Neurofibromatosis associated with intra- and extracranial aneurysms and extracranial vertebral arteriovenous fistula

Neurofibromatosis associated with vascular disorders is generally known as a vascular neurofibromatosis. In the neurosurgical aspect, it has been pointed out that "moyamoya disease" -like stenosis and/or occlusion of the internal carotid artery are seen in some of the patients with neurofibromatosis. This 58-year-old female with multiple café-au-lait spots and neurofibromas all over the body was admitted to our hospital with complaints of left orbital tumor and tinnitus. She had been suffering from the left intraorbital neurofibroma since ten years of age. Radiological examinations revealed left intraorbital neurofibroma extending into the intracranial cavity, multiple aneurysms and arteriovenous fistula. The aneurysms were located at the left vertebral-posterior interior cerebellar artery junction and at the extracranial internal carotid artery. The arterio-venous fistula was demonstrated at the left extracranial vertebral artery. Pathological changes of the blood vessels in the vascular neurofibromatosis are essentially obstructive lesions due to intimal thickening. Only few cases of neurofibromatosis associated with aneurysms and arteriovenous fistula have been reported, and eleven cases of them could be reviewed in the literature. Six are with intracranial aneurysms, two with extracranial aneurysms and three with extracranial vertebral arteriovenous fistula,. It is interesting that in all cases the extracranial vertebral arteriovenous fistula are located on the left side. This is corresponding to the fact that the bone defect around the asterion is predominantly seen on the left side in cases of neurofibromatosis.     

Pediatric orbital eosinophilic granuloma with intra- and extracranial extension--case report.

A rare case of eosinophilic granuloma of the orbit in a 3-year-old boy presented as right upper eyelid swelling and proptosis. Computed tomographic scanning revealed a soft-tissue dense mass in the lateral wall of the right orbit. Magnetic resonance (MR) imaging demonstrated tumor extension into the orbit, anterior and middle cranial fossae, and extracranial region. The tumor was completely removed. Histological diagnosis was eosinophilic granuloma. No evidence of recurrence was found 14 months later. MR imaging is useful for diagnosis of the lesion, and particularly for surgical management.     

Hemodynamic analysis of associated extracranial atraumatic vertebral artery aneurysm and arteriovenous fistula. Case report

A case of atraumatic arteriovenous (AV) fistula of the extracranial vertebral artery associated with an atraumatic aneurysm of the contralateral extracranial vertebral artery is reported. The fistulous lesion was excised after distal and proximal ligation of the vessel. Subsequently, the contralateral aneurysm underwent spontaneous dissolution. Seven cases of extracranial vertebral AV fistulae associated with ipsilateral vertebral artery aneurysms (four traumatic and three as part of vascular dysplastic syndromes) have been reported previously.     

Glomus jugulare tumor with intra- and extracranial extension: a case report with MRI study

A case of glomus jugulare tumor with intra- and extracranial extension is described. The patient was a 63-year-old woman who complained of gait and memory disturbances. On admission neurological examination revealed recent memory disturbance, left deafness, left XI, XIIth cranial nerve palsies, and slight ataxic gait. Roentgenogram of the skull showed an enlarged left jugular foramen with bone erosion. Plain X-ray computerized tomography scan (X-CT) indicated obstructive hydrocephalus and X-CT with contrast enhancement revealed a mass lesion in the left posterior cranial fossa extending through enlarged left jugular foramen to the extracranial space toward the level of C2. Cerebral angiography demonstrated a large mass with blood supply from branches of left external carotid and vertebral arteries. The tumor stain was not remarkable. Left internal jugular vein was completely obstructed at the level of the second cervical vertebral body. Magnetic resonance imaging (MRI) clearly showed the tumor extending from the anterolateral portion to the second cervical vertebral body through the enlarged jugular foramen to the posterior cranial fossa. Brain stem and cerebellar hemisphere which were markedly compressed by the mass were clearly visualized. At first a ventriculo-peritoneal shunt was made and four weeks later subtotal removal of the tumor was undertaken. Histopathology of tumor specimen showed typical glomus jugulare tumor. MRI was considered to be very useful for the diagnosis and treatment of the glomus jugulare tumor with intra- and extracranial extension.     

Fatal intracranial hemorrhage after balloon occlusion of an extracranial vertebral arteriovenous fistula. Case report

A patient is presented who developed a fatal hemorrhage immediately after balloon occlusion of an extracranial vertebral arteriovenous fistula. The fistula was associated with marked retrograde flow not only from the contralateral vertebral artery but also from the carotid artery system through the posterior communicating artery and the basilar artery. The bleeding appeared to be caused by acute hemodynamic effects following abrupt occlusion of the long-standing fistula. A gradual staged occlusion or trapping procedure should be considered for the treatment of such vertebral arteriovenous fistulae.     

先天性动静脉畸形的新分期与治疗选择

目的 探讨颅外先天性动静脉畸形的临床分类方法及相应的治疗选择。方法 通过对6年内83个病例的临床表现、影像诊断、介入治疗、外科手术治疗结果的系统随访和分析，探讨该疾病的临床分期，解剖区域分布规律、各分期的治疗选择、不同介入材料对复发率的影响、进行未痊愈病例原因分析。结果 根据临床表现可将先天性动静脉畸形分为静止期、扩张期和失代偿期，前者以单纯介入治疗为主，后者以手术治疗为主，介入治疗的方法与材料选择对复发率等有重要影响，未栓塞病例的手术治疗要求依据DSA和CTA完整切除和具有丰富血供的组织移植重建。结论 对先天性动静脉畸形的临床分期和合理的治疗方案设计将使此类疑难病例的治疗大大易化。     

Unusual aneurysms of the extracranial carotid artery

Aneurysms of the extracranial internal carotid artery are rare and only 4 cases have been treated at the Wellesley Hospital since 1969. These aneurysms usually present as a painless lump in the neck and the 4 cases to be described manifest unusual presentations. One patient with a history of facial sepsis presented in the Emergency Department with what was thought to be a neck abscess. This was incised and drained before the true nature of the swelling was appreciated. Two patients presented with nerve palsies, one with a hypoglossal palsy due to stretching of the hypoglossal nerve over a large aneurysm and the second with recurrent laryngeal nerve palsy as a result of rupture of a false aneurysm from disruption of a saphenous vein patch inserted following carotid endarterectomy 9 months earlier. The fourth patient presented with a carotid bruit and had a stenosis distal to the aneurysm. Although rare, carotid aneurysms require recognition and early treatment if neurological sequelae are to be avoided.     

Unusual posterior fossa dural arteriovenous malformation in a neonate: case report

We report the case of an infant born with an unusual posterior fossa dural arteriovenous malformation that was supplied by the entire intracranial circulation. The lesion caused hydrocephalus and congestive heart failure that led to the infant's death.     

Cranial fasciitis of a neonatal case with massive intra- and extracranial extension

We report a neonatal male case of cranial fasciitis in childhood. He was born with a large skull tumor in his left fronto-parietal region. The day after his birth, he was admitted to our hospital. On inspection, the mass was firm, non mobile, and had two humped peaks. The size of the mass was 7 x 5 x 4 cm, and it was located on the left coronal suture and fontanelle. The overlying skin was normal. Skull X-rays revealed osteolytic defect and linear bone remnant skirted the outer rim of the tumor. CT scan showed a slightly high-density large frontal mass with a low density area in the center of it and marked enhancement. MR images revealed a large mass with massive intra- and extracranial extension. Cerebral angiography showed the remarkable vascularization of the tumor. Preoperatively, the branches of the external carotid artery were partly embolized. On surgery, the profusely bleeding, elastic hard, yellowish-white mass arising from the dura was partially removed with the surrounding bone. A small mass remained subdurally. The boundary of the mass and the dura was not well demarcated. After the operation, no neurological deficits were seen. 16 months after the surgery, MRI revealed the unfolded brain with no evidence of tumor. Histological examination showed characteristic proliferation of spindle-shaped fibroblasts embedded in a collagenous stroma. Inflammatory lymphocytes and acidophils were also noted. Electron micrograph revealed a tumor cell rich in rough endoplasmic reticulum and nuclei with numerous indentations. Immunostaining confirmed that these cells were positive for vimentin but negative for smooth muscle actin, GFAP, S-100 protein, and desmin. No recurrence of this tumor has been detected at four years of follow-up.     

Gliosarcoma with multiple extracranial metastases. Case report

A 68-year-old male was hospitalized because of headache, nausea, and disturbance of consciousness. Neurological examination on admission disclosed somnolence, disorientation, marked neck stiffness, papilledema, and quadriparesis. Computed tomography (CT) scanning demonstrated a round mass with marked contrast enhancement in the right sylvian fissure and small contrast-enhanced masses in the interpeduncular, quadrigeminal and ambient cisterns. CT also showed marked peritumoral edema, a midline shift, and hydrocephalus. The patient's consciousness level and respiration deteriorated 3 days after admission and a craniotomy was performed. The tumor, which was well demarcated, firmly attached to the sphenoidal ridge, and grossly appeared to be a meningioma, was totally removed. Histologically, the tumor had two well defined components, glioblastoma and fibrosarcoma. The patient underwent ventriculoperitoneal shunting, chemotherapy, and radiotherapy after surgery, but the primary tumor soon recurred, with scalp metastasis, and he died 5 months postoperatively. Autopsy revealed metastases to the liver, spleen, and spinal cord. The histogenesis of this mixed tumor and the mechanism of extracranial metastasis are discussed, and the literature is reviewed.     

Unusual posttraumatic porencephaly--case report.

An unusual case of posttraumatic porencephaly preceded by neither overt cerebral contusion nor hemorrhage is reported. The cerebral cortex just above the porencephalic cyst was found intra-operatively to be partially herniated into a fracture line, while the cortex elsewhere was completely intact. The porencephalic cyst communicated with the lateral ventricle. Apparently, brain herniation and the cyst-ventricle communication can be causative factors in the occurrence and growth of posttraumatic porencephaly.     

A case of fibromuscular dysplasia associated with intra- and extracranial multiple aneurysms]

A case of fibromuscular dysplasia (FMD) with intra- and extracranial multiple aneurysms is reported. A 42-year-old woman was admitted to Kagawa Central Hospital with severe headache and stiffness of the neck. CT scan showed subarachnoid hemorrhage predominantly in the left side of the basal cisterns and hydrocephalus. Angiography at admission revealed marked stenosis and dilatation of the extracranial major arteries and multiple aneurysms in the lt. PCA, lt. ICA, bil. VA, and the lt. renal artery. String-of-beads appearance was also seen in the branches of the lt. external carotid artery. During the operation, the PCA aneurysm which has been diagnosed as the ruptured one, was found to arise from the posterior communicating artery itself. It was thus a so-called true posterior communicating aneurysm. The aneurysm was trapped by clipping the artery on both the ICA and the PCA sides. The giant aneurysm of the lt. ICA was successfully treated by lt. STA-MCA anastomosis and ligation of the lt. ICA. Postoperative angiography demonstrated no visualization of the aneurysm and total occlusion at the origin of the lt. VA that had been patent preoperatively. On histological examination, intimal and medial hyperplasia was seen in the aneurysmal wall and occipital artery biopsied at operation. There has been no report of FMD associated with multiple and very rare posterior communicating aneurysms. Wide involvement of vascular change and advance of arterial occlusion noted by serial angiography indicates that FMD is a disease in which pathology would be progressive in some cases.     

Portosystemic arteriovenous fistula. Case report.

A case of the rare portosystemic arteriovenous fistula is presented. This lesion, although basically simple, is not trivial. The predominant cause of death is variceal bleeding. Most often, the fistulas are due to rupture of an aneurysm of the hepatic or splenic artery, gunshot wound of the abdomen, or the use of mass ligature during abdominal surgery. The treatment is excision when possible, reconstruction of the vessels when vessels cannot be sacrificed, and ligation when everything else fails. Angiography is indispensible in defining the fistula. Because of the circulatory strain produced by these fistulas and of the danger of portal hypertension even in the asymptomatic patient, the presence of a portosystemic arteriovenous fistula is sufficient indication for surgical treatment.     

Traumatic arteriovenous fistula. Case report

A young man with a fistula between the right femoral artery and vein due to bullet wound presented 6 years later with exertional dyspnea and palpitations and venous insufficency of the right leg. Angiography showed a wide (greater than 20 mm) arteriovenous fistula. Moderate cardiac hypertrophy and cardiac output 14.8 l/min were found. During division and closure of the fistula, 8 l/min flow was measured in the right external iliac artery. The cardiac symptoms disappeared immediately and the venous insufficiency gradually.     

Unusual type of spinal arteriovenous malformation

We describe an uncommon type of spinal arteriovenous malformation that is intradural rather than dural and that consists of a true fistula rather than a nidus of abnormal vessels between the anterior spinal artery and a draining vein. Its clinical significance and treatment are discussed.