A case report of fibromuscular dysplasia presenting symptoms like moyamoya disease: "string of beads" appearance of the pericallosal artery

We report a case of fibromuscular dysplasia (FMD) presenting a transient ischemic attack (TIA) like Moyamoya disease. The patient, a 16-year-old woman, had recurrent attacks of right hemiparesis induced by hyperventilation. Neurological examinations revealed no abnormality. Angiography showed severe stenosis of both anterior cerebral arteries at the proximal portion with "string of beads" appearance in the left pericallosal artery, and tubular stenosis of the left internal carotid artery at the level of the second cervical vertebra. Renal artery stenosis was not seen. A cerebral-blood-flow (CBF) study with 133Xe SPECT showed marked reduction of CBF in the left cerebral hemisphere at times of hyperventilation. Extracranial-intracranial bypass surgery was performed and histological examination of the superficial temporal artery (STA) revealed intimal fibroplasia which was compatible with FMD. Postoperative angiography showed good filling of the middle cerebral arteries from the STA. The patient has had no recurrence of TIA for a year since the operation. CBF study showed mild low CBF in the region of the left anterior cerebral arterial circulation at hyperventilation. There is no report of intracranial FMD presenting an ischemic symptom induced by hyperventilation. Clinical diagnosis of Moyamoya disease should be made carefully when extracranial vascular lesion accompanies it.     

Successful recanalization by in-stent percutaneous transluminal angioplasty with distal protection for acute carotid stent thrombosis

A 71-year-old male presented with severe left cervical internal carotid artery stenosis manifesting as repeated transient ischemic attacks consisting of right hemiparesis and motor aphasia. Carotid artery stenting (CAS) under distal protection was performed to prevent further ischemic events. This procedure was uneventful. However, the patient exhibited progressive right hemiparesis and motor aphasia 3 days after CAS. Emergent angiography revealed carotid artery occlusion due to in-stent thrombosis. In-stent percutaneous transluminal angioplasty (PTA) was performed under distal protection. The carotid artery was recanalized with small residual thrombus. The neurological deficits almost completely disappeared after PTA. Follow-up angiography 9 months after stenting showed restenosis but no in-stent thrombosis. Carotid thrombosis after CAS can be resolved by in-stent PTA under distal protection and subsequent treatment with antithrombotic agents.     

A case of adult moyamoya disease progressed after vascular reconstructive surgery

We report an adult onset patient with moyamoya disease showing acute progress after contralateral vascular reconstructive surgery. A 47-year-old female developed cerebral infarction in the left corona radiata. A magnetic resonance (MR) angiography and a cerebral angiogram revealed severe stenosis extending from the terminal portion of left internal carotid artery (ICA) to the M1 portion. The right ICA showed slight stenosis. We performed direct bypass surgery (STA-MCA anastomosis) on the affected left side. MR angiography 1 month after surgery revealed the progressive stenosis of the C1 portion of the right ICA. While measurement of cerebral blood flow (CBF) showed a slight impairment of vascular reactivity to acetazolamide loading in the region of the right MCA, we continued without vascular reconstructive surgery for the right side because there was no ischemic attack. The patient had a transient sensory disturbance of the left upper extremity 16 months after surgery. MR angiography and a cerebral angiogram revealed more progressive stenosis extending from the right ICA to the M1 portion. CBF study showed a low CBF at rest and a negative response to acetazolamide loading in the region of the right MCA. Direct bypass surgery was performed on the right hemisphere. Follow-up study revealed an increment of rest CBF and improvement of vascular reactivity. We underlined the necessity for careful postoperation observation of progressive contralateral arterial stenosis using MR angiography and CBF study in adult onset patients with moyamoya disease.     

Improvement of ischemic symptoms and cerebral blood flow after percutaneous transluminal angioplasty for renovascular hypertension. Report of a case with multiple cerebrovascular occlusive disease]

A 53-year-old male complained of frequent left motor-sensory transient ischemic attack for 4 months. On admission, he demonstrated mild right hemiparesis, dysarthria, and right hemisensory disturbance of all modalities. Cerebral angiography demonstrated complete occlusion of the left internal carotid artery just above the origin of the ophthalmic artery and a stenotic lesion at the horizontal segment of the right middle cerebral artery. Renal angiography showed severe stenosis of the right renal artery. Systolic blood pressure was over 200 mmHg and marked circadian variation of blood pressure was noted. Serum renin was 4.0 ng/ml/hr. Four months after superficial temporal artery-middle cerebral artery anastomosis, left carotid angiography showed good patency of the bypass and the ischemic symptoms completely disappeared. Single photon emission computed tomography (SPECT) showed increased cerebral blood flow (CBF), especially in the left hemisphere after surgery. Six months after the bypass surgery, he complained of mild right hemiparesis again. Shortly after percutaneous transluminal angioplasty (PTA) for renal arterial stenosis, his hemiparesis was improved and the systolic blood pressure stabilized to 150-170 mmHg. SPECT showed the CBF had also recovered in both hemispheres. The improvement in ischemic symptoms and increased CBF after PTA were probably related to stabilization of the systemic blood pressure or inhibition of serum renin-angiotensin.     

Carotid artery stenting in a patient with right-sided aortic arch with an aberrant left subclavian artery

A right-sided aortic arch with an aberrant left subclavian artery is a rare anatomical variation. We report a case treated with carotid artery stenting (CAS) for a patient with a right-sided aortic arch with an aberrant left subclavian artery. A 72-year-old man presented right hemiparesis due to acute brain infarction. Neck CT angiography showed 70% stenosis in the left internal carotid artery (ICA). We diagnosed acute brain infarction as artery-to-artery embolism due to ICA stenosis and decided to perform carotid artery stenting (CAS) for symptomatic ICA stenosis. CT angiography to evaluate an access route to the lesion incidentally showed the right-sided aortic arch with an aberrant left subclavian artery. An intraoperative aortogram showed a right-sided aortic arch. The guiding catheter was carefully introduced up to the left common carotid artery. CAS was performed with a proximal balloon and distal filter protection. The stenotic area was restored, and the patient was discharged without suffering recurrent attacks. Although a right-sided aortic arch with an aberrant left subclavian artery is a very rare anatomical variation, it can be encountered in neuroendovascular treatment, and therefore knowledge of this anatomical variation is important.     

Common carotid artery dissection propagated from acute aortic dissection: a case successfully treated by PTA

We reported a rare case, which was successfully treated by PTA, of right common carotid artery dissection propagated from acute aortic dissection (AAD) type A. A 45-year-old male with a past history of hypertension and an artificial graft replacement of the abdominal aorta due to AAD type B, 7 years ago, was brought into our hospital by ambulance 30 minutes after an attack of fainting and left hemiparesis. On admission, the patient complained not of chest pain or left hemiparesis, but nausea. At that time his consciousness level was JCS 1. During examinations, he had the same attack twice and his consciousness level deteriorated to JCS 2. Brain MRI showed no abnormality, but cervical MRA did not visualize the right carotid artery and thoracic CT depicted acute aortic dissection including branches of the aorta. Emergent angiography disclosed that the dissecting 99% stenosis of the right common carotid artery had developed from AAD type A with poor collateral blood flow. PTA was carried out 8 times and reduced the residual stenosis to about 50% with shortened circulation time. The patient's consciousness disturbance improved. After the replacement of the whole aortic arch in an artificial graft, the residual stenosis disappeared. The patient recovered without neurological deficit but right frontal silent embolic infarction caused by the artificial graft replacement was detected. AAD is a catastrophic illness and sometimes accompanied by devastating ischemic cerebral disease (ICD) because of propagation of dissecting to extracranial vessels. This is the first report that shows the efficacy of PTA for treatment of ICD associated with AAD.     

Surgical results of two cases of simultaneous surgery of carotid and coronary occlusive disease]

Two cases of symptomatic extracranial carotid artery stenosis associated with ischemic heart disease are reported. The first case was 72-year-old male, who was admitted because of transient ischemic attack due to the stenosis of left internal carotid artery. He had a history of myocardial infarction and coronary angiography revealed three vessel disease. The second case was 74-year-old female with diabetes mellitus. She was admitted because of cerebral infarction. The carotid angiography revealed critical stenosis of bilateral internal carotid arteries. Her coronary angiography revealed three vessel disease. Her chest symptom became unstable after her admission. In both cases, simultaneous carotid endarterectomy and coronary bypass grafting were performed with successful outcome. In the patients with symptomatic extracranial carotid occlusive disease associated with severe ischemic heart diseases, we advocate simultaneous operation both for carotid and coronary artery occlusive disease.     

A case of recurrent internal carotid stenosis, after carotid endarterectomy, due to strangulation by scarring

A case of recurrent internal carotid stenosis, one year after carotid endarterectomy, due to strangulation by scarring composed of digastric muscle, hypoglossal nerve and a branch of external carotid artery (ECA) is reported in this paper. A 34-year-old male suddenly became nauseated and developed right hemiplegia and dysphasia. He was admitted to a hospital, and the symptoms and signs improved gradually during a period of several weeks. A left carotid angiography, performed in another hospital, revealed a stenosis and wall irregularity around the highly-situated carotid bifurcation. He was transferred to our clinic with a view to being operated on. On admission, neurological examination disclosed a mild right motor weakness and motor dysphasia. A right carotid angiography revealed good cross circulation to the opposite. An EEG monitoring under the Matas test showed no outbreaks. With diagnosis of an embolism arising from carotid atheromatous plaque, a carotid endarterectomy was performed successfully. A postoperative angiography revealed good patency without stenosis nor wall irregularity around the carotid bifurcation. The postoperative and out-clinic condition was uneventful. However, one year after the operation, a left carotid angiography revealed recurrent internal carotid stenosis in a high degree. A second operation was performed and the operative findings showed an internal carotid stenosis due to strangulation by scarring composed of digastric muscle, hypoglossal nerve, a branch of ECA and connective tissue. The scarring was decompressed and a postoperative angiography revealed an improvement of the stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

右侧肱动脉入路左颈动脉支架成形术治疗颈内动脉狭窄合并牛型主动脉弓1例报告

2013年10月我科收治1例63岁女性左颈动脉狭窄,有左侧脑梗死病史并频繁出现一过性脑缺血发作（transient ischemic attack,TIA）。术前CTA检查为牛型主动脉弓,左侧颈内动脉起始部重度狭窄,但位于C2水平不适合行颈动脉内膜切除术。采用全麻下经右侧肱动脉人路穿刺,造影导管选人左颈外动脉,加硬导丝引导F6长鞘选人左颈总动脉建立手术通路,然后常规进行保护伞下的颈动脉球囊扩张和支架成形术。手术成功,未发生任何并发症。术后1个月随访,TIA症状完全消失,颈动脉超声提示左颈内动脉支架术后血流通畅。我们认为经右侧肱动脉人路行支架成形术治疗牛型主动脉弓变异的左侧颈内动脉狭窄是安全、可行的。     

"Internal cross-clamping" for symptomatic internal carotid artery thrombus. Report of two cases

Both carotid endarterectomy and carotid artery stent placement with filter embolic protection present a higher risk for patients with internal carotid artery (ICA) lesions containing intraluminal thrombus. Despite the risk associated with intervention, patients with symptomatic intraluminal thrombus who were enrolled in the North American Symptomatic Endarterectomy Trial did better with surgical than medical treatment. We describe the novel use of an endovascular "internal cross-clamping" technique in two patients with symptomatic intraluminal thrombus in the ICA. A 57-year-old woman presented with a history of multiple episodes of left upper-extremity numbness, mild dysarthria, and agraphia occurring over the previous 24 hours. Cranial magnetic resonance imaging revealed a scattered watershed infarction of the right hemisphere and a critical stenosis of the right ICA. An 81-year-old man awoke with hemiplegia and inability to follow commands after undergoing a complicated carotid endarterectomy. Computed tomographic perfusion imaging demonstrated an increased time to peak in the left middle cerebral territory, and emergent angiography demonstrated both intimal flaps and thrombus in the endarterectomy bed. The lesions in both patients were treated with endovascular stent placement using both proximal and distal flow occlusion--a functional "internal cross-clamping"--for embolic protection. To our knowledge, this is the first report of internal trapping and stent placement for symptomatic carotid stenosis containing intraluminal thrombus. This treatment strategy should be added to the armamentarium of endovascular surgeons in selected patients with symptomatic carotid intraluminal thrombus.     

Retrograde carotid stenting for isolated stenosis of the proximal common carotid artery--case report

A 49-year-old female with a history of systemic hypertension and diabetes mellitus suffered transient right hemiparesis. Carotid angiography with arch-aortography detected severe stenosis of the proximal portion of the left common carotid artery. The left carotid bifurcation was surgically exposed and retrograde catheterization was performed to approach the proximal common carotid artery stenosis. The lesion was dilated with a balloon catheter and successfully stented without complications. The left internal carotid artery was clamped during the procedure to avoid embolism. Retrograde carotid stenting for stenosis of the proximal common carotid artery is a safe and effective alternative to conventional surgery in selected patients.     

Percutaneous transluminal angioplasty in subclavian artery stenosis

The technique of percutaneous transluminal angioplasty (PTA) that was introduced initially by Dotter and Judkins in 1964 has been applied to various arterial stenoses and some occlusive lesions and has advanced dramatically with the development of the Grüntzig catheter in the treatment of peripheral vascular disease. However, the application to occlusive disease in brachiocephalic arteries is still considered to be disputable and has been made only in selective cases, especially in Japan. We report here a case with stenosis of the left subclavian artery successfully treated with PTA. The patient was 66 year-old man, who had been suffering from transient attacks of vertigo, ataxia, visual disturbance and the left arm claudication. Blood pressure was 150/92 mmHg in the right arm and 110 mmHg in systole in the left arm. Diagnostic arteriography identified the right carotid artery occlusion at its origin and significant stenosis in the proximal left subclavian artery. PTA under fluoroscopic control was performed by passing dilating catheter in antegrade fashion by Seldinger method through the left femoral artery. Anatomic correction was achieved without hemorrhagic or embolic complications. Systolic blood pressure gradient measured at the brachial artery level disappeared immediately after PTA and symptomatic relief was obtained completely. One month later, wide patency of the left subclavian artery and sufficient antegrade flow in the vertebral artery were confirmed angiographically. During follow-up period of 4 months, the patient was asymptomatic. We reviewed the literature reporting PTA for occlusive disease in brachiocephalic arteries and discussed its problems and possibilities, especially for the treatment of subclavian artery stenosis.     

A 10-year follow-up of extracranial–intracranial bypass for the treatment of bilateral giant internal carotid artery aneurysms in a patient with fibromuscular dysplasia: case report

Cervicocephalic fibromuscular dysplasia (FMD) is an idiopathic, non-inflammatory and non-atherosclerotic arteriopathy which usually affects small- and medium-sized cervical arteries distributed at the atlas and axis interspace. Few cervicocephalic FMD patients are associated with multiple intracranial aneurysms which may rupture or develop. So the authors describe a cervicocephalic FMD patient with a history of right oculomotor palsy in 2000. Angiography revealed bilateral internal carotid artery (ICA) aneurysms and a fusiform aneurysm in right vertebral artery. Typical “string-of-beads” phenomenon was observed in V2 segment of left vertebral artery. The right ICA giant aneurysm was treated by right ICA occlusion and superficial temporal artery (STA)-middle cerebral artery (MCA) bypass at that time. Five years later, the patient presented with paroxysmal weakness in right limbs. The subsequent angiography showed the enlargement of left ICA aneurysm. It was treated satisfactorily with left external carotid artery-saphenous vein-MCA bypass and left ICA ligation. During the long-term follow-up, the patient kept no neurological deficit and the angiography showed good patency of bilateral grafts and the lesions in bilateral vertebral arteries remained unchanged.     

Fibromuscular dysplasia with cerebral infarction in children. Case report

A 15-year-old boy suddenly developed a headache, followed by progressive weakness of the right extremities. A computed tomographic scan obtained on admission showed a low-density area in the left putamen extending to the internal capsule. No neurological deficits were observed other than slight dysarthria and right hemiparesis. Left carotid angiography showed the characteristic "string of beads" deformity of fibromuscular dysplasia (FMD) extracranially and complete occlusion of the internal carotid artery at the C2 portion. The right carotid artery and both vertebral arteries were normal. Anastomosis of the left superficial temporal and middle cerebral arteries was performed 1 month after the onset. The patient recovered quickly from the hemiplegia and was discharged with no neurological deficits. Angiograms obtained 3 months later showed not only patency of the bypass, but also recanalization of the intracranial internal carotid artery. Cervico-cephalic FMD mainly affects middle-aged women, and is uncommon in children; only nine childhood cases have been reported. This disorder may play an important role in the genesis of strokes among children.     

A case of transient cerebral ischemia of the vertebrobasilar system caused by carotid ulcerative lesion and persistent proatlantal intersegmental artery: a case report

A rare case with the episodes of vertebrobasilar TIAs which were caused by carotid ulcerative lesion and by persistence of proatlantal intersegmental artery is reported. This 69-year-old man was admitted on 14th Feb. 1985 with complaints of three attacks of vertigo, diplopia, nausea, vomiting and dysequilibrium lasting about 1 hour. His past history and family history were unremarkable. On admission, physical and neurological examinations revealed nothing particular, except bilateral carotid bruits. On the right side, innocent low pitched bruit was detected, however on the left side, carotid bruit was high pitched and was regarded as pathological. Blood pressure was 180/80 mmHg. Pulse rate was 66/min and cardiac arrhythmia was not detected by EKG. He also had no history of cardiac arrhythmia as mentioned above. CT scans and MRI showed only mild brain atrophy and no brain stem nor cerebellar lesions. Left carotid angiogram revealed ulcerative lesion of the common carotid bifurcation which was compatible with high pitched bruit. And at the level of the C2 vertebra, persistent proatlantal intersegmental artery originated from the left internal carotid artery which, after running upward, joined to the horizontal portion of the left vertebral artery above the atlas. Internal carotid and the vertebrobasilar vasculature beyond the proatlantal intersegmental artery were normal except slight irregularity of carotid siphon. Right carotid and vertebral angiograms revealed no abnormality and basilar artery was opacified clearly again via right vertebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)     

The use of a modified vertical mandibular osteotomy for exposure of a distal cervical internal carotid artery

We report herein the case of a 56-year-old woman who presented with repeated transient ischemic attacks (TIAs). Cerebral angiography revealed that the left redundant internal carotid artery (ICA) uncharacteristically curved medially. These findings were highly suggestive of an internal carotid artery aneurysm at the level between the first and second cervical vertebral bodies. The lesion was presumed to be difficult to access by the surgical procedure usually adopted in carotid endarterectomy. Thus, we decided to employ a modified vertical mandibular osteotomy. The operative view revealed that the lesion was atherosclerotic stenosis with ulceration, so carotid endarterectomy was indicated. This mandibular osteotomy not only provided us with wide, satisfactory exposure of the distal cervical internal carotid artery but also afforded good mandibular stability during the postoperative period.     

Carotid endarterectomy in chronic renal failure patients: report of two cases

Chronic renal failure is one of the risk factors for carotid atherosclerosis. We report two cases of stenosis of the carotid bifurcation treated by carotid endarterectomy. A 66-year-old man with a 17-year history of hemodialysis experienced repeated episodes of right hemiparesis. Cerebral angiography showed severe stenosis of the cervical carotid bifurcation bilaterally. Left and right carotid endarterectomy operations were performed one month apart. The postoperative course was uneventful, and the patient returned home without neurological symptoms. The second case was in a 49-year-old woman with a 15-year history of hemodialysis had vertigo of one month duration. Cerebral angiography revealed occlusion of the left subclavian artery, and the distal left axillary artery was filled by retrograde flow from the left vertebral artery. Stenosis of the right carotid bifurcation was also noted. Right carotid endarterectomy was performed without any complications. Although a high incidence of intraoperative complications and of recurrent stroke after carotid endarterectomy (CEA) has been reported in chronic renal failure patients, the poor prognosis of the natural history of severe carotid stenosis in chronic renal failure should be taken into consideration. The cases reported indicate that carotid endarterectomy is safe and justified for carotid stenosis in chronic renal failure patients.     

Internal carotid artery stenosis and external carotid artery occlusion with unusual extracranial collateral pathway: case report

A patient with stenosis of the internal carotid artery and occlusion of the external carotid artery associated with an unusual extracranial collateral pathway is presented. A 63-year-old man was hospitalized for sudden onset of black-out after urination. He was alert, and no neurological deficit was found. MRI showed multiple lacunae in the bilateral putamens. Cerebral angiogram demonstrated severe stenosis of the left internal carotid artery at its cervical segment and complete occlusion of the left external carotid artery at its origin. The ascending pharyngeal artery originated from the left internal carotid artery above its stenotic lesion and the superior thyroid artery originated from the left common carotid artery. The sternocleidomastoid branch from the left superior thyroid artery and the muscular branch from the left vertebral artery anastomosed with the muscular branch of the ascending pharyngeal artery. The ascending pharyngeal artery maintained patency of the internal carotid artery. It is important to perform vertebral angiography when there is proximal occlusion and severe stenosis of the internal carotid artery, and when the ascending pharyngeal artery has not been clearly identified as a branch from the ipsilateral external carotid artery on the common carotid angiogram.     

Surgical reconstruction for radiation-induced extracranial vertebral artery stenosis: a case report]

We report a case of symptomatic extracranial vertebral artery stenosis after radiation therapy. This 49-year-old female received radiation therapy to the neck for nasopharyngeal carcinoma 11 years earlier, was admitted because of continuous dizziness and a floating sensation. Magnetic resonanse imaging showed no abnormalities, but an aortography demonstrated complete occlusion of the right common carotid artery as well as occlusion of the right vertebral artery and severe stenosis of the left vertebral artery at its origin, which was presumed to be the result of previous radiation therapy. Percutaneous transluminal angioplasty (PTA) for the left vertebral artery was performed using conventional balloon treatment, which resulted in wall dissection. Because of this, she underwent end-to-side vertebral artery to subclavian artery transposition, and she has had no further ischemic events science that time. PTA has been successfully performed as the first treatment of choice for vertebral artery stenosis, but surgical reconstruction can be a therapeutic management of choice for cases of failed PTA.     

A case of common carotid artery dissection showing remission in short-term

We report a case of left common carotid artery dissection showing complete remission in the short term. A 48-year-old man presented a 1-week history of weakness in the right arm. Neurological findings revealed mild motor weakness of the right upper extremity. Magnetic resonance imaging (MRI) showed sporadic cerebral infarction and hemorrhagic infarction in the area of the left middle cerebral artery (MCA) and the posterior border zone. Computed tomographic angiography (CTA) revealed irregular stenosis with a smooth surface in the left common carotid artery and ultrasonography indicated an iso-echoic lesion in the same area. ECD-single-photon emission tomography (SPECT) showed low perfusion only in the infarcted territory. Conservative therapy using a free radical scavenger and aspirin was carried out and the patient displayed full neurological recovery. After a 3-week follow-up period, carotid angiography and CTA showed almost complete disappearance of the stenotic lesion.