BACKGROUND: Alagille syndrome (AGS) is a dominantly inherited multisystem disorder involving the liver, heart, eyes, face, and skeleton, caused by mutations in Jagged1. Intracranial bleeding is a recognized complication and cause of mortality in AGS. There are multiple case reports of intracranial vessel abnormalities and other vascular anomalies in AGS. ...

15 years ago, the treatment of incidentally discovered intracranial aneurysms was straightforward with a good evidence base behind it. When intracranial aneurysms were identified, people were referred to neurosurgeons who would offer surgical repair if the patient was in reasonable health and had a good life expectancy. Since that time, ...

The authors describe a novel approach to the management of high-output heart failure secondary to an intracranial high-flow dural arteriovenous fistula (DAVF) by using extracorporeal membrane oxygenation (ECMO). To the best of the authors' knowledge, this represents the first report of an embolization performed in conjunction with the use of ...

PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial haemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had ...

Extracranial-intracranial arterial bypass was frequently utilized in the 1970s and early 1980s to treat patients with atherosclerotic occlusive carotid arterial lesions not amenable to extracranial arterial revascularization procedures. After a large randomized trial reported in 1985 that there was no benefit of surgery in these patients, the procedure was generally ...

Three cases of re-rupture of intracranial aneurysms during cerebral angiography (RIADCA) between June and September, 2001 are reported. All cases underwent emergency craniotomy and aneurysm clipping. The subarachnoid blood and the extravasating contrast medium were removed intraoperatively as completely as possible. There was no mortality in this series. The incidence, ...

OBJECTIVES: To evaluate the frequency of intracranial atherosclerosis among patients with steno-occlusive extracranial carotid artery disease and to determine if there are factors related to the combined intracranial atherosclerosis. DESIGN: Cross-sectional study. SETTING: A tertiary referral hospital. Patients We studied 142 consecutive patients who had atherosclerotic steno-occlusive lesions (defined as ...

OBJECTIVE AND IMPORTANCE: The association of Graves' disease with multiple intracranial arterial stenoses is rare. CLINICAL PRESENTATION: We report on two Japanese women who experienced the concurrence of Graves' disease and cerebral ischemia attributable to multiple intracranial arterial stenoses around the circle of Willis. Clinically, these patients demonstrated hyperthyroidism, goiter, ...

We report 2 patients who had clinical and neuroimaging signs of spontaneous intracranial hypotension and who developed cerebral sinus venous thrombosis. This sequence of events -- known after dural puncture but not in spontaneous intracranial hypotension -- was suggested by the change in the pattern of headache, from a postural ...

Moyamoya disease is a progressive vascular disorder of unknown etiology. Theories of inflammatory and immunologic mechanisms have been proposed as the pathogeneses. We have designed a new method of administering N-acetylmuramyl-L-alanyl-D-isoglutamine (MDP) for experimental induction of moyamoya disease using an intravascular interventional technique combined with rod-shaped embolic materials made from ...

Intracranial aneurysms are frequently present with subarachnoid hemorrhage. Less often they produce suggestive symptoms of cranial nerve dysfunction or intracranial tumor when very large. Their association with epilepsy has rarely been reported; such concurrence may not be a coincidence. When the patient presents with epileptiforme attacks the presence of an ...

BACKGROUND: Intracranial atherosclerosis accounts for 8% to 10% of all ischemic strokes, and intracranial angioplasty is increasingly performed to treat stenotic lesions. We report an autopsy case and discuss the effects of intracranial angioplasty for atherosclerotic arteries. CASE DESCRIPTION: A 77-year-old patient died 9 days after angioplasty of the left ...

Screening patients with autosomal dominant polycystic kidney disease (ADPKD) for asymptomatic intracranial aneurysms has been proposed as a method of reducing the morbidity and mortality associated with aneurysm rupture. However, recent studies have shown lower spontaneous rupture rates of small aneurysms and higher risks of significant complications with interventions than ...

OBJECTIVE: Although the cause of cerebral aneurysms remains unclear, there is clear evidence that genetic predisposition plays a role. Ten percent of patients report an aneurysm in a first-degree family member. However, studies to date have largely involved Caucasian populations. Our goal was to characterize the familial aggregation of intracranial ...

OBJECTIVE: Massive intraoperative swelling (IOS) is a potential complication of intracranial surgery for traumatic hematomas. We present a novel design of dural opening to minimize the risks of IOS. METHODS: Over the last eight years, we have used and evaluated a "reversed U-shaped" durotomy incision, which leaves the dura intact ...

OBJECT: Intracranial stent placement combined with coil embolization is an emerging procedure for the treatment of intracranial aneurysms. The authors report their results using intracranial stents for the treatment of intracranial aneurysms. METHODS: A prospectively maintained database was reviewed to identify all patients with intracranial aneurysms that were treated with ...

Summary: Traumatic intracranial aneurysms in children are rare and mostly related to skull fracture or rapid decelerating closed head injury.We report the case of an infant who developed intracranial aneurysm after minor head trauma and managed by endovascular treatment. A seven-month-old infant presented with delayed intracranial hemorrhage following minor head ...

OBJECTIVE: Chlamydia pneumoniae (C pneumoniae) is a common cause of a usually mild, community acquired pneumonia. This organism, however, can spread from the respiratory tract into other parts of the body and has been detected in up to 70% of atheromatous lesions in blood vessels. Although the exact mechanism of ...

BACKGROUND: The acronym PHACE describes a rare neurocutaneous syndrome encompassing the following features: posterior fossa brain malformation, hemangioma, arterial anomalies, coarctation of aorta and cardiac defects, and eye abnormalities. This report describes the cervico-cerebrovascular anomalies (CCVA) in 7 patients with PHACE syndrome. METHODS: The medical records of 7 children (4 ...

While the mortality rate of subarachnoid haemorrhage is very high in haemodialysis (HD) patients, the prevalence of unruptured intracranial aneurysms in HD patients has not yet been elucidated. We performed cerebral magnetic resonance angiography (MRA) on 123 HD patients who did not have symptomatic cerebrovascular disease, and on 52 control ...

Despite many reports investigating the relationship of subarachnoid haemorrhage and seasonal variation in different parts of the world, there is no clear correlation. As far as we know this subject has not been reported from Istanbul yet. The purpose of this study is to determine if there is any correlation ...

Extracranial-intracranial bypass surgery was performed prior to carotid resection in eight patients with head and neck carcinoma that involved the carotid artery near the skull base. Four patients underwent the standard one-stage extracranial-intracranial bypass procedure. A two-stage procedure was performed in the remaining four patients. The procedure first involved an ...

OBJECTIVE: Elevated serum levels of lipoprotein(a), a risk factor for atherosclerosis (AS), are also associated with the presence of asymptomatic intracranial aneurysms. AS is present in some aneurysms, but its contribution to aneurysm formation and growth is unclear. Apolipoprotein(a) [apo(a)], the active moiety of lipoprotein(a), is present in atherosclerotic circle ...

We report a case of spontaneous intracranial hypotension that underwent cerebral angiography. The angiogram showed prominence of the veins and venous phase of the angiogram. This is thought to be secondary to decreased intracranial pressure and subsequent dilation of the venous system to attempt to replace the lost intracranial CSF ...

Summary: Formation of de novo intracranial aneurysms is rare. Their etiology is not known, but they are seen in patients with inherited collagen disorders, polycystic kidney disease, and familial history of aneurysms. Most de novo intracranial aneurysms are found 3-20 years after diagnosis of the initial aneurysm. We report the ...

OBJECT: Vascular bypass is performed in neurosurgery for a variety of pathological entities, including extracranial atherosclerotic disease, extra- and intracranial aneurysms, and tumors involving the carotid artery (CA) at the skull base or cervical regions. Creation of an interposition saphenous vein graft (SVG) is the typical method of choice when ...

OBJECTIVE AND IMPORTANCE: A unique case of delayed ischemic deficit after resection of a large intracranial dermoid is presented. CLINICAL PRESENTATION: A 23-year-old woman, 36 hours after the uneventful gross total resection of a large intracranial dermoid cyst, slowly developed a progressive mixed aphasia and right hemiparesis. Magnetic resonance imaging ...

BACKGROUND: Although vasculopathy is a recognized complication during acute meningitis, to our knowledge, no previous reports have been published of this phenomenon developing months after successful treatment. OBJECTIVE: To report a unique case of a late-developing vasculopathy after pyogenic meningitis in an adult. REPORT OF A CASE: A 51-year-old woman ...

Intracranial internal carotid artery (ICA) aneurysms are frequently treated either by microsurgical clipping of the aneurysm neck, by endovascular coiling of the aneurysm sac or by balloon occlusion of the parent vessel. For some broad-based aneurysms that may not be suitable for any of these options, microsurgical wrapping of the ...

Childhood intracranial aneurysms are exceedingly uncommon. Diagnosis of intracranial aneurysms in infancy may be difficult because of their infrequency and confusing clinical presentation. Findings with routine radiographic methods may be misleading and difficult to interpret. We present a case of the rupture of an anterior communicating artery aneurysm in a ...

BACKGROUND: We aimed to prospectively assess the usefulness of computer tomographic angiography (CTA) in streamlining the management of symptomatic intracranial aneurysms in a tertiary neurosurgical unit, from admission to surgery. METHODS: We performed a prospective evaluation over a 2-year period of all symptomatic intracranial aneurysms managed according to a standardized ...

BACKGROUND AND PURPOSE: The clinical pattern of stroke and the angiographic distribution of cerebral atherosclerosis in the Japanese and Chinese are different from those in whites. Our purpose was to evaluate the location and distribution of severe atherosclerotic stenoses in Korean patients by using cerebral angiography. METHODS: We retrospectively reviewed ...

When intracranial aneurysms are deemed nonclippable, an accepted alternative neurosurgical treatment is to reinforce the aneurysm wall by wrapping or coating it with various materials, including muslin (cotton gauze). Granulomatous or "foreign-body" reactions, sometimes referred to as "muslinomas" or "gauzomas," and adhesive arachnoiditis are known but rare complications. Experience with ...

The persistent otic artery is a rare remnant of early fetal development that, to our knowledge, has been convincingly documented only once before with angiography. Other persistent vessels such as the trigeminal, hypoglossal, and proatlantal intersegmental arteries have been found to be associated with a variety of vascular anomalies. We ...

Summary: Acute subdural haematoma (ASDH) is rarely caused by an aneurysmal rupture. We report four cases of pure acute subdural haematomas caused by ruptured intracranial aneurysms. Aneurysms were localized in the posterior communicating artery in two cases, in the anterior communicating artery in one case and in the middle cerebral ...

Oculomotor palsy related to the presence of an intracranial aneurysm arising from the supraclinoid internal carotid artery (ICA) is a well known and described clinical condition. Recent microanatomical and clinical evidences seem to demonstrate that the pathophysiology of the aneurysm-related III nerve palsy could be interpreted as that of any ...

Intracranial aneurysms (IAs) are found more often in patients with aortic coarctation (AC) than in the general population and aneurysm rupture occurs much earlier in the lives of these patients when there is coexistent AC. The diagnosis of AC is frequently made only after a serious cerebrovascular complication has developed. ...

A 59-year-old man with abnormal vascular features (intracranial aneurysm, a cervical arteriovenous shunt, bilateral internal jugular vein occlusions, and left transverse sinus hypoplasia), as well as left optic atrophy was suspected to have familial polycystic kidney disease. The possibility of autosomal dominant polycystic kidney disease complicated by Ehlers-Danlos syndrome type ...

Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin-twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition ...

BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and primary angiitis of the central nervous system (PACNS) share several clinical and radiological features. However, digital subtraction angiogram (DSA) is generally reported as normal in CADASIL, whereas lumen irregularities in distal cerebral arteries indicate PACNS. OBJECTIVE: To describe ...

OBJECTIVE: To investigate the occurrence of microembolic signals (MES) and hemodynamic features in patients with acute symptomatic intracranial cerebral artery stenoses by transcranial Doppler (TCD). MATERIAL AND METHODS: Twelve patients with acute hemispheric ischemic events and corresponding intracranial cerebral artery stenoses as identified by TCD, and exclusion of extracranial or ...

BACKGROUND AND PURPOSE: Many cases of subarachnoid hemorrhage are due to rupture of small cerebral aneurysms. Our purpose was to evaluate the usefulness of helical CT angiography (CTA) in the detection and characterization of very small (<5 mm) intracranial aneurysms. METHODS: One hundred eighty consecutive patients underwent CTA for suspected ...

Magnetic resonance angiography (MRA) revealed silent but rapid growth of a small unruptured intracranial aneurysm until it was surgically treated to prevent rupture. Modern neuroimaging methods such as MRA and 3-dimensional computed tomography have increased opportunity to detect small unruptured cerebral aneurysms. Strict follow up is an option for the ...

The use of a microanastomotic device for direct connection of intracranial vessels can be helpful to facilitate removal of distally located middle cerebral artery (MCA) aneurysms. The authors report on two patients who presented for treatment with large aneurysms distally located on the MCA. The aneurysms were completely excised and ...

Two patients who developed subarachnoid haemorrhage are presented. The first patient was a 41-year-old woman whose angiograms showed right extracranial vertebral artery (VA) dissection starting at the C2 level extending to the intracranial VA near the VA union. Proximal occlusion of the right VA by the endovascular approach was performed. ...

The authors describe a case of intracranial vascular calcifications that mimicked small saccular aneurysms on CT angiography (CTA). The density of two vascular calcifications was visually similar to that of contrast in the parent vessel, making them indistinguishable from aneurysmal outpouchings. Comparison of the CTA with the non-contrast head CT ...

BACKGROUND AND PURPOSE: The development of intracranial collateral circulation is associated with a lower risk of stroke. A noninvasive technique that can reliably detect the presence of intracranial collaterals would be a valuable factor in the assessment of risk in patients with occlusive cerebrovascular disease. METHODS: Color velocity imaging quantification ...

OBJECTIVE: The treatment of giant intracranial aneurysms is a challenge because of the limitations and difficulty of direct surgical clipping and endovascular coiling. We describe the indications, surgical technique, and complications of saphenous vein extracranial-to-intracranial bypass grafting followed by acute parent vessel occlusion in the management of these difficult lesions. ...

BACKGROUND: Coronary abnormalities after Kawasaki disease (KD) may be associated with endothelial dysfunction due to intimal hypertrophy. The purpose of this study was to evaluate myocardial flow reserve (MFR) and endothelial function in regressed aneurysmal regions after KD. Methods and Results- Subjects were 12 patients aged 16.0+/-2.6 years who suffered ...

Dissection of the internal carotid artery usually occurs in the cervical segment, but rarely may involve the artery in the intracranial course (1). The clinical course of intracranial dissection is often catastrophic, with rapid onset of profound neurological deficit, as a result of middle and/or anterior cerebral artery involvement. When ...