伽玛刀治疗脑内海绵状血管瘤

目的：评估伽玛刀治疗脑内海绵状血管瘤的效果。探讨治疗的适应证和制定治疗计划的注意点。方法：回顾性分析1994年至1995年间,用伽玛刀治疗57例脑内海绵状血管瘤,边缘剂量平均20．6Gy(9-25.2Gy)30%-80%的等剂量曲线包绕病灶。结果：53例平均随访4．2年。4例失访。1例死亡。影像随访中见19例病灶缩小。5例治疗后4月到39月再次出血。21例治疗后癫痫发作频度和程度减轻,6例主诉癫痫未改善。4例额叶（A）停药后未再出现癫痫。17例病灶位于额顶叶及额叶内侧功能区的患者治疗后4月至25月出现肢体肌力减退、失语等症状。33例病灶周围出现中到重度水肿;5例出现明显的肢体运动障碍。2例治疗后14月和16月再次外科手术切除病灶。3例出现明显视力下降。结论：（1）以边缘剂量16Gy为界,边缘剂量较高时治疗后再出血率明显减低。（2）癫痫发作频度和程序减轻与较高的治疗剂量和水肿的程度较重有关。额叶的绵状血管瘤疗效较显著。（3）同样剂量照射相同体积的血管畸形和海绵状血管瘤,后者较前者临床实践中出现严重放射性脑损伤的比率明显增高。（4）从治疗后再手术切除的病理标本中,未找到血管瘤内完全机化闭塞的确切证据。（5）脑内海绵状血管瘤的伽玛刀治疗必须慎重。     

脑海绵状血管瘤选择性治疗

目的观察选择手术切除及伽玛刀治疗脑海绵状血管瘤效果。探讨伽玛刀治疗的适应证。方法回顾1996~2005年手术10例,伽玛刀治疗26例,随访4个月~9年。对癫痫、出血、病灶及局部神经功能障碍控制、变化、改善观察和评估。结果手术10例,8例恢复正常,2例遗留轻度限局神经功能障碍,可做轻工作。CT、MRI复查:9例病灶消失,1例疑有病灶部分残留行伽玛刀治疗,无再出血及癫痫发作病例。伽玛刀治疗26例,随访21例。随访时间4个月~28个月。局部神经功能障碍观察:治疗前4例有锥体束征,治疗后3例恢复,1例无变化。癫痫发作观察:治疗前发作者19例,16例治疗后半年无发作而停药始无发作。3例治疗后偶有发作,服药半年停药,再未发作。CT及MRI复查:治疗后2~28个月,复查1~3次。病灶消失1例,4例无变化,缩小16例,其中2例灶周水肿、1例水肿伴灶内出血症状加重,经治疗逐好转。结论神经外科显微手术切除是首选根治方法。对病灶位于难以手术部位,多发灶难以一次切除,病人难以承受手术或不接受手术症状显著者,伽玛刀是一种安全可选择的治疗方法。对降低出血率、癫痫及病灶控制、改善症状是有效的。对病灶缩小或消失、降低出血率的机制等问题,尚需进一步研究。     

Genetics of cerebral cavernous malformations

The past few years have seen rapid advances in our understanding of the genetics and molecular biology of cerebral cavernous malformations (CCM). This article summarizes the recent cloning of the CCM1, CCM2, and CCM3 genes, which are responsible for autosomal dominant CCM, and also describes current hypotheses for their roles in integrin and p38 mitogen-activated protein kinase-mediated regulation of angiogenesis. A mouse model of CCM has been generated by mutation of the Ccm1 gene, and it indicates a role for that protein in arterial development. Future studies will probably focus on integration of data from each of the three CCM genes into a single model of the pathogenesis of cavernous malformation.     

γ-刀治疗脑内海绵状血管瘤

脑内海绵状血管瘤(cavernous angioma, CA)并不少见,但由于其生长缓慢,自然病史不十分清楚,故理想的治疗方法尚未确定.我们分析了北京奥海γ-刀中心1996年11月至2002年12月收治的84例脑内海绵状血管瘤的临床特点和治疗情况,并复习文献,旨在确定γ-刀治疗海绵状血管瘤的疗效和适应证.     

颅内海绵状血管瘤

海绵状血管瘤(CM)是较为常见的隐匿性脑血管畸形,常规脑血管造影不能显示病变.可发生于任何年龄,发病高峰为30～60岁,男女比例相等.有反复出血倾向,常呈自限性;病灶内可合并血栓和钙化.影像学可见病灶内不同演变时期的出血产物,周围组织可见陈旧性出血所致含铁血黄素沉积.X线检查仅见钙化,难以定性诊断.脑血管造影常无异常发现,合并静脉畸形时可见静脉聚集.CT检查显示,病灶多呈类圆形或分叶状略高密度影,不均匀,其内可见钙化和出血,病灶无明显占位征象和水肿.有30％～ 50％的海绵状血管瘤CT检查可无异常表现.MRI检查为首选诊断方法,可直接显示病灶内不同时期的出血征象(T1WI和T2WI序列)及其周围含铁血黄素沉积[梯度回波序列(GRE)和磁敏感加权成像(SWI)].     

Intracerebral cavernous angioma

Thirty four cases (18 operated, 16 non-operated), of cavernous angioma are reported. The presenting symptoms and signs were epilepsy in 22 cases, neurologic deficit in 9 and cerebromeningeal hemorrhage without vigilance disorders in 3. In the operated group 15 angiomas were supratentorial, 3 were subtentorial. In the non-operated group symptoms and signs were epilepsy in 11 cases and a neurologic deficit related to a brain stem lesion in 4/5 cases. Follow-up of 2 to 39 years after the first fit episode and of 1 to 7 years after initial diagnosis suggested that the risk of hemorrhage is low. Indications for neurosurgery are discussed as a function of the site of the angiomas and of the operative hemorrhagic risk.     

Intracranial cavernous angioma]

Cavernous angiomas are vascular malformations that belong to the group of hamartomas. Evidence to support the hypothesis that some intracranial cavernous angiomas are benign vascular tumors is lacking. As they are angiographically occult, cavernous angiomas were largely overlooked and were considered rare before CT scan and MRI became available. Both of these techniques provide a quick and accurate diagnosis in cases of epilepsy, brain hemorrhage or intracranial hypertension. Most of the hemispheric cavernous angiomas are situated subcortically, in the neighborhood of the rolandic fissure. In the brain stem, they can be found mostly in the pons. Cerebellum and spinal cord are seldom involved. Cavernous angiomas may occur in children, as well as in familial forms, or in association with other visceral locations. It is important to know that some cavernous angiomas are able to grow, owing to a variety of mechanisms, which may result in rupture and cerebral hemorrhage. The improvement of both imaging and surgical techniques have reduced the risks of death or heavy sequelae. Some deeply located cavernous angiomas, however, are still inaccessible to surgery.     

脑内海绵状血管瘤的伽玛刀治疗(附85例报告)

目的评价脑内海绵状血管瘤伽玛刀(γ-刀)治疗的效果。方法105例脑内海绵状血管瘤进行γ-刀治疗,病灶直径大小0.4￣1.8cm(平均1.2cm)。采用多个等中心照射,周边剂量12￣15Gy,平均13Gy,40%￣60%的等剂量曲线包绕病灶,中心剂量20￣30Gy,平均24Gy。85例获得随访。结果随访期6￣72个月(平均36个月)。5.8%(5/85)再出血;31%(26/85)病灶缩小,69%(59/85)无变化;20%(17/85)病灶周围脑水肿,经治疗后脑水肿明显减轻好转。50%(15/30)癫痫发作频率和程度减轻。41%(35/85)临床症状改善,40%(34/85)症状无明显改善,19%(16/85)症状加重,17.6%(15/85)经治疗后恢复,1例遗留明显肢体活动障碍。结论当海绵状血管瘤位于主要功能区或深部组织,手术风险极大时,可选择γ-刀治疗;控制周边剂量<15Gy,可以获得较好的疗效和较低的并发症。     

以癫痫起病的幕上脑海绵状血管瘤的治疗

目的 探讨以癫痫起病的幕上脑海绵状血管瘤（CCA）的治疗方法及影响术后癫痫控制率的相关因素。方法 2008年1月至2014年1月收治以癫痫起病的幕上CCA患者76例,其中药物组（不愿接受手术者）25例,口服抗癫痫药物治疗。显微手术组51例,显微手术切除病灶及致痫灶,术后继续抗癫痫药物治疗。所有患者出院后随访1~5年,按Engel分级评定疗效,Engel分级Ⅰ~Ⅱ级为疗效满意。结果 显微手术组疗效满意45例（88.2%）,药物治疗组癫痫疗效10例（40.0%）,两组疗效满意率差异有统计学意义（P<0.05）。癫痫病程≤1年的患者术后疗效满意率明显高于癫痫病程>1年的患者（P<0.05）。>结论 以癫痫起病的幕上CCA显微手术治疗效果良好,建议尽早手术治疗;影响术后癫痫控制率的因素为癫痫的病程。     

Spinal epidural dumbbell-shaped cavernous angioma

A 23 year-old man, with a two month-history of back pain, presented with spastic paraplegia and hypesthesia of both lower limbs. A myelo C.T. revealed a spinal epidural dumb bell-shaped mass extending in the intra and extra vertebral spaces through the enlarged intervertebral foramen. Total recovery was observed three months after surgery. Pathological examination of the tumor showed it to be a cavernous angioma. Primary epidural angioma is rare: in 2 reported dumb bell-shaped spinal epidural cavernous angiomas, radiological findings were also suggesting a spinal neurinoma.     

Cerebral cavernous angioma in children

Cerebral cavernous angioma is a rare vascular malformation at any age and is very rare in childhood. In the literature available to us, we have been able to trace only 50 cases, to which we have added the 6 cases from our own series. The incidence in pediatric group is higher at 0–2 years (26.8%) and at 13–16 years (35.7%). The clinical onset shows epilepsy in 45.4% of cases, hemorrhagic syndrome in 27.3%, intracranial hypertension in 16.4%, and focal neurological deficits in 10.9%. Furthermore, we discuss the neuroradiological features (CT, angiography, and MRI) and the therapy of pediatric cavernous angioma.     

显微手术治疗小脑海绵状血管瘤

目的总结小脑海绵状血管瘤的临床表现及手术治疗效果。方法对12例均经手术切除和病理证实的小脑海绵状血管瘤进行回顾性分析。结果11例急性起病,表现为小脑自发性出血。慢性起病1例,表现为渐进性共济失调、行走不稳。头颅MRI平扫与增强多表现为T1高信号,T2高或混杂信号,病灶周围有环形低信号带。磁敏感加权成像序列（SWI）更有助于诊断。12例均采用显微手术治疗,所有病灶均完整切除,疗效满意。结论自发性出血是本病的主要症状,多有共济失调等小脑受损体征。MRI具有较高的诊断特异性,手术是处理小脑海绵状血管瘤安全和有效的首选方法。     

Trigonal cavernous angioma: case report

The authors report on an intraventricular cavernous angioma located at the right trigone in a 25-year-old male patient presented with a predominantly intralesional haemorrhage. Neuroimaging led to an accurate preoperative diagnosis although the typical low intensity perilesional ring of gliosis and hemosiderin was not present. The lesion was microsurgically removed using an stereotactically guided posterior temporal transsulcal approach.     

Familial cavernous angioma without clinical haemorrhage

The incidence and natural history of cerebral cavernous malformations remain poorly defined. MRI now provides anaccurate means of diagnosis and follow-up of these lesions. Hereditary cavernous angiomata have been reported in several racial groups, revealing asymptomatic family members with significant numbers of lesions. We report a family of Anglo-Saxon origin with nine members among three generations affected via an autosomal dominant mode of inheritance. MRI of seven affected family members revealed 74 lesions. Autopsy of two further family' members who died of unrelated causes also demonstrated the presence of cavernous angiomata. No lesion was associated with anomalous venous drainage. Two affected family members had seizures. Although six lesions exhibited the characteristic mixed intensity T2 spin echo signal suggesting haemorrhage, no lesion has caused a clinically apparent haemorrhage. This family offers a unique perspective of the natural history of this disease. No clinically apparent haemorrhage has occurred in 2418 lesion years. The benign nature of these lesions suggests that, in clinically unruptured familial cavernous angiomas, without accompanying anomalous venous drainage, a conservative approach may be warranted irrespective of their location.     

Genetics of cavernous angiomas

Cerebral cavernous malformations (CCM) are vascular malformations that can occur as a sporadic or a familial autosomal dominant disorder. Clinical and cerebral MRI data on large series of patients with a genetic form of the disease are now available. In addition, three CCM genes have been identified: CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10. These recent developments in clinical and molecular genetics have given us useful information about clinical care and genetic counselling and have broadened our understanding of the mechanisms of this disorder.     

脊髓海绵状血管瘤的显微外科治疗

目的总结脊髓海绵状血管瘤的手术经验。方法回顾性分析39例海绵状血管瘤病人的临床表现、术前MRI表现、术中所见、手术切除情况、术后MRI表现及随访结果。均在显微镜下行手术治疗,根据肿瘤与脊髓有无明确边界,决定肿瘤切除的程度。手术前后采用McCormick分级评价脊髓功能。结果镜下见肿瘤有明确边界者行手术全切除,共34例;肿瘤有相对边界但不明确者仅行近全切除,共4例;肿瘤无边界且病人术前功能较差者行部分切除,计1例。病理证实均为海绵状血管瘤。术后McCormick分级改善30例,无变化5例,加重4例。结论MRI是脊髓海绵状血管瘤术前诊断最有效的方法;手术全切除病变是最主要的治疗方法,有症状者应积极行手术治疗。     

De novo development of intracranial cavernous angioma

We report on the de novo development of intracranial cavernous angiomas. The patient is a 16-year-old boy with multiple cavernous angiomas who has experienced four episodes of intracerebral haemorrhage. Our investigations suggest that repeat magnetic resonance imaging (MRI) is recommended for routine follow-up monitoring.