视神经鞘脑膜瘤50例临床分析

目的 探讨视神经鞘脑膜瘤的临床表现、影像学特征及治疗原则,以求更好的治疗效果.方法 回顾性分析1999年6月至2006年5月诊治的视神经鞘脑膜瘤患者50例(53眼),对全部病例的临床资料、标准化A/B超、彩色多普勒成像、CT和MRl图像、治疗方案和手术方式进行分析.结果视神经鞘脑膜瘤多发生于中年女性.主要表现为视力下降或丧失、眼球突出,眼底检查可见视盘慢性水肿性萎缩和视睫状静脉.标准化A/B超和CT有特征性表现,可以显示视神经增粗、眶内软组织肿块,MRI对于观察视神经管内及颅内蔓延情况,效果更好.治疗原则包括观察、放射治疗、手术切除或与放射治疗结合等.结论 视神经鞘脑膜瘤是一种较常见的眼眶肿瘤,早期正确诊断较难,影像学检查有助于诊断和鉴别诊断.确诊后可根据病变位置、大小、范围、发展趋势和患者具体情况予以个体化治疗.     

眶尖部及视神经肿瘤的早期诊断

目的探讨眶尖部及视神经肿瘤在眼球突出前的临床表现、影像学检查特征及临床诊断要点。方法回顾性分析22例无明显眼球突出单侧眼眶肿瘤患者的病历资料,包括主要症状、首发表现、临床诊治过程及影像学检查结果。结果22例肿瘤患者中,海绵状血管瘤6例,神经鞘瘤、视神经鞘脑膜瘤、鼻窦及鼻咽腔恶性肿瘤眶内蔓延各4例,后组筛窦黏液囊肿2例,神经纤维瘤和视神经胶质瘤各1例。以视力下降为首发症状者17例,视力下降合并复视者3例。全部患者均在CT或MRI检查后确诊。肿瘤位于眶尖部或起源于视神经。结论眶尖部及视神经肿瘤的早期症状是视功能障碍,无眼球突出表现。视力呈缓慢进行性下降,按视神经炎治疗效果差。CT或MRI检查对早期诊断起重要作用。     

视神经鞘脑膜瘤的诊断及治疗

目的 探讨视神经鞘脑膜瘤的早期诊断要点及合理的治疗方案.方法 回顾性分析12例(12只眼)原发性视神经脑膜瘤的早期临床及影像检查特征.结果 单侧眼球突出,视力减退,视盘萎缩和眼球运动障碍,是其主要表现,CT和MRI能确诊.结论 CT和MRI必须做多层面强化扫描,MRI必须做脂肪抑制技术.在早期诊断基础上,制定手术或临床观察方案.     

眼眶横纹肌肉瘤临床分析

探讨眼眶横纹肌肉瘤的临床诊断及治疗方法。方法 :对 2 0年间收治的 2 6例原发性眼眶横纹肌肉瘤患者的临床表现、影像学特征和治疗进行分析。结果 :2 5例有眶内占位病变及炎症体征 ,如眼球突出、眶部肿块、眼睑及结膜充血水肿、眼球运动受限等。B超、彩色多谱勒超声、CT、X线等影像学检查提示眶内占位病变。结论 :影像学检查可提供定性、定位诊断 ,治疗方法为手术、化学治疗及放射治疗相结合。     

原发于眼眶脑膜瘤的CT图像分析

目的分析原发于眼眶脑膜瘤的常见CT图像特征,探讨CT影像对原发于眼眶脑膜瘤的诊断价值。方法回顾性分析51例经病理确诊的原发于眼眶脑膜瘤患者的临床资料和CT图像表现。探讨CT对脑膜瘤的诊断及鉴别诊断价值。结果 51例原发于眼眶脑膜瘤患者中视神经鞘脑膜瘤43例,眶骨膜脑膜瘤8例。视神经鞘脑膜瘤43例中肿瘤形状呈管状20例,块状12例,其他形状11例;呈现车轨征16例,钙化斑6例。8例眶骨膜脑膜瘤均有蝶骨大翼和(或)小翼骨膜增厚及眶外壁骨质增生,其中视神经向内移位6例。所有病例中肿瘤蔓延者8例,双眼眶发病2例。结论 CT图像可清晰显示肿瘤的位置、形状、范围,尤其是眶骨壁改变,对于原发于眼眶脑膜瘤的诊断及治疗具有重要的临床指导意义。     

眶颅沟通性肿瘤的诊断和手术治疗

目的：探讨眶颅沟通肿瘤的临床特征、种类、诊断方法及手术治疗。方法：选择60例手术病理或随访结果证实的眶颅沟通肿瘤患者，所有患者均行CT扫描，54例行MRI扫描，其中增强扫描42例。20例行视神经管X光像。经翼点入路切除肿瘤12例，经额入路切除肿瘤21例。结果：最常见的阳性体征是眼球突出、眶压升高和视力下降等，最常见的症状是头痛。较多见的眶颅沟通肿瘤是脑膜瘤、骨纤维异常增生症和转移癌。眶颅沟通肿瘤的CT、MRI扫描及视神经孔X光像有一定影像学特征。眶颅沟通的途径包括视神经管、眶上裂和眶颅交界骨壁。全切肿瘤32例，次全切肿瘤1例。结论：掌握各种眶颅沟通肿瘤的临床特征、病种分类，综合运用CT、MRI和X光等检查方法可提高诊断和鉴别诊断水平。翼点入路和经额入路是眶颅沟通肿瘤的重要手术方法。     

眶脑膜瘤误诊1例

眶脑膜瘤误诊１例南京军区总医院王义，黄振平眼眶脑膜瘤是常见肿瘤之一，如发生于眶尖部，早期多误诊为视神经炎或视神经萎缩，直至发生了眼球突出方确诊，结果使患者丧失了视力，增加了手术治疗的难度［１］。我院曾遇１例左眼眶脑膜瘤误诊１１年，现报告如下。患者女４...     

视神经肿瘤36例临床分析

分析视神经源性肿瘤患者的病史、临床表现、影像学检查和治疗方法,为其诊断和治疗提供帮助。 方法：对视神经胶质瘤和视神经鞘脑膜瘤患者的临床资料进行分析。 结果：视神经胶质瘤的15例患者中以发现眼球突出为主诉的病例为6例,以发现视力下降为主诉的5例,眼球突出合并视力下降的2例;视神经鞘脑膜瘤组21例患者中以眼球突出为主诉的病例为13例,视力下降5例,眼球突出合并视力下降的3例。超声检查、CT、MRI检查均有典型表现;30例患者经手术治疗,6例经伽玛刀治疗。 结论:视神经源性肿瘤在眼部检查及影像学表现上有一定的特征性,对于辅助诊断具有重要价值;治疗方案应视患者的具体情况综合制定,目前以手术及放射治疗为主。     

眶尖部肿瘤的诊断与治疗

本文回顾性分析了我院诊治的眶尖部肿瘤２９例。病理检查为海绵状血管瘤１０例，神经鞘瘤８例（其中复发性神经鞘瘤３例），视神经鞘脑膜瘤７例，继发肿瘤及转移瘤３例。眶尖肿瘤主要表现早期视力障碍，轻度眼球突出，眼底视盘水肿或萎缩，早期易误诊。ＣＴ及ＭＲＩ显示眶尖部肿瘤，手术难度较大，合并症多。本文讨论了几种常见眶尖肿瘤的临床诊断特点、手术治疗原则及注意事项     

眼眶组织细胞增生症X临床分析

目的 探讨眼眶组织细胞增生症X的临床表现、影像学特征、诊断方法及治疗原则。方法 对47例(54只眼)眼眶组织细胞增生症X患者的临床症状、眼部体征、影像学检查、治疗方法及随访情况等临床资料进行分析,其中嗜酸性肉芽肿患者17例(19只眼),韩—薛—柯病患者30例(35只眼)。结果 嗜酸性肉芽肿好发于青少年,多单发于眶内。韩—薛—柯病多见于5岁以下的男性患儿,以多灶性组织细胞增生为特征,除眼眶肿瘤外,还有肝、脾、淋巴结肿大等眶外表现,典型患者具有三联征(眼球突出、尿崩症及多灶性颅骨缺失)。患者多以眼球突出、眶区发现肿块就诊。颅部X线片示颅骨多灶性缺失,B超及彩色多普勒示眶内软组织肿物,CT可同时显示眶骨缺失和软组织肿块,MRI可示眶内肿瘤及颅内受累情况。结论 特征性的影像学表现有助于组织细胞增生症X的诊断。治疗宜采用综合疗法,即在手术治疗的基础上配合局部放疗及化疗。预后与分型有关,嗜酸性肉芽肿预后最好,婴儿型组织细胞增生症预后最差。     

CT和MRI检查在视神经鞘脑膜瘤诊断中的价值

目的探讨CT和MRI检查在诊断视神经鞘脑膜瘤中的价值。方法本研究15例视神经鞘脑膜瘤均经手术及病理检查确诊,并均于术前行CT和MRI检查。结果15例视神经鞘脑膜瘤患者中,增强CT仅显示2例颅内蔓延;平扫MRI显示2例颅内蔓延;而增强MRI显示9例颅内有肿瘤。结论增强MRI在诊断视神经鞘脑膜瘤颅内蔓延中非常有价值,可显示增强CT和MRI平扫未显示的病变,应将其作为视神经鞘脑膜瘤术前、术后常规检查。     

CT、MRI在眼眶脑膜瘤诊断中的应用

本文对２２例进行ＣＴ、ＭＲＩ影像检查的眼眶脑膜瘤进行分析，其中，视神经鞘脑膜瘤１０例，眶内脑膜瘤５例，蝶骨嵴脑膜瘤６例，眶内、颅内与副鼻窦三腔脑膜瘤１例，术后得到随访者７例。ＣＴ、ＭＲＩ相互补充提高诊断的符合率，对肿瘤做出定位诊断，有利于制定治疗方案。术后随访中ＭＲＩ比ＣＴ更有优势，可鉴别肿瘤复发与术后瘢痕组织，给早期治疗提供了可能性。本文讨论ＣＴ、ＭＲＩ的优缺点及眼眶脑膜瘤的ＣＴ、ＭＲＩ表现。     

眶尖部肿瘤及其外科治疗

为探讨眶尖占位性病变的早期诊断要点及恰当的手术入路，分析１４例（１４只眼）原发性眶尖占位的临床表现及手术治疗，表明眶尖占位的早期临床特征是视功能减退而不伴有眼球突出，ＣＴ及ＭＲＩ能确诊。在早期诊断的基础上，根据肿瘤与视神经的位置关系，采用３种手术入路有助于全切肿瘤和保护视功能。     

Neural orbital tumors

Primary tumors of the optic nerve (optic nerve sheath meningiomas and optic nerve astrocytomas) are important causes of visual morbidity. They may elude clinical detection and have the potential to be lethal. Their treatment remains controversial but often includes radiation. The optic nerve may be involved secondarily by peripheral nerve tumors in the orbit or by metastatic and infiltrative neoplasms. Optic neuropathy may also develop as a result of treatment of these tumors. Recent contributions to the literature concerning the diagnosis and management of neural orbital tumors are reviewed.     

The optic nerve pathology in magnetic resonance imaging

Magnetic resonance imaging (MRI) delineates the orbital soft tissue excellently and is a valuable diagnostic tool in optic nerve pathology. By using different imaging parameters some examples are shown of demarcation of optic nerve tumors, optic nerve inflammations and orbital diseases surrounding the optic nerve. Whereas CT appears to be superior to MRI only in the detection of calcified perioptic meningiomas, MRI provides unique visualization of optic canal and even subarachnoidal space of retrobulbar optic nerve in peculiar cases.     

关于视神经鞘脑膜瘤诊断和治疗的新观点

视神经鞘脑膜瘤是视神经鞘最常见的肿瘤,占原发性视神经瘤的三分之一。随着先进的神经影像技术的发展,其发生率提高了。神经影像技术对该病的早期诊断具有重要的意义。大多数临床所见的病例诊断甚是可疑,许多病例并未做活组织检查,只是通过神经影像学支持诊断。视神经鞘脑膜瘤治疗的目的是确保一定的视力,控制肿瘤的局部增长,减少死亡率。对视力轻微下降或无视力障碍以及视力保持稳定的患者进行随访观察,分次立体定向放射疗法对进行性和早期的病例可以维持视力。外科切除术和活组织检查的致盲率比较高,适用于向外生长到硬脑膜的肿瘤。     

Aggressive orbital optic nerve meningioma with benign microscopic features: a case report

Primary optic nerve meningiomas occur at lower ages than meningiomas arising from the coverings of the brain and spinal cord. Here we report the case of a 20-year-old female with an aggressive orbital meningioma referred to the Ophthalmology Department of the Farabi Hospital in Tehran. The patient had a history of orbital meningioma from 10 years ago and several surgical resections due to tumor recurrence during these 10 years. On admission, the patient had a large orbital mass and severe proptosis. MRI images revealed a large left orbital mass with optic nerve involvement and extension to the left maxillary sinus, pterygoid fossa and the dura in the floor of the anterior fossa. Fine-needle aspiration cytology of the mass confirmed tumor recurrence. The patient first received radiotherapy due to the inoperable mass, and the tumor was resected 1.5 month later. Microscopic study showed meningotheliomatous meningioma with extensive involvement of the optic nerve and invasion of the optic disc, sclera and choroid. The interesting aspect of this case was the aggressive behavior of the tumor with intraocular invasion, despite its benign histopathological features, which led to wide exenteration of the eye together with resection of the upper and lower lids.     

Tumors of the optic nerve

This is a review of primary and secondary tumors of the optic nerve. The emphasis is on optic nerve gliomas and meningiomas.Optic nerve gliomas are slowly growing astrocytic neoplasms of the anterior visual pathways, the majority of which occur within the first two decades of life with equal sex incidence in about 1 of 200,00 patients presenting with eye complaints. The incidence is greater in neurofibromatosis. The typical presentation is visual impairment in a verbal pre-school child with optic canal enlargement and optic atrophy. An intraorbital location of the tumor leads to axial, irreducible, non-pulsatile proptosis. An intracranial location may disturb hypothalamic and pituitary function and produce hydrocephalus. Ocular findings may also include limited motility on a mechanical-restrictive basis, a pupillary relative afferent defect, nystagmus, and variable, non-specific visual field defects. Roentgenographic studies may show concentric unilateral enlargement of the optic canal with preservation of a well corticated margin, a fossa under the anterior clinoid process in continuity with the optic canal (J-shaped sella), and findings of increased intracranial pressure. On pathologic examination the tumor is a smooth, fusiform, intradural enlargement of the optic nerve. Histologically there is proliferation of elongated (pilocytic) astrocytes in reticulated patterns with intervening microcystic spaces containing mucosubstance and surrounding reactive hyperplasia of the arachnoid. Mitoses are not found. The diagnosis is clinical X-ray studies and brain scan should be performed. The differential diagnosis is that of unilateral proptosis in a child and includes acute ethmoiditis, hyperthyroidism, craniostenosis, other neoplasms, Hand-Schuller-Christian disease, and orbital hemorrhage due to trauma. Surgical resection is performed in cases with unilateral optic nerve involvement, the surgical approach being determined by tumor location. Bilateral or chiasmal cases are treated with radiotherapy when progression occurs. Malignant optic nerve gliomas and optic nerve hyperplasia are also discussed.Optic nerve meningiomas arise from the nerve sheath and are to be distinguished from orbital meningiomas arising from ectopic arachnoidal cells or those secondarily involving the orbit by extension from adjacent sites. Up to 80% of orbital meningiomas occur in females, in two age peaks, 25% in the first decade, and the rest in the 5th decade. Meningiomas present with visual loss and may produce proptosis, papilledema and/or optic atrophy, retinal striae, opticociliary shunts, limitation of extra-ocular movements, and lid edema. Signs of von Recklinghausen's disease should be sought. X-rays are the mainstay of diagnosis. Orbital meningiomas are composed of cells in sheets or in whorls with some spindle shaped cells. Calcifications are typical. Usually the dura is penetrated and the orbit invaded. Primary orbital meningiomas are locally infiltrating but do not metastasize. Complete local excision en bloc is recommended. Local recurrences may require reoperation; however, long term survival is excellent.Optic nerve head tumors are mostly benign hamartomatous proliferations of glial, melanocytic, or vascular tissue in the form of the astrocytic hamartomas of tuberous sclerosis, melanocytomas, and angiomatous malformations. There may also be primary or reactive proliferations of the juxtapapillary pigment epithelium. Metastatic lesions of the optic nerve are rare, breast and lung carcinomas accounting for most. The optic nerve may be infiltrated by neopastic cells in the leukemias and lymphomas. The optic nerve may also be involved by inflammatory processes, notably sarcoidosis. Optic nerve medulloepitheliomas are highly malignant and must receive aggressive surgical treatment.From the Edward S. Harkness Eye Institute, Columbia-Presbyterian Medical Center, New York, New York 10032.