Mycotic Aneurysm of the Internal Carotid Artery in an Unusual Intra-cranial Location

A child is reported who developed a mycotic aneurysm of the cavernous portion of the internal carotid artery following staphylococcal cellulitis and septicemia. The unique feature of this case was the conspicuous absence of clinical or arteriographic evidence of carvernous sinus thrombosis. The pathogenesis of mycotic aneurysms is discussed.     

侵袭性真菌性鼻窦炎的神经系统损害

目的：探讨侵袭性真菌性鼻窦炎致神经系统损害的诊断和治疗。方法：回顾性分析9例的临床表现、影像学及病理学。结果；全部病例既往无鼻窦炎病史，其中7例免疫功能低下，均以神经系统损害首诊于神经科，多呈急性暴发起病，病情凶险，表现复杂，以视觉障碍、头痛、偏瘫等常见。影像学和病理学提示真菌感染由鼻窦侵袭眶尖，海绵窦、颈内动脉、脑膜和脑实质等，鼻内窥镜检查有助于确诊，抗真菌药物治疗结合鼻窦切开清除术治疗，5例好转，4例死亡。结论：本病是免疫功能缺陷病人重要的致死性疾病，常引起严重的神经系统损害，应引起重视。     

Rhino-cerebral fungal infection successfully treated with supplementary hyperbaric oxygen therapy

Rhino-cerebral fungal infections are rare and difficult disorders to cure. We report the case of a woman presenting a left trigeminal neuralgia complicated by ophthalmoplegia and blindness. MRI demonstrated a lesion of the left orbital apex with extension into the cavernous sinus. Fungal infiltration (aspergillosis or mucormycosis), was seen on biopsy. High-dose liposomal Amphotericin B (5mg/kg/day) for six weeks was unsuccessful. Adjunctant hyperbaric oxygen therapy led to clinical and radiological improvement. Hyperbaric oxygen therapy is discussed in the medical management of rhino-cerebral yeast abscesses.     

Bacterial cavernous sinus aneurysm treated by detachable balloon technique

We describe a patient who developed bilateral cavernous sinus septic thrombosis secondary to a suppurative lesion on the left cheek. Despite clinical improvement, left oculomotor symptoms recurred suddenly. A carotid artery aneurysm within the cavernous sinus was diagnosed by means of magnetic resonance imaging and confirmed by digital angiography. Follow-up angiograms showed an initial decrease in the aneurysm size, with subsequent enlargement. A latex contrast-filled balloon was successfully placed within the aneurysm, preserving the carotid parent artery blood flow. Our case illustrates the usefulness of the detachable balloon technique in the treatment of bacterial aneurysms of the cavernous sinus as an alternative treatment to carotid artery ligation.     

曲霉菌病性痛性眼肌麻痹

目的:讨论导致痛性眼肌麻痹的曲霉菌病的病例特点及诊断。方法:对1例患者进行临床相关资料分析和文献复习。结果:曲霉菌病是痛性眼肌麻痹的原因之一,导致痛性眼肌麻痹的曲霉菌病起病急骤,病变进展迅速,可累及海绵窦、鼻窦、眶尖、颈内动脉,而出现眼肌麻痹,眶后、眶周疼痛,视力障碍,偏瘫等临床表现。活检可确诊曲霉菌病。结论:导致痛性眼肌麻痹的曲霉菌病容易误诊,通过病史及体格检查,并结合CT、MRI、活检可以确诊。     

Relevant imaging findings of cerebral aspergillosis on MRI: a retrospective case-based study in immunocompromised patients

The aim of the study was to assess neuroimaging patterns of cerebral aspergillosis with magnetic resonance imaging (1.5 T). The clinical and imaging data of nine patients were reviewed. Patients were included in the study if the diagnosis of aspergillosis was confirmed by either biopsy, autopsy, aspergillus antigen determination and/or neuroradiological and clinical response to specific treatment. Four patients had single or multiple abscesses presenting as ring-enhancing lesions on T1-weighted images, hypointensity of the ring on T2-weighted MR images and low to high signal intensity on diffusion-weighted imaging. Four patients had single or multiple infarctions affecting all compartments of the brain with hyperintensities on T2-weighted images in three of four patients, irregular parenchymal contrast enhancement in all patients and hemorrhagic transformation of the infarcted parenchyma in one patient. Diffusion-weighted images were positive in all ischemic areas. One patient with paranasal sinusitis developed a mycotic aneurysm of the internal carotid artery. Cerebral aspergillosis presents three principal neuroimaging findings: areas consistent with infarction; ring lesions consistent with abscess formation following infarction; and dural or vascular infiltration originating from paranasal sinusitis or orbital infiltration. Recognition of these patterns in cerebral aspergillosis may lead to more timely and effective diagnosis and treatment.     

海绵窦造影在颈动脉-海绵窦瘘及其并发症中的诊断和治疗意义

目的 了解颈动脉一海绵窦瘘(carotid-cavernous fistula,CCF)的血流动力学变化和颈内动脉海绵窦段及其周围静脉窦和静脉的病理改变,为制定更为合理的治疗方案提供依据。方法 1990年4月～1999年4月共收治CCF患者124例,28例施行眼上静脉(superior ophthalmic vein,SOV)海绵窦造影,其中A型17例,B型1例,D型10例。结果 海绵窦造影显示,海绵窦接近正常者12例(42.86％),其中海绵窦狭窄3例,网状海绵窦9例。海绵窦扩大者16例(57.14％),包括局限性扩张呈静脉湖状14例,其中海绵窦囊性占位者5例,即囊中囊表现,提示存在外伤性动脉瘤;海绵窦分隔2例。辅助动脉造影检查发现,CCF合并同侧颈动脉闭塞者2例,颈动脉狭窄3例,合并外伤性动脉瘤5例,其中2例发生严重鼻出血。28例均经眼上静脉入路或眼上静脉+动脉入路闭塞CCF及假性动脉瘤,治疗效果良好。结论 眼上静脉-海绵窦造影与颈动脉造影结合,于动脉外闭塞CCF瘘口或闭塞海绵窦瘘,可提高CCF治疗的成功率并保持载瘘动脉的通畅率,有助于CCF合并颈内动脉海绵窦段损伤及外伤性动脉瘤的诊断与治疗。     

Fatal rhinocerebral mucormycosis with intracavernous carotid aneurysm and thrombosis: a late complication of transsphenoidal surgery?

Mucormycosis is a rare opportunistic fungal infection. Rhinocerebral form of the disease mainly affects diabetic or immunocompromised patients. Mucormycosis have specific tropism for blood vessels leading to mucorthrombosis and less often to mycotic aneurysms. We report on a patient initially presenting with a severe sphenoid sinusopathy, who progressively evolved to cavernous sinus syndrome, internal carotid aneurysm followed by spontaneous thrombosis, chronic meningitis and ultimately fatal hypertensive hydrocephalus. Necropsy revealed a purulent infiltrate containing thin-walled, aseptate, right-angle branching, hyphae consistent with mucormycosis. His only relevant previous medical history was a transsphenoidal surgery for pituitary macroadenoma 21 years before. We hypothesize that post-surgical mucosal changes in the sphenoid sinus have been a favoring factor for delayed and invasive mucor infection.     

A case of cavernous sinus syndrome following a mycotic aneurysm of extracranial carotid artery]

A 66-year-old man was admitted to our department with left abducens palsy and pain in the territory of the left trigeminal nerve. He had a history of left mandibular osteomyelitis that had been treated for five years in the dental department. However, the osteomyelitis was resistant to therapy. Two months before this admission, he had an infectious aneurysm of the left extracranial carotid artery with occlusion. On admission, the ESR was 140 mm/hour. P-ANCA and antinuclear antibody were negative. Lumbar puncture revealed elevated cell counts (43% neutrophils) and protein. Microbiological studies were negative. Cranial MR images showed an enhanced lesion in the left cavernous sinus. His condition gradually improved with high dose of penicillin and low dose of corticosteroid. However, he died of pulmonary embolism after 81 days. At autopsy, the left extracranial carotid aneurysm was highly fibrose. The left CCA, ICA, and ECA were occluded from the origin of the left common carotid artery to the ICA in the cavernous sinus. There were also fibrosis, hemosiderin, and macrophages around these arteries, and parts of these arteries were destroyed. The left cavernous sinus lesion was also highly fibrose. These pathological findings indicated that there was old inflammation around the left extracranial carotid aneurysm, left carotid artery, and left cavernous sinus. We believe that the left cavernous sinus syndrome in our patient was caused by left carotid artery vasculitis induced by the left infectious extracranial carotid aneurysm. We also believe that this infectious aneurysm was caused by the left mandibular osteomyelitis.     

头颈部少见类型动-静脉瘘的血管内栓塞治疗

目的探讨头颈部少见类型动-静脉瘘血管内栓塞治疗的方法及临床价值。方法创伤性伴有基底动脉动脉瘤的基底动脉-基底静脉丛瘘、原始三叉动脉动脉瘤破裂臻致三叉动脉-海绵窦瘘、自发性和创伤性颈外动脉-海绵窦瘘、创伤性颈内动脉-海绵窦瘘伴有椎动-静脉瘘及创伤性颈内动脉-海绵间窦瘘各1例，对此6例少见类型动-静脉瘘患分别采用机械解脱弹簧圈、电解脱弹簧圈以及可脱性球囊技术进行血管内栓塞治疗。结果创伤性伴有基底动脉动脉瘤的基底动脉-基底静脉丛瘘和创伤性颈内动脉-海绵窦瘘伴有椎动-静脉瘘患分两次伶塞，而原始三叉动脉动脉瘤破裂致三叉动脉-海绵窦瘘、自发性和刨伤性颈外动脉-海绵窦瘘、刨伤性颈内动脉-海绵间窦瘘患均一次完成栓塞治疗。手术后临床症状和体征完全消失。无并发症发生。结论血管内栓塞是治疗头颈部少见类型动-静脉瘘的最佳方法，其操作简单．安全可靠，疗效好，并发症发生率低。应作为首选。     

Multiple aneurysms associated with bilateral carotid occlusion and venous angioma: surgical management risk-case report.

A unique case of multiple aneurysms associated with bilateral carotid artery occlusion and venous angioma is described. A 42 year old female presented with subarachnoid haemorrhage. Cerebral angiograms demonstrated(1) a ruptured saccular aneurysm in the right posterior cerebral artery,(2) bilateral occlusion of internal carotid arteries,(3) a rete mirabile in the subtemporal fossa fed by left external carotid artery which connected with the internal carotid artery at the cavernous portion where a saccular aneurysm had formed, and(4) a venous angioma in the posterior fossa. The ruptured aneurysm of the posterior cerebral artery was obliterated preserving the anterior choroidal arteries. However, a left hemiparesis developed and CT scan revealed a small low density area in the right posterior limb of the internal capsule postoperatively. A ruptured aneurysm associated with bilateral extracranial carotid occlusion poses a clinical dilemma and treatment of such cases is challenging and difficult. The non-surgical and surgical outcomes of ruptured cerebral aneurysms associated with internal carotid occlusion are reviewed.     

A case of TSH-secreting pituitary adenoma associated with an unruptured aneurysm: successful treatment by two-stage operation and gamma-knife]

We report a successfully treated case of invasive TSH-secreting pituitary adenoma associated with an unruptured internal carotid artery aneurysm by two-stage operation and gamma-knife radiosurgery. A 64-year-old woman was admitted to our department with a 3-year history of general fatigue and 1-year history of anxiety, palpitation and hyperhydrosis. Endocrinological examination revealed hyperthyroidism with elevated TSH, GH and somatomedin C. Magnetic resonance images demonstrated a tumor in the sella turcica which extended into the left cavernous sinus, furthermore, indicated aneurysm-like flow void at the ventral part of the left internal carotid artery. The aneurysm was confirmed by conventional angiography. Neck clipping of the aneurysm was performed through pterional approach as the first operation. One month later, at the second operation, the pituitary adenoma except for cavernous sinus portion was resected via the transsphenoidal approach. Immunohistological examination revealed positive for TSH and GH. Gamma-knife radiosurgery with a central dose of 33.3 Gy and peripheral dose of 17 Gy was carried out for residual tumor at the cavernous sinus under both MRI and CT guidance. Posttreatment course was uneventful with normalization of thyroid function at 16 months after gamma-knife. Two-stage operation and gamma-knife radiosurgery is effective for TSH-secreting adenoma extending into the cavernous sinus associated with an unruptured aneurysm.     

Cavernous aneurysm and pituitary adenoma: management of dual intrasellar lesions.

A 53-year-old female with a functioning pituitary adenoma was found to also have an unruptured asymptomatic aneurysm in the cavernous sinus portion of the internal carotid artery on MRI. The adenoma had a suprasellar extension with optic chiasm compression and extended into the right cavernous sinus. An aneurysm-like flow-void adjacent to the left internal carotid artery in the sella and embedded in the adenoma was also found. The aneurysm was confirmed by conventional angiography. We used a right fronto-pterional approach to clip the aneurysm and to remove the pituitary tumor in a one-stage procedure. The outcome was good on long-term follow-up. It is important to thoroughly evaluate the anatomic relations around the sella turcica prior to procedures with limited exposure, including transsphenoidal surgery, to avoid unrecognized complications and morbidity.     

Early operation of ruptured basilar artery aneurysm associated with bilateral carotid occlusion (moyamoya disease)

A 34-year-old Caucasian man presented with subarachnoid hemorrhage. Angiography revealed bilateral carotid occlusion at the cavernous sinus and an aneurysm at the basilar artery bifurcation. The whole brain was supplied with blood from the basilar artery and posterior cerebral arteries through a large number of collateral vessels to the internal carotid artery bifurcation, middle cerebral and anterior cerebral arteries: the moyamoya phenomenon. The aneurysm was clipped within hours of the subarachnoid hemorrhage. The relation between moyamoya disease and basilar artery aneurysms is discussed and some surgical and management considerations are given.     

Recurrent lymphocytic hypophysitis and bilateral intracavernous carotid artery occlusion. an observation and review of the literature

OBJECTIVES: Bilateral carotid artery occlusion associated with lymphocytic hypophysitis is exceedingly rare. We describe this association and review the literature.METHODS: The authors describe a 38-year-old woman with a history of severe headaches. Magnetic resonance (MR) imaging showed an intrasellar mass with invasion of both cavernous sinuses. Lymphocytic hypophysitis was diagnosed by transphenoidal biopsy. In the course of the disease, she developed symptoms of cerebral ischemia attributable to bilateral occlusion of her internal carotid arteries in both cavernous sinuses. She underwent bilateral superficial temporal artery-middle cerebral artery bypass surgery.RESULTS: The patient experienced progressive neurological recovery after surgery. A literature search revealed no other cases describing this unique association.CONCLUSIONS: Bilateral carotid artery occlusion may develop in the course of lymphocytic hypophysitis with cavernous sinus involvement. If indicated, cerebral revascularization should be performed to reverse cerebral ischemia.     

A case of fibromuscular dysplasia presenting with Wallenberg syndrome, and developing a giant aneurysm of the internal carotid artery in the cavernous sinus]

A 25-year-old man developed Wallenberg syndrome (WS). At that time his carotid angiography was normal. When he was 28 years old, he suffered from retinal artery embolism in the left eye. At the age of 30 years, he had an acute onset of abducens nerve palsy in his right eye. The carotid angiography showed a giant aneurysm at the cavernous sinus portion in the right internal carotid artery. At his age of 38, the right oculomotor, trochlear and trigeminal nerves were involved. A vertebral angiography revealed a bead-like formation, and a diagnosis of fibromuscular dysplasia (FMD) was made. An intensive angiographic examination revealed many stenotic or dilated lesions in the carotid, vertebral, coronary, renal, and hepatic arteries. A sural nerve biopsy specimen revealed that the sural vein was involved. In Japan only one case of FMD presenting with WS is known. FMD should be under consideration as an underlying disease, when WS occurred in younger patients with few risk factors. In this patient an angiography revealed no abnormality in the cavernous sinus portion of the internal carotid artery, when he suffered from WS. However, eight years later he was proved to have a giant aneurysm in the cavernous sinus portion. In conclusion, we support the hypothesis that aneurysm may originate from angiographically normal arterial wall in FMD.     

Usefulness of ultrasonography in a case of spontaneous carotid-cavernous fistula: a case report]

Since spontaneous carotid-cavernous fistula (CCF) is a dural arterio-venous fistula at the cavernous sinus, which is different from traumatic CCF and CCF associated with a ruptured aneurysm at the cavernous internal carotid artery, cerebral angiography is required in order to differentiate these condition. We here report a case of spontaneous CCF, in which a result of ultrasonographic evaluation of cervical arteries well corresponded with that of cerebral angiography. Ultrasonography showed increased blood flow and decreased pulsatility index in the ipsilateral external carotid artery, contralateral internal and external arteries, and these values in all arteries resolved within normal range after the interventional embolization. Ultrasonography is less invasive examination and can be easily performed even in outpatients for observation of spontaneous CCF.     

An autopsied case of pachymeningitis associated with a ruptured, cerebral aneurysm due to Aspergillus infection]

We reported a 64-years-old woman with pachymeningitis associated with a ruptured mycotic cerebral aneurysm due to Aspergillus infection. She had suffered from diabetes mellitus and been treated since she was 49 years old. She complained of headache at the age of 62 and loss of her left visual acuity three months later. She was treated by the pulse therapy of methylprednisolone as neuritis retrobulbaris and her visual acuity recovered. But her headache continued. Three months later, her right visual acuity was lost, and the pulse therapy was not effective this time. Six months later, she died of subarachnoid hemorrhage following acute meningitis. The autopsy was granted, but limited to the cranial cavity. Macroscopically, it disclosed brownish thickened dura around sella turucica involving trigeminal ganglion and optic nerve, and fresh subarachnoid hemorrhage in the basal cisterns and a ruptured aneurysm (3 mm in diameter) between internal carotid and posterior cerebral artery on the left side. Histologically, the brownish thickened dura was infiltrated by lymphocytes, plasma cells, and multinucleated giant cells. The wall around the aneurysm was infiltrated by lymphocytes and plasma cells as well as many fungi. Immunohistochemistry demonstrated the presence of Aspergillus in the thickened dura and the arterial wall around the aneurysm. There were lymphocytes and plasma cell infiltration in the basal subarachnoid space and scattered microabcesses in the brain. Although the first entry of Aspergillus to the dura was unclear, we assume that the final intravascular dissemination of Aspergillus from the dura caused meningitis and mycotic aneurysm.     

Cavernous sinus thrombophlebitis caused by sphenoid sinusitis--report of autopsy case.

A 34-year-old man developed fever, headache, nausea, double vision, exophthalmus, ptosis, disturbance of vision and oculomotor nerve palsy. Magnetic resonance imaging and cerebral angiography led to the clinical diagnoses of cavernous sinus thrombophlebitis and suspicion of bacterial aneurysm of the left internal carotid artery, respectively. Peptostreptococcus was detected in blood culture analysis. He died 15 days after admission. Systemic organs showed several septic changes. In particular, the bilateral cavernous sinuses were enlarged and showed severe neutrophilic leukocyte infiltration of the walls and organization, recanalization and abscesses in thrombi. In anterior to the middle cranial fossa, abscess-forming, necrotic, hemorrhagic meningitis, purulent sphenoid sinusitis, pyogenic osteomyelitis of the sphenoid bone, suppurative encephalitis, and inflammatory necrosis of the hypophysis were seen. Based on these findings, we diagnosed the patient with cavernous sinus thrombophlebitis caused by sphenoid sinusitis.     

A case of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome]

A 48-year-old female was seen because of left orbital pain. The neurological findings were normal at her first visit. She presented temporary double vision during conservative period. Plain CT revealed no mass around the sellar region. Enhanced CT revealed enhanced mass in the left cavernous sinus. MRI revealed low intensity lesion on both T1 and T2 weighted images. Enhanced MRI showed strongly enhanced mass extended from the left cavernous sinus to the dura of sellar floor, the contralateral cavernous sinus, and cerebellar tentorium. Angiography showed stenosis of the left internal cerebral artery. Biopsy from the dura of the tentorium was performed via anterior temporal approach. Abundant collagen fibers with hyalinization were seen in the thickened dura. A final diagnosis of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome was made. Symptoms were free just after the surgery, however, the regrowth of the tentorial lesion was found on MRI after one year. The size of the lesion decreased after administration of steroid.