眶尖是眼眶锥形向后的尖端。有视神经管通过,视神经管眶口通眶腔,颅口通颅中窝,里面有视神经、视神经被膜、眼动脉等。其外下方紧邻眶上裂,眶上裂(Superiororbitalfissure)位于眶腔上部的开口。动眼神经、滑车神经、三叉神经眼神经支(泪腺N、额N、鼻睫状N)、外展神经、脑膜中动脉的眶支、泪腺动脉脑膜返支以及眼上静脉经由此处入眶。视神经管、眶上裂为沟通眼眶和颅中窝的交通要道,管内、裂内有诸多重要的神经、血管通行。无论是眶尖的还是颅中窝的,无论是视神经管的还是眶上裂的肿瘤、炎症、脓肿以及外伤骨折与血肿,都可累及这些神经血管,导致复杂的临床表现,称为眶尖综合征(orbitapexsyndrome)。
先回顾下海绵窦区和眶上裂的解剖:
VisuallossandophthalmoplegiaareoftentheinitialmanifestationsofanOAS.Thus,theophthalmologistmaybethefirstphysicianconsultedbyapatientwithanOAS.
视力丧失和眼肌麻痹通常是眶尖综合征的最初表现。因此,OAS患者可能咨询的第一个医生是眼科医生。
Periorbitalorfacialpainmayreflectinvolvementoftheophthalmic(V1)ormaxillary(V2)branchofthetrigeminalnerve.PeriorbitalpainisoneofthediagnosticcriteriaforTolosa–Huntsyndrome(THS),anidiopathicinflammatorysyndromeoftheorbitalapex.However,theabsenceofpaindoesnotexcludeaprocesswithintheorbitalapex.Itisimportanttotesttheperiorbitalskinandthecornealreflexestodetectasymmetryinsensation.
眶周或面部疼痛可能反映了三叉神经的眼支(V1)或上颌支(V2)分支的受累。眶周痛是TolosaHunt综合征(THS)的诊断标准之一,这是一种眶尖的特发性炎性综合征。然而,没有疼痛并不能排除眶尖疾病。对眶周皮肤和角膜反射进行感觉是否对称的检测是很重要的。
Infectious,inflammatory,andneoplasticconditionsmaybeassociatedwithproptosis.Vascularcausesofacavernoussinussyndrome,suchasacarotid–cavernousfistula,classicallyareassociatedwithpulsatileproptosis.
感染性、炎症性和肿瘤性疾病可能与突眼有关。海绵窦综合征的血管原因通常与搏动性突眼相关,如颈动脉海绵窦瘘。
Toassessforopticnervedysfunction,measurementofbest-correctedvisualacuity,examinationofthepupilsforthepresenceofanafferentpupillarydefect,andcolorvisiontestingshouldbeincluded.Visualfieldtestingwithkineticorstaticperimetrymayrevealsubtlevisualfielddeficitswhenvisualacuityisnormal.WeroutinelyuseHumphreyautomatedperimetrytoassessforvisualfielddefects.
视神经功能障碍的评估包括测量最佳矫正视力,检查瞳孔是否有光反射障碍,以及色觉测试。当视力正常时,使用动态或静态视野的视野测试可能会显示出细微的视野缺陷。我们经常使用Humphrey自动视野测量来评估视野缺陷。
OpticatrophytypicallydevelopsoverweekstomonthsinpatientswithanOAS.Thus,opticatrophymayormaynotbepresentinapatientwithanOAS,anditsabsenceshouldnotprecludefurtherevaluationifotherclinicalfindingssuggestthatanopticneuropathyispresent.
在OAS患者中,视神经萎缩通常会持续数周到数月。因此,视神经萎缩可能或不存在于OAS的患者中,如果其他临床表现表明视神经病变存在,则不应终止进一步的评估。
DiplopiamaybethepresentingsymptominSOFS,OAS,orCSS.Thepatternofanoculardeviationisespeciallyimportantintheevaluationofasingleocularmotornervepalsy(eg,anesotropiagreateringazeipsilateraltoasixthnervepalsyorhypertropiaincreasingincontralateralgazeandonipsilateralheadtiltinafourthnervepalsy).However,becausemultiplecranialnervesmaybeinvolved,adistinctpatternmaybedifficulttodetect.
复视可能是眶上裂综合征、眶尖综合征或海绵窦综合征中的表现症状。在单眼运动神经麻痹的评估中,眼位的模式尤其重要。然而,由于可能累及到多个颅神经,因此很难发现一个明显的模式。
SeveralauthorshavereportedacorrelationbetweentheinitialnumberofcranialnervesinvolvedandthelikelihoodofhavingaCSS.Intheirretrospectivestudyof68patientswithcranialneuropathies,CSSwasfoundin7of34patients(18%)whopresentedwithacranialmononeuropathyandinsevenofninepatients(78%)whopresentedwithinvolvementoffourcranialnerves.Theoculomotorandabducensnerveswerethemostfrequentlyinvolvedcranialnerves,followedbythetrochlearnerve
有作者报告说,最初受累的颅神经数目与海绵窦综合征的可能性有关联。在对68例颅神经病变患者进行的回顾性研究中,34例海绵窦综合征患者中有7例(18%)患者出现颅神经病变,9例患者中有7例(78%)累及了四条颅神经。动眼神经和外展神经是最受累的脑神经,其次是滑车神经。
Causes
Orbitalapexsyndromesmaybecausedbyinflammatory,infectious,neoplastic,iatrogenic/traumatic,orvascularprocesses:
眶尖综合征可能是由炎症、感染、肿瘤、医源性/创伤或血管性疾病引起。
Inflammatory
1.Sarcoidosis2.Systemiclupuserythematosus3.Churg–Strausssyndrome4.Wegenergranulomatosis5.THS6.Giantcellarteritis7.Orbitalinflammatorypseudotumor8.Thyroidorbitopathy
1.结节病2.系统性红斑狼疮3.Churg–Strauss综合征(变应性肉芽肿血管炎或过敏性肉芽肿性血管炎)4.韦格纳肉芽肿5.THS6.巨细胞动脉炎7.眼眶炎性假瘤8.甲状腺眼病
Infectious
1.Fungi:Aspergillosis,Mucormycosis2.Bacteria:Streptococcusspp.,Staphylococcusspp.,Actinomycesspp.,Gram-negativebacilli,anaerobes,Mycobacteriumtuberculosis3.Spirochetes:Treponemapallidum4.Viruses:Herpeszoster
感染1.真菌:曲霉病,毛霉菌病2.细菌:链球菌,葡萄球菌,放线菌,革兰氏阴性杆菌,厌氧菌,结核分枝杆菌3.螺旋体:梅*螺旋体4.病*:带状疱疹
Neoplastic
1.Headandnecktumors:nasopharyngealcarcinoma,adenoidcysticcarcinoma,squamouscellcarcinoma
2.Neuraltumors:neurofibroma,meningioma,ciliaryneurinoma,schwannoma
3.Metastaticlesions:lung,breast,renalcell,malignantmelanoma
4.Hematologic:Burkittlymphoma,non-Hodgkinlymphoma,leukemia
5.Perineuralinvasionofcutaneousmalignancy
肿瘤1.头颈肿瘤:鼻咽癌,腺样囊性癌,鳞状细胞癌2.神经系统肿瘤:神经纤维瘤、脑膜瘤、睫状神经瘤、神经鞘瘤3.转移瘤:肺、乳腺、肾细胞、恶性黑素瘤4.血液系统肿瘤:Burkitt淋巴瘤,非霍奇金淋巴瘤,白血病5.皮肤恶性肿瘤的神经浸润
Iatrogenic/traumatic
A.Iatrogenic1.Sinonasalsurgery2.Orbital/facialsurgery
B.Traumatic1.Penetratinginjury2.Nonpenetratinginjury3.Orbitalapexfracture4.Retainedforeignbody
医源性/创伤A.医源性1.鼻腔鼻窦术后2.眶/面部手术B.创伤性1.穿透性伤2.非穿透伤3.眶尖骨折4.异物
Vascular1.Carotidcavernousaneurysm2.Carotidcavernousfistula3.Cavernoussinusthrombosis4.Sicklecellanemia
血管1.颈动脉动脉瘤2.颈动脉海绵窦瘘3.海绵窦血栓形成4.镰状细胞贫血
OtherA.Mucocele
其他A.粘液囊肿
葱花