Intracranial Vascular Malformations Research Articles

Multiple cerebral cavernous malformations in a pediatric patient with Turner syndrome

Abstract Turner syndrome (TS; 45,X0) is a relatively common chromosomal disorder that is associated with characteristic phenotypic stigmata: short stature, webbed neck, broad (“shield”) chest with widely spaced nipples, cubitus valgus, ovarian dysgenesis (“streak ovary”), primary amenorrhea, renal anomalies, lymphedema of the hands or feet, and various vascular abnormalities. Abnormalities of the cardiovascular system are commonly reported in patient with TS, and vascular anomalies affecting various other organ systems are also frequently reported. To date, however, few reports of intracranial vascular malformations exist. The authors report the case of a patient with TS who was found to have multiple cerebral cavernous malformations on imaging.

Intracranial Dural Arteriovenous Fistulae.

Intracranial dural arteriovenous fistulae (dAVFs) are typified by pathological anastomoses between meningeal arteries and dural venous sinuses or cortical veins. These fistulae frequently reside within the dural leaflets surrounding a venous sinus, characteristically at the transverse-sigmoid junction but also at the cavernous sinus (CS), superior sagittal sinus, anterior cranial fossa, tentorium, and other locations. dAVFs are distinguished from their pial counterparts (eg, arteriovenous malformations) by their arterial supply from vessels that perfuse the dura mater and lack of a parenchymal nidus.1 dAVFs are rare lesions, accounting for 10% to 15% of all intracranial vascular malformations: 6% of supratentorial and 35% of infratentorial vascular malformations.2 Most frequently, dAVFs affect patients in their middle-to-later years of life (eg, 50–60 years of age).3 Less commonly, dAVFs occur in younger age groups, including children. There is neither a clear sex predilection nor genetic component to these lesions. Clinical manifestations vary widely and depend on both the hemodynamic properties and location of the fistula. Multiple classification systems have been proposed in an effort to predict which dAVFs are at high risk for new neurological events. Most of these schemes stratify risk based on angiographic appearance of the dAVFs, in particular involvement of a venous sinus and presence or absence of retrograde cortical venous drainage (CVD). Other schemes combine angiographic appearance with mode of presentation, given the impact symptoms related to intracerebral hemorrhage (ICH) and nonhemorrhagic neurological deficits (NHND) have on dAVF natural history.4–6 In the following review and synthesis, the authors describe the pathophysiology, classification, mode of presentation, imaging characteristics, natural history, and therapeutic armamentarium for intracranial dAVFs. Particular attention will be given to recent evidence that certain clinical and angiographic features of dAVFs can be used to help stratify risk of future ICH and NHND in an effort …

Optimization of the Surgical Approach in AVMs Using MRI and 4D DSA Fusion Technique : Atechnical note.

Previously published data demonstrated the possibility of displaying the angioarchitecture of intracranial vascular malformations using time-resolved 3D imaging (4D digital subtraction angiography [DSA]). The purpose of our study was to prove the technical feasibility of creating fused images of time-resolved 3D reconstructions and MPRAGE MRI data sets and to check the reliability of the correct anatomical display of the angioma nidus and the venous drainage in the fused images of patients with intracranial arteriovenous malformations (AVM). In this study 20patients with intracranial AVM underwent pretherapeutic DSA and time-resolved 3D DSA in addition to MRI including MPRAGE sequences. The images were post-processed with the fusion software tool on adedicated research workstation. The fusion of both imaging modalities was done semi-automatically with automatic co-registration software followed by amanual co-registration. Co-registered DSA/MRI data sets of 20 untreated AVMs were evaluated independently by two reviewers. Image fusion was successful in all 20cases with an acceptable additional set-up time. The fused images were highly scored by the two raters in respect to their congruency of the dedicated regions. Precise anatomical localization of the nidus, the feeding arteries and the draining veins were possible with the merged images. Creating fused images of time-resolved 3D DSA and contrast-enhanced T1-weighted MPRAGE MR images might be beneficial for the preoperative and intrasurgical workflow in patients with AVMs. This new software tool fulfils the required quality and accuracy of the merged images. The clinical validation has to be proven in further studies.

Epilepsy and Intracranial Vascular Malformations, a Possible Missing Link!

Epilepsy and Intracranial Vascular Malformations, a Possible Missing Link!

Epilepsy and Intracranial Vascular Malformations: A Possible Missing Link!

The development of brain arteriovenous malformations (AVMs) remains enigmatic. Their usual presentation with hemorrhage could be a devastating event. Their second mode of presentation; Seizure poses a mechanistic, diagnostic and therapeutic challenge. For the most part, vascular development and flow abnormalities are implicated in the development of seizures in the context of brain AVMs. Proposed is a hypothesis in which common cortical and vascular development pathways lead to perturbation of the vascular phenotype resulting in arteriovenous shunting while simultaneously leading to focal form of cortical dysplasia conducive of seizure.

Three-dimensional model printing for surgery on arteriovenous malformations.

Surgery for intracranial complex vascular malformations can be quite exigent and involves considerable preoperative planning. Here, we present the advantages of using three-dimensional (3D) printed models as a preoperative investigational modality. 3D printed models were made from thin-slice computed tomography (CT) angiography scans of 6 patients with arteriovenous malformations over an18-month period from August 2015 to December 2016. The locations of the arteriovenous malformations were in the Sylvian fissure in 1 patient, posterior frontal region in 2 patients, subfrontal region in 1 patient, and parietal region in 2 patients. The CT angiography was performed on a 64-slice CT scanner. Thin-slice axial CT sections were acquired and a volume file was created of the arteriovenous malformation and the required skull bones. The file was then transferred to the 3D printer for creating the model. The model depicted the precise nature of the compactness and location of the nidus in relationship to the skull. It was possible to clearly delineate the course, size, and number of feeding vessels and draining veins. The model made identification of the normal and abnormal vessels easier and assisted in the preparation and conduct of surgery. The model was made to scale and was placed beside the surgeon during the operation. The limitation of current technology was that the exact differentiation of arteries and veins by color coding was not possible. 3D printed models can be helpful in getting information regarding the architecture and character of the arteriovenous malformation. The models are cost-effective and easy to build.

Chapter 10 - Surgical management of cerebral dural arteriovenous fistulae

Dural arteriovenous fistulae are high flow, low resistance intracranial vascular malformations defined by an aberrant connection between an artery and dural vein or sinus. Symptomatology and presentation are highly dependent on location, generally categorized as supratentorial, tentorial, or infratentorial, and consist primarily of sequelae secondary to local venous hypertension, insufficiency, and cortical venous reflux. Surgery is generally reserved for high risk or persistently symptomatic lesions that are unamenable or unresponsive to endovascular therapy. For surgical lesions, familiarity with skull base approaches, specific fistula anatomy, and technical nuances based on fistula location offer patients the best chance of a favorable outcome.

Chapter 11 - Endovascular management of intracranial dural arteriovenous fistulas

Dural arteriovenous fistulas are a heterogeneous group of lesions that comprise 10-15% of intracranial vascular malformations. The treatment strategy is devised after careful consideration of the arterial supply, venous drainage, clinical presentation, and risk of progression, hemorrhage, or neurologic decline. With recent advancements in endovascular technology, the majority of dural arteriovenous fistulas can be treated with either transarterial or transvenous embolization. Those that cannot be fully treated by endovascular means are approached with either adjuvant surgery or radiotherapy.

Facial nerve palsy after transarterial embolization of dural arteriovenous fistula

Dural arteriovenous fistulas are abnormal connections between the dural arteries and dural veins or venous sinuses. These intracranial vascular malformations can present with a variety of features ranging from completely asymptomatic to fatal intracranial haemorrhage. With the advent of newer endovascular treatment techniques, most dural arteriovenous fistulas can be effectively managed with transvenous or transarterial embolization procedures. However, these procedures are not without complications. Herein, we report a case of facial nerve palsy following embolization of posterior fossa dural arteriovenous fistula.

Evolutionary History of Multiple Dural Fistula.

Intracranial dural arteriovenous fistulas (DAVFs) are abnormal communications between arteries and veins or dural venous sinuses, which sit between the sheets of the dura. They represent 10% to 15% of intracranial vascular malformations. Clinical manifestations and prognosis depend on the pattern of venous drainage and location. The clinical presentation of DAVF may be mistaken for vascular or nonvascular brain pathologies. For that reason, within the differential diagnosis come a wide range of conditions, such as secondary headaches, encephalopathies, dementias including those with rapid progression, neurodegenerative diseases, inflammatory processes, or tumors typically at the orbital level or in the cavernous sinus. Diagnosis requires a high degree of suspicion because of the multiplicity of symptoms and presentations, making this pathology an entity that provides a major challenge for clinicians, yet early and multidisciplinary treatment of high-grade fistulas improve the possibility of avoiding poor or unfavorable outcomes for the patient.

Time-resolved 3D rotational angiography: display of detailed neurovascular anatomy in patients with intracranial vascular malformations

PurposeThe purpose of this pilot study was to demonstrate the applicability of time-resolved three-dimensional (3D) reconstructions from 3D digital subtraction angiography (DSA) rotational angiography (RA) datasets (four-dimensional (4D) DSA) to...

Dementia risk after spontaneous intracerebral haemorrhage: a prospective cohort study

Dementia occurs in at least 10% of patients within 1 year after stroke. However, the risk of dementia after spontaneous intracerebral haemorrhage that accounts for about 15% of all strokes has not been investigated in prospective studies. We aimed to determine the incidence of dementia and risk factors after an intracerebral haemorrhage. We did a prospective observational cohort study in patients with spontaneous intracerebral haemorrhage from the Prognosis of Intracerebral Haemorrhage (PITCH) cohort who were admitted to Lille University Hospital, Lille, France. We included patients aged 18 years and older with parenchymal haemorrhage on the first CT scan. Exclusion criteria were pure intraventricular haemorrhage; intracerebral haemorrhage resulting from intracranial vascular malformation, intracranial venous thrombosis, head trauma, or tumour; haemorrhagic transformation within an infarct; and referral from other hospitals. Median follow-up was 6 years. We studied risk factors (clinical and neuroradiological [MRI] biomarkers) of new-onset dementia as per a prespecified subgroup analysis, according to intracerebral haemorrhage location. Dementia diagnosis was based on the National Institute on Aging-Alzheimer's Association criteria for all-cause dementia. We did multivariable analyses using competing risk analyses, with death during follow-up as a competing event. From the 560 patients with spontaneous intracerebral haemorrhage enrolled in the PITCH cohort between Nov 3, 2004 and March 29, 2009, we included 218 patients (median age 67·5 years) without pre-existing dementia who were alive at 6 months follow-up. 63 patients developed new-onset dementia leading to an incidence rate of 14·2% (95% CI 10·0-19·3) at 1 year after intracerebral haemorrhage, and incidence reached 28·3% (22·4-34·5) at 4 years. The incidence of new-onset dementia was more than two times higher in patients with lobar intracerebral haemorrhage (incidence at 1 year 23·4%, 14·6-33·3) than for patients with non-lobar intracerebral haemorrhage (incidence at 1 year 9·2%, 5·1-14·7). Disseminated superficial siderosis (subhazard ratio [SHR] 7·45, 95% CI 4·27-12·99), cortical atrophy score (SHR per 1-point increase 2·61, 1·70-4·01), a higher number of cerebral microbleeds (SHR for >5 cerebral microbleeds 2·33, 1·38-3·94), and older age (SHR per 10-year increase 1·34, 1·00-1·79) were risk factors of new-onset dementia. There is a substantial risk of incident dementia in dementia-free survivors of spontaneous intracerebral haemorrhage; our results suggest that underlying cerebral amyloid angiopathy is a contributing factor to the occurrence of new-onset dementia. Future clinical trials including patients with intracerebral haemorrhage should assess cognitive endpoints. French Ministry of Education, Research, and Technology, Adrinord, Inserm U1171.

Neurovascular 4DFlow MRI (Phase Contrast MRA): emerging clinical applications

Recent advances in 4DFlow MRI (Phase Contrast MRA) acquisition and reconstruction enable high resolution exams to be obtained in practical imaging times. 4DFlow MRI provides images of vascular morphology and quantitative measurements of blood velocity throughout a 3D imaging volume. Hemodynamic parameters such as flow volume, relative wall shear stress, streamlines, vorticity and pressure gradients can be derived from the velocity data. The combination of anatomic vessel wall imaging, lumen visualization and physiologic data derived from accelerated 4DFlow MRI augments the characterization of intracranial arterial stenosis, aneurysms, vascular malformations and dural sinus pathology. This review provides an update for clinicians interested in 4DFlow MRI of the brain.

Dural Arteriovenous Fistulae: Imaging and Management.

Intracranial dural arteriovenous fistulae (DAVF) are pathologic arteriovenous shunts between meningeal arteries and dural venous sinuses or veins. They account for approximately 10% to 15% of all intracranial vascular malformations and are most common in middle-aged patients. DAVF are the most common vascular cause of pulsatile tinnitus. Digital subtraction angiography remains the gold standard for diagnosing these lesions. The pattern of venous drainage determines the type of DAVF and their risk for hemorrhage. Endovascular treatment has become a first-line option for their management. This article describes the natural history, clinical presentation, classification, imaging features, and management options of intracranial DAVF.

Vein of Galen aneurysmal malformation—clinical and angiographic spectrum with management perspective: an institutional experience

Background and purposeVein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically...

Abstract WP393: Cervical Arterial Fibromuscular Dysplasia in a Bi-ethnic Population: a Retrospective Study in U.S.-Mexican Border

Background: There have been studies trying to characterize Fibromuscular Dysplasia (FMD), however most of them are based in mainly non-Hispanic samples. The objective of this study is to describe the epidemiology, clinical characteristics, angiographic findings, and associated vascular conditions of cervical arterial fibromuscular dysplasia in Hispanics in the US-Mexican border. Methods: We retrospectively reviewed the cerebral angiograms performed in our center in a period of 3.5 years under different indications (cervical and intracranial steno-occlusive arterial disease, intracranial vascular malformation, cranial and facial tumor embolization, and subarachnoid hemorrhage) looking for angiographic patterns of FMD involving the cervical (internal carotid and vertebral) arteries. Exclusion criteria included subjects younger than 18 years old. Patients were subdivided by their self-reported ethnicity in Hispanics and non Hispanics. Of those patients found to have FMD, we looked for the association with the demographics, co-morbidities and related supra-aortic vascular abnormalities. Results: We analyzed 467 angiograms among patients >18 years of 323 (69.1%) were Hispanics and 144 (30.8%) were non-Hispanics. We found that 27 (8.3%) Hispanics patients with FMD involving the cervical arteries (internal carotid, vertebral artery isolated and in different combinations), while 20 (13.8%) cases of FMD among the non-Hispanics. There was no significant difference between the two groups regarding the frequency of FMD. In the Hispanic FMD group the mean age was 57.5 ± 15.76 years and 77.7% were females (p-value=0.007). The most common intracranial and cervical arterial conditions associated with FMD in the Hispanic group were aneurysms and arterial dissections that account for 60% and 25.9% respectively. There was a significant association between Hispanic with Female FMD and intracranial aneurysm (p=0.03). Conclusion: Our study suggests that FMD might follow the same demographic and clinical characteristics in Hispanics and non Hispanic populations in North-America. FMD in Hispanics has a clear predilection for women and intracranial aneurysm and supra-aortic arterial dissection are most commonly associated conditions.

Abstract WP436: Comparing Risk of Stroke and Death in Treated and Untreated Patients With Unruptured Brain Arteriovenous Malformations (BAVM)

Background: Management of unruptured BAVM remains controversial despite recent clinical trial results demonstrating a 3-fold increased risk of stroke and death in treated versus medically managed patients. We compared outcomes between treated and untreated patients with unruptured BAVMs using observational data from the Multicenter AVM Research Study (MARS). Methods: MARS consists of 3 unruptured cohorts: UCSF (n=416), Columbia (n=223), and the Scottish Audit of Intracranial Vascular Malformations (n=70). We used a propensity score adjusted analysis with Cox proportional hazards regression of time to adverse event (stroke or death) from date of diagnosis or treatment, censoring at last follow-up (up to 5 years) to compare the group receiving any interventional treatment of embolization, gamma knife, or surgical resection (‘treated’) to the group receiving medical management (‘untreated’). The propensity score for treatment was computed using logistic regression with predictors of age, age2, sex, Caucasian race, cohort, Spetzler-Martin score, and reason for presentation. Results: There were 709 unruptured BAVM patients (565 treated/144 untreated). A total of 81 adverse events (68 strokes/13 deaths) occurred during 1632 patient-years of follow-up for an annual rate of 5.0% (95% CI: 4.0%-6.2%) overall, 5.6% (4.4%-7.2%) in the treated group, and 4.6% (2.0%-5.4%) in the untreated group. There was a trend for increased risk of adverse events in the treated group (HR=1.5, 95% CI:0.8-2.7, p=0.180). In stratified analysis, subjects in the highest propensity quintile (i.e., those most expected to receive treatment) seemed to have lower rates of adverse events in the treated versus untreated group (HR=0.4, 95% CI:0.1-1.5, p=0.178). An analysis using all patients except those in the highest quintile suggested outcomes were worse in the treated group (HR=1.81, 95% CI:0.96-3.4, p=0.065). Conclusion: Our results suggest an increased risk of adverse events in unruptured BAVMs that are treated. However, taking into account propensity for treatment revealed a possible subgroup that may benefit from treatment. Larger sample sizes are needed to identify risk factors and develop risk stratification models for unruptured BAVMs.

Is it time for routine use of the retromolar fiberscope?

Purpose:This study aimed to determine the effectiveness, safety, and oral intubation time (IT) using a retromolar Bonfils fiberoptic scope compared with a conventional Macintosh laryngoscope.Materials and Methods:Sixty patients (16-60 years old, American Society of Anesthesiology I/II) scheduled for general anesthesia for elective ear-nose-throat and plastic surgery were randomly divided into a Bonfils group (Group B, n = 30) and a Macintosh group (Group M, n = 30). Exclusion criteria included Mallampati IV, thyromental distance ≤4 cm, mouth <4 cm, cervical spine problems, body mass index >35, sleep apnea, reflux esophagitis, coronary artery disease, intracranial vascular malformation, elevated intracranial pressure, bleeding disorders, allergies to planned drugs, and patient refusal. Mallampati scoring, mouth opening, and thyromental distance were used for airway assessment. The time needed for successful intubation (IT), number of attempts, number of failures, systolic (SBP), diastolic (DBP), mean blood pressure (MBP), heart rate (HR) and Oxygen saturation (O2) and damage to the lips, dentures, and pharyngeal or laryngeal structures were recorded. Continuous variables are presented as mean ± standard deviation and categorical variables are presented as frequency and percentage. Chi-square tests and Student's t-tests were used to compare the groups. P < 0.05 was considered statistically significant.Results:The groups were comparable regarding demographic data, preoperative airway parameters, IT, the number of attempts, O2, and the incidence of complications (P > 0.05). However, Bonfils intubation was associated with more stable HR, SBP, DBP and MBP (P < 0.05).Conclusion:The Bonfils fiberoptic scope is comparable to a Macintosh laryngoscope but assures better hemodynamic stability. In difficult cases, the Bonfils scope is a better choice because of its ability to navigate.

Intracranial dural arteriovenous fistula as a cause for symptomatic superficial siderosis: A report of two cases and review of the literature.

Background:Superficial siderosis (SS) is the occult deposition of hemosiderin within the cerebral cortex due to repeat microhemorrhages within the central nervous system. The collection of hemosiderin within the pia and superficial cortical surface can lead to injury to the nervous tissue. The most common presentation is occult sensorineural hearing loss although many patients have been misdiagnosed with diseases such as multiple sclerosis and amyotrophic lateral sclerosis before being diagnosed with SS. Only one case report exists in the literature describing an intracranial dural arteriovenous fistula (dAVF) as the putative cause for SS.Case Description:We describe two cases of SS caused by a dAVF. Both patients had a supratentorial, cortical lesion supplied by the middle meningeal artery with venous drainage into the superior sagittal sinus. In both patients, symptoms improved after endovascular embolization. The similar anatomic relationship of both dAVFs reported presents an interesting question about the pathogenesis of SS. Similar to the pathologic changes seen in the formation of intracranial arterial aneurysms; it would be possible that changes in the blood vessel lining and wall might predispose a patient to chronic, microhemorrhage resulting in SS.Conclusions:We describe the second and third cases of a dAVF as the cause of SS, and the first cases of successful treatment of SS-associated dAVF with endovascular embolization. As noninvasive imaging techniques become more sensitive and easily obtained, one must consider their limitations in detecting occult intracranial vascular malformations such as dAVF as a possible etiology for SS.

Sonographic Diagnosis of Fetal Adrenal Hemorrhage Complicating a Vein of Galen Aneurysmal Malformation.

The vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which causes end-organ ischemia or venous congestion due to heart failure. Adrenal hemorrhage associated with VGAM has not been reported in the literature. We present the imaging findings of a fetal VGAM with adrenal hemorrhage. A 26 year-old primigravida woman whose fetus with VGAM and mild cardiomegaly was scanned in the 34th week. On fetal ultrasound, a hyperechoic, well-circumscribed mass in the left suprarenal region was shown. Fetal and postnatal magnetic resonance imaging confirmed the diagnosis of adrenal hemorrhage. The baby died after delivery. Adrenal hemorrhage can complicate VGAM in fetuses with severe heart failure. Evaluation of the adrenal gland in affected cases may contribute to the prenatal counseling, and postnatal management.