脑干海绵状血管瘤影像学

目的：结合临床表现特点，对４８例有组织学结果的脑干海绵状血管瘤的影像学表现进行分析。方法：４８例依发作方式分为两种类型。一类表现为突然发作的颅神经及传导束症状，部分为反复类似发作；另一种为静止性发作。所有病人均进行了ＣＴ及ＭＲＩ检查。并通过手术得到病理证实。结果：所有病例ＣＴ及ＭＲＩ均表现出海绵状血管瘤的特点。在非增强ＣＴ扫描表现为等或稍高密度，在增强ＣＴ扫描，病灶表现为轻至中度强化，水肿及占位效应均不明显。ＭＲＩ扫描表现为高信号或混杂信号，代表不同时相的出血及胶质样变。部分病例可见典型的桑椹样或网络样结构。除１８例病灶位于延髓或中脑外，大多病灶位于桥脑。３例在梯度回波扫描可见多发病灶。有１例组织学可见海绵状血管瘤的血管腔内存在弹性纤维；４例可见引流静脉及供血动脉。结论：桥脑是脑干海绵状血管瘤最常见部位；影像学特别是ＭＲＩ表现与病程及病变构成有关；部分组织病理学具有与其它血管畸形重叠的特点。手术彻底切除病灶对防止复发非常重要。     

脑内海绵状血管瘤的CT及MRI诊断

目的探讨CT及MRI对脑内海绵状血管瘤(cavernous angiomas,CA)的表现和诊断价值。方法收集资料完整经手术病理证实的36例脑内CA患者的CT及MRI资料,重点分析其影像表现特征。结果 CA可位于脑内任何部位,单发病灶多见,周围无或轻度水肿,无占位效应,CT平扫为稍高密度影,钙化占68.7%,增强扫描大都无强化;MRI表现为桑椹状混杂高信号,周围有云絮状低信号环,增强后病灶大都无强化。结论脑内海绵状血管瘤的MRI表现有特异性,MRI对脑实质内CA的检出率优于CT,特别是MRI的T2WI像有助于明确诊断。     

主要表现为癫痫的脑海绵状血管瘤的治疗

目的探讨以癫痫为主要表现的脑海绵状血管瘤合理有效的治疗方法。方法收集我科2003年1月～2009年12月治疗的以癫痫为主要临床表现的脑海绵状血管瘤患者资料35例。单纯药物治疗组(17例):按照血药浓度检测结果进行药物剂量调整及换药,且全部病例疗程都在3年以上;手术联合药物治疗组(18例):严格根据癫痫手术要求进行术前评估及术中监测,切除致痫灶,术后继续使用抗癫药平均1年以上。对两组患者癫痫控制情况及抗癫药使用种类进行对比分析(平均随访2.9年)。结果手术联合药物治疗组患者有16例癫痫得到良好控制,药物治疗组仅6例得到控制(2=8.4777,P0.05),而且手术联合组较药物治疗组抗癫药使用种类明显减少。结论对于主要表现为癫痫的脑海绵状血管瘤手术联合药物比单纯药物治疗更有效。     

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。     

Intracranial cavernous angiomas: diagnosis and therapy

The authors reviewed the case histories of 10 patients with intracranial cavernous angiomas treated from 1985 till 1987. Two patients are described in detail and are illustrated by CT scan, MRI scan and angiography. The diagnostic and therapeutic problems of intracranial cavernous angiomas are discussed.     

脑内海绵状血管瘤的MRI和CT诊断

目的分析脑内海绵状血管瘤的CT和MRI影像学特点。方法回顾性分析68例CT和MRI表现,并比较CT和MRI优缺点。结果海绵状血管瘤位于幕上55例,幕下脑干4例,小脑9例。68例病灶有出血54例,钙化17例。MRI检查。T1加权像病灶显示为短T1高信号,周边为轻度低信号影;T2加权像病灶中央呈高、低混杂信号影,周边为极低黑色信号环。增强有轻度强化或无强化。CT平扫为斑片状不规则形高密度区。增强后有轻度强化或无强化。结论应用MRI诊断脑内海绵状血管瘤,其敏感性高,特异性强。对脑内海绵状血管瘤的显示MRI明显优于CT。     

Management of cerebral cavernous angiomas in children presenting with seizures

Until recently intracranial cavernous angiomas were thought to be rare vascular malformations that usually presented in adulthood as an intracerebral hemorrhage, an expanding mass lesion, or with the new onset of seizures. Prior to the advent of computed tomography (CT), and more recently magnetic resonance imaging (MRI), their diagnosis in childhood was extremely rare. However, the CT and MRI features of cavernous angioma are quite distinctive and allow early diagnosis and treatment. Advances in surgical techniques permit successful removal of these potentially devastating lesions and amelioration of the associated seizure disorder. Seven children with cerebral cavernous angiomas have been treated at the Children's Hospital Medical Center since 1980. Six children presented with seizures and one with an intracerebral hemorrhage. All had characteristic findings on CT and/or MRI and underwent surgical excision of symptomatic lesions. Intraoperative sonography, electrocorticography, and cortical mapping were used when indicated and were found to be helpful in the surgical management of these patients. Our experience suggests that symptomatic cerebral cavernous angiomas in children are not as rare as previously thought and that surgical treatment using modern neurosurgical techniques is both safe and appropriate and can be helpful in the management of associated seizures.     

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.     

Surgical treatment of intracranial cavernous angiomas.

We present a surgical series of 35 patients (25 males and 10 females) with histopathologically verified intracranial cavernous angiomas. The 35 malformations were located as follows: 21 were in the cerebral hemispheres; 4 in the lateral ventricles, 4 in the brain stem; and 6 in the cerebellum. Seizures and focal neurological deficits were the main clinical features observed in patients with intracranial cavernous angiomas. A number of these vascular malformations were misdiagnosed by computerized tomography. In the last 10 years, magnetic resonance imaging has been the most sensitive method for detecting these lesions. Thirty-five cavernous angiomas were treated surgically; in 33 patients a complete excision, and in 2 patients subtotal excision were obtained. One of the patients died one year after the operation. The overall outcome was good in all of the 34 remaining patients, resulting in improved seizure control or neurological deficit. The rationale for neurologic differential diagnosis and surgical treatment and follow up results are discussed.     

Carotid cavernous aneurysm presenting as pituitary apoplexy.

The authors report an interesting case with a ruptured internal carotid artery aneurysm that presented as a sellar haematoma mimicking radiologically a pituitary adenoma, and clinically a pituitary apoplexy. A 53-year-old woman presented with a 2-week history of episodic severe headache and vomiting associated, 3 days prior to admission, with left ophthalmoparesis and transient right hemiparesis. Brain MRI showed a large intra- and suprasellar mass suggestive of a pituitary macroadenoma. Hormonal profiles showed hyperprolactinaemia and subsequent cerebral angiography demonstrated a carotid cavernous aneurysm. The patient underwent surgery via a subfrontal approach to manage both lesions. At operation, the suspected pituitary adenoma was revealed to be a sellar haematoma; the aneurysm was successfully clipped. Postoperatively, the patient developed hypotension and right hemiparesis which, as well as the third nerve paresis, progressively improved to full recovery. At 12 months follow-up the patient is neurologically intact and generally well. The clinical features, the management of such a case and the importance of differential diagnosis in the acute stage are emphasised and discussed along with relevant literature.     

颅内海绵状血管瘤的显微手术治疗

海绵状血管瘤 (cavernous angiomas, CA),约占隐匿性血管畸形的20%[1].由于其"隐匿性"的特点,使得临床诊断、治疗较为困难.本文报道了1999年6月至2004年6月间,同济医院经手术及病理证实、资料完整的颅内CA患者21例,介绍其临床特点及处理方法.     

脊髓海绵状血管瘤的外科治疗

脊髓海绵状血管瘤是一种少见的脊髓髓内血管畸形,常致严重的脊髓损害.本文总结四年来作者治疗此病12例的经验.病人多为青壮年,肢体瘫痪和二便失禁为其主要症状.病变为一团浆果样薄壁血管窦,出血后血块上皮化使病变体积增大,多发生于脊髓胸段,病程可为急性发病型、双峰型和慢性进行型.反复小量出血和脊髓微循环失调是脊髓受损的主要机理.MRI为确诊的唯一手段.手术完全切除病变是最主要的治疗方法.     

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（CA）的临床特征和治疗策略。方法回顾性分析30例颅内CA的临床资料,其中脑内型29例,脑外型1例。采取临床观察4例,显微手术治疗26例。结果 4例临床观察病人,随访无特殊不适。26例显微手术治疗,均镜下全切病变,效果满意,无死亡病例。术后随访3个月～3年,病人均恢复良好。结论对有症状的CA积极行显微手术治疗可获得良好效果,无症状的CA可临床观察。脑内型CA行伽玛刀治疗需要慎重。     

The surgical management of cerebral cavernous angiomas

Abstract

Caver~ou: angioma (CA) is a h.amartomatous hemorrhagic lesion which has received a great deal of atention In recent years due to Improvement of neuroimaging with magnetic resonance and heightened clInIcal awareness. Long consIdered to be rare, its actual prevalence is now recognized to be of 0.9%. Cavernous angiomas may be multiple, particularly in patients with familial form. It may be associated with a variety of clinical syndromes attributed to focal microhemorrhages or less frequently to gross bleeding. CA are usually dIagnosed between the age of 20 and 50 with a highest clinical. incidence in the fourth decade. A female predominance is observed in regard to bleeding. The male patients are more at risk for seizures. The recent series ofMR ima~ing confirm that CA even when multiple can be asymptomatic in a significant number of cases. Surgery IS the treatment of choice in order to eliminate the risk of hemorrhage and improve the control of seizures. Minimally invasive approaches are now adopted with reduced postopertive .morbldlty. We report our expenence in surgical management of cerebral CA and suggest a clasSIfication of the lesIons accordIng to surgical accessibility and residual morbidity. [Neural Res 1998 20: 597–606]     

颅内海绵状血管瘤的临床治疗经验

目的 探讨颅内海绵状血管瘤(ICCA)的治疗策略.方法 回顾性分析55例经病理证实的颅内海绵状血管瘤病人的临床资料.其中采用常规开颅方式手术30例,神经导航手术19例,立体定向手术6例.结果 全切除52例,大部切除3例.术后无死亡,无颅内出血.术后主要并发症为感染、脑水肿和脑梗死,对症治疗后均好转.结论 神经导航手术是...