Lindau氏病一个家系调查报告

我科先后收治同患脑血管网状细胞瘤父子俩,家系调查发现其一家6名男性均患此病。报告如下。例1　先证者。1984年(23岁)因头晕、头痛、呕吐在某医院以“脑积水”行“脑室分流术”,症状无缓解。1年后在另一医院手术切除左小脑肿瘤,病理诊断为“血管网状细胞瘤”。术后肿瘤复发2次并均行手术治疗。1992年因肿瘤复发在我科行手术及Ｘ-刀治疗,术中见左小脑半球内肿瘤呈囊性,壁内侧见红色蚕豆粒大小瘤结节,把瘤结节连同囊壁一并切除,病理证实为血管网状细胞瘤。现工作生活正常,双眼检查未发现异常。随访3年,肿瘤无复发。例2　先证者之四弟。1988年…     

显微外科手术治疗后颅凹血管网织细胞瘤临床分析

目的探讨后颅凹血管网织细胞瘤的诊断与治疗方法,以降低术后并发症和致残率及病死率。方法回顾性分析21例颅内血管网织细胞瘤患者,术中采用显微外科手术治疗,分析血管网织细胞瘤的诊断与鉴别诊断、手术技巧及术中注意事项。结果 21例患者中,17例囊性肿瘤者行瘤结节全切除,4例实性肿瘤在介入栓塞后全切除;术后症状临床痊愈者16例,好转4例,无明显变化1例,无死亡病例。结论后颅凹血管网织细胞瘤是一种良性血管性肿瘤,影像学如头颅MRI或CT诊断具有较高的特异性,临床显微外科手术切除瘤结节和实性肿瘤切除有明显效果。     

内镜控制显微神经外科手术治疗小脑囊性血管网状细胞瘤（附9例报告）

目的探讨应用内镜控制显微神经外科手术治疗小脑囊性血管网状细胞瘤的疗效。方法回顾性分析9例采用内镜控制神经外科手术治疗的小脑囊性血管网状细胞瘤患者的临床资料。结果9例小脑囊性血管网状细胞瘤患者的瘤结节均被全切除,手术并发症少,术后患者恢复良好。结论内镜控制显微神经外科手术治疗小脑囊性血管网状细胞瘤创伤小、安全、有效,有利于提高手术疗效,降低手术并发症。     

微侵袭手术切除脑内血管网状细胞瘤

目的：探讨神经导航及显微技术下的微侵袭手术切除囊性血管网状细胞瘤瘤结节及血供丰富的实体肿瘤的疗效。方法：本组共10例，对瘤结节直径＜1cm的3例肿瘤于神经导航下行显微手术切除，其余直接行显微手术，血供丰富的大型肿瘤在显微镜下逐步显露供瘤动脉，分别阻断后除之。结果：导航定位准确，平均误差仅2mm，9例全部切除，本组术后无神经功能缺失。1例肿瘤少量残留于术后15d因原瘤床出血而死亡。结论：神经导航及显微技术是脑内血管网状细胞 瘤有效的微侵袭手术方法，对血供丰富的瘤体应尽可能做到全切除。     

家族性多发血管网织细胞瘤1例

患者,女性,27岁,因头痛头晕并行走不稳2月入院。查体:神志清,双眼视乳头水肿,左手指鼻试验及轮替动作差,Romberg征阳性,同时双下肢感觉减退。CT示左侧小脑半球一4cm×4cm囊性占位,MRI示小脑囊性占位,强化后发现囊内偏外侧一5mm×5mm结节;同时发现C5~T12脊髓空洞,T10平面脊髓空洞内一瘤结节。于2003年12月3日行小脑肿瘤切除术,术中切除小脑瘤结节后,探查发现延髓背侧表面一瘤结节约3mm大小,呈黄红色桑椹样,一并切出。病理检查证实均为血管网织细胞瘤。两周后再次手术切除脊髓内瘤结节,病理证实为血管网织细胞瘤。术后病人症状消失。追问…     

颅内血管网状细胞瘤的治疗方法及临床特征分析

目的分析颅内血管网状细胞瘤的治疗方法以及临床特征。方法回顾性分析我院2000—2010年收治的31例颅内血管网状细胞瘤患者的临床资料,总结治疗方法以及临床特征。结果本组41个肿瘤灶,32个囊性肿瘤,其中29个肿瘤行瘤结节完全切除,3个次全切除或大部分切除。本组31例患者,9例实性肿瘤,5例肿瘤行完全切除,4例大部分切除;治愈24例,术后随访至今未见复发;好转6例,其中3例术后1～4a复发,二次手术后随访至今未见复发;病死1例,病死率3.23%。结论颅内血管网状细胞瘤患者临床表现多为头晕、头痛、恶心、呕吐,以及不同程度的行走不稳和视力下降,依靠CT及MRI检查可以基本明确诊断,手术治疗为首选治疗方案,术后残余患者给予γ-刀放射治疗。     

12例小脑血管网织细胞瘤诊治体会

目的探讨小脑血管网织细胞瘤的诊断和治疗。方法回顾分析12例血管网织细胞瘤患者的临床特征、影像学表现和治疗方法。结果11例囊性肿瘤行瘤结节全切，1例实体性肿瘤行全切除。术后无并发症。结论CT及MRI对小脑血管网织细胞瘤诊断具有特异性，手术治疗效果好。瘤结节和瘤体的完整切除是手术成功的关键。     

脑干肿瘤的手术治疗

手术治疗6例脑干肿瘤病人，次全切除2例，大部切除1例，部分切除3例。术后临床表现明显改善1例，改善3例，无变化2例，无一例术后死亡。作认为脑干肿瘤手术治疗成功的关键在于手术适应证的选择。弥漫性脑干肿瘤不适合手术治疗，局限性脑干肿瘤，囊性脑干肿瘤，颈髓延髓交界处脑干肿瘤及背侧外生性脑干肿瘤适合手术治疗。其中囊性脑干肿瘤，颈髓延髓交界处脑干肿瘤，背侧外生性脑干肿瘤常能做到肿瘤的全切除或次全切除。     

脑干肿瘤的手术治疗

手术治疗6例脑干肿瘤病人,次全切除2例,大部切除1例,部分切除3例.术后临床表现明显改善1例,改善3例.无变化2例,无一例术后死亡.作者认为脑干肿瘤手术治疗成功的关键在于手术适应证的选择.弥漫性脑于肿瘤不适合手术治疗,局限性脑于肿瘤、囊性脑干肿瘤、颈髓延髓交界处脑于肿瘤及背侧外生性脑干肿瘤适合手术治疗.其中囊性脑干肿瘤、颈髓延髓交界处脑于肿瘤、背侧外生性脑干肿瘤常能做到肿瘤的全切除或次全切除.     

脑干血管网状细胞瘤

本文报告了８例经手术证实的脑干血管网状细胞瘤。结合临床及ＭＲ表现，将其分为延髓近端组和延髓远端组，文中较详细地分析了两组的症状、体征，并论述了手术要点；特别提出了大的血管网状细胞瘤术后发生应激性消化道出血的可能机理及防治措施。本组除１例术后死于胃穿孔，其余均获得理想的预后。     

延髓肿瘤的外科临床表现（附68例临床报告）

目的：分析68例不同性质延髓肿瘤临床表现，有助于早期诊断，早期治疗。万法：全面分析68例延髓肿瘤临床症状与体征、神经放射学特征，评估术前呼吸循环功能，并结合术中所见和术后病理诊断，对不同性质肿瘤进行对比研究。结果：延髓肿瘤以后组预神经功能受累最为多见。良性肿瘤生长较缓慢，常见肿瘤巨大而临床症状轻微，很少发生明显呼吸循环功能改变。出血性肿瘤如海绵状血管瘤常突然发病，症状突然加重，表现出呼吸、循环改变，恶性度高的胶质瘤，病情进展迅速，临床症状较重。结论：延髓肿瘤早期症状元明显特异性，如果出现后组颅神经功能障碍及长束征或特发呼吸功能障碍者，宜进一步行MRI检查以明确诊断。     

延髓海绵状血管畸形出血致呼吸障碍的手术治疗

目的 探讨延髓海绵状血管畸形出血导致严重自主呼吸障碍患者的手术适应证及显微外科治疗.方法 回顾性分析4例延髓海绵状血管畸形出血导致严重自主呼吸障碍的临床资料,术前KPS评分平均17.5分,均行枕下后正中人路病灶切除.结果 4例延髓海绵状血管畸形均手术全切,自主呼吸均改善,肢体麻木和行走困难无显著改善,术后KPS评分平均60分,生活质量改善2例,稳定2例.平均随访15.0个月,无症状加重,随访KPS评分平均70分.结论 手术治疗延髓海绵状血管畸形出血伴自主呼吸障碍可取得良好预后.

Abstract：

Objective To discuss the surgical management of cavernous malformation (CM) in medulla oblongata with dyspnea after hemorrhage.Methods The clinical data of four patients with CM in medulla oblongata with dyspnea after hemorrhage were analyzed retrospectively.The mean preoperative Karnofsky Performance Scale (KPS) was 17.5.All of the patients underwent operation through posterior midline suboccipital approach.Results Complete resection was achieved in four patients.The main clinical symptoms of dyspnea, facial paralysis and pain were totally improved.But there was no change of motor deficits or sensory disturbance.The mean postoperative KPS was 60.Two patients were improved and others were stabilized.The mean follow - up duration was 15.0 months without recurrent hemorrhage.The recent mean KPS was 70.Conclusions Patients with CM in medulla oblongata suffering from dyspnea could obtain favorable prognosis through surgical treatment.     

Germinoma of medulla

Germinoma occurring in the medulla oblongata is extremely rare. We report a case of primary intracranial germinoma arising in the medulla oblongata of a 24-year-old postpartum female who presented with progressive weakness of upper and lower limbs, seventh nerve palsy, and decreased palatal movements. Her MR imaging showed a heterointense mass lesion in the posterior portion of upper medulla, the histology of which was reported as germinoma. Germ cell tumors should be considered in the differential diagnosis of tumors occurring in the brain stem.     

延髓髓内血管母细胞瘤的诊断与治疗

目的 探讨延髓髓内血管母细胞瘤的诊断与治疗。方法 回顾性分析7例患者的MRI表现及其手术治疗的效果。结果 这类肿瘤在MRI上可分为二型：A、实体型，B、囊肿型。所有患者均经手术全切除肿瘤，并经病理证实为血管母细胞瘤。术后患者神经系统状态好转者6例，加重1例。结论 颈髓MRI能对延髓髓内血管母细胞瘤作出定位、定性诊断，并可将其分型，以利选择不同的手术方法。延髓髓内血管母细胞瘤宜行积极手术治疗，手术方法随肿瘤类型不同而各异，应在离断供血动脉后沿正确的界面分离，最后切除肿瘤。     

脑干神经节胶质瘤的诊断及治疗

目的总结脑干神经节胶质瘤的诊治经验。方法回顾性分析10例脑干神经节胶质瘤的临床资料。肿瘤均位于延髓,其中单纯位于延髓3例,累及上颈髓3例,累及脑桥3例,累及小脑1例。均经枕下后正中入路,行显微手术治疗。结果肿瘤近全切除6例,大部分切除2例,部分切除2例。术后辅助放疗2例。病理诊断为神经节胶质瘤。随访3个月～12年,失访2例;存活7例,未见肿瘤复发;术后4个月因急性呼吸道梗阻死亡1例。结论显微神经外科手术是脑干神经节胶质瘤的首选治疗方法,手术主要切除突出到脑干外及脑干内相对表浅的肿瘤。脑干神经节胶质瘤预后较好,后组脑神经麻痹是其主要术后并发症。     

显微神经外科手术联合血管内栓塞治疗延髓背侧实质性多血性血管母细胞瘤

目的 探讨应用显微神经外科手术联合血管内栓塞治疗延髓背侧实质性多血性血管母细胞瘤的方法.方法 回顾性地分析11例延髓背侧实质性血管母细胞瘤患者采用显微神经外科手术联合血管内栓塞治疗方法,临床效果通过KPS评分进行评价.所有的患者均进行了术前的神经系统影像学检查,包括核磁共振成像（MRI）和全脑血管造影（DSA）.首先在全麻插管下对异常血管团进行血管内栓塞治疗,在凝血指标正常后,即一般在介入治疗后2周内再进行显微神经外科手术切除实质性血管母细胞瘤.结果 11例患者栓塞过程顺利,栓塞后即刻造影显示肿瘤血管和肿瘤染色大部分消失,未出现并发症.肿瘤均经显微神经外科手术全切除,术中均未输血,无手术死亡.术后神经系统的功能障碍改善和恢复者10例,1例因术后急性心肌梗死死亡.术后的随访显示除死亡病例外,肿瘤全切者无肿瘤复发,且KPS评分均有所增加.手术并发症主要有：交通性脑积水,切口感染,肺炎,切口脑脊液漏等.而且无论血管栓塞还是手术治疗,术中及术后均无正常灌注压突破综合征（NPPB）的发生.结论 对延髓背侧实质性血管母细胞瘤患者进行术前栓塞,对于提高手术的安全性,减少手术并发症具有一定的意义,而且术前对于供瘤动脉的栓塞范围应个体化,以不损伤重要脑组织供血为原则.     

Intractable Hiccup as the Presenting Symptom of Cavernous Hemangioma in the Medulla Oblongata: A Case Report and Literature Review

A case of intractable hiccup developed by cavernous hemangioma in the medulla oblongata is reported. There have been only five previously reported cases of medullary cavernoma that triggered intractable hiccup. The patient was a 28-year-old man who was presented with intractable hiccup for 15 days. It developed suddenly, then aggravated progressively and did not respond to any types of medication. On magnetic resonance images, a well-demarcated and non-enhancing mass with hemorrhagic changes was noted in the left medulla oblongata. Intraoperative findings showed that the lesion was fully embedded within the brain stem and pathology confirmed the diagnosis of cavernous hemangioma. The hiccup resolved completely after the operation. Based on the presumption that the medullary cavernoma may trigger intractable hiccup by displacing or compression the hiccup arc of the dorsolateral medulla, surgical excision can eliminate the symptoms, even in the case totally buried in brainstem.     

Isolated medulla oblongata function after severe traumatic brain injury

The objective was to report the first pathologically confirmed case of partly functionally preserved medulla oblongata in a patient with catastrophic traumatic brain injury.A patient is described with epidural haematoma with normal breathing and blood pressure and a retained coughing reflex brought on only by catheter suctioning of the carina. Multiple contusions in the thalami and pons were found but the medulla oblongata was spared at necropsy. In conclusion, medulla oblongata function may persist despite rostrocaudal deterioration. This comatose state ("medulla man") closely mimics brain death.     

Diffusion tensor imaging findings of the brainstem in subjects with tonsillar ectopia

We aimed to evaluate the differences between apparent diffusion coefficient (ADC), fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD) values obtained from different cranial sites in subjects with Chiari I Malformation (CM-I) and borderline tonsillar ectopia (BTE), and to determine correlations between diffusion tensor imaging (DTI) metrics and the severity of tonsillar ectopia. A total of 73 subjects with CM-I and BTE and 35 control underwent MRI and DTI. In our study, ADC values measured from the level of medulla oblongata and the RD values measured in middle cerebellar peduncles, thalamus, and globus pallidus were higher in CM-I patients than in controls. FA values at the medulla oblongata level and AD values at the medulla oblongata and pons level higher in patients with CM-I. ADC and AD values measured at the pons level were higher in BTE subjects than in controls. Compared with BTE, the CM-I subjects’ ADC values at the medulla oblongata and AD values at the pons level were higher. In addition, FAs at the pons and medulla oblongata level were higher. At the medulla oblongata level, a positive correlation was observed between ADC and the size of tonsillar ectopia. AD and FA values measured at the level of medulla oblongata and pons were positively correlated with the size of tonsillar ectopia. These findings may be related to the severity of microstructural changes involving neuronal tracts at the brainstem level due to tonsillar ectopia. DTI may be useful in determining the extent of microstructural changes at the tissue level in subjects with tonsillar ectopia.     

延髓损伤可能会引发局限型和广泛型急性胃粘膜出血

目的：通过动物实验，观察延髓损伤引起胃粘膜出血的表现形式和特点。方法：对１９只成年健康杂种狗，进行延髓一侧性损伤手术（１３只）和对照假损伤性手术（６只）；在损伤后，对胃粘膜表现进行胃镜的动态观察。结果：延髓损伤，可引起胃粘膜出血，而且发生率很高，达１００％；胃粘膜出血的严重程度及其预后有轻、重不同。结论：分析观察结果后认为：胃粘膜出血分可能有局限型和广泛型两个类型；延髓损伤的严重程度与胃粘膜出血的类型密切相关。