Intracranial extra-axial cavernous (HEM) angiomas: tumors or vascular malformations?

INTRODUCTION: Extra-axial cavernous hemangiomas or angiomas [(hem)angiomas] are relatively rare lesions. They usually arise in relation to the dura mater intracranially or at the spinal level. Most of these lesions have been described in the middle cranial fossa at level of the cavernous sinus. Controversy still exists regarding the exact nature of these extra-axial cavernous angiomas: vascular tumor versus vascular malformation similar to intra-axial cavernomas. It has been suggested that they could represent an adult form of the hemangioma of infancy. Extra-axial cavernous (hem)angiomas often mimic meningiomas and their clinical behavior and imaging appearance are quite different than those of intra-axial cavernous angiomas. SUBJECTS AND METHODS: Five patients ranging in age from 24 to 63 years with a histologically proven dural cavernous angioma were retrospectively included. The lesions were located at level of the cavernous sinus (4 cases) and falx. CT and MR scans were performed in all cases and angiography in three patients. Four patients underwent surgery and a biopsy was performed in one case. One lesion was embolized before biopsy. Histology was available in all patients. RESULTS: In the operated patients, the lesion was totally resected in 2 cases and partially in the other 2. No postsurgical complication was noted. Histology revealed a vascular malformation composed of large vascular channels lined by flat endothelium and separated by fibroconnective tissue stroma. The pathological diagnosis was cavernous angioma. CONCLUSION: On the basis of the analysis of the literature and of our cases, intra-cranial extra-cerebral so-called cavernous (hem)angiomas present findings suggesting that they are vascular malformative lesions, analogous to the intra-axial cavernous angioma. A relationship with the hemangiomas of infancy seems unlikely. Correct terms for extra-cerebral cavernous (hem)angiomas are cavernoma, cavernous angioma, or venous vascular malformation of cavernous type . The term hemangioma should be avoided and reserved for the common vascular tumor of infancy.     

Therapeutical considerations in patients with intracranial venous angiomas

The clinical presentation of intracranial venous angiomas are usually headache, seizures or dizziness. Very often these anomalies are found incidentally on magnetic resonance imaging (MRI). We reviewed 35 patients, which were examined in our department between 1994 and 2000. Only one patient became symptomatic with an intracranial haematoma, which was removed surgically. In three patients an associated cavernous angioma was found, which was removed successfully with preservation of the coexisting venous angioma. There is no indication in operating a venous angioma because the risk of postoperative deterioration caused by venous infarction is high. When removing cavernous angiomas the associated venous angioma has to be preserved.     

Familial cerebral cavernous angiomas

Three families with two or more members affected by cavernous angiomas of the brain are described and the other 17 reported in the literature are reviewed. The familial occurrence of cerebral cavernous angiomas has been considered a rare event; nevertheless, the experience of the authors (positive familiarity in three patients where two or more relatives have been explored radiologically) suggests that cavernous angiomas of the brain might be, at least in most cases, a familial disease. Therefore, when a patient with cerebral cavernous angioma is observed, a careful familial history and the exploration of the familial members by computerized tomography or better still by magnetic resonance must be performed. The high frequency of multiple lesions in familial cases, the surgical indications and the role of the ultrasonic prenatal diagnosis are also discussed.     

Intramedullary spinal cord cavernous angioma in children: case report and literature review

Introduction Intramedullary cavernous angiomas (ImCA) of the spinal cord are very uncommon malformations especially in the pediatric population where only ten cases have yet been reported within the available literature. Case report In this paper, the authors report the case of a 12-year-old girl presenting with a T11 level hematomyelia because of a cavernous angioma. Microsurgical excision was performed with good clinical outcome and no magnetic resonance imaging evidence of residual cavernoma 30 months later. Discussion Despite the small number of cases reported in children, this one contributes to the literature identifying special features of presentation and management of ImCA for pediatric patients. Because a higher risk of recurrent bleeding has been demonstrated for ImCA, with dramatic clinical consequences, microsurgical removal remains the only definitive treatment.     

非海绵窦区硬膜海绵状血管畸形2例报道并文献复习

目的 探讨非海绵窦区硬脑膜海绵状血管畸形的影像学特点、诊断及治疗。方法 回顾性分析2例经术后病理证实的非海绵窦区硬脑膜海绵状血管畸形的临床资料，结合相关文献进行分析。结果 1例术前考虑大脑镰旁脑膜瘤，另1例术前考虑左侧横窦及天幕脑膜瘤。2例病灶均全切除，术后病理均证实为硬脑膜海绵状血管畸形，术后均恢复良好，未出现手术并发症，无需放疗等后续治疗。术后随访1年以上无复发。结论 非海绵窦区硬膜海绵状血管畸形极少见，容易误诊为脑膜瘤。手术切除时，应先阻断肿瘤位于硬膜或静脉窦的肿瘤基底，并尽量整块切除，否则可能遭遇汹涌的出血。该类疾病手术往往可安全有效的全切除病变，预后良好。     

Cavernous angioma of the cavernous sinus: imaging findings

Cavernous angiomas (cavernomas) of the cavernous sinus are uncommon, with only scattered reports in literature. Unlike their parenchymatous counterparts, they are intensely enhancing para-sellar masses, iso/hypointense in T1- and hyperintense in T2-weighted images. Differential diagnosis with para-sellar meningiomas and schwannomas can be difficult. We report three cases of this condition, describing findings of diffusion-weighted imaging in this kind of lesion for the first time in literature.     

Multiple spinal intramedullary cavernous angioma: case report

Spinal cavernous angiomas frequently accompany to cranial cavernous angiomas. Multiple spinal cord cavernous angiomas are very rare and to authors knowledge, only one case has been described having multiple intramedullary cavernous angiomas without cranial involvement until now. In this report, we present a case with acute paraplegia who had thoracic and cervical intramedullary cavernous angiomas and normal cranial magnetic resonance imaging.     

Development of a Posterior Fossa Cavernous Malformation Associated With Bilateral Venous Anomalies: Case Report

Venous angiomas (VAs) and cavernous malformations (CMs) are common cerebrovascular malformations. Frequently, these lesions are found in close proximity. The interrelationship between VAs and CMs has not yet been adequately defined. The authors report a case of a 48-year-old man with progressive dysarthria, dysmetria, and ataxia. Eight years previously, magnetic resonance imaging (MRI) revealed a solitary CM and bilateral posterior fossa VAs. Later imaging after neurological progression revealed the presence of 2 rather than 1 CM adjacent to the VAs. The sequential imaging suggests a causal relationship between VAs and some CMs. Furthermore, the detailed MRI permitted radiosurgical treatment of these CMs. The occurrence of de novo CMs adjacent to VAs on future imaging studies in other patients may help confirm the etiology of at least a subset of CMs.     

幕上脑内海绵状血管瘤与癫痫(46例报告)

目的探讨幕上脑内海绵状血管瘤所致癫痫的临床特点及治疗方法。方法1998—2003年在北京天坛医院顺序入院的72例幕上脑内海绵状血管瘤患者中，46例伴有癫痫发作。详细记录和分析癫痫发作的临床类型和特点，同时给予手术和药物治疗。并对治疗效果进行随访。结果46例患者行显微手术治疗，其中40例术前均给予抗癫痫药物治疗。药物治疗后27例发作次数减少，13例病情无改善。手术治疗后，癫痫均得到良好控制。结论幕上脑内海绵状血管瘤伴发癫痫单纯通过药物控制效果不佳，而显微外科手术效果良好。     

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.     

海绵窦断层及三维重建

目的对海绵窦断层结构进行研究,并建立海绵窦的三维模型.方法收集数字化可视头颅连续断层标本数据,从水平、矢状和冠状三个方位观察海绵窦大小、形态、内部结构和毗邻;利用医学图像可视化软件对海绵窦及其毗邻结构进行三维重建.结果海绵窦内各结构在断层层面上可清晰显示,所获得的海绵窦三维重建模型可以清楚显示海绵窦内神经、血管等重要组织结构的空间位置关系.结论海绵窦的断层研究和三维重建对于该区域的外科手术治疗具有重要意义.     

Progressive vision loss. A rare manifestation of familial cavernous angiomas.

We studied four generations of a family in which the index case had progressive loss of vision secondary to a cavernous angioma of the optic nerve and chiasm. Magnetic resonance imaging of the brain revealed multiple, asymptomatic intracerebral cavernous angiomas. Brain magnetic resonance imaging scans of the family members revealed multiple cavernous angiomas in the brother and paternal grandfather, but none in the father or his siblings. Autopsy reports of the paternal great grandfather noted multiple cavernous angiomas in the brain and abdominal viscera. We believe our patient to be the sixth reported case in which a cavernous angioma involved the optic chiasm and optic nerve. Magnetic resonance imaging is a sensitive and specific method of detecting cavernous angiomas. Cavernous angiomas have an autosomal dominant pattern of inheritance with variable penetrance. Surgical intervention in patients with symptomatic cavernous angiomas depends on the location and size of the lesion and associated surgical risks.     

小脑海绵状血管瘤（18例分析）

目的 :研究小脑海绵状血管瘤的临床。方法 :回顾性分析经手术病理证实的小脑ICCA 18例。结果 :18例共 19个病灶 ,单发17例 ,多发 1例 2个病灶。 11例表现为小脑自发性出血 ,7例表现为颅高压及小脑症状。 15例行头颅CT检查 ,诊断小脑ICCA 2例 ,2个病灶 ;头颅MRI检查 16例 ,诊断小脑ICCA 15例 ,16个病灶。 3个病灶为囊性变。 18例均行手术治疗 ,其中 2例急诊手术清除血肿并全切除病灶。手术无死亡 ,疗效满意。结论 :自发性出血是本病的主要症状。MRI具有较高的诊断价值。首选手术切除病灶。囊性小脑ICCA可能为实质性ICCA内部慢性出血的结果。     

显微手术治疗脑内型海绵状血管瘤43例

目的:探讨脑内型海绵状血管瘤(CA)的诊断和显微手术治疗效果。方法:回顾性分析2007年5月至2010年10月收治的43例经病理证实的脑内型海绵状血管瘤患者。结果:全部病例术前均行头颅MRI或CT检查,其中30例MRI检查结果为海绵状血管瘤,与病理结果相符,4例MRI检查结果与病理结果不相符。其他9例CT检查结果为脑内血肿。显微手术全部切除海绵状血管瘤,手术无死亡,效果满意。结论:MRI对脑内型海绵状血管瘤的诊断具有较高的敏感度和特异度。对于有明显临床表现的脑内型海绵状血管瘤患者,采用显微手术切除病灶,可获得良好的治疗效果。     

The cavernous sinus syndrome an anatomical and clinical study

The cavernous sinus is often involved pathologically, which can cause ocular motor nerve palsies with or without facial sensory disturbances. Consequently several clinical features of ocular motor nerve palsies have been described.

In this article we present a study of the cavernous sinus syndrome, and compare this syndrome with other nerve palsy syndromes caused by lesions in or adjacent to the cavernous sinus.

The clinical features are explained by means of an anatomical study of the cavernous sinus.     

Total removal of an intramedullary cavernous angioma by transthoracic approach

Intramedullary cavernous angiomas are rare vascular malformations; all published cases have been surgically approached posteriorly by standard laminectomy. We describe the case of a 63-year-old man with an intramedullary cavernous angioma, anteriorly located in the thoracic spinal cord. The angioma was operated on by transthoracic approach and totally removed.     

经额颞硬膜外入路进行海绵窦手术

目的:研究经硬膜外人路进行海绵窦直接手术的方法。方法:在经福尔马林固定的尸体头颅标本上,模拟硬膜外入路进行海绵窦及其邻近结构的显露和解剖。结果与结论:经额颞硬膜外入路可以充分显露海绵窦,且神经、血管损伤少,优于传统的硬膜内入路,适合于大多数海绵窦手术。     

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.     

巨大海绵窦海绵状血管瘤的硬膜内入路显微外科治疗

目的探讨巨大海绵窦海绵状血管瘤(cavernous sinus cavernous hemangiomas,CSHs)的临床特征及显微外科治疗方法,为CSHs的治疗提供临床经验。方法回顾性分析2009年1月—2018年12月解放军东部战区总医院神经外科收治的15例CSHs患者的临床及随访资料;分析患者的临床表现、颅神经受累情况、手术方式、术后并发症及随访结果。结果15例CSHs患者占同期收治的颅内海绵状血管瘤(CHs)患者的5.6%。临床表现为头痛者11例、视神经损害者5例、动眼神经损害者6例、面部感觉异常者2例、肢体无力者1例。15例患者通过扩大翼点、翼点或颞前小切口入路经硬膜内切除血管瘤,均完整切除;无死亡病例。10例患者术后出现同侧Ⅲ、Ⅳ、Ⅴ及Ⅵ神经部分麻痹。术后获得随访的患者9例,随访时间5~117个月,平均48.9个月,均无肿瘤复发表现;其中4例患者遗留长期海绵窦颅神经功能障碍。结论CSHs多具备特征性影像学表现,术前多可明确诊断。经硬膜内入路手术切除是治疗巨大CSHs的有效方法,术中应尽量按界限整块切除,以减少出血。海绵窦内神经功能的保留仍是目前巨大CSHs手术治疗的难点。     

海绵窦海绵状血管瘤的影像特征和显微手术

目的 总结海绵窦海绵状血管瘤的诊断和手术治疗经验,探讨其影像特征和手术要点.方法 对2001年10月至2008年10月收治并手术切除的13例海绵窦海绵状血管瘤进行回顾性分析.病灶最大径3.5～6 0 cm,均经MRI证实和显微手术切除.结果 病变被全切除9例,大部切除3例,活检1例,无手术死亡.海绵窦神经保留情况:8例保留动眼神经;9例保留三叉神经第Ⅱ、Ⅲ支;其余均未能保留.结论 海绵窦海绵状血管瘤可以根据其MRI的特征性影像获得确诊.合适的手术人路、娴熟的显微外科技术是全切肿瘤的必备条件.海绵窦内神经的保留仍然是未能完全解决的问题.

Abstract：

Objective To analyze the imaging character and surgical therapy of cavernous hemangiomas in cavernous sinus ( CSHs) .Method From 2001 to 2008, 13 patients with CSHs were surgically treated in our department.The diameters of CSHs varied from 3.5 cm to 6.0 cm.Results All the patients were operated on with no mortality.Complete tumor removal was achieved in 9 cases, partial tumor removal in 3 cases, and biopsy in 1 case.Cranial nerve Ⅲ was preserved in 8 cases and cranial nerves V2,V3 in 9 cases.In the other cases, cranial nerves in cavernous sinus were sacrificed unavoidably.Conclusions CSHs can be diagnosed preoperatively according to MRI and can be removed successfully by experienced surgeons who are familiar with operative approaches and microsurgical technique.However,great efforts should be made to decrease postoperative cranial nerve deficits.