Pial Arteriovenous Malformations Research Articles

An Unusual Cause of Neck Pain and Headache in Pregnancy.

A previously healthy 21-year-old Caucasian female G1P0 at 32 weeks gestation presented to the ED for an episode of syncope. She also complained of headaches, neck pain, and blurry vision. Physical examination revealed a healthy pregnant female. Neurological examination demonstrated Grade III papilledema but was otherwise unremarkable. CT brain revealed hydrocephalus and intraventricular hemorrhage of unclear etiology MRI of the head was negative for a mass lesion. MRA/MRV of the head was negative, ruling out cavernous sinus thrombosis. Lumbar puncture was bloody but negative for infection. Infectious workup, including HSV, toxoplasmosis, and neurocysticercosis, was negative. An intraventricular drain was placed for hydrocephalus. While in the hospital, she developed sudden left-sided weakness, prompting an emergency C-section. Further workup with CT angio of the brain and neck revealed an arteriovenous malformation (AVM) involving the anterior spinal artery and adjacent venous plexus. Digital subtraction angiography showed a C2-3 pial AVM with a partially thrombosed nidal aneurysm. She was transferred to an outside hospital for embolization. Embolization obliterated the aneurysm, but residual flow remained in the AVM. Blood products are visible on sagittal MRI after embolization. At hospital discharge, her left-sided weakness had resolved, and her neurological examination was normal. The hydrocephalus had resolved.

Case of a Pure Arterial Malformation and Review of the Literature. (P12-5.029)

<h3>Objective:</h3> NA <h3>Background:</h3> Pure arterial malformations are dilated, overlapping, and tortuous arteries forming a mass of arterial loops without a venous component. These malformations are rare, with limited cases reported in the literature. Affecting any of the intracranial arteries, they are usually found incidentally and often have a benign course. <h3>Design/Methods:</h3> We present a case of a pure arterial malformation in a young female with hyperprolactinemia. Furthermore, we review the literature and provide an updated summary of these malformations. <h3>Results:</h3> A 28-year-old female with a history of infantile hemangiomas, polycystic ovary syndrome, and hyperprolactinemia presented with acne, mild hirsutism, and galactorrhea. Her neurological exam was normal. Magnetic Resonance Imaging (MRI) of the brain with and without contrast ruled out a prolactinoma but revealed a 3 cm vascular malformation at the margin of the left cavernous sinus extending posteriorly into the anterior left perimesencephalic cistern. Initial differentials included a pial arteriovenous malformation or less likely a dural arteriovenous fistula. A diagnostic cerebral angiogram revealed a pure arterial malformation without arteriovenous shunting. This was composed of dysplasia of the left posterior communicating artery and anterior choroidal arteries. Stenosis of the left internal carotid artery distally with collateral support into the left anterior cerebral artery and middle cerebral artery was also noted. Genetic testing for vascular malformation syndromes, including genes known to be associated with hereditary hemorrhagic telangiectasia, was negative. Repeat MRI/MRA done 8 months from the initial scan revealed no abnormal large arterial venous shunting or definite saccular aneurysm formation. <h3>Conclusions:</h3> Although rare, pure arterial malformations should be considered in the differential diagnosis for patients presenting with a cranial vascular malformation. Management of these lesions is usually conservative given their lack of arteriovenous shunting and low risk of rupture, with only a few cases undergoing surgery or endovascular treatment. <b>Disclosure:</b> Dr. Ahmed has nothing to disclose. Dr. Regenhardt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Rapid Medical. Dr. Regenhardt has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Johnson and Bell Trial Lawyers. The institution of Dr. Regenhardt has received research support from National Institutes of Health. The institution of Dr. Regenhardt has received research support from Society of Vascular and Interventional Neurology. The institution of Dr. Regenhardt has received research support from Heitman Foundation. Dr. Vranic has nothing to disclose. Dr. Dmytriw has nothing to disclose. James D. Rabinov has stock in Pfizer.

Abstract Number ‐ 236: Pure Arterial Malformations: A Case Report

Introduction Pure arterial malformations are dilated, overlapping, and tortuous arteries forming a mass of arterial loops without a venous component. These malformations are rare, with limited cases reported in the literature. Affecting any of the intracranial arteries, they are usually found incidentally and often have a benign course. Methods We present a case of a pure arterial malformation in a young female with hyperprolactinemia. Furthermore, we review the literature and provide an updated summary of these malformations. Results A 28‐year‐old female with a history of infantile hemangiomas, polycystic ovary syndrome, and hyperprolactinemia presented with acne, mild hirsutism, and galactorrhea. Her neurological exam was normal. Magnetic Resonance Imaging (MRI) of the brain with and without contrast ruled out a prolactinoma but revealed a 3 cm vascular malformation at the margin of the left cavernous sinus extending posteriorly into the anterior left perimesencephalic cistern. Initial differentials included a pial arteriovenous malformation or less likely a dural arteriovenous fistula. She underwent a diagnostic cerebral angiogram which revealed a pure arterial malformation without arteriovenous shunting. This was composed of dysplasia of the left posterior communicating artery and anterior choroidal arteries. Stenosis of the left internal carotid artery distally with collateral support into the left anterior cerebral artery and middle cerebral artery was also noted. Lesser changes were noted in the right side with reconstitution of the right anterior cerebral artery from collaterals. Genetic testing for vascular malformation syndromes, including genes known to be associated with hereditary hemorrhagic telangiectasia, was negative. Repeat MRI/MRA done 8 months from the initial scan was stable with no abnormal large arterial venous shunting or definite saccular aneurysm formation noted. Conclusions Although rare, pure arterial malformations should be considered in the differential diagnosis for patients presenting with a cranial vascular malformation. Management of these lesions is usually conservative given their lack of arteriovenous shunting and low risk of rupture, with only a few cases undergoing surgery or endovascular treatment.

Prenatal diagnosis and characterization of extra-axial, supratentorial pial arteriovenous malformation using high-resolution transvaginal neurosonography.

Prenatal diagnosis and characterization of extra-axial, supratentorial pial arteriovenous malformation using high-resolution transvaginal neurosonography.

Diagnostic performance of dynamic 3D magnetic resonance angiography in daily practice for the detection of intracranial arteriovenous shunts in patients with non-traumatic intracranial hemorrhage.

Identification of treatable causes of intracranial hemorrhage (ICH) such as intracranial arteriovenous shunt is crucial to prevent recurrence. However, diagnostic approaches vary considerably across centers, partly because of limited knowledge of the diagnostic performance of first-line vascular imaging techniques. We assessed the diagnostic performance of dynamic three-dimensional magnetic resonance angiography (dynamic 3D MRA) in daily practice to detect intracranial arteriovenous shunts in ICH patients against subsequent digital subtraction angiography (DSA) as reference standard. We reviewed all adult patients who underwent first-line dynamic 3D MRA and subsequent DSA for non-traumatic ICH between January 2016 and September 2021 in a tertiary center. Sensitivity, specificity, accuracy, positive and negative predictive values of dynamic 3D MRA for the detection of intracranial arteriovenous shunt were calculated with DSA as reference standard. Among 104 included patients, 29 (27.9%) had a DSA-confirmed arteriovenous shunt [19 pial arteriovenous malformations, 10 dural arteriovenous fistulae; median onset-to-DSA: 17 (IQR: 3-88) days]. The sensitivity and specificity of dynamic 3D MRA [median onset-to-dynamic 3D MRA: 14 (3-101) h] for the detection of intracranial arteriovenous shunt were 66% (95% CI: 48-83) and 91% (95% CI: 84-97), respectively. The corresponding accuracy, positive and negative predictive values were 84% (95% CI: 77-91), 73% (95% CI: 56-90), and 87% (95% CI: 80-95), respectively. This study suggests that although first-line evaluation with dynamic 3D MRA may be helpful for the detection of intracranial arteriovenous shunts in patients with ICH, additional vascular imaging work-up should not be withheld if dynamic 3D MRA is negative. Comparative prospective studies are needed to determine the best imaging strategy to diagnose arteriovenous shunts after non-traumatic ICH.

Intracranial pial arteriovenous malformation in an infant treated with endovascular glue embolization

Intracranial pial arteriovenous fistulas (AVFs) are rare vascular malformation in children. Children usually present with macrocrania and localized neurological impairments, whereas infants typically present with congestive heart failure. Congenital pial AVF may have syndromic association with Rendu-Osler-Weber disease and Klippel-Trenaunay-Weber syndrome. Here, we are reporting the case of a 5-month-old child who presented with multiple episodes of seizures and magnetic resonance imaging brain revealed pial AVF from the left middle cerebral artery. Following digital subtraction angiogram, glue embolization was done and achieved complete obliteration of venous sac and flow across fistula. Child had an uneventful postoperative period and developed no complications.

Angiographic analysis on posterior fossa hemorrhages and vascular malformations beyond aneurysms by CT angiography and digital subtraction angiography

BackgroundPosterior fossa malformations are less common than supratentorial malformations, but hemorrhages in posterior fossa are more serious due to presence of vital structures within this region. Most common cause of bleed in posterior fossa apart from hypertension is aneurysms but other vascular malformations are also there which cause hemorrhage. Here we discuss other causes of posterior fossa bleed beyond aneurysms.ResultsA total of 80 patients were evaluated: 47 (58.8%) had aneurysms, 29 (36.3%) had arteriovenous malformations, one each had developmental venous anomaly and brainstem cavernoma. Thirty vascular malformations were detected, of which 18 (62.1%) were pial arteriovenous malformations (AVMs), 11 (37.9%) were dural arteriovenous fistulas (dAVF), and one had developmental venous anomaly (DVA). Six patients of AVM underwent both DSA and CTA, and CTA could correctly diagnose only 2 of 4 pial AVMs detected by DSA. Among two dAVFs detected by DSA, CTA could demonstrate dAVF only in one patient.ConclusionCTA could be used as alternative to DSA in diagnosis and characterizing aneurysms in posterior fossa but for AVMs, 3D-CTA cannot replace DSA; however potential of time-resolved CTA (TR-CTA) appears promising.

Anomalous location of intracranial vessels in adults

Anomalous intracranial vessels are not uncommon, and this finding is not always associated with arteriovenous malformations. Other conditions such as anomalous connections between arteries or phlebitc patterns can also present as vessels with abnormal intracranial locations. Noninvasive diagnosis is important to determine whether to do more invasive tests such as cerebral digital subtraction angiography or to estimate the risk of bleeding in arteriovenous malformations and therefore to evaluate the need for endovascular/surgical treatment. In this paper, we present an algorithm for the differential diagnosis of anomalous intracranial vessels according to their location (intra/extra-axial) and function (whether the vessels are arterialized). Moreover, we analyze the important points of the angioarchitecture of the principal arteriovenous malformations with risk of intracranial bleeding, such as pial arteriovenous malformations and dural fistulas.

Vasos intracraneales en localización anómala en adultos

Anomalous intracranial vessels are not uncommon, and this finding is not always associated with arteriovenous malformations. Other conditions such as anomalous connections between arteries or phlebitc patterns can also present as vessels with abnormal intracranial locations. Noninvasive diagnosis is important to determine whether to do more invasive tests such as cerebral digital subtraction angiography or to estimate the risk of bleeding in arteriovenous malformations and therefore to evaluate the need for endovascular/surgical treatment. In this paper, we present an algorithm for the differential diagnosis of anomalous intracranial vessels according to their location (intra/extra-axial) and function (whether the vessels are arterialized). Moreover, we analyze the important points of the angioarchitecture of the principal arteriovenous malformations with risk of intracranial bleeding, such as pial arteriovenous malformations and dural fistulas.

The efficacy of surgical treatment of cerebral arteriovenous malformations in a single academic institution: a case series

AimTo report on patients who underwent surgical treatment of arteriovenous malformations (AVMs) at our institution.MethodsThis retrospective single-center case series enrolled the patients who underwent surgical treatment of pial AVM at the Department of Neurosurgery, University Hospital Brno, between 2005 and 2019. The data are summarized as descriptive statistics presenting basic characteristics in all the patients and in sex or age subgroups.ResultsFifty patients were enrolled. The majority of AVMs were of Spetzler-Martin grade II (n = 27; 54%), localized supratentorialy (n = 43; 86%), and half of AVMs were ruptured. A total resection was performed in 48 patients (96%), and a good overall outcome was achieved in 44 patients (88%). Surgery-associated morbidity was 2%, and the mortality rate was 0% due to meticulous selection of patients for surgical treatment..ConclusionMicrosurgery is an appropriate method of treatment for S-M grade I-III pial AVMs. Microsurgery may be used to treat the majority of small-nidus AVMs with a low mortality and morbidity, when precisely planned and performed by an expert vascular team. The meticulous selection of patients for surgical treatment is crucial.

YouTube as a source of information for arteriovenous malformations: A content‐quality and optimization analysis

AimYouTube (YT) is not only a source of entertainment but also, a popular source of medical knowledge. Our goal was to evaluate the quality of videos concerning arteriovenous malformations (AVM) available on YT. Materials and methodsA YT search was conducted for the terms “Arteriovenous malformation”, “AV malformations”, “Intracranial AVM”, “Cerebral AVM”,” Brain AVM” and ”Pial AVM.” The first 70 videos for each search were evaluated and yielded 122 unique videos after the exclusion was applied. The videos were examined by two independent Raters using a validated quality criteria such as the Quality Criteria for Consumer Health Information (DISCERN), the Journal of the American Medical Association instrument (JAMA), and the Global Quality Score (GQS). ResultsThe mean DISCERN score was 30 out of 75 possible points, with an intraclass correlation coefficient for the absolute agreement of 0.98, indicating an excellent interrater reliability. Most videos were produced in the United States (51.6%) and were uploaded by educational channels. Videos had statistically higher DISCERN, JAMA, and GQS scores when they presented clear information, AVM symptoms, angioarchitecture, AVMs with concomitant aneurysms, risks of bleeding, Spetzler-Martin Grading, treatment information, treatment risks, treatment results, follow-up, diagrams, and a doctor as a speaker. Videos that featured animations observed higher popularity as indicated by the Video Power Index (VPI) and observed a higher DISCERN and GQS score. Videos with the overall highest VPI generated poor DISCERN scores. ConclusionsYT does not provide high content quality medical information regarding AVMs and, in some cases, offer misleading information.

Characteristics of pial brain arteriovenous malformations with transdural arterial supply

PurposeTransdural blood supply (TDBS) to pial brain arteriovenous malformations (BAVM) is uncommon and believed to be related to vascular endothelial growth factor - induced angiogenesis. The aim of this study was to define the BAVM characteristics in relation to presence and volume of TDBS. MethodsBAVMs managed at our institution between January 2006 and December 2016 who subsequently underwent complete digital subtraction angiography (DSA) were included. They were classified based on presence of TDBS as well as volume of TDBS. ResultsOf the 641 BAVM patients managed during the recruitment period, 387 (391 BAVMs) had complete pretreatment DSAs. Forty-three (11.0 %, 10 ruptured) BAVMs exhibited TDBS. With TDBS group had a significantly greater proportion of large nidus (> 3.1 cm) than the Without TDBS group (85.1 % vs 19.5 %, p < 0.01) and were more frequently temporal (32.6 % vs 14.7 %, p < 0.01) and occipital (25.6 % vs 13.5 %, p < 0.05) in location. In unruptured BAVMs, the presence of headaches was significantly more prevalent when the malformation harboured TDBS compared to not (57.6 vs 34.8 %, p < 0.05). The annual rupture rate among unruptured BAVMs treated by conservative management was 4.7 % in the With TDBS (n = 12) group and 0% (n = 21) in BAVMs with TDBS that underwent treatment including surgery, endovascular therapy, or radiosurgery. ConclusionBAVMs with TDBS are more likely to be associated with a large nidus and located in the temporal and occipital lobes. Headache is more frequently associated with the presence of TDBS. Rupture rate of unruptured BAVMS with TDBS can be effectively reduced following treatment.

Microsurgical Anatomy of the Meningeal Branch of the Dorsolateral Medullary Plexus

Meningeal branches originating from intradural arteries may be involved in several diseases such as meningeal tumors and arteriovenous lesions. These "pial-dural" arterial connections have been described for anterior cerebral, posterior cerebral, and cerebellar arteries. However, to the best of our knowledge, meningeal supply originating from the arterial plexus over the dorsolateral aspect of the medulla oblongata (dorsolateral medullary plexus [DLMP]) has not been described. To define the microsurgical anatomy of the meningeal branch of DLMP. A total of 20 cadaver heads (40 sides) underwent far-lateral craniotomy and the cerebellomedullary cisterns were explored to find the DLMP and any meningeal branches. Additionally, de-identified intraoperative images of 85 patients with vertebral artery (VA)/posterior inferior cerebellar artery aneurysms who had undergone far-lateral craniotomy were studied to find any meningeal branches of DLMP. The meningeal branches of DLMP were identified in 4 cadavers/sides. These branches reached the region of jugular tubercle (JT) after crossing the accessory nerve. In 3 specimens, these branches were joined by a small twig from V4-VA before penetrating the dura. DLMP meningeal branches were found in 12 patients of the studied cohort (14%) with similar anatomical features as those found in the cadaveric study. DLMP may give rise to meningeal branches to the adjacent dura of JT. The actual prevalence of this anatomic variation is difficult to estimate using our data. However, when present, these branches may have important clinical implications, ie, diseases such as dural arteriovenous fistulas, pial arteriovenous malformations (AVMs), and meningeal-based tumors.

Pediatric intracranial arteriovenous shunts: Advances in diagnosis and treatment.

Pediatric intracranial arteriovenous shunts (IAVSs) comprise a wide range of lesions, including pial arteriovenous malformation (AVM) and arteriovenous fistula (AVF), dural arteriovenous fistula (DAVF) and vein of Galen aneurysmal malformation (VGAM). We provide an overview of pediatric IAVSs, encompassing both diagnosis and endovascular treatment. We include references from pertinent literature and representative cases from our hospital. Pediatric IAVS are frequently associated two broader conditions, such as capillary malformation-arteriovenous malformation (CM-AVM) and hereditary hemorrhagic telangiectasia (HHT). These conditions and the associated genetic mutations have only recently been described. Their impact on the brain will be different in the prenatal period, in neonates, in infants, and in children, with variable symptoms according to each age group and the current strategies of endovascular treatment in the management of these vascular lesions will be discussed. This review could improve the understanding of pediatric IAVSs and their diagnosis and treatment.

Microsurgical Excision of T12-L1 Pial Arteriovenous Malformation: 3-Dimensional Operative Video.

This 29-yr-old man presented with progressive paraparesis, sensory loss, allodynia, bowel, and bladder dysfunction for 9 mo, acutely exacerbated in the preceding 24 h. Magnetic resonance imaging scan showed multiple dilated vessels involving the thoracic cord. Spinal angiogram revealed a T12-L1 pial arteriovenous malformation (AVM)/arteriovenous fistula on the left side. It was fed by an L1 radicular artery that filled the anterior spinal artery, which in turn had multiple feeders to a pial AVM. Because of the supply from the anterior spinal artery with multiple feeders to the AVM, and the danger of infarction of the conus, embolization was not performed. He underwent T11-L1 laminectomy laterally to the pedicles and excision of AVM. There was one large arterialized vein in the midline that had a fistulous connection with an artery coursing up from inferiorly. Despite occlusion of this fistula, the vein was still arterialized. On further exploration, there was a large artery coming in to the subarachnoid space at the T11 level and coursing inferiorly, and entering the intradural pial AVM with a glomus of vessels located at the T12 level in the left anterolateral subpial aspect of the cord. This major artery as well as multiple smaller vessels going into it were cauterized and divided, and the AVM was totally excised. Postoperative angiogram showed complete excision of the nidus. At 1 mo follow-up, he had complete recovery of motor and bladder functions but bowel dysfunction persisted. He was independent for his daily activities. Informed consent was obtained from the patient prior to the surgery that included videotaping of the procedure and its distribution for educational purposes. All relevant patient identifiers have also been removed from the video and accompanying radiology slides.

Embolization of cerebral arteriovenous shunts in infants weighing less than 5 kg.

Endovascular treatment in children, especially neonates, can be more challenging than analogous procedures in adults. This study aimed to describe the clinical and radiological findings, type and timing of endovascular treatment, and early outcomes in children who present with neurovascular malformations, who are treated with embolization, and who weigh less than 5 kg. The authors carried out a retrospective review of all consecutively treated children weighing less than 5 kg with neurovascular arteriovenous malformations (AVMs) at a single institution over a 10-year period. Fifty-two patients were included in the study. Thirty-eight had a vein of Galen aneurysmal malformation, 3 a pial AVM, 6 a pial arteriovenous fistula, and 5 a dural sinus malformation. The endovascular treatment goals were control of cardiac failure or hydrocephalus in cases of nonhemorrhagic malformations or to prevent new bleeding in cases of previous hemorrhage. A hemorrhagic complication occurred in 12 procedures and an ischemic complication in 2. Both complication types were correlated with the age of the infant (age cutoff at 3 months) (p = of 0.015 and 0.049, respectively). No correlation was found with the weight of the infant or the duration of the procedure. The embolization of AVMs in these patients prevented adverse cardiac effects, hydrovenous disorders, and rebleeding. The risk of major cerebral complications seems mainly correlated with age, with a threshold at 3 months. A multidisciplinary team involved in the treatment of these children may help to improve treatment success and management.

Letter by Gross and Du Regarding Article, “Intracranial Dural Arteriovenous Fistulae: Clinical Presentation and Management Strategies”

We read with interest the review by Miller and Gandhi1 on dural arteriovenous fistulae (dAVF). Limited by word count of a letter to the editor, we cannot provide more references/details; however, we have several inquiries. The authors state that dAVF may recruit pial arterial supply. Do they have a radiographic example of such a case? It would seem almost by definition that dAVF are supplied exclusively by meningeal arteries. Perhaps a pial arteriovenous fistula or arteriovenous malformation may be of mixed supply, but it is difficult to understand from a pathophysiologic basis that how a dAVF may recruit pial arterial supply. The authors credit an article in Neurosurgery from 1984 as one of the first to identify cortical venous drainage as the main risk factor for serious sequelae from dAVF. In their oft-cited 1972 article in Radiology , …

Pediatric cerebral arteriovenous malformations

Pial arteriovenous malformations (AVMs) of the brain are vascular abnormalities located in the subpial space that consist of direct communications from artery to vein via intervening nidus. AVMs are the most common intracranial pathology and frequent cause of intracranial hemorrhage in children. Moreover, the epidemiology and clinical findings particular to chil- dren with AVMs can be ascribed to the distinct angioarchitecture of pediatric AVMs. However, the natural history and treatment of pediatric AVMs is relatively unknown. In this article, we review current knowledge of pediatric pial AVMs.

Cerebral Arteriovenous Shunts in Children

Intracranial arteriovenous shunts (AVSs) in children can be divided into pial arteriovenous malformations, vein of Galen malformations, and arteriovenous fistulae (AVF). Dural AVF and dural sinus malformations are rare entities within this group. The relative immaturity of the anatomy and physiology of the neonatal and infant brain results in the inability of the hydrovenous system to compensate in the face of such disorders. Thus, the clinical presentation reflects this difference in the underlying anatomy, physiology, and disorder between children and adults. In this article, we briefly review the presentation, natural history and management of these entities.

E-018 Embolisation of Intracranial Arteriovenous Malformations and Dural Arteriovenous Fistulae Using a Novel Low Profile Distal Access Microcatheter: Initial experience

IntroductionSuccessful endovascular embolisation of intracranial arteriovenous malformations (AVMs) or arteriovenous fistulae (AVFs) requires distal navigation of very small microcatheters to the nidus or fistulous point of the malformation. Such small...