Unique vascular structures of a radicular arteriovenous fistula at the craniocervical junction along the first cervical spinal nerve: A case report.

Background: An arteriovenous fistula (AVF) at the craniocervical junction (CCJ) is a rare vascular malformation. Definitive diagnosis and curative treatment of CCJ AVF are challenging. Case Description: A 77-year-old man presented with subarachnoid hemorrhage. Cerebral angiography showed an AVF at the CCJ, which drained into a radicular vein. The lesion was fed by a vertebral artery, anterior and lateral spinal arteries (LSAs), and the occipital artery (OA). There were two unique structures: the LSA originating from the posterior inferior cerebellar artery of the extracranial V3 segment and the OA feeding the shunt. Curative treatment involved two steps: endovascular embolization of feeders using Onyx and surgical shunt disconnection. Feeding arteries were blackened by Onyx, which helped identify the location of the shunt. The shunt was located behind the first cervical (C1) spinal nerve, and the draining vein was confirmed on the deep side of the nerve. A clip was applied to the draining vein distal to the shunt. Tiny vessels supplying the shunt were then coagulated referring to blackened arteries. Conclusion: A radicular AVF at the CCJ along the C1 spinal nerve had unique vascular structures. Definitive diagnosis and curative treatment were achieved by combining endovascular embolization using Onyx and direct surgery.

Successful carotid artery stenting with a double-layer micromesh stent for spontaneous extracranial internal carotid artery dissection: a case report.

Extracranial internal carotid artery dissection is a relatively rare disease in Japan. We herein report a case of a 60-year-old woman with spontaneous left internal carotid artery dissection with a dilated and dissected cavity. Following the identification and measurement of the true and false lumens using intravascular ultrasound, a double-layer micromesh stent (Casper stent; Microvention, Terumo, Tustin, CA, USA) was deployed for post-dilation. No perioperative complications were observed, and the patient was discharged on postoperative day 6.

Usefulness of an Electromagnetic Navigation System for Direct Percutaneous Puncture of the Superior Ophthalmic Vein.

Objective: We report a case of dural arteriovenous fistula (dAVF) at the cavernous sinus treated by direct puncture of the superior ophthalmic vein (SOV) using an electromagnetic navigation system. Case Presentation: The case involved a 70-year-old male patient who presented with mild chemosis, proptosis, and abducens palsy of the right eye. In this case, we used an electromagnetic navigation system for direct puncture of the SOV. Angiographic obliteration of the fistula was confirmed and the visual symptoms recovered well after surgery. There were no complications associated with direct puncture of the SOV using the electromagnetic navigation system. Conclusion: Direct puncture of the SOV to obliterate a dAVF is a possible alternative choice of treatment when the usual transvenous access route fails. To reduce the risk of complications, an electromagnetic navigation system is useful.

Sinus Pericranii with Dominant Venous Outflow in the Superior Eyelid.

Sinus pericranii (SP) located in the superior eyelid is an unusual clinical presentation. Here, we report a case of 72-year-old woman with an unruptured cerebral aneurysm presented with an SP located in the left superior eyelid. The SP was found to have a dominant venous outflow from the bilateral frontal region with an arterialized blood flow pattern on color Doppler ultrasonography (CDUS). During the aneurysmal surgery, intraoperative monitoring of the dominant venous outflow with CDUS was useful for the prevention of venous outflow obstruction. Physicians should carefully consider intracranial vascular anomalies in the differential diagnosis of vascular lesions of the superior eyelid.

[A case of moyamoya disease with a subarachnoid hemorrhage treated with endovascular technique].

We report a case of a moyamoya disease presenting with subarachonoid hemorrhage (SAH) due to a ruptured aneurysm. A 40-year-old woman presented with sudden onset of headache and vomiting. Computed tomography (CT) showed diffuse thick SAH localized around basal cistern. 3D-CT Angiography (3D-CTA) and digital subtraction angiography (DSA) demonstrated a saccular aneurysm at the bifurcation of the left superior cerebellar artery and basilar artery. In addition, the both carotid arteries were occluded at the terminal portion and the territory of both middle cerebral arteries were perfused by abnormal moyamoya vessels. The aneurysm was completely embolized by endovascular embolization. The SAH due to a ruptured aneurysm associated with moyamoya disease is rare. We think endovascular therapy is safe and effective. However, a vasospasm of the catheter technique occurred during the operation. This fact is very important to consider when we treat diseases such as this in the future.

Cerebral hemorrhage and cerebral infarction in 30 cases of adult moyamoya disease: comparison between conservative therapy and superficial temporal artery-middle cerebral artery anastomosis.

To clarify the effect of surgery on the prevention cerebral hemorrhage in adult moyamoya disease, we compared postoperative courses between superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and conservative therapy. The study subjects were 30 adults with moyamoya disease. Unilateral STA-MCA anastomosis was conducted in 7 of these 30 cases, and bilateral STA-MCA anastomosis was conducted in 8 of these 30 cases. Therefore, anastomosis was performed in a total of 23 sides. The postoperative clinical course was observed for more than 5 years after the STA-MCA anastomosis. Cerebral hemorrhage occurred after operation in 2 sides (8.7%) among the 23 sides that received STA-MCA anastomosis. On the contrary, hemorrhage occurred during conservative therapy in 5 sides (13.5%) among 37 non-operation sides (no significance in chi2 test). Cerebral infarction occurred in 3 sides (13%) among 23 sides treated with STA-MCA anastomosis. However, the infarction occurred in 2 sides (5.4%) among the 37 non-operation sides (no significance in chi2 test). Cerebral hemorrhage tended to occur less frequently after STA-MCA anastomosis, and bypass surgery was suggested to have some beneficial effect in preventing cerebral hemorrhage in adult moyamoya disease. However, it was revealed that STA-MCA anastomosis exacerbated the brain ischemia. Therefore, strict management is mandatory in the perioperative period.

Atherothrombotic lesion of the middle cerebral artery: report of 21 cases with stenotic and obstructive lesions.

This was a retrospective analysis of 12 consecutive cases of middle cerebral artery stenosis and 9 consecutive cases of middle cerebral artery occlusion that presented to our hospital with acute cerebral ischemia. The degree and area of the cerebral infarctions were assessed with the Alberta Stroke Program Early CT Score (ASPECTS) and ASPECTS-DWI (APSECTS with assessment of white matter lesion using diffusion-weighted image). As for cerebral infarctions in the region of the perforating artery, lesions that were more than 20 mm long in the caudal-cranial direction were diagnosed as branch atheromatous disease (BAD). Activities of daily living (ADL) were poorer in the cases with lower ASPECTS and ASPECTS-DWI. ADL tended to be worse in cases with BAD than in those without. The prognosis was significantly poorer in the group with ASPECTS< or =7 points. ASPECTS tended to be lower in cases with BAD than in those without. ADL, ASPECTS and the presence of BAD were not significantly different between the stenosis and obstruction groups. In summary, the neurological prognosis was dependent on the extent of the cerebral infarction and was related to BAD to some extent. These findings will be important when considering medical treatment at the outpatient clinic setting.

Visual disturbance following shunt malfunction in a patient with congenital hydrocephalus.

A 25-year-old woman presented with complaints of nausea and headache. She had been treated with a ventriculoperitoneal shunt for hydrocephalus when she was 7 months old. Her bilateral optic discs showed moderate atrophy. Right visual acuity allowed only perception of hand movement and left visual acuity was 0.02 (1.2). Computed tomography (CT) showed mild ventricular dilation but no periventricular lucency. Intracranial pressure (ICP) was not high when the shunt valve was punctured. Her visual acuity deteriorated 5 days after the consultation. She was referred again 8 days after the first consultation. The bilateral optic discs were completely pale. Both pupils were dilated on admission, and the bilateral direct light reflexes were absent. The patient could slightly detect only green light stimulus. CT showed moderate enlargement of the ventricle. ICP was 47 cmH(2)O when the shunt valve was punctured. Shuntgraphy showed obstruction of the shunt at the distal end of peritoneal catheter. Emergent total shunt revision was performed. She could detect dark stimulus and the still-dilated left pupil had recovered direct light reflex on the next day. The visual acuity was 0.01 (0.7) on the left 6 months after the operation, although she was blind in the right eye and the bilateral optic discs were completely pale. Visual loss associated with shunt failure remains a major morbidity in shunted congenital hydrocephalus. Early diagnosis and shunt revision may allow visual recovery.

Inflammatory index and treatment of brain abscess.

This study retrospectively analyzed 12 patients with brain abscesses. Half of the patients were diagnosed inaccurately in the initial stage, and 7.2 days were required to achieve the final diagnosis of brain abscess. The patients presented only with a moderately elevated leukocyte count, serum CRP levels, or body temperatures during the initial stage. These markers changed, first with an increase in the leukocyte count, followed by the CRP and body temperature. The degree of elevation tended to be less prominent, and the time for each inflammatory index to reach its maximum value tended to be longer in the patients without ventriculitis than in those with it. The causative organisms of a brain abscess were detected in 10 cases. The primary causative organisms from dental caries were Streptococcus viridians or milleri, and Fusobacterium nucleatum. Nocardia sp. or farcinica were common when the abscess was found in other regions. The primary causative organisms of unrecognized sources of infection were Streptococcus milleri and Prolionibacterium sp. Nocardia is resistant to many antibiotics. However, carbapenem, tetracycline and quinolone were effective for Nocardia as well as many other kinds of bacteria. In summary, the brain abscesses presented with only mildly elevated inflammatory markers of body temperature, leukocyte and CRP. These inflammatory markers were less obvious in the patients without ventriculitis and/or meningitis. The source of infection tended to suggest some specific primary causative organism. It was reasonable to initiate therapy with carbapenem.

Treatment of a cerebral dissecting aneurysm in anterior circulation: report of 11 subarachnoid hemorrhage cases.

This report presents 8 cases of internal carotid artery aneurysms, 1 case of a middle cerebral artery aneurysm, and 2 cases of anterior cerebral artery aneurysms, together with a discussion of the treatment of aneurysms in anterior circulation. All cases showed subarachnoid hemorrhage. Two of the 8 internal carotid artery aneurysms were trapped with a low-flow bypass; however, both patients died of an immediate hemodynamic infarction or vasospasm-induced infarction. Five of the 8 internal carotid artery aneurysms were trapped after revascularization with high flow bypass. Four of those patients were self-supporting at discharge, but one patient was discharged in a vegetative state due to the sacrifice of arterial branches which were included in the dissecting portion. One case of the dissecting aneurysm in the M2 portion of the middle cerebral artery was trapped after low-flow bypass. This patient was self-supporting at discharge. In 2 cases of anterior cerebral artery aneurysms, the lesions were first wrapped with Bemsheets, and then the aneurysmal clip was applied on the wrapped dome. Trapping following high-flow bypass is the best method for treating a dissecting aneurysm in the internal carotid artery. Trapping also can be used to treat a dissecting aneurysm of the middle cerebral artery, after low-flow bypass. Clipping on the wrapped aneurysm can also be performed successfully in the anterior cerebral artery aneurysm.

Air vent of vein graft in extracranial-intracranial bypass surgery.

Revascularization with a vein graft is a mandatory method for treatment of dissecting or pseudoaneurysms of the internal carotid artery. We report the necessity for an air vent from the vein graft and explain its use in our two cases. In Case 1, we searched for a great saphenous vein around its junction with a femoral vein during the harvest of vein graft. An accessory branch of that great saphenous vein was also found around the junctional region with a femoral vein, and was temporarily ligated. At first, anastomosis was completed on both the distal and proximal sides. After the proximal side of a vein graft was opened and the ligation of the branch was loosened, an air vent could be made through the branch of the vein graft. Multiple air bubbles and a large quantity of white microemboli were discharged through this branch. The postoperative course was uneventful. In Case 2, the air vent was omitted to shorten ischemia. During the opening of the vein graft, the migrated air was observed to move to the middle cerebral artery. A computed tomography scan demonstrated that brain infarction and dysarthria occurred postoperatively. The air vent of the vein graft is essential in extracranial-intracranial bypass surgery, because the air bubbles or microthrombi are easily trapped around the valve and cause cerebral infarction. An air vent can be easily made if the branch of a vein graft exists in the outflow pathway, because intraluminal air or thrombus can be washed out through the branch at the final stage of surgery.

Neuromyelitis optica spectrum disorder: 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography findings--case report.

A 51-year-old female with a history of rheumatoid arthritis rapidly developed anterior neck pain and paresis in the left upper and lower extremities and right lower extremity, sensory disturbance in the left upper and lower extremities, and bladder and rectal disorder. Adduction of the left eye and abduction of the right eye were also disturbed. Spinal magnetic resonance imaging demonstrated severe edema in the C1-T5 levels, which then deteriorated rapidly over 3 days, and lesions enhanced with gadolinium in the C1-C3 and C5-T3 levels. 2-Deoxy-2-[18F]fluoro-D-glucose positron emission tomography study demonstrated the inflammatory sites as segmental enhanced accumulation in the C1-C3, C5-C6, and T1 levels. The serum anti-aquaporin 4 antibody level was positive and she was diagnosed with neuromyelitis optica spectrum disorder. Marked improvement in the neurological conditions, concomitant with reduced spinal cord edema, was obtained by steroid pulse therapy.

Postoperative recovery from unilateral blindness caused by tuberculum sellae meningioma.

A 47-year-old female had noticed diminished visual acuity in both eyes 2 months previously. The patient had vision loss (no light perception) in her right eye on admission. Her left visual acuity was 1.2 (naked vision) and an upper temporal quadrant hemianopsia was revealed in her left eye. Optic disc atrophy was also found bilaterally during a fundus examination. The tumor was located at the tuberculum sella. The first operation was performed using a right pterional approach. The right optic nerve was thin and atrophic and was severely encased by the tumor. Considering the deterioration of her visual evoked potential, the operation was terminated in the remaining major part of the tumor. Postoperatively, the patient suffered visual loss in her right eye (no light perception), decreased visual acuity (naked: 0.6 (corrected: 1.0)), and deteriorated visual field defects (upper temporal quadrant hemianopsia) in her left eye. The tumor remnant was resected again 2 weeks later using the right frontobasal and pterional approaches. The tumor around the bilateral internal carotid arteries and optic nerves was not resected. Light perception in the right eye appeared 2 weeks after the operation. Although an opthalmological examination revealed right optic atrophy, finger counting was possible in the upper nasal visual field of the right eye three months after the second operation. Her visual acuity was 0.7 (1.0), and the upper temporal quadrant hemianopia of the left eye improved in comparison with the preoperative one. Our case demonstrated the possibility of a recovery from blindness.

Repair of carotid blow-out using a carotid sheath in a patient with recurrent thyroid cancer.

The patient had thyroid cancer and underwent subtotal thyroidectomy. Local recurrence occurred on both sides 5 years and 6 months later. The sterno-hyoid muscle and sterno-thyroid muscle were severed and the tumor around the cricoid cartilage was removed. The tumor extended into the space between the right common carotid artery and internal jugular vein and was located under the right common carotid artery and vagus nerve on the lateral side. The carotid sheath was peeled off of the carotid artery quite easily. The right common carotid artery ruptured abruptly at the distal side during this procedure. The right common carotid artery had two layers, which were very fragile, so the direct suture or repair with a graft was impossible. The carotid artery could not be trapped with ligation because the cerebral vascular supply was not examined preoperatively. This portion was repaired using the remaining carotid sheath. However, re-bleeding occurred at the proximal portion of the previous laceration spontaneously. Fibrin glue with oxidized cellulose was initially used to seal the second small hole in this lesion. The second ruptured section was repaired using the remaining sterno-thyroid and sterno-hyoid muscles. The proximal portion of the right common carotid artery was reinforced with the harvested external jugular vein. These procedures resulted in hemostasis. Three-dimensional CT angiography showed irregular stenosis just after the operation, but it recovered 11 days later. No cerebral infarction occurred after the operation and the patient's general condition was good.

Schwannoma originating from lower cranial nerves: report of 4 cases.

Four cases of schwannoma originating from the lower cranial nerves are presented. Case 1 is a schwannoma of the vagus nerve in the parapharyngeal space. The operation was performed by the transcervical approach. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia happened transiently after the operation. Case 2 is a schwannoma in the jugular foramen. The operation was performed by the infralabyrinthine approach. Although only the intracapsular tumor was enucleated, facial palsy, hoarseness, dysphagia and paresis of the deltoid muscle occurred transiently after the operation. The patient's hearing had also slightly deteriorated. Case 3 is a dumbbell-typed schwannoma originating from the hypoglossal nerve. The hypoglossal canal was markedly enlarged by the tumor. As the hypoglossal nerves were embedded in the tumor, the tumor around the hypoglossal nerves was not resected. The tumor was significantly enlarged for a while after stereotactic irradiation. Case 4 is an intracranial cystic schwannoma originating from the IXth or Xth cranial nerves. The tumor was resected through the cerebello-medullary fissure. The tumor capsule attached to the brain stem was not removed. Hoarseness and dysphagia happened transiently after the operation. Cranial nerve palsy readily occurs after the removal of the schwannoma originating from the lower cranial nerves. Mechanical injury caused by retraction, extension and compression of the nerve and heat injury during the drilling of the petrous bone should be cautiously avoided.

Surgical results of parasagittal and falx meningioma.

Sixteen operative cases of parasagittal and falx meningioma were analyzed retrospectively. Parasagittal meningioma totaled 12 cases and falx meningioma numbered 4 cases. Preoperative symptoms were paresis of a lower extremity in 7 cases and disturbed consciousness or mentality in 6 cases. Paresis and/or consciousness deteriorated just after the operation in 11 cases. The deterioration was identified in paresis (6 cases), consciousness (3 cases), paresis and consciousness (2 cases). Motor function further deteriorated postoperatively when the patients had shown preoperative paresis. The cause of postoperative deterioration of motor function and/or consciousness level was intracerebral hematoma in 1 case, and newly-developed brain edema in 1 case. There was no obvious explanation for the symptomatic exacerbation in the other 9 cases. At discharge, 5 cases showed deterioration of motor function in comparison to their preoperative condition, and 3 cases showed an improvement. Eleven cases showed no change of consciousness in comparison to the preoperative condition, and 5 cases showed improvement at discharge. Surgical result was good for consciousness or mentality, but was relatively poor for motor function. It was considered that surgery should be performed carefully in patients with preoperative paresis.

Acute subdural hematoma in patients with medication associated with risk of hemorrhage.

The characteristics of 90 cases of acute subdural hematoma were analyzed in 25 patients taking medication related to bleeding tendency, the bleeding tendency group, and 65 patients without such medication, the control group. Acute subdural hematoma was significantly associated with over-turning or without accident in the bleeding tendency group (92%) compared with the control group (56.9%). The time from trauma to admission was significantly longer in the bleeding tendency group (32.7 hours) compared with the control group (9.7 hours). The mean Glasgow Coma Scale score on admission was 13.0 points in the bleeding tendency group and was significantly better than 10.9 points in the control group. The international normalized ratio of prothrombin time on admission was 3.59 in the patients treated with warfarin. All patients taking warfarin received reversal agents and this value decreased significantly to 1.38. In the bleeding tendency group, hematoma size increased in 20% after the operation, almost the same as in the control group (25%). Although good recovery tended to be observed less frequently in the bleeding tendency group than in the control group, the mean age of the bleeding tendency group was significantly higher than that of the control group, so the prognosis might be affected by this age-related factor. Cautious diagnosis, follow-up imaging, and strict management are mandatory in patients with bleeding tendency.

[Intracranial metastasis of thymoma: report of three cases].

Thymoma with metastasis to the central nervous system is extremely rare. Herein, three rare cases of thymoma with intracranial metastasis are reported. Case 1 (thymoma, B3 in the WHO classification); cranial magnetic resonance imaging (MRI) revealed a mass lesion with a ring enhancement effect in the right temporal lobe. The cystic and hemorrhagic lesion was subtotally removed and radiation therapy of 40 Gy was administered to the tumor cavity containing the remains of the tumor around the ependyma. The patient received combination chemotherapy with carboplatin and gemcitabine hydrochloride, and he was discharged 3 months after the operation. Case 2 [(thymoma, C (thymic carcinoma)]; multiple cerebral metastases of 8 lesions were found and whole brain irradiation of 40 Gy was performed. Three months later, rapid development of 2 metastatic lesions resulted in the patient's death. At autopsy, neoplastic lesions were found in the neck lymph node, right shoulder, chest frame, pleural cavity, diaphragm, lung, periaortic lymph node, liver and pancreas. Case 3 (thymoma, B2); computed tomography (CT) scan and MRI showed a tumorous lesion over the cerebellar hemisphere. At operation, a vascular, elastic-soft and grey tumor was found to originate from the dura mater and had invaded the occipital bone. The tumor had also invaded the arachnoid membrane and sinus wall but the pia mater was free from invasion. Thereafter local irradiation of 40 Gy was performed to the tumor cavity containing the remains of the tumor around the sinus. However local recurrence reoccured 3 years later. After stereotactic irradiation of 40 Gy to the recurrent tumor, the tumor diminished. The patient is wheelchair-bound 3 years and 4 months after the operation. Most cerebral metastases are extremely rare. Outcome remains poor and life expectancy is very short when brain metastasis is present. Treatment for thymoma is multimodal, including surgery, irradiation and chemotherapy.

[Epidermoid tumor originating from the petrous bone: report of two cases].

In case 1, the tumor was incidentally found in the right petrous bone, middle cranial fossa and cerebello-pontine angle. T1 weighted MRI demonstrated a low intense mass and T2 weighted imaging demonstrated hyperintensity. Through the subtemporal extradural approach, an epidermoid in the middle cranial fossa was partially removed. Postoperative course was uneventfull but an episode of rhinorrhea occurred 15 months later. Bone-window CT scan disclosed air cells of the petrous bone were exposed to the previous surgical cavity. Using the same approach, an epidermoid was totally removed. With the sealing of the tumor cavity with the vascularized muscle flap, the patient became free from rhinorrhea. Case 2 had complainted of sensory impairment in the left trigeminal nerve distribution, atrophy of the left temporal and masseter muscle, and diplopia. T1 weighted imaging of MRI demonstrated a low-intense mass in the left petrous bone, middle cranial fossa, temporal lobe, and cerebello-pontine angle, and T2 weighted imaging demonstrated hyperintensity. The intradural tumor under the temporal lobe was removed at another hospital. As the diplopia deteriorated 5 years later, the patient was re-introduced to our hospital. At first, the tumor in the cerebello-pontine angle was removed using the left retromastoid lateral suboccipital approach. Later, the tumor in the petrous bone and middle cranial fossa was removed through the left subtemporal extradural approach. With the sealing of the tumor cavity with the vascularized muscle flap, postoperative cerebrospinal fluid leakage was prevented. The epidermoid tumor in the petrous apex is a congenital and rare disease. The obstruction of the petrous air cell and dural defect using the vascularized flap is most important to prevent postoperative cerebrospinal fluid leakage.