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PubMed2001

Surgical treatment of moyamoya disease in pediatric age.

BACKGROUND: Moyamoya is a progressive occlusive cerebrovascular disease which affects the distal internal carotid artery and its main branches, and is characterized, from a clinical point of view, by a variety of ischemic manifestations ranging from transient ischemic attacks (TIA) to stroke. Although considered until recently as almost specific of Japanese population, it can also affect individuals of different origin, and there is an increasing interest for this rare disease in Western medical literature. Nonetheless papers dealing with moyamoya disease in pediatric age are still very rare and almost exclusively limited to isolated case reports. These considerations prompted us to review our experience with the diagnosis and treatment of such unusual disease. METHODS: Nine children (seven months to nine years of age) presented with clinical manifestations of occlusive cerebrovascular disease (TIAs or stroke), epilepsy, and/or psychomotor retardation. Neuroradiological investigation, and in particular cerebral angiography, demonstrated the classical pattern of moyamoya disease. Eight of these children underwent cerebral revascularization utilizing the encephalomyosynangiosis (EMS) technique, with a total of 14 operations (six on both sides, two on one side). RESULTS: There was no mortality neither major morbidity related to surgery. One patient with renovascular hypertension, died at home approximately six months after a successful operation, probably from cerebral hemorrhage. In all the cases postoperative angiography revealed a marked improvement in cerebral circulation. However, in spite of the apparently normalized cerebral blood flow, clinical manifestations were affected less significantly. In fact, there was only minimal if any improvement in children operated on with an already established hemiplegia; whereas improvement in motor ability was obtained in those presenting with hemiparesis or TIAs, as well as in epilepsy and psychomotor retardation. CONCLUSIONS: On the grounds of our results we conclude that cerebral revascularization (by means of a simple operation like the EMS) in children with moyamoya disease is worthwhile, provided that the operation be performed before irreversible cerebral damage is established; however, even under these circumstances the operation can at least stop the progression of the disease.

Age Factors↗
PubMed1984

Can neuropsychological tests detect the sites of cerebrovascular stenoses and occlusions?

Conventional neuropsychological data may not mirror the site of cerebrovascular stenosis in a patient with mild to moderate symptoms. In this study, three groups of cerebral revascularization candidates failed to differ on an extended Halstead-Reitan battery: patients with symptoms and angiographic results referable to the carotid arterial system, subjects with clinical signs and neuroradiological findings referable to the vertebrobasilar arterial system, and patients with symptoms of cerebral ischemia but negative angiograms. Consistent with previous work, these patients generally were mildly impaired on neuropsychological tests, even though many were not symptomatic at the time of assessment. Neuropsychological tests seem to be sensitive to the presence and severity of cerebral ischemia, but in isolation may not be sufficient to determine the loci of vascular stenoses. The incongruence between angiographic and neuropsychological findings underlines the importance of distinguishing among different levels and types of measurements in defining subgroups of cerebral revascularization candidates. Collaboration among neuroscientists will further our understanding of the interrelationships among neurodiagnostic tests.

Carotid Artery Diseases↗
PubMed1998

Intellectual functions and regional cerebral haemodynamics after extensive omental transplantation spread over both frontal lobes in childhood moyamoya disease.

Most accepted operations for childhood moyamoya disease have attempted to increase cerebral blood flow (CBF) in the ischaemic cortical areas around the central fissure. Developed ischaemic brain damage in the prefrontal area may lead to poor intellectual outcome and restrict patients' daily lives. Thus, extensive cerebral revascularization in both the ischaemic anterior and middle cerebral artery territories is mandatory. We describe the long-term follow-up results for intellectual outcome and performance status and make an evaluation of regional cerebral haemodynamics after extensive omental transplantation spread over both frontal lobes performed as the initial management. In the past 10 years, 10 moyamoya patients less than 12 years of age consecutively underwent omental transplantation. The omental flap was spread over not only the symptomatic hemisphere but also the contralateral frontal lobe after a large craniotomy. Superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis was accomplished simultaneously. On the contralateral hemisphere, STA-MCA anastomosis combined with encephalomyosynangiosis was subsequently performed. The clinical observation period averaged 6.7 years (ranged 1.9 to 9.2 years). Apart from 2 patients in whom severe mental retardation had been disclosed pre-operatively, full-scale intelligence quotient scores have been maintained at over 90, that is, within the normal intellectual range. With respect to quality of life (QOL), these 8 patients have been leading normal daily lives since the operation. The focal decrease in CBF observed in the frontal lobe pre-operatively in 7 cases had disappeared after surgical treatment. In these patients, serial post-operative MR angiography revealed developed omental vessels and STAs. Deterioration of intellectual functions and QOL as well as cerebral ischaemic events in paediatric moyamoya patients can be prevented by extensive omental transplantation spread over both frontal lobes combined with STA-MCA anastomosis.

Cerebral Revascularization↗
PubMed2006

Case report: Aggressive blood pressure management for carotid endarterectomy hyperperfusion syndrome.

PURPOSE: Cerebral hyperperfusion syndrome (CHS) is a rare but potentially devastating complication following cerebral revascularization. Management of CHS requires aggressive blood pressure control to prevent stroke and intracerebral hemorrhage. This case report documents a severe case of CHS, and outlines a successful outcome associated with aggressive blood pressure control. CLINICAL FEATURES: A 67-yr-old gentleman, nine days post left carotid endarterectomy, required tracheal intubation and intensive care unit admission following seizures and acute right-sided weakness. A computed tomography scan and magnetic resonance imaging revealed significant vasogenic edema in the left middle cerebral artery territory, without evidence of infarction. The history and radiographic findings suggested CHS. As such, a systolic blood pressure target was set at 90-140 mmHg. This blood pressure parameter was lower than typically targeted following acute ischemic or hemorrhagic stroke. Rapid clinical improvements were seen by day five, and tight blood pressure control was maintained throughout. Repeat computed tomography and magnetic resonance imaging revealed improved edema and no evidence of infarct or hemorrhage. CONCLUSION: Cerebral hyperperfusion syndrome is believed to occur following restoration of blood flow to a brain with impaired autoregulation due to chronic hypoperfusion. Massive brain edema and hemorrhage can result from higher pressures. Clinicians should be aware of this potential complication following cerebral revascularization procedures, and the importance of establishing blood pressure targets which are considerably lower than for other patients with similar clinical presentations.

Aged↗
PubMed1990

[Surgical strategy in polyarterial disease. Value and results of combined surgery].

Patients with coronary disease associated with carotid artery stenosis and/or abdominal aortic aneurysm often raise problems of operative strategy; in particular, the order in which these lesions must be treated is a frequent source of controversy. We report the results of sequential or simultaneous combined surgery for multiple arterial lesions. Between 1979 and 1988, 65 patients with such lesions underwent either cerebral revascularization simultaneously with coronary bypass (n = 48) or repair of infrarenal aortic aneurysm a few weeks after myocardial revascularization (n = 17). After simultaneous surgery on the carotid and coronary arteries, the postoperative mortality rate was 4.2 per cent (2 patients). Myocardial infarction occurred in 3 cases (6.2 per cent). No neurological disorder consecutive to carotid endarterectomy was observed. Late postoperative mortality involved 5 patients; in 2 of these the cause of death was non-cardiovascular. No other late complication due to coronary or carotid artery disease was noted. The 5-year survival rate, operative mortality included, was 74.8 +/- 8.6 per cent. After coronary bypass followed by abdominal aortic aneurysm repair, there was no operative death, and no perioperative complication due to coronary disease was observed. Late mortality involved 2 patients. One patient underwent bilateral femoro-popliteal bypass 4 years after repair of an aortic aneurysm. Percutaneous angioplasty for proximal stenosis of a venous coronary graft implanted 8 years previously was performed in one patient with primary success. All other patients are now asymptomatic as regards both coronary and peripheral arteries. The 7-year survival rate is 85.7 +/- 9.4 per cent. The absence of neurological disorders after simultaneous myocardial and cerebral revascularization, and the absence of cardiac complications after aortic aneurysm repair preceded by coronary bypass enable us to recommend systematic combined or sequential vascular surgery. The long-term survival rate obtained in this series supports this recommendation.

Aged↗
PubMed2006

Delayed re-opening of an STA-MCA bypass graft.

We describe the case of a 47-year-old female with symptomatic right MCA stenosis who had undergone cerebral revascularization through a superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass. Despite clear patency in the operating room, post-operative angiography showed no flow in the bypass. Her ipsilateral internal carotid artery (ICA) was widely patent. She remained asymptomatic and follow-up angiography four years later showed a widely patent bypass graft in the setting of critical stenosis of the ipsilateral ICA. That the graft was found opened up and supplying the hemisphere was presumably stimulated by an increased "demand" and flow gradient promoting its patency.

Blood Pressure↗
PubMed1999

Liquid jets, accelerated thrombolysis: a study for revascularization of cerebral embolism.

A prior study has reported that a rapid recanalization therapy of cerebral embolism, using liquid jet impacts generated by the interaction of gas bubbles with shock waves, can potentially penetrate through thrombi in as little as a few microseconds with very efficient ablation (Kodama et al. 1997). The present study was undertaken to examine the liquid jet impact effect on fibrinolysis in a tube model of an internal carotid artery. First, the conditions for generating the maximum penetration depth of liquid jets in the tube were investigated. Gelatin was used to mimic thrombi. The shock wave was generated by detonating a silver azide pellet weighing about a few micrograms located in a balloon catheter. The collapse of the inserted gas bubbles and the subsequent liquid jet formation were recorded with high-speed photography. Second, thrombi were formed using fresh human blood from healthy volunteers. The fibrinolysis induced by the liquid jet impact with urokinase was explored. This was conducted under selected conditions based on the experiment using the gelatin. Fibrinolysis was calculated as the percentage of the weight loss of the thrombus. Fibrinolysis with urokinase alone and with a single liquid jet impact with urokinase was 1.9 +/- 3.7% (n = 16) and 20.0 +/- 9.0% (n = 35), respectively, for an incubation time of 60 min. Statistical differences were obtained between all groups (ANOVA). These results suggest that liquid jet impact thrombolysis has the potential to be a rapid and effective therapeutic modality in recanalization therapy for patients with cerebral embolism and other clinical conditions of intra-arterial thrombosis.

Carotid Artery, Internal↗
PubMed1996

Management and long-term outcome of adenoid cystic carcinoma with intracranial extension: a neurosurgical perspective.

Adenoid cystic carcinoma is a tumor of minor and major salivary glands that often invades the cranial base and intracranial cavity via local and perineural spread. In the past, the role of neurosurgeons in managing these tumors has been limited. The growth of interdisciplinary cranial base surgical approaches has now increased the involvement of neurosurgeons in the management of these tumors. We present a series of 16 patients with adenoid cystic carcinomas with cranial base and intracranial extension, the largest series reported in the neurosurgical literature. We have focused our approach on a neurosurgical perspective and made recommendations for the treatment of these tumors in relation to the following specific aspects of this disease. 1) The management of the carotid artery: In our experience, when the carotid artery is involved by tumor, a preliminary cerebral revascularization procedure with a cervical carotid to middle cerebral artery vein bypass graft should be performed before tumor resection. 2) The management of the cavernous sinus and orbit: Cavernous sinus tumor should be removed as fully as possible, but every effort should be made to preserve the IIIrd and IVth cranial nerves to achieve optimal functional and cosmetic results. The orbit should be exenterated when there is intraconal involvement; otherwise, intraorbital tumor can be removed with orbital preservation. 3) The use of palliative surgery: We have found that the use of palliative surgery can be considered even in patients whose extent of local disease precludes a surgical cure. The slow progression of the disease allows for long-term survival of many patients with advanced local disease and even of those with metastatic disease. 4) The long-term survival of these patients: In our series, six patients had no evidence of local disease and a mean survival of 72 months, one living patient had evidence of local disease and has survived 56 months, eight patients died of disease, with a mean survival of 137 months, and one patient died of complications after surviving for 63 months.

Adult↗
PubMed1978

Protective effects of methyl prednisolone and dimethyl sulfoxide in experimental middle cerebral artery embolectomy.

Acute arterial embolism continues to be a major cause of stroke morbidity in children and young adults. Potential therapy modalities include medical management and/or cerebral revascularization. The canine middle cerebral artery (MCA) was embolized by means of a pliable cylinder, 8 mm long by 1.6 mm in diameter, via the internal carotid artery. Control and experimental embolectomies were performed 6 hours following embolization. The experimental animals were treated with either dimethyl sulfoxide (DMSO) or methyl prednisolone. In the control animals, the average area of infarction in the brain was 1.45 cu cm. The animals treated with methyl prednisolone (2 mg/kg) or DMSO (2 gm/kg) showed no infarction of the brain, whereas methyl prednisolone (30 mg/kg) did not prevent infarction.

Animals↗
PubMed2003

Combined off-pump coronary revascularization: ascending aorta to carotid bypass.

A 76-year-old man with symptomatic cerebral vascular disease and coronary artery disease was treated with simultaneous off-pump bypass and cerebral revascularization. With the emergence of off-pump coronary artery operation as a viable option for coronary revascularization we believe that this procedure will become our method of choice for combined cerebral and coronary artery disease.

Aged↗
PubMed1985

Cerebral angiography and neuropsychological measurement: the twain may meet.

In a sample of 31 cerebral revascularization candidates, severity and dissemination of atherosclerosis on the cerebral angiogram were correlated with two of three global indicators of neurobehavioral impairment. Additionally, the angiographic rating was correlated with age and with an index of medical risk factors, but not with duration of the longest symptomatic episode. It seems likely that several variables, particularly collateral circulation, help to determine whether a given pattern of stenoses results in neuropsychological dysfunction and what type of behavioral deficit occurs. In many cases, the configuration of neuropsychological test scores may not directly mirror the pattern of cerebrovascular stenoses.

Adult↗
PubMed2005

Superficial temporal artery-to-middle cerebral artery bypass.

The superficial temporal artery-to-middle cerebral artery bypass represents the mainstay of bypass operations for cerebral revascularization. The role of this operation in the setting of cerebrovascular occlusive disease, with compromised cerebral hemodynamic reserve, is a topic of current investigation. In this report, we describe technical nuances to avert potential pitfalls in the performance of this procedure. The use of intraoperative quantitative blood flow measurements as a reliable method of assessing the success of the bypass is also described.

Brain Ischemia↗
PubMed1997

EC-IC bypass improves chronic ischemia in a patient with moyamoya disease secondary to sickle cell disease: an in vivo microdialysis study.

Patients with sickle cell disease may develop intracranial vascular disease, with the occlusion or obstruction of the large or small arteries, which may lead to the secondary development of moyamoya disease. In this report, we describe the neurochemical changes in the brain before, during, and after an extracranial-intracranial bypass procedure on a patient with sickle cell disease and a moyamoya disease like pattern on angiography. We used the in vivo microdialysis technique to measure the on-line pH, lactate and amino acid concentrations in the extracellular fluid. There were relatively high resting glutamate levels and a lower-than-normal pH in the extracellular fluid prior to the bypass, associated with chronic ischemia. During the bypass there was a short-lived increase in the glutamate levels. After revascularization, there was a rapid decrease in the glutamate levels and an increase in the pH value. The patient's preoperative neurological deficit improved post-operatively, corresponding to the biochemical changes towards normal values. These changes after revascularization suggest that chronic biochemical abnormalities due to brain ischemia may improve after cerebral revascularization.

Adult↗
PubMed1990

Effects of medical and surgical treatment on cerebral perfusion and cognition in patients with chronic cerebral ischemia.

The effects of medical treatment with and without cerebral revascularization procedures on cognition and cerebral blood flow were compared among 36 patients with extracranial occlusive cerebrovascular disease and cognitive impairments. Three comparable groups were studied. The first group (N = 18) received only medical treatment by control of risk factors for stroke (including hypertension, diabetes, and hyperlipidemia) and antiplatelet aggregant medication. The second group (N = 10) had the same medical treatment plus superficial temporal-to-middle cerebral artery bypass, and the third group had the same medical treatment plus carotid endarterectomy. Regional cerebral blood flow and cognition were monitored in all three treatment groups over a 3-year interval. All groups showed stabilization without expected rates of decline for both cerebral blood flow and cognition, but no statistically significant differences emerged among the treatment groups.

Aged↗
PubMed1999

Spontaneous occlusion of supraclinoid aneurysms after the creation of extra-intracranial bypasses using long grafts: report of two cases.

OBJECTIVE: We describe two cases of giant supraclinoid aneurysms, treated by means of saphenous vein grafting between the external carotid artery and the middle cerebral artery, which unexpectedly spontaneously occluded. CLINICAL PRESENTATION: Two patients presented with subarachnoid hemorrhage and headache, respectively. In the first case, angiography showed an aneurysm of the right internal carotid artery (ICA), which had been treated by clipping. Repeat angiography showed a giant aneurysm of the right ICA, the formation of which was probably caused by sliding of the clip that had been applied during the previous operation. The patient was operated on again, but it was impossible to exclude the aneurysm because no clear neck could be identified. In the second case, magnetic resonance imaging and cerebral angiography showed a large, partially thrombosed aneurysm of the supraclinoid segment of the left ICA. TECHNIQUE: In view of the patients' ages and the statuses of compensatory circulation, each patient underwent cerebral revascularization with a long saphenous vein graft placed between one branch of the middle cerebral artery and the external carotid artery, in anticipation of subsequent endovascular treatment of the aneurysm and/or closure of the ICA in the neck. Postoperative angiography demonstrated spontaneous occlusion of the aneurysms. CONCLUSION: Thrombosis of an aneurysm may occur spontaneously or after explorative surgery. However, it should be remembered that spontaneous occlusion of an aneurysm may be induced or favored by hemodynamic vascular alterations that take place inside the aneurysm after a high-flow extra-intracranial bypass has been created.

Adult↗