视神经鞘脑膜瘤的显微外科治疗(附13例报道)

目的探讨视神经鞘脑膜瘤的显微神经外科手术方法。方法收治13例视神经鞘脑膜瘤，根据肿瘤位置采用3种不同经颅入路：单侧额部入路、经额经眶上缘入路及经额颧经眶上缘入路，利用显微外科技术安全显露眶上裂外侧部、眶上裂中央部切除肿瘤。结果肿瘤全切11例，部分切除2例。术后13例患者的眼球突出均恢复正常，11例其它症状和体征也恢复或改善。术后动眼神经麻痹1例，患侧眼失明2例。结论熟悉显微神经外科解剖学，利用显微外科技术选择合适的经颅入路，可以提高视神经鞘脑膜瘤的全切除率，并使颅眶区的正常结构得到最大程度的保护。     

颅眶及球后眶内肿瘤手术治疗：附16例临床分析

背景和目的：颅眶区解剖复杂，有许多孔隙、开口与颅内、眶内相通。由于肿瘤邻近重要的颅神经和血管，手术切除和重建颅底往往困难。本文探讨经颅入路切除颅眶沟通性肿瘤和球后眶内肿瘤的手术方法。方法：回顾性分析16例经颅入路手术切除的颅眶沟通性肿瘤和球后眶内肿瘤的临床资料，分别采用经额经眶上缘入路和经眶上一翼点入路进行。结果：全切除肿瘤14例，次全切除2例。手术后患眼失明1例，动眼神经麻痹2例，无手术死亡，手术效果满意。结论：经颅入路切除颅眶沟通性肿瘤和球后眶内肿瘤时，根据肿瘤所在眶内的位置选择恰当的手术入路是手术成功的前提，合理的手术方式和熟练的手术技巧是提高全切率、减少并发症的关键。     

颅底沟通瘤扩大额下入路10例

（目的〕介绍扩大额下前颅窝底入路的手术方法，并报告10例中线部位累及广泛的巨大颅底沟通瘤显微手术体会。（方法）采用额部开颅同时行眉弓-眶顶成形，蝶骨平台及眶顶磨除，经硬股外、硬膜下联合入路在显微镜下一次切除肿瘤，最后行颅底重建。（结果）10例中肿瘤全切5例，近全切3例，大部切除2例。病理报告良性肿瘤7例，恶性肿瘤3例。无手术死亡及严重并发症；术后视力恢复正常，鼻塞流涕及吞咽呛咳等临床症状均有明显改善。术后均恢复正常生活。（结论）该入路适用于颅底中线部位累及广泛的肿瘤；具有暴露充分，脑组织损伤小，切除较彻底及术后并发症少等优点。颅底重建是手术成功的关键之一。     

儿童颅眶沟通肿瘤的诊断治疗附16例临床分析

背景与目的:一般前中颅窝底肿瘤在目前的手术技术和设备条件下切除并不困难,但侵及颅眶并沟通的肿瘤入路困难,全切除率低,手术难度较大,仍是神经外科医生棘手难题。本文讨论了儿童颅眶沟通肿瘤的生长特点、诊断,具体手术入路,治疗方法与预后。方法:16例患儿根据肿瘤生长部位及侵及范围不同而分别采用了翼点眶外侧壁入路、改良翼点额底硬膜外眶顶入路二种手术方式,前一种手术入路7例,后一种手术入路9例。原则上一般先切除颅内肿瘤再开眶切除眶内肿瘤。结果:全切除10例,次全切除6例。术后病理证实脑膜瘤6例,视神经纤维瘤6例、海绵状血管瘤2例,脊索瘤2例。术后患儿眼球突出均有不同程度改善,眼球活动障碍4例,术后视力好转10例,上睑下垂3例,术前已失明2例及4例视力仅为光感患儿无恢复。结论:手术治疗是儿童颅眶沟通肿瘤这类疾病唯一有效的治疗方法。既便是单眼失明不能恢复也仍可保住患儿生命及眼睛外形,术后残余肿瘤行放疗可减少复发。     

经颅显微切除眶颅沟通瘤九例

眼眶与颅腔解剖关系密切，眶顶即为前颅底的一部分，并经视神经管、眶上裂相交通，原发于眶内或颅内的肿瘤突破前颅窝底或视神经管、眶上裂入颅内或眶内形成眶颅沟通瘤，我院已成功经颅显微切除眶颅沟通瘤9例，结果总结报道如下。     

改良经颅眶上外侧壁入路治疗眶尖区肿瘤的临床分析

目的探讨改良经颅眶上外侧壁入路治疗眶尖区肿瘤的手术方法、适应证及效果。方法对2011年1月至2012年12月间经颅眶手术治疗的50例眶尖区肿瘤患者的临床资料进行分析。结果眶-翼点入路手术患者20例,眶-额与眶-颧以及颧-翼联合入路手术患者30例。肿瘤全切除35例(70.0%),肿瘤次全切除8例(16.0%),大部分或者部分切除6例(12.0%),活检1例(2.0%)。眶尖区肿瘤病理类型主要为脑膜瘤、神经源性肿瘤、血管/淋巴管源性肿瘤和泪腺源性肿瘤,分别占42.0%、32.0%、20.0%和6.0%。50例患者出院时,视力功能较治疗前有明显改善或无加重者20例,单纯眼睑下垂5例,眼球轻微活动受限7例(4例伴有眼睑下垂,3例复视),严重动眼神经损伤5例(1例神经鞘瘤,2例转移瘤,2例脑膜瘤),视力下降或者失明3例(2例视神经本身伴有肿瘤,1例颅眶沟通性神经鞘膜瘤)。术后6个月至1年随访观察显示,单纯眼睑下垂患者完全恢复,眼球轻微活动受限的临床症状完全消失,严重动眼神经损伤患者症状明显减轻,视力下降患者视力恢复正常。本组患者无复发和死亡。结论临床上采取改良经颅眶上外侧壁入路治疗眶尖区肿瘤是可行的,操作中应选取合适的角度,并加强神经功能的保护,降低并发症的发生,提高临床治疗总体效果。     

颅眶沟通肿瘤的临床特征与手术治疗

目的 研究同时侵犯颅内和眶部肿瘤的临床及病理特征与神经外科显微手术治疗方法.方法 对102例经临床分析与影像学检查而获诊断的病例采取不同的手术入路,并根据术中冰冻活检的结果采取显微外科技术切除肿瘤,术中对需要保留视力的患者行闪光视觉诱发电位(F-VEP)监测.全组病例中,肿瘤位于前颅凹-眼眶67例,中颅凹-眼眶26例,前颅凹-中颅凹-眼眶9例.其中91例(89.2%)出现突眼、视力下降、眼肌麻痹等视器症状.62例(60.8%)有头痛、头晕等颅内压增高表现.结果 本组81例(79.4%)肿瘤获手术全切除,21例(20.6%)为次全切除,无死亡病例.对93例经信访或门诊随访6个月至14年(平均5.6年),70例(75.3%)恢复满意,7例(7.5%)复发,余16例(17.2%)有不同程度后遗症.结论 颅眶沟通肿瘤同时侵犯颅内及眼眶,多以视器改变为主要临床表现.采取不同的手术人路并根据术中冰冻活检的结果指导显微外科技术切除该类肿瘤,预后良好,术中采取F-VEP监测,可以减少手术对视神经的损伤.     

颅-颌面联合入路切除颅内外沟通性肿瘤

背景与目的：颅内外沟通性肿瘤范围广泛，同时累及颅内、颅外多个结构。单一的手术入路常不能充分暴露肿瘤，手术难度很大。本文重点介绍颅一颌面联合入路切除颅内外沟通性肿瘤的手术方法及要点。方法：对22例颅内外沟通性肿瘤采用颅一颌面联合入路进行手术，其中21例一期行颅-颌面成型。(1)颅眶颧入路：即额颞开颅伴眶骨颧骨颧弓截骨术，用于14例颅眶颞下窝沟通性肿瘤。(2)颅前窝底结合鼻侧切入路：用于3例颅-眶-筛窦沟通性肿瘤及2例颅-筛、蝶窦-鼻腔沟通性肿瘤。(3)乳突-乳突后入路：用于切除2例同时累及乳突、外耳道、岩骨及颅内的肿瘤。(4)开颅结合上颌骨切除：用于1例晚期恶性肿瘤同时侵犯颅内、眶内、筛窦、蝶窦及上颌窦，局部用颞肌瓣添充并行植皮。结果：肿瘤全切18例，近全切4例。术后病理诊断为脑膜瘤12例，恶性脑膜瘤2例，神经鞘瘤2例。腺样囊性癌4例，胆脂瘤1例，低分化鳞癌1例。术后除1例外，余没有遗留明显的颅面畸形．复查增强颅MRI或CT检查未发现明显的肿瘤残留病灶，术后行预防性放疗6例。随访3年，复发2例。结论：采用颅一颌面联合入路可以充分的显露颅内外沟通性肿瘤，可基本做到影像学意义上的肿瘤全切．对恶性肿瘤术后辅以放疗可减少肿瘤复发。     

脑肿瘤

68例脑胶质瘤术后治疗及相关预后因素分析;儿童脑肿瘤的临床治疗及病理特点研究;显微外科治疗颅眶及球后眶内肿瘤20例临床分析;经单鼻孔入路内窥镜下垂体腺瘤切除术;内镜引导下手术切除内耳道内听神经鞘瘟     

儿童颅咽管瘤的手术治疗27例体会

背景与目的：儿童颅咽管瘤是好发于鞍区的良性肿瘤,目前主要的治疗方法是手术切除肿瘤,选择适当的入路既可将肿瘤完整切除又能避免肿瘤周围重要的神经结构损伤,是减少肿瘤复发及术后并发症的关键。我们选择27例手术治疗儿童颅咽管瘤的临床资料以探讨治疗儿童颅咽管瘤的手术入路选择、手术技巧。方法：回顾分析27例儿童颅咽管瘤患者手术治疗的临床资料,总结治疗过程中的体会。结果：27例中肿瘤镜下全切22例,次全切3例,部分切除2例。手术经额硬膜下入路18例,经翼点入路4例,经蝶入路切除3例,经半球间-纵裂入路2例。24例获随访2个月至5年,效果良好者21例,生活需照顾者2例,死亡1例,最常见的术后并发症为垂体功能低下及尿崩。结论：显微外科手术全切颅咽管瘤,效果良好,额下入路能提供鞍上大颅咽管瘤手术切除良好的暴露。     

Transnasal Endoscopic Optic Nerve Decompression in Post Traumatic Optic Neuropathy

To quantify the successful outcome in patients following optic nerve decompression in post traumatic unilateral optic neuropathy in form of improvement in visual acuity. A prospective study was carried out over a period of 5 years (January 2011 to June 2016) at civil hospital Ahmedabad. Total 20 patients were selected with optic neuropathy including patients with direct and indirect trauma to unilateral optic nerve, not responding to conservative management, leading to optic neuropathy and subsequent impairment in vision and blindness. Decompression was done via Transnasal-Ethmo-sphenoidal route and outcome was assessed in form of post-operative visual acuity improvement at 1 month, 6 months and 1 year follow up. After surgical decompression complete recovery of visual acuity was achieved in 16 (80%) patients and partial recovery in 4 (20%). Endoscopic transnasal approach is beneficial in traumatic optic neuropathy not responding to steroid therapy and can prevent permanent disability if earlier intervention is done prior to irreversible damage to the nerve. Endoscopic optic nerve surgery can decompress the traumatic and oedematous optic nerve with proper exposure of orbital apex and optic canal without any major intracranial, intraorbital and transnasal complications.     

Orbital apex syndrome: a rare presentation of extramedullary hematopoiesis: case report and review of literature

We describe a case of compressive neuropathy in the orbital apex due to extramedullary hematopoiesis (EMH). A 64-year-old man with Polycythemia Rubra Vera developed unilateral visual loss, proptosis, complete ophthal-moplegia, and facial paresis. Bone marrow biopsy showed myelofibrosis. Magnetic resonance imaging demonstrated enhancement at the orbital apex and subtle optic canal narrowing. Decompression of the optic nerve with biopsy of surrounding bone showed EMH. The patient received a course of radiation without benefit. We suggest including the diagnosis of EMH of the orbital apex bones in the differential diagnosis of patients with myeloproliferative disorders who develop an orbital apex syndrome.     

Lymphomatous proptosis as a novel feature of mantle cell lymphoma

We describe eight cases of acute proptosis due to mantle cell lymphoma (MCL), among 26 consecutive MCL cases. The median time of onset was 29 months (range 0-102) from diagnosis. Two cases presented initially with orbital masses while five as sudden disease progression after multiple courses of chemotherapy. In each case, there was dramatic loss of vision and severe proptosis, which uniformly responded to radiotherapy and/or further chemotherapy. Unilateral blindness only occurred in two cases, with recurrent orbital relapse and repeated retinal irradiation and retro-orbital optic nerve involvement, respectively. The high incidence, as well as bilateral and recurrent nature, of orbital involvement suggested a homing mechanism of MCL to this site. Despite the absence of central nervous system involvement, most cases died of refractory disease. Apart from lymphomatous polyposis of the gut, MCL cells also show predilection to ocular presentation, and must be considered as a differential diagnosis to maltoma in the two anatomical sites.     

Proptosis and decreased vision secondary to prostate cancer orbital wall metastasis

The case of a 66-year-old gentleman who presented with unilateral proptosis, eye pain and partial loss of vision seven years after his original prostate cancer diagnosis is reported. MRI of the orbits revealed a 2-cm lesion in the posterolateral right orbital wall near the optic foramen with compression of the optic nerve. Metastatic orbital lesions are relatively uncommon in prostate cancer. Treatment is palliative and varies according to the time of presentation in the course of the disease. This patient's symptoms resolved after reinitiation of combined androgen blockade.     

Fibro-osseous lesions of the maxillo-ethmoid complex with orbital involvement

Fibro-osseous lesions involving the maxillo-ethmoid complex are rare. Extensive lesions leading to proptosis are rare still. We report here 22 cases of fibro-osseous lesions with orbital involvement. CT scan was the mainstay of radiological investigation. Transfacial excision was done in all cases, except three which necessitated a cranio-facial resection. Surgery resulted in significant reduction of proptosis and improved cosmesis in all cases. In three instances, a sliver of the tumor tissue was left behind due to its proximity to the dura or optic nerve. Two of these cases however went on to develop subsequent visual loss secondary to disease progression. Six cases necessitated revision surgery for residual or recurrent lesion. Disease extension to the proximity of vital structures should be excised as continuing tumor growth may prove unsafe.     

Measurement of Proptosis (Exophthalmos) by Computerised Tomography

As computerised tomography is now accepted as a method of choice for the investigation of suspected orbital masses, measurement of the degree of proptosis (exophthalmos) should be useful in assessing response to therapy. Review of a number of orbital CT scans (GE 8800) revealed that both scanning and measuring techniques must be standardised to obtain consistent results. The following requirements must be met: The plane of the scan must be parallel to the plane passing through the optic nerve head and lens, i.e. 15 degrees cephalad to the orbitmeatal line. The eyelids must be open, with the patient looking straight ahead. Window levels must be set appropriately when the measurements are made, i.e. +500 HU for the corneal surfaces. A line between the bony lateral orbital margins is drawn on the slice containing the optic nerve head and lens, and the distance of the cornea in front of this line is measured. When the radiographic criteria are met, proptosis can be measured with accuracy of 0.8 mm.     

Primary intraosseous meningioma of orbit and anterior cranial fossa: A case report and literature review

Primary intraosseous meningiomas of the skull base are rare. Extensive involvement of the skull base by the tumour may result rarely in loss of vision. Surgical management requires extensive decompression and reconstruction. A case of primary intraosseous meningioma of orbits and anterior cranial fossa presenting with painless proptosis and loss of vision is presented here and the literature reviewed.     

Preliminary visual outcomes after three-dimensional conformal radiation therapy for optic nerve sheath meningioma

PURPOSE: We assessed visual outcomes, local control, and toxicity associated with three-dimensional conformal radiation therapy (3D-CRT) for primary optic nerve sheath meningiomas (ONSM). METHODS: Twenty-three patients diagnosed with ONSM were evaluated at the University of Michigan between 1986 and 2001. Fourteen patients were treated with 3D-CRT. Detailed pre- and postradiation treatment ophthalmologic examinations and MRIs were performed on all patients. Clinically significant visual acuity change was defined as a >or=three line change on the Snellen chart. Mean deviation change of >or=three decibels was defined as a clinically significant visual field change. Radiographic progression was defined as any increase in size on MRI. Acute and late toxicity was scored according to RTOG criteria. RESULTS: Median follow-up was 51.3 months. Five patients had a clinically significant improvement in visual acuity. Seven had stable acuity, and only 2 worsened. Nine patients had clinically significant visual field improvement. One patient developed early radiation retinopathy, 1 experienced orbital pain, 1 developed dry eye, and 2 developed iritis. No patient has required additional treatment, and none have demonstrated radiographic progression. CONCLUSION: 3D-CRT is effective in controlling tumor growth while improving or preserving vision in most patients with optic nerve sheath meningiomas.     

Objective evaluation of improvement in optic neuropathy following radiation therapy for thyroid eye disease

PURPOSE: While the literature supports the use of radiation therapy for thyroid eye disease, it does not sufficiently describe in detail the results of radiation therapy for optic neuropathy associated with thyroid eye disease. The objective of this study is to quantify the changes in parameters of optic neuropathy after orbital irradiation for thyroid eye disease. METHODS AND MATERIALS: Twelve consecutive patients with optic neuropathy from thyroid eye disease were followed by a single neuro-ophthalmology practice and treated by one radiation oncologist with radiation therapy from 1991 through 1995. All cases were prospectively followed for visual acuity, color vision, mean deviation, and/or foveal sensitivity and afferent pupillary defect. All patients received 2000 cGy in 10 fractions with megavoltage irradiation to the orbits. RESULTS: Ten of 12 patients were evaluated for follow-up (one moved out of this country and one had a stroke, which confounded interpretation of examination results). An analysis was performed retrospectively while treatment and evaluation remained uniform. Five men and five women formed the basis of this study with a median age of 60 years (35-76 years). Nineteen eyes were evaluated for thyroid optic neuropathy. Improvement in optic nerve function occurred in eight of ten patients. Improvement was seen either during radiotherapy or within 2 weeks of completion. No long-term adverse effects were noted. CONCLUSION: This study objectively demonstrates improvement in optic neuropathy from radiation therapy for thyroid eye disease.     

Fibrous dysplasia of the skull with acromegaly and sarcomatous transformation

Summary Two cases of fibrous dysplasia of the skull are reported. Both patients were young women with acromegaly and were treated with radiotherapy. Progressive pareses of cranial nerves, pain, and a malignant course of the disease were characteristic in both patients, and the diagnosis of osteogenous sarcoma proved in one of them by histological examination.The clinical picture of fibrous dysplasia of the skull and the role of radiotherapy with the risk of development of malignancy is discussed.