectinresponsetoconfrontation.Extraocularmotilitywasnormal.Threedaysafterthisvisit,magneticresonanceimaging(MRI)oftheorbits,performedaftertheintravenousadministrationofcontrastmaterial,revealedmild-to-moderateenlargementoftheinferior,medial,andsu-
periorrectusmusclesintherightorbit,whichhadresultedinnarrowingoftheposteriororbitalapex.Therewasenhancementoftheenlargedextraocularmuscles,aswellasabnormalhyperintensesignalonT2-weightedimages.ConcurrentMRIoftheheadwasnormal,aswerebloodlevelsofthyrotropinandfreethyroxine.Prednisone(40mgperday)wasprescribed.
5天后，患者回到眼科诊所,并述右眼视力模糊1天。有持续的突出、上睑下垂和眼睑肿胀。再次右眼检查,视力为20/50,并有一个新的视野缺损的对抗反应。眼外运动正常。本次访视3天后,静脉注射造影剂，MRI显示右眼眶内下直肌、内直肌、上直肌轻度至中度增大，导致眶后收缩，眼外肌增大，t加权图像显示异常高信号。示异常高信号。同期头部MRI正常，血促甲状腺素和游离甲状腺素水平正常。处方是强的松(每天40毫克)。
Duringthenext2weeks,thepainandeyelidswellingdiminishedandthevisualacuitynormalized,buttheproptosisandvisual-fieldrestrictionpersisted.Thepatientalsohaddecreasedcolorvisionintherighteye,withoutarelativeafferentpupillarydefect.Thedoseofprednisonewasdecreasedto20mgperday;after2weeksofthisdosingregimen,theproptosisandptosispersistedbuttheremainderoftheophthalmologicexaminationwasnormal.Thedoseofprednisonewasgraduallydecreasedoveraperiodof7.5monthsandthenstopped;symptomsresolvedandtheophthalmologicexaminationwasnormal.
2周后，疼痛及眼睑肿胀减轻，视力恢复正常，但眼球突出及视野受限持续。患者右眼也有色觉减退，没有相对的瞳孔传入缺陷。强的松剂量降低至每天20mg，服药两周后，突出和上睑下垂持续，但持续时间不明显。其余眼科检查均正常。强的松的剂量在7.5个月后逐渐减少，然后停止;症状消失，眼科检查正常。
Sevenmonthsafterdiscontinuationofprednisone(17monthsbeforethisevaluation),thepatientreturnedtotheophthalmologyclinicbecauseofa1-weekhistoryofrecurrenteyepainandperiorbitalswellingontherightside.Prednisonewasadministeredfor5daysatadoseof40mgperdayandthenwascontinuedatadoseof20mgperday.Duringthenext2.5weeks,thepainpersistedandtheswellingworsened.Thedoseofprednisonewasincreasedto60mgperday,andoralmethotrexateandfolicacidwereprescribed;thepainandswellingdiminished.Thedoseofprednisonewasgraduallydecreasedduringthenext2.5monthsandthenstopped.
停用强的松7个月后(本次评估前17个月)，患者因有1周复发性眼部疼痛和右侧眼眶周围肿胀的病史再次就诊。强的松给药5天,剂量为每天40毫克，然后继续剂量为每天20毫克。在接下来的2.5周，疼痛持续，肿胀加剧。强的松剂量增加到每天60mg，并规定了口服甲氨蝶呤和叶酸；疼痛和肿胀消失。在随后的2.5个月，强的松的剂量逐渐减少,然后停止。
Onemonthlater(13monthsbeforethisevaluation),thepainintherighteyerecurredandthepatientreturnedtotheophthalmologyclinic.Prednisone(40mgperday)wasprescribed,andthedoseofmethotrexatewasincreased.Duringthenext3months,painrecurredeachtimethedoseofprednisonewasdecreasedto20mgperday;naproxenwasprescribed.
一个月后(本次评估前13个月),右眼疼痛复发，患者回到眼科诊所。开强的松每天40毫克，甲氨蝶呤的剂量增加。在接下来的3个月，当强的松的剂量降低到每天20mg时，疼痛复发，加用萘普生。
Tenmonthsbeforethisevaluation,thepatientwasseenintherheumatologyclinicattheotherhospital.Hereportedweightgainbutnofever,fatigue,eyepain,visionproblems,dryeyes,dry
mouth,oralornasalulcerations,shortnessofbreath,arthralgias,orrash.Onexamination,hehadacushingoidappearanceandwasinnoapparentdistress.Thevitalsignswerenormal.Thebody-massindex(BMI;theweightinkilogramsdividedbythesquareoftheheightinmeters)was29.7.Therewasnoconjunctivalinjection,andextraocularmotilitywasnormal.Hedidnothavethyromegaly,lymphadenopathy,rash,orsynovitis;theremainderoftheexaminationwasnormal.LaboratorytestresultsareshowninTable1.Prednisoneandfolicacidwerecontinued,andoralmethotrexatewaschangedtosubcutaneousmethotrexate.Duringthenext4months,thedoseofprednisonewasgraduallydecreasedto2.5mgperday,withoutrecurrenceofpain.Thealanineaminotransferaselevelincreasedto81Uperliter(referencerange,0to50);thedoseofmethotrexatewasdecreased,andthelevelnormalized.
评估前10个月,医院的风湿病门诊就诊。体重增加，但没有发烧、疲劳、眼痛、视力问题、眼干、口干、口腔或鼻溃疡、呼吸短促、关节痛或皮疹。经检查，他有库欣式综合征患者的外貌，没有明显的痛苦。生命体征正常。身体质量指数;体重(公斤)除以身高(米)的平方是29.7。无结膜注射，眼外运动正常。无胸腺肥大、淋巴结病、皮疹或滑膜炎:；其余的检查都很正常。实验室试验结果见表1。继续使用强的松和叶酸，口服甲氨蝶呤改为皮下甲氨蝶呤注射。在接下来的4个月里，强的松的剂量逐渐减少到每天2.5毫克，没有疼痛的复发。丙氨酸转氨酶水平提高到81∪/升(参考范围，0~50);甲氨蝶呤剂量降低,水平归一。
Fourmonthsbeforethisevaluation,pressuretionattherheumatologyclinic,thebloodpressurewas/77mmHgandtheothervitalsignswerenormal.TheBMIwas30.7.Thepatienthadacushingoidbodyhabitusandfacialacne.Hedidnothaveproptosisoreyelidedema.Extraocularmotilitywasnormal,aswastheremain-deroftheexamination.Thedoseofsubcutaneousmethotrexatewasincreased,andthedoseofprednisonewasincreasedto20mgperday.Duringthenext6weeks,thepaininthelefteyepersisted,andperiorbitalswellingandproptosisdevelopedontheleftside.Thedoseofprednisonewasincreasedto40mgperday,withoutimprovement,andthento60mgperday.Duringthistime,cellulitisoftherightlowerabdominal
walldeveloped.Acourseoforaldoxycyclinewasadministered,followedbyacourseoforalcephalexinandtopicalmupirocin;methotrexatewasdiscontinued.Eightweeksbeforethisevaluation,theproptosispersisted.Mycophenolatemofetilwasprescribed,andthedoseofprednisonewasdecreasedto40mgperday.Thepatientwasreferredtoarheumatologistandophthalmologistatthishospitalforfurtherevaluation.
在此评估的四个月前，左眼出现了压迫性疼痛。在风湿门诊就诊，血压为/77毫米汞柱,其他生命体征正常。BMI是30.7。患者有库欣综合征身体体征和面部痤疮。无眼球突出或眼睑水肿。眼外运动正常，其余的检查也是如此。皮下甲氨蝶呤剂量增加，强的松剂量增加至20mg/天。接下来的6周，左眼持续疼痛，左侧眼眶周围肿胀和眼球突出。强的松剂量增加到每天40毫克，没有改善，然后增加到每天60毫克。在此期间，右下腹壁出现蜂窝织炎。给予一个疗程的口服强力霉素，接着是一个疗程的口服头孢氨苄和局部莫匹罗星;甲氨蝶呤停止。在此评估前8周，突出持续存在。处方是霉酚酸酯，强的松剂量减少至每天40mg。,医院的风湿病专家和眼科专家作进一步评估。
Onevaluationintheophthalmologyandrheumatologyclinicsatthishospital,thepatientreportedproptosisanda2-weekhistoryofdiplopiainalldirectionsofgaze,withoutpain.Hehadseasonalallergicrhinitisandaremotehistoryofesotropia,asthma,tonsillectomy,adenoidectomy,andmyringotomy.Ninemonthsearlier,hehadhadanepisodeofbilateralsubmandibularsialadenitis;atestformumpsvirusinfectionhadbeennegative.Medicationsincludedprednisone,mycophenolatemofetil,cholecalciferol,andSaccharomycesboulardii.Ibuprofenhadcauseditching.ThepatientwasacollegestudentandlivedinthesoutherncentralUnitedStates.Hedidnotdrinkalcohol,smoketobacco,oruseillicitdrugs.HisgrandmotherhadGraves’disease.
经本院眼科及风湿病门诊评估，患者有眼球突出及双耀复视病史2周，无疼痛。有季节性变应性鼻炎和内斜视、哮喘、扁桃体切除术、腺样体切除术和鼓膜切开术的长期病史。九个月前，他曾出现过1次双侧下颌下涎腺炎;对流行性腮腺炎病毒感染的检测呈阴性。药物包括强的松、霉酚酸酯、胆钙化醇和布洛芬。病人是一名大学生,住在美国中南部。他不喝酒，不吸烟，也不使用违禁药物。他的祖母患有格雷夫斯病。
Onexamination,thepatienthadacushingoidappearance.Hehadexotropiaandminimalproptosisofthelefteye,palloroftherightopticdisk,andarightinferiorcentraldefectonvisualfieldtesting.Examinationoftheskinrevealedstriaeandacne(Fig.1).Theremainderoftheexaminationwasnormal.LaboratorytestresultsareshowninTable1.Urinalysiswasnormal;theurineproteinlevelwas15.7mgperdeciliter(referencerange,0.0to13.5),andtheurineprotein:creatinineratiowas0.05(referencerange,0.15).Imagingstudieswereobtained.
经检查，病人表现为库欣样。他的左眼有外斜视和轻微凸出，右侧视盘苍白，在视野检查中有右下中心缺损。皮肤检查显示条纹和痤疮(图1)。其余其余检查正常。实验室试验结果见表1。验尿是正常的;尿蛋白水平为15.7毫克/分升（参考范围0.0—13.5），尿蛋白质:肌酐的比率为0.05（参考范围：＜0.15）。进一步安排了影像学检查。
Figure1.ClinicalPhotographs.
Thepatienthascushingoidfaciesandacne(PanelA),anadductiondefectandmildproptosisofthelefteye(PanelsBandC),andprominentcutaneousstriae(PanelD).
图1临床图片。患者有丘疹样面容和痤疮（图A），内收缺陷和轻度左眼突出（图B），以及突出的皮肤纹（图D）
MRIoftheorbitswasperformed(Fig.2).Contrast-enhancedT1-weightedimages,obtainedwithandwithoutfatsuppression,showedabnormalfusiformenlargementandincreasedenhancementoftheleftmedialandinferiorrectusmuscles,withacentralareaofhypoenhancementoftheleftmedialrectusmuscle.Therewasrelativesparingofthemusculotendinousinsertionsandnosubstantialsurroundingfatstranding.Inaddition,T2-weightedimagesshowedhyperintensesignalintheleftmedialandinferiorrectusmuscles,afindingsuggestiveofedema.Therewasmildproptosisoftheleftglobe.Therightorbitandparanasalsinuseswereessentiallyunremarkable.
进行了眼眶的核磁共振成像检查（图2）。对比增强t1加权图像，观察有无脂肪抑制，出现了左侧内、下直肌呈异常梭状膨大强化，与一个中心左侧内直肌增强不全区。肌腱鞘的插入相对较少，周围没有实质性的脂肪绞合。此外，t2加权图像显示左侧为高信号内直肌和下直肌，这个发现暗示了水肿的产生。有轻微的眼球突出。鼻窦和副鼻窦等区域基本不明显。
表1
Toconvertthevaluesforcalciumtomillimolesperliter,multiplyby0..Toconvertthevaluesforureanitrogentomillimolesperliter,multiplyby0..Toconvertthevaluesforcreatininetomicromolesperliter,multiplyby88.4.Toconvertthevaluesforglucosetomillimolesperliter,multiplyby0..Toconvertthevaluesforbilirubintomicromolesperliter,multiplyby17.1.Toconvertthevaluesforcholesteroltomillimolesperliter,multiplyby0..Toconvertthevaluesfortriglyceridestomillimolesperliter,multiplyby0.01.
将钙的值转换为每升的毫摩尔值，乘以0.。将尿素氮的值转换为每升的毫摩尔，乘以0.。要把肌酐的值转化为每升的微摩尔，乘以88。4。将葡萄糖的值转换为每升的毫摩尔，乘以0.。将胆红素的值转换为每升的微摩尔，乘以17.1。将胆固醇值转换为每升的毫摩尔值，乘以0.。将甘油三酯的值转换为每升的毫摩尔数，乘以0.01。
Referencevaluesareaffectedbymanyvariables,includingthepatientpopulationandthelaboratorymethodsused.TherangesusedatMassachusettsGeneralHospitalareforadultswhoarenotpregnantanddonothavemedicalconditionsthatcouldaffecttheresults.Theymaythereforenotbeappropriateforallpatients.
参考值受到许多变量的影响，包括患者群体和所使用的实验室方法。医院使用的范围是为成年人谁没有怀孕，没有医疗条件，可能影响结果。因此，它们可能并不适合所有患者。
Ifthepatientisblack,multiplytheresultsby1.21.
如果病人是黑种人，则将结果乘以1.21。
行项目：变量，医院参考范围，评估十个月前在医院风湿病科，医院参考范围，这次评估在医院风湿病科的结果。
列项目：血细胞比容，血红蛋白，白血球计数，微分数，中性粒细胞，不成熟的细胞，淋巴细胞，单核细胞
。嗜碱性粒细胞，嗜酸性粒细胞，血小板计数，红细胞数，平均微粒体，平均红细胞血红蛋白，平均红细胞血红蛋白浓度，红细胞分布宽度，红细胞沉降率，钠，钾，氯化物，二氧化碳，阴离子间隙，尿素氮，肌酐，肾小球滤过率，葡萄糖，丙氨酸转氨酶，天冬氨酸转氨酶，碱性磷酸酶，总胆红素，蛋白总计，白蛋白球蛋白，胆固醇总计，高密度脂蛋白，低密度脂蛋白，甘油三酯，糖化血红蛋白，促甲状腺激素，C3，C4，免疫球蛋白，IgG1，IgG2，IgG3，IgG4，IgA，IgM，IgE，血清蛋白电泳，胞质抗体，乙肝核心抗体总计，干扰素—4释放检测结核分枝杆菌。
Figure2.MRIoftheOrbits.
Anaxial,contrastenhanced,fat?saturatedT1-weightedimageoftheorbits(PanelA)showsfusiformenlargementandincreasedenhancementoftheleftmedialrectusmuscle,withacentralareaofhypoenhancement(arrow).Thereissparingofthemusculotendinousinsertionandnosubstantialsurroundingfatstranding.Acoronal,contrast?enhancedT1-weightedimageoftheorbits(PanelB)showsabnormalenlargementandcentralhypoenhancementoftheleftmedialrectusmuscle(arrow)andabnormalenlargementoftheleftinferiorrectusmuscle(arrowhead).AnaxialT2-weightedimageoftheorbits(PanelC)showsincreasedsignalintensityintheenlargedleftmedialrectusmuscle(arrow),afindingsuggestiveofedema.Thereismildproptosisoftheleftglobe.
图2。轨道磁共振成像。轨道轴向增强脂肪饱和t1加权图像(A图)显示左侧内直肌梭形增大，增强增强，中央区域增强减退(箭头)。肌肉腱的插入保留，周围没有实质性的脂肪滞留。日冕状增强t1加权成像(B面)显示左侧内直肌异常增大和中央低强化(箭头)，左侧下直肌异常增大(箭头)。轨道轴向t2加权图像(C图)显示增大的左内直肌信号强度增加(箭头)，提示水肿。左眼球轻度突出。
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