术中磁共振和功能神经导航在脑干海绵状血管瘤手术中的应用

目的评价高场强术中磁共振(iMRI)和功能神经导航在脑干海绵状血管瘤手术中临床应用价值。方法16例脑干海绵状血管瘤患者,术前根据弥散张量成像重建白质纤维束,确定最佳安全手术入路及手术策略,术中常规应用基于弥散张量成像技术的纤维束镜下导航结合术中电生理监测辅助手术,术后定期临床及影像学随访。结果本组16患者中,2例患者因术中磁共振扫描发现海绵状血管瘤残留需继续手术切除残留病变。术中末次扫描证实所有患者的脑干海绵状血管瘤完全切除。3例患者术后有短期新发神经动能障碍,其中2例长期随访时恢复至正常功能水平。长期神经功能致残为1例。无iMRI相关不良事件发生。结论对于脑干海绵状血管瘤手术,iMRI可以实时评估海绵状血管瘤的切除程度。镜下功能神经导航术中显像脑干白质纤维束结合术中电生理监测和电刺激定位脑干颅神经核团,对最大限度切除病变、保护患者的神经功能有很大帮助。     

桥脑海绵状血管瘤手术指征和入路选择（附12例报告）

目的探讨桥脑海绵状血管瘤的手术指征和手术入路。方法显微手术切除桥脑海绵状血管瘤12例。11例位于桥脑背侧的海绵状血管瘤,采用枕下正中经菱形窝入路切除;1例位于桥脑腹外侧的海绵状血管瘤,采用枕下经乙状窦后入路切除。结果均镜下全切除病灶,术后病理证实为脑海绵状血管瘤。术后临床表现改善者6例,3例无变化;1例术后面瘫加重;1例术后出现外展神经瘫痪;死亡1例。术后3个月,9例MRI复查,均未见病灶,脑干组织影像学修复良好。结论正确选择手术指征和手术入路,采用显微外科技术切除桥脑CM是安全和有效的。     

脑干海绵状血管瘤手术入路选择

目的探讨脑干海绵状血管瘤手术适应证和手术入路的选择。方法回顾分析70例手术治疗的脑干海绵状血管瘤，其中位于：中脑15例、中脑-桥脑交界8例、桥脑34例、桥脑-延髓交界5例、延髓8例。我们共采用8种手术入路：经菱形窝27例、颞下或颞-枕入路14例、远外侧经髁7例、外侧小脑上幕下7例、中线小脑上幕下8例、枕经天幕4例、乳突后2例，以及眶颧1例。结果本组病例的年出血率为2．9％（77／2364），占同期颅内海绵状血管瘤的44％（70／159）；97％的病例手术选择在亚急性或慢性期、且所有患者均有颅神经症状和（或）运动功能障碍、感觉障碍、共济失调（包括失平衡）。中脑病变手术入路选择以中线小脑上（46．7％，7／15）、颞下或颞-枕（26．7％，4／15）为主；桥脑病变多选择菱形窝（58．8％，20／34）、颞下或颞-枕（23．5％，8／34）；而病变位于延髓者以远外侧经髁（62．5％，5／8）和菱形窝入路（37．5％，3／8）为主。结论脑干海绵状血管瘤表现为进行性神经功能缺失、具有占位效应、接近脑干表面者可考虑手术治疗；个体化地选择手术入路、术中神经电生理监测以及直接的电刺激是手术成功的关键。     

脑干海绵状血管瘤的影像学诊断和显微外科治疗

目的 探讨脑干海绵状血管瘤的影像学特点与显微外科治疗效果. 方法 第四军医大学唐都医院全军微创神经外科中心自2006年5月至2011年9月共行显微外科手术切除术治疗脑干海绵状血管瘤患者15例,回顾性分析患者的影像学资料和治疗效果. 结果 脑干海绵状血管瘤在MRI上有特征性的表现,可见“桑椹状”或“网格状”结构,肿瘤周边可见低信号环,以T2WI像更为明显,提示有含铁血黄素沉积的陈旧性出血.本组肿瘤全切14例,1例次全切.术后1周,12例较术前症状有明显改善,2例无明显变化,1例延髓病变者出现饮水呛咳加重.术后3月,随访13例症状较术前明显改善,2例无明显改善.术后12月,随访12例,11例症状较术前明显改善,1例无明显改善. 结论 MRI及其增强显影可准确地显示海绵状血管瘤的大小位置,显微外科手术治疗是有效的治疗脑干海绵状血管瘤的方法.     

以颅神经损害症状首发的脑干海绵状血管瘤的外科治疗

目的探讨以颅神经损害症状首发的脑干海绵状血管瘤手术治疗的临床效果。方法对17例以颅神经损害症状首发的脑干海绵状血管瘤出血手术患者,观察、随访1年和疗效评定(NIHSS评分,mRS评分);并对患者的资料进行回顾性分析。结果本组患者术前与术后1、6、12个月NIHSS评分和mRS评分比较,差异均无统计学意义(均P0.05)。结论首次有症状性出血并位于脑干表面的海绵状血管瘤,尤其是有颅神经损害症状者,可根据病灶部位选择合理的手术入路,采取手术切除脑干海绵状血管瘤,对预防再出血,提高临床治愈率,改善患者预后具有积极作用。     

脑内海绵状血管瘤的临床特点与微侵袭手术治疗

目的 总结脑内海绵状血管瘤（CA）的临床特点及手术治疗经验.方法 回顾性分析21例脑内型CA的临床资料和手术方法.首发症状为癫（癎）者16例,偏瘫或偏身感觉障碍者4例,无症状1例.采用神经导航辅助显微手术,其中4例同时做了颞叶部分切除,10例切除了胶质疤痕和含铁血黄素沉积带,2例在皮层脑电图监测下切除病灶和癫（癎）灶,余5例以含铁血黄素沉积带为界面切除病灶.结果 病灶均全切.21例患者随访0.5～4年恢复良好或无变化.结论 脑内型CA应积极手术治疗.合理采用微侵袭神经外科技术并遵循个体化原则有利于提高手术疗效.     

以癫痫起病的颞叶海绵状血管瘤的手术治疗

目的 探讨以癫痫起病的颞叶海绵状血管瘤的临床特征、显微手术治疗方法及效果。方法 对14例以癫痫起病的颞叶海绵状血管瘤的临床资料、手术方式和手术效果进行回顾性分析。结果 6例单纯瘤周放电和1例电极描记阴性行单纯病变和含铁血黄素层切除;7例病变侵及颞叶内侧和岛叶,加行前颞叶以及内侧结构切除术。术后随访1~2年,癫痫缓解率满意,按照Engel 癫痫手术预后标准,Ⅰ级8例,Ⅱ级4例,Ⅲ级1例,Ⅳ级1例;所有患者未检测到血管瘤复发。结论 对颞叶海绵状血管瘤继发癫痫,术前积极评估,采取个体化手术方案,争取血管瘤和含铁血黄素层全切,同时恰当处理好存在的致痫灶,是治愈海绵状血管瘤和控制癫痫发作的有效手段。     

脑干海绵状血管瘤的治疗策略与技巧

脑干海绵状血管瘤（BSCM）的特性是易反复出血,从而导致病变周围含铁血黄素及胶质样变范围扩大,肿瘤像"滚雪球"样逐渐增大。完整、安全地切除病灶可获得治愈,避免肿瘤增大或瘤内反复出血。然而,在脑干这一狭小区域内布满了神经核团和上下行神经纤维束,手术存在很大的风险和挑战性。本文重点介绍BSCM的手术目的和原则、手术技巧和最新进展,治疗的效果,以便于指导临床手术治疗,对于安全切除病灶、降低再出血风险至关重要。     

颞叶海绵状血管瘤继发癫痫的外科治疗

目的探讨颞叶海绵状血管瘤继发癫痫的临床特点及手术疗效。方法收集自2010年1月—2019年1月于武汉脑科医院神经外科以癫痫起病的颞叶海绵状血管瘤行手术治疗的患者38例,其中男17例,女21例,年龄8～57岁,平均(40.05±14.64)岁;病程1～10年,平均(1.25±2.19)年。临床表现为复杂部分性发作7例,部分性发作继发全身性发作8例,全身强直-阵挛性发作23例。所有患者术前均行头部断层扫描(CT)和核磁共振(MRI)检查,同时行长程视频脑电图(VEEG)监测。结合临床特点、影像学及VEEG监测结果,在皮层脑电图(ECoG)监测下行海绵状血管瘤显微手术切除,必要时加行前颞叶切除或皮层热灼术,术后随访通过Engel分级以评估手术的疗效。结果所有患者术后病理检查均证实海绵状血管瘤的诊断,术后随访1～9年发现,36例患者疗效满意,癫痫无发作,效果较差2例,手术有效率为94.74%(36/38)。结论对于颞叶海绵状血管瘤继发癫痫的患者,在手术切除海绵状血管瘤的同时,应一并切除胶质瘢痕和含铁血黄素沉积带,必要时可加行前颞叶切除术或皮层热灼术。若术中探查困难,可借助神经导航和术中B超对病灶进行定位,且术后癫痫控制一般均较好。     

脑干海绵状血管瘤的显微手术治疗

目的探讨脑干海绵状血管瘤的诊断和治疗经验。方法本组18例脑干海绵状血管瘤中,病灶位于一侧大脑脚3例,桥脑及桥脑臂9例,桥脑一延髓4例,延髓2例。分别采用经幕下小脑上人路、一侧颞枕小脑幕人路、桥小脑角人路和枕后正巾人路显微手术治疗。结果血管瘤镜下全切17例,次全切除1例。眩晕病人术后症状明显减轻,除1例术后新出现复视外,其余颅神经损害患者症状减轻,或无变化。结论显微手术治疗脑干海绵状血管瘤是可行的,早期手术可能取得较好的效果。     

幕上与癫痫有关的海绵状血管瘤的显微外科治疗

目的 探讨幕上表现于癫痫的颅内海绵状血管瘤外科治疗方法和效果.方法 25例幕上表现于癫痫的颅内海绵状血管瘤患者,根据术前影像学、脑电图表现与运动区的关系分为A、B二组.手术在皮层脑电图(ECoG)监测下进行,包括单纯切除血管瘤、切除血管瘤和切除含铁血黄素层,以及辅助部分皮层痫灶切除、皮层热灼或软膜下横切等.结果 本组海绵状血管瘤均令切除,2例术后有一过性轻度肢体功能障碍,无手术死亡.随访10个月-4.5年,A组9例,Engel Ⅰ级6例;Engel Ⅱ B级2例;Engel Ⅲ级1例.B组16例,均无发作.结论 幕上与癫痫有关的海绵状血管瘤在ECoG监测下做病灶和痫灶切除,是控制术后癫痫发作的有效手段;运动区和附近海绵状血管瘤由于手术没有完全切除致痫灶可能是术后癫痫控制率差的原因.     

Treatment strategies in cavernomas of the brain and spine

The management of brain and spinal cavernomas includes two main options: (i) conservative treatment or (ii) surgical removal. Clinical experience related to cavernoma patients falls into four major categories: the surgical or conservative treatment of incidental or symptomatic cavernomas. In many patients, cavernomas exist as fairly benign lesions, frequently remaining clinically silent for life. This observation argues against the active treatment of all cavernoma patients; rather for the meticulous selection of only those more likely to benefit from surgery. Thus, the most crucial task in successful management of cavernomas is appropriate patient selection. In this review, we present our institutional experience on cavernoma management supplemented with data from the literature.     

Direct trans-sylvian approach to the ventrolateral pons in surgical management of large cystic cavernous malformations of the brain stem in children

OBJECTIVES: In the study surgical indications are reviewed and the effect of the applied surgical technique on long-term treatment outcome in children with large cystic brainstem cavernous malformations is evaluated. METHOD: Clinical data of 5 patients treated surgically for large cystic cavernous malformations of the pons in the years 1995-2001 were retrospectively evaluated. In all the cases the transsylvian approach to the ventrolateral pons with splitting of the crus cerebri was used to reach cavernoma within the cystic cavity. The long-term follow up (mean 4.9 years) included a complete neurological examination and annual MRI studies. RESULTS: At least one hemorrhagic episode was diagnosed in all the cases, while bleeding to the pons, with clinical course mimicking that of stroke, was noted twice in 3 patients. All the episodes confirmed by MRI imaging appeared to be intralesional. The malformations ranged in size from 30 to 50 mm (mean 36.9 mm). Their most characteristic MRI features were not only the localization in the pontine structure exclusively i.e. within the natural anatomical barriers separating the pons from the midbrain and medulla, but also the growth pattern corresponding to that of brainstem benign gliomas. The patients' mean preoperative KPS score was 60 (30 to 90). Cavernous hemangiomas were totally removed in 3 out of 5 cases. One patient with partially removed lesion presented after a year recurrent hemorrhage and new neurological deficits that required a second stage surgery. In all but one case transient complications included some new cranial nerve, motor and sensory deficits that influenced the patients' immediate postoperative KPS assessment, with the mean score of 48. In a follow-up assessment at 1.5 to 7 years, a significant amelioration of both their neurological status and quality of life was found, with KPS scores from 70 to 90 (mean score 80). Annual MRI examinations showed extensive hemosiderin deposits within the pontine tissue in 3 cases of totally removed cavernous hemangiomas and this image was stable during the follow-up period. CONCLUSIONS: The findings suggest that resection of large pontine cavernomas in children is indicated in cases of symptomatic hemorrhage with lesions approaching the pial surface, or surrounded by a small margin of normal tissue. Recurrent hemorrhages are intralesional (i.e. limited to pontine structures by the natural anatomical barriers), resulting in a "cyst-like" growth of malformations mimicking that of focal neoplasms and in compression of the brain stem tissue rather than in a direct hemorrhagic insult.     

Brain-stem compression in vertebrobasilar dolichoectasia. A multimodal electrophysiological study.

OBJECTIVE: To evaluate the effects of mechanical compression of the brain-stem in patients with vertebrobasilar dolichoectasia (VBD). METHODS: In the framework of a prospective, observational study that collected clinical and laboratory data in patients with VBD, we studied 20 patients with compression of the brain-stem due to ectatic, tortuous basilar or vertebral arteries. Patients with cerebral lesions other than small lacunae in the white matter of the cerebral hemispheres were excluded from the study. Patients underwent vestibular and auditory function testing, including brain-stem auditory evoked potentials (BAEPs), blink reflex (BR), somatosensory evoked potentials (SEPs), and motor evoked potentials (MEPs). RESULTS: Almost all of the patients complained of auditory or vestibular symptoms and none had symptoms or signs of impairment of long tracts or the facial and trigeminal nerves. The most consistent findings were BR abnormalities with prolongation of ipsilateral R1 latency in cases of compression of the pons (10/16) and prolongation of the R2 and R2c latencies with compression of the medulla oblongata (5/15). Subclinical impairment of corticospinal pathways was found in 13 out of 25 instances of compression, and this was more frequent with compression of the pons. Abnormal BAEPs or SEPs were less frequently encountered, and only in cases with compression of the pons. CONCLUSIONS: Neurovascular compression of the brain-stem, even with severe distortion, is seldom associated with overt clinical signs, whereas subclinical dysfunctions are relatively frequent. The central pathways of the BR and the corticospinal pathways are more susceptible to compression than acoustic and sensory pathways. BR, MEP and BAEP data provide a functional evaluation of the brain-stem and some cranial nerves, which is lacking in imaging studies. Functional investigations may be useful in the long-term management of these patients, since VBD may be progressive and surgical correction may be required at some stage.     

A single institution series of cavernomas of the brainstem

We retrospectively analyzed a series of patients with brainstem cavernomas of the medulla, pons, or midbrain to attempt to define the natural history, indications for surgery, and outcome after surgical resection. Between 1993 and 2008, 52 patients with cavernomas of the brainstem presented to our institute and were managed either surgically or conservatively. Twenty-three patients underwent surgical excision using standard skull base approaches. Outcomes were correlated to the number of preoperative hemorrhages, location of the cavernoma, timing of surgery in relation to the hemorrhage and the preoperative neurological status. Nine patients improved after surgery, 12 deteriorated and two died. In the conservatively managed group, 15 patients had a good outcome, 11 deteriorated and one died. Multiple hemorrhages, poor preoperative neurological status and surgery during the acute phase were predictive of the surgical outcome. Excision of brainstem cavernomas should be considered in patients with symptomatic hemorrhages whose lesions approach the pial surface. Patients with minimal stable neurologic deficits without recurrent bleeds should be managed conservatively.     

颅内海绵状血管瘤的显微外科治疗

目的探讨颅内海绵状血管瘤的诊断和治疗的进展。方法对我院连续收治的31例颅内海绵状血管瘤患者的资料进行回顾性分析。病灶位于额叶6例,额顶区皮层至室管膜下巨大病灶1例,顶叶功能区4例,岛叶基底节1例,颞后1例,一侧大脑脚3例,桥脑及桥脑臂9例,桥脑一延髓4例,延髓2例。病灶大小1～7cm,半球病灶采用最近的骨瓣开颅;脑干病灶采用4种手术入路：经幕下小脑上入路、经一侧颞枕小脑幕入路、桥小脑角入路、枕后正中入路。结果全切30例,次全切除1例。偏瘫5例术后4例肌力增加,1例无变化;癫痫4例3例术后1个月内无发作,1例有1次轻微发作;眩晕患者症状明显减轻,除1例术后新出现复视外,其余颅神经损害或减轻,或保持原状。病理结果：海绵状血管瘤。结论显微手术治疗颅内海绵状血管瘤是可行的,早期手术可能取得较好的效果。     

Prediction of postoperative outcome with special respect to removal of hemosiderin fringe: a study in patients with cavernous haemangiomas associated with symptomatic epilepsy.

PURPOSE: In this study 30 patients with symptomatic epilepsy caused by cavernomas were investigated in a postoperative follow up study to assess predictors for postoperative outcome with respect to indications, time and approach of surgery. METHODS: Thirty patients with cavernomas refractory to medical treatment were scheduled for surgery based on the findings of high-resolution MR imaging and intensive EEG-video monitoring. Postoperative outcome of epilepsy was assessed by follow-up examinations based on the basis of classification by Engel and the International League against epilepsy (ILAE). RESULTS: The following variables were associated with good postoperative outcome: (1) complete resection of hemosiderin fringe surrounding the cavernoma was correlated to less postoperative seizure frequency versus incomplete resection of the hemosiderin fringe according to the outcome protocol of ILAE. (2) Lower duration of epilepsy at the time of operation was correlated to a better postoperative outcome with a benefit for recovery. (3) Absence of hemorrhage before surgery and unifocal seizure onset was a predictor for a favorable outcome, whereas bilateral or multifocal seizure onset zones showed poorer postoperative outcomes. (4) In patients with dual pathology (hippocampal sclerosis in addition to a cavernoma), lesionectomy plus hippocampectomy as opposed to lesionectomy only, had a better outcome than single lesionectomy. CONCLUSION: Postoperative outcome in patients with cavernomas should be the topic of further prospective multicenter studies involving a large number of patients. In addition to the ideal operation time and handling of dual pathology the role of extended resection including perilesional hemorrhages should be taken into account.     

Cavernoma of the trochlear nerve

Here we present the case of a 53-year old man with progressive double vision due to isolated left trochlear nerve palsy. Cranial magnetic resonance imaging (MRI) showed a small tumor within the left quadrigeminal cistern that did not increase in size after several months. Explorative neurosurgical intervention revealed a left trochlear nerve cavernoma. The lesion was microsurgically excised followed by end-to-end anastomosis of the trochlear nerve. After a one-year follow up, double vision totally disappeared and cranial MRI showed no recurrence. Cerebral cavernous malformations usually become symptomatic in seizures or focal neurological deficits after intracerebral hemorrhage. Rarely, cavernomas arise from cranial nerves. To the authors' knowledge, this is the first report on a symptomatic cavernous malformation arising from the trochlear nerve and on its successful surgical management.     

Virtual endoscopy assisted pure ventriculoscopic resection of cavernomas occluding foramen of Monroe: Technical note and literature review

ObjectiveCavernomas at Foramen of Monroe (FoM) are rare cases among the intracranial cavernomas. Pure ventriculoscopic removal of cavernoma at FoM through a single burr hole is challenging and rarely reported.MethodsWe herein introduced the virtual endoscopy (VE) assisted ventriculoscopic resection to treat the cavernomas at FoM. Two cases diagnosed with cavernomas at FoM, a 31-year-old male patient (case 1) and a 26-year-old male patient (case 2), were included. Both of them suffered from headache, nausea and vomiting. The pre-operative MRI revealed masses at the FoM. We reconstructed the VE on a free and open-source platform (3D Slicer) for the pre-surgical evaluation. And then ventriculoscopic operation through a single burr hole was made to remove the cavernomas at FoM.ResultsThe VE displayed a 14×19×16 mm lesion in case 1 and an 18×20×29 mm lesion in case 2 and both cases revealed some attachment between the lesions and the periventricular tissue. The ventriculoscopic operations indicated by VE were performed to achieve total resection of the cavernomas without neurological deficit.ConclusionsAlthough the neuroendoscopic treatment to cavernoma at FoM through a single burr hole was rarely reported among the previous literatures, it was a quite effective and useful method in our cases. And the application of VE before ventriculoscopic operation could help to provide a three-dimensional and panorama view of the intraventricular lesions.