Intracavernous schwannoma of the abducens nerve: a review of the clinical features, radiology and pathology of an unusual case.

We report a case of an intracavernous abducens nerve schwannoma presenting in a 19 year old male of Mediterranean origin. This is only the second report of an abducens nerve schwannoma located entirely within the cavernous sinus. The lesion resulted in a progressive diplopia and focal abducens palsy. The clinical, radiological and pathological features are presented. A literature search has identified only four other abducens nerve schwannomas arising completely or partly within the cavernous sinus. We review these cases and aspects of other ocular cranial nerve schwannomas.     

上颌窦-翼腭窝-海绵窦手术入路的显微解剖学研究

目的对上颌窦-翼腭窝-海绵窦手术入路相关结构进行显微外科解剖学研究,为手术入路提供解剖学基础.方法利用10例经福尔马林固定的国人成人尸头共20侧,完全模拟该手术入路,对入路相关解剖标志进行了详细地显微解剖、观察、拍摄、测量和统计.结果上颌窦后壁与翼腭窝区结构复杂,有重要的神经和血管经过,是颅前、中窝与鼻腔、窦肿瘤互相蔓延的通道.该入路可较好地显露海绵窦前外侧相关的结构.结论该手术入路是海绵窦入路的一个特殊方法,拓展海绵窦手术入路的路径,为利用颅底硬膜外间隙进行入路提供了依据.     

A case of Tolosa-Hunt syndrome accompanied by facial and vestibular nerve damage]

A 37-year old man, who had repeatedly suffered from transient ophthalmoplegia in his left eye at the age of 29 and 36, developed left painful ophthalmoplegia accompanied by ipsilateral facial nerve palsy in August, 1991. Neurological examination revealed involvement of the left oculomotor, trochlear, ophthalmic division of the trigeminal, abducens, facial and vestibular nerves. Gadolinium-enhanced MRI which was taken at the acute phase of the illness demonstrated markedly enhanced left cavernous sinus and adjacent thickened dura mater in the middle cranial fossa. At the remission phase after starting corticosteroid therapy, these enhanced lesions were no longer observed even in enhanced MRI studies. We diagnosed him as suffering from Tolosa-Hunt syndrome presently accompanied by facial and vestibular nerve damage because of his history of illness, confined lesion in the left cavernous sinus and steroid-induced remission. We concluded that Tolosa-Hunt syndrome may be accompanied by damage of other cranial nerves in its course and that repeated gadolinium-enhanced MRIs are necessary for diagnosis and observation of the patients.     

手术切除从颅底侵犯海绵窦的肿瘤

目的探讨手术切除侵犯海绵窦的颅底肿瘤的指征及手术要点。方法自1998年11月至2002年5月，中日友好医院神经外科与中国医学科学院肿瘤医院头颈外科合作，连续切除侵犯海绵窦的颅底肿瘤32例，其中鼻咽纤维血管瘤7例，脊索瘤5例，鼻咽癌和鼻咽囊腺癌5例，副鼻窦癌5例，神经鞘瘤3例，嗅神经母细胞瘤1例，颞下翼腭窝低分化癌2例，颞下翼腭窝肉瘤3例，恶性纤维组织细胞瘤1例。23例曾经1次或多次手术切除后复发。对临床资料进行回顾性总结。结果根据肿瘤主体的部位分别选用经上颌骨入路、颞下耳前入路、或额眶入路。全部病例术中显露满意，肿瘤均得到肉眼切除，受累的颅神经一并切除，无手术死亡，术后无偏瘫等严重并发症。、术后辅以放射治疗。随访3—50个月，平均19个月，失访3例，4例术后3—6个月死亡，4例带瘤生仔，21例健在。结论由下向上侵犯海绵窦的颅底肿瘤可以手术切除，近期效果满意。对颅底正常和病理性解剖结构的熟练掌握以及多学科医生的密切协作是手术取得成功的关键。     

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.     

Parasellar syndromes

The parasellar compartments are located lateral to and on either side of the sella turcica. The cavernous sinuses are the most prominent anatomic feature of the parasella. Each sinus consists of a plexus of veins through which runs the intracavernous portion of the internal carotid artery. Ocular motor nerves three and four travel within the dural covering of the cavernous sinus to the superior orbital fissure, and cranial nerve six travels through the carotid sinus itself, giving rise to parasellar syndromes, which have distinctive clinical features. Ophthalmoplegia occurs as a result of damage to these ocular motor nerves and variable involvement of oculosympathetic nerves. Facial pain, dysesthesia, and paraesthesia are caused by damage to one or more of the divisions of the fifth cranial nerve, travelling in the dural wall of the cavernous sinus. Tumors, such as meningiomas, frequently cause parasellar syndromes, as do aneurysms of the intracavernous portion of the internal carotid artery, carotid-cavernous fistulas, and cavernous sinus thrombosis. Inflammatory conditions such as Tolosa-Hunt syndrome, ischemia to small vessels supplying the cavernous portion of the cranial nerves, and infections can cause this syndrome. Magnetic resonance imaging is the investigation of choice and therapy is specific to the cause of the parasellar syndrome, but now includes more aggressive endoscopic and microsurgical intervention, and radiosurgery.     

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.     

Multiple cranial nerve palsies: analysis of 979 cases

BACKGROUND: To my knowledge, no large series of multiple cranial neuropathies is available. OBJECTIVES: To examine the seats and causes of multiple cranial neuropathies in a large group of inpatients. DESIGN: Personal case series. SETTING: Wards of a large municipal hospital and affiliated rehabilitation hospital. PATIENTS: A consecutive series of 979 unselected inpatients with simultaneous or serial involvement of 2 or more different cranial nerves. RESULTS: Cranial nerves VI (565 cases), VII (466 cases), V (353 cases), and III (339 cases) were most commonly affected. The locations and causes were diverse, with cavernous sinus (252 cases), brainstem (217 cases), and individual nerves (182 cases) being the most frequent sites, and tumor (305 cases), vascular disease (128 cases), trauma (128 cases), infection (102 cases), and the Guillain-Barré and Fisher syndromes (91 cases total) being the most frequent causes. Recurrent cranial neuropathy was uncommon (43 cases, 106 episodes, 136 nerves), with diabetes mellitus (14 cases), self-limited unknown causes (14 cases), and idiopathic cavernous sinusitis (10 cases) being the usual causes. CONCLUSION: While the locations and causes of multiple cranial neuropathy are highly diverse, the fact that tumor composes more than one quarter of cases places a premium on prompt diagnosis.     

采用Dolenc入路处理海绵窦病变

目的 探讨海绵窦手术入路方法，以期提高手术效果。方法 在8个尸头上（16侧）按Dolenc手术入路的基本要领进行解剖练习，应用于临床12例，其中10例为海绵窦内肿瘤（脑膜瘤5例，垂体瘤3例，脊索瘤2例），1例为累及海绵窦的眼动脉巨大动脉瘤，1例为海绵窦段颈内动脉巨大动脉瘤。结果 Dolenc手术入路主要分两步；首先从硬膜外去除遮挡海绵窦的部分骨质（前床突，蝶骨翼等），然后将海绵窦上壁和外侧壁的硬膜广泛剥离开，暴露其深面的颅神经。采用此入路，10例海绵窦内肿瘤有5例全切。3例近全切，动脉瘤顺利夹闭，除2例持久的外展神经麻痹，未带来新的神经功能缺损。结论 Dolenc手术入路显露充分，安全，为彻底根除某些海绵窦病变提供了机会。     

The antero-medial triangle: the risk for cranial nerves ischemia at the cavernous sinus lateral wall. Anatomic cadaveric study

OBJECTIVE: Vascular damage in the cavernous sinus can cause ischemic injury to the cranial nerves. An appropriate anatomical knowledge of the blood supply to the cranial nerves can help to reduce the morbidity associated with cavernous sinus surgery. MATERIAL AND METHODS: Three formalin-fixed and six adult cadaveric fresh heads, with common carotid arteries injected, were used for anatomical dissection in this study. A fronto-temporal craniotomy was performed and the cavernous sinus was explored according to the Dolenc technique. With microsurgical dissection and photographic documentation, we demonstrate the anatomy of the superior orbital fissure artery in the antero-medial triangle. RESULTS: The 12 explored cavernous sinuses demonstrated the presence of two principal branches directly from the intracavernous internal carotid artery that supply the cranial nerves: the infero-lateral trunk and the meningohypophyseal trunk. The artery of the Superior Orbital Fissure (SOF), originated more often from the infero-lateral trunk, and vascularized the III, IV, VI, and VI, and ophtalmic division of the trigeminal nerve (TGN VI) at their entry in the fissure. CONCLUSION: In this study we demonstrate that the superior orbital fissure artery is a branch from the infero-lateral trunk which runs immediately under the reticularis layer at the level of the anteromedial triangle in the lateral wall of the cavernous sinus. The blood supply to all cranial nerves in the SOF is at risk to injury when the lateral wall of the cavernous sinus is transgressed at the anteromedial triangle since the SOF-artery runs superficially at this level.     

海绵窦肿瘤的诊断和显微手术切除

报告13例海绵窦肿瘤,包括6例垂体腺瘤、5例脑膜瘤、1例神经纤维瘤和1例脊索瘤,主要表现为头痛、视力减退、突眼、眼球活动障碍和内分泌紊乱。CT和MR对确定肿瘤是否侵入海绵窦内具有重要意义。13例肿瘤分别经海绵窦外侧壁、上壁和内壁切除,无手术死亡。5例发生颅神经损伤(38.5％)。作者介绍了手术方法及防止术中出血和颅神经损伤的体会。     

海绵窦内颅神经血液供应显微外科解剖研究

目的 为减少海绵窦显微外科手术时损伤海绵窦内颅神经血供而研究这些颅神经的血液供应解剖。方法 50具成人尸体海绵窦标本,在解剖显微镜下解剖和分析。结果（１）９０侧（占90％）的标本示动眼神经由颈内动脉的下外侧动脉分支供血;（2）滑车神经近段有74例（占74％）的标本示其接受下外侧动脉的分支供血,有26例（占26％）标本示其接受脑膜垂体动脉分支天幕动脉的供血,滑车神经远段主要由下外侧动脉的分支供血;（3）在Dorellos管区域,外展神经近段接受脑膜背侧动脉的血供,中段及远段由下外侧动脉的分支供血;（4）三叉神经的眼支和上颌支由下外侧动脉的分支供血,三叉神经节的内侧部由下外侧动脉和天幕动脉的分支供血,中部由下外侧动脉的分支供血,外侧部由下外侧动脉的分支或由脑膜中动脉供血。结论 下外侧动脉在海绵窦内颅神经供血中起重要作用。术中我们应保护这些血供,就可减少术后颅神经功能障碍。     

Intraoperative electrical stimulation for identification of cranial nerve nuclei.

The purpose of this study was to evaluate the feasibility and usefulness of cranial nerve nuclei monitoring during resection of brainstem cavernous malformations. Eleven patients with brainstem cavernous malformations underwent resection of their malformations utilizing cranial nerve nuclei monitoring. Cranial nerves V and VII were monitored by placing electrodes in muscle groups innervated by these nerves and recording manipulation-induced neurotonic discharges and triggered electromyographic (EMG) activity, after electrical stimulation of the corresponding brainstem nuclei. Seven of 11 procedures (64%) with cranial nerve nuclei monitoring were noted to have cranial nerve nuclei activity corresponding to manipulation of the nuclei. The cavernous malformation was completely resected in 5 of 7 cases with cranial nerve nuclei activity and in all 4 cases without activity. In the remaining 2 cases, the cavernous malformation was not resected due to the proximity of the monitored cranial nerve nuclei to the cavernous malformation and to increasing neurotonic activity as the cavernous malformation was approached. None of the 11 patients had new permanent postoperative deficits corresponding to the cranial nerve nuclei monitored; 1 patient had a transient partial facial palsy lasting 2 days. Preliminary results indicate that cranial nerve nuclei monitoring proves useful in preserving neurologic function and reducing surgical morbidity during resection of brainstem cavernous malformations, particularly indicating when lesion resection places these nuclei at risk.     

A case of Tolosa-Hunt syndrome starting from abducens nerve palsy alone]

A 59-year-old man was admitted to our hospital because of right abducens nerve palsy without orbital pain. One month later right orbital pain appeared and two months later the pain changed to an aching severe pain and the right oculomotor, trochlear and trigeminal nerves were also involved. The administration of corticosteroid dramatically reduced these symptoms. The diagnosis was Tolosa-Hunt syndrome (THS) based on these findings. THS starting with abducens nerve palsy alone is rare, representing only 5.1% of 118 patients. In our patient, the newly developed dynamic MRI was able to reveal the right cavernous sinus lesion. The diagnostic usefulness of dynamic MRI for cavernous sinus lesions has been demonstrated for such cases.     

Meningiomas of the cavernous sinus: the surgical resectability and complications

The optimal management for patients with cavernous sinus meningiomas is to evacuate tumor without causing mortality or morbidity. The records of 16 patients, including 11 women and 5 men ranging in age from 31 to 63 years, underwent surgical treatment for this condition were reviewed. Completeness of tumor resection, cranial nerve morbidity, complications, mortality, the internal carotid artery encasement and outcome were studied. Total removal was achieved in six patients. Of ten patients who underwent subtotal resection there was one death and four were sent to radiotherapy. Morbidity was 24% for cranial nerves controlling extraocular motor function; trigeminal nerve function did not improve after surgical treatment. Symptomatic recurrence occurred in two patients who underwent subtotal tumor resection and in one who underwent complete tumor resection. The average follow-up period was of 26 months. According to our findings, we conclude: 1) the resectability of meningiomas of cavernous sinus depends on the degree of internal carotid artery involvement; 2) total resection of meningiomas confined in cavernous sinus is rare; 3) morbidity of the cranial nerves is significant; 4) subtotal resection is an effective mean to obtain control of the disease.     

A case of Raeder's syndrome caused by metastatic malignant lymphoma

A case of Raeder's syndrome caused by metastatic malignant lymphoma was reported. The patient was 67-year-old male. He had complained of diplopia, ptosis and frontal headache at the left side. Neurological examinations revealed left incomplete Horner's syndrome (miosis and ptosis, but normal facial sweating) and left abducens palsy, which was considered to be Raeder's syndrome Group 1 (Boniuk and Schlazinger's classification). CT scan, MRI and angiography demonstrated a mass lesion in the left cavernous sinus extending to the sphenoparietal sinus, and a mass lesion in the anterior part of the superior sagittal sinus. During his hospitalization, enlargement of the left cervical lymph nodes was noticed. "Malignant lymphoma (non-Hodgkin)" was diagnosed on the basis of biopsy. Group 1 of Raeder's syndrome is rare, but it is important to define the site of lesion, which is located around the paratrigeminal region at the middle cranial fossa. Because these lesions are very small and metastatic in many cases, various neuroradiological investigations, especially MRI, are necessary for early diagnosis and early treatment.     

经耳前颧弓硬膜外入路切除海绵窦肿瘤60例分析

目的 总结回顾2002年至2009年经治的海绵窦病变60例的临床特点、手术人路及手术效果.方法 神经鞘瘤18例,海绵状血管瘤23例,皮样囊肿9例,脑膜瘤4例,脊索瘤3例,垂体瘤3例.均经耳前颧弓硬膜外入路切除.结果 神经鞘瘤18均全切,海绵状血管瘤23例,全切18例,5例有残留.皮样囊肿9例全切,脑膜瘤4例,全切3例,次全切1例.脊索瘤3例,结合经鼻蝶窦入路手术,均达到了全切.垂体瘤3例全切.结论 经耳前颧弓硬膜外入路切除海绵窦病变是一个理想的手术入路,可以充分显露病变,减少对脑组织的牵拉,也可以明确Ⅲ～Ⅵ脑神经和颈内动脉的位置,减少神经和血管损伤的概率.对与动脉或神经粘连无法彻底切除的病变可以辅以立体定向放射治疗.

Abstract：

Objective To review our experience of microsurgery for 60 cavernous sinus tumors from 2002 to 2009.The clinical features,surgical techniques and outcome of cavernous sinus tumor in 60 cases were investigated retrospectively.Methods The patients included 23 hemangiomas,18 shwannomas,9 dermoid cysts,4 meningiomas,3 chordomas,3 pituitary adenomas.AIl the tumors were removed with subtomperal preauricular extradural approach.Results The tumors were removed satisfactorily.The shwannomas were totally removed. The hemangiomas were totally removed in 18,near-totally removed in 5 cases.Nine dermoid cysts were removed totally.For the 4 meningiomas,3 were removed completely,neartotallv removed in 1 cases.The 3 Chordomas were resected near-totally and achieved a completely removal with combined approach.Conclusion The subtomperal preauricular extradural approach is a rational choice.It can reveal the cranial nerve branches and artery at an early stage so that cranial nerves Ⅲ～Ⅵ and internal carotid artery can be preserved during operation.The tumor exposure is ideal and brain traction and contusion are slightly.The adjunctive radiotherapy is demanded for residual tumors adhering to nerves and arteries severely.     

小脑脑桥角海绵状血管畸形三例并文献复习

目的 总结小脑脑桥角(CPA)区海绵状血管畸形(CMs)的临床表现、影像学特征及治疗特点.方法 2005年6月至2010年6月我科共收治CPA区CMs病例3例,进行报告.搜索MEDLINE上自1985年至2010年文献查到此类病例5例,进行文献复习.结果 文献复习的5例CMs临床表现为脑神经及小脑受累症状,但无脑干受压症状;影像见CMs均呈囊实性,与内听道及颅底硬膜无粘连;术中见CMs与周边结构有粘连,但可进行分离;手术治疗效果满意.本文报告的3例临床特征与之相符.结论 当CPA区见囊性病变与内听道及颅底硬膜无粘连时,应考虑到CMs的可能,显微外科手术切除多能获得良好的疗效.

Abstract：

Objective To investigate the clinical presentation, imaging features and treatment experience of cystic cavernous malformations ( CMs ) in the cerebellopontine angle ( CPA ) . Methods Three patients with the CMs of the CPA between 2005 and 2010 in our institute were summarised. Five patients from MEDLINE search of the English literature from 1985 to 2010 for CMs of the CPA were identified. We reviewed the characteristics of these 5 cases and our cases. Results Their presenting symptoms of 5 cases in the literature included headache, injury of cranial nerves in the CPA and cerebellar symptoms. But they did not show signs of brain stem compression. MRI and /or CT scans showed that the lesions in the eight cases of CMs all had solid and cystic components, and no adhesion with internal auditory canal and skull base dura mater was present in the CPA. Despite the fact that cystic CMs also involved cranial nerves, cerebellum, brainstem and arteries, they could be separated from these surrounding structures thanked to the presence of well - margined adhesion, which was contrary to solid CMs. We reported three cystic CMs of the CPA in our institute that was the same as the above. Conclusion Although cystic CMs of the CPA had no established imaging features, a diagnosis of CMs may be suspected when a cystic lesion is present in the CPA and does not involve internal acoustic meatus or dura matter of the skull base. Skillful microsurgical techniques will secure good outcomes for patients with cystic CMs in the CPA.     

Serial Neuroimaging in Tolosa‐Hunt Syndrome with Acute Bilateral Complete Ophthalmoplegia

Tolosa‐Hunt syndrome (THS) is a very rare, relapsing, and remitting painful ophthalmoplegia caused by nonspecific granulomatous inflammation in the cavernous sinus. To our knowledge, bilateral complete, simultaneous palsies of all 3 cranial nerves associated with extraocular movement have not been reported. We describe the first such patient with bilateral THS that responded quickly to corticosteroid therapy. A 54‐year‐old man presented with a periorbital and frontal headache with acute bilateral severe blepharoptosis and fixed eyes, which dramatically responded to corticosteroid therapy. He had diabetes mellitus type II. Brain MRI showed granulomatous inflammation in both cavernous sinuses and thickening of the surrounding dura mater of the cranial base, suggesting the coexistence of focal hypertrophic cranial pachymeningitis. Our experience indicates that steroid therapy with strict control of blood sugar should be considered in patients with THS complicated by diabetes. MRI is a valuable tool for serially monitoring the response of lesions to treatment in THS.     

Cavernous malformation of the hypoglossal nerve: case report and review of the literature

OBJECTIVE: To describe a patient who presented with a hypoglossal nerve palsy caused by a cavernous malformation, review the literature on cavernous malformations associated with cranial nerves and the differential diagnosis of hypoglossal palsy. RESULTS: Partial resection of the lesion was achieved and the diagnosis of cavernous malformation proven histologically. CONCLUSIONS: Involvement of a cranial nerve by a cavernous malformation is very uncommon and the facial nerve is the example most frequently reported. This case report adds another possible site for this rare occurrence.