Pial Arteriovenous Fistula Research Articles

Outcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula

BackgroundCongenital cerebral arteriovenous fistulas (AVFs), including vein of Galen malformations, presenting in infancy carry variable mortality and morbidity. This study aimed to describe the outcome of neonates with cerebral AVFs who present with refractory cardiac failure. MethodsRetrospective chart review of neonates with refractory cardiac failure due to cerebral AVFs presenting before 28 days of age in a single-center neuro-intensive care nursery over a 12-year period (2008-2020) was conducted. ResultsSeventeen neonates were included. Twelve had a vein of Galen malformation, four a non-galenic pial AVF, and one a dural AVF. Seven neonates (41%) died without receiving an embolization procedure. The remaining ten were critically ill. Seven (70%) were mechanically ventilated and on nitric oxide, 5 (50%) were on pressors, and 6 (60%) had renal and/or hepatic dysfunction. Seven (70%) had pre-existing brain injury on imaging. The first embolization procedure occurred at a median age of 4 days (range: 0-8 d). Complications included intracranial hemorrhage in 8 of 10 (80%) and seizures in 5 of 8 (62%). Five (50%) neonates who underwent embolization died. Among the 5 neonates who survived, all have motor impairment. Four (80%) developed hydrocephalus requiring a ventriculoperitoneal shunt, and 2 (40%) developed epilepsy and are nonverbal. ConclusionIn this cohort of critically ill neonates with cerebral AVF, all seven who did not receive embolization and half of ten who were treated died. The five survivors all have neurodevelopmental impairment. This information may be helpful to parents and providers who make decisions regarding life-sustaining treatments for neonates with cerebral AVFs and refractory cardiac failure.

Spontenous regression of hemorrhagic multiple pial arteriovenous fistula: case report and review of the literature

Spontenous regression of hemorrhagic multiple pial arteriovenous fistula: case report and review of the literature

Transarterial Embolization of Intracranial Arteriovenous Fistulas with Large Venous Pouches in the Form of Venous Outlet Ectasia and Large Venous Varix or Aneurysm : Two Centers Experience.

ObjectiveThere are different types of cerebral vascular malformations. Pial arteriovenous fistulas (PAVFs) and dural arteriovenous fistulas (DAVFs) are two entities; they consist of one or more arterial connections to a single venous outlet without a true intervening nidus. The high turbulent flow of PAVFs and aggressive DAVFs with cortical venous reflux can result in venous outflow varix and aneurysmal dilatation. They pose a significant challenge to transvenous embolization (TVE), stereotactic radiosurgery, and surgical treatment. We aim to share our centers’ experience with the transarterial embolization (TAE) for arteriovenous fistulas (AVFs) with large venous pouches and to report the outcome. MethodsThe authors’ two institutions’ databases were retrospectively reviewed from February 2017 to February 2021. All patients with intracranial high flow PAVFs and aggressive DAVFs with venous outlet ectasia and large venous varix and were treated by TAE were included. ResultsFifteen patients harboring 11 DAVFs and four PAVFs met our inclusion criteria. All patients underwent TAE in 17 sessions. Complete angiographic obliteration was achieved after 14 sessions in 12 patients (80%). Four patients (25%) had residual after one TAE session. Technical failure was documented in one patient (6.7%). Fourteen patients (93.3%) had favorable functional outcome (modified Rankin score 0–2). ConclusionsTAE for high flow or aggressive intracranial AVFs is a safe and considerable treatment option, especially for those associated with large venous pouches that are challenging and relatively high-risk for TVE.

A neonatal case of pial arteriovenous fistulas and the review of literature

Pial arteriovenous fistulas (PAVFs) are extremely rare vascular malformations of the brain, and associated with greater morbidity and mortality in neonates and infants. We reported a neonatal case of giant multi-hole PAVFs presented with heart failure and local brain atrophy. The challenges associated with the complexity, outcome and therapeutic options of neonatal PAVFs was discussed. In addition, a review of the published literature on cases of PAVFs diagnosed at neonatal age, over the last two decades, was performed. Comprehensive prognostic evaluation and an optimal treatment strategy offered by a multidisciplinary team at specialized centers should be assured for each case affected.

Intradural spinal cord arteriovenous shunts in a personal series of 210 patients: novel classification with emphasis on anatomical disposition and angioarchitectonic distribution, related to spinal cord histogenetic units.

Few classifications of intradural spinal arteriovenous shunts (ID-SAVSs) have considered their anatomical localization in relation to their phenotype and angioarchitectonics. The authors propose another vision of ID-SAVSs allowing a reappraised classification based on analysis of the anatomical disposition, angioarchitecture, and histogenetic location of these vascular malformations. The radiological and clinical records of 210 patients with ID-SAVSs were retrospectively reviewed, considering their localization, vascular architectonics, and correlation with the 5 histogenetic units of the spinal cord. Among these, 183 files with complete data allowed precise analysis of the ID-SAVSs. Among these 183 files (162 and 21 cases with single and multiple lesions, respectively), different entities were identified: 13 pial macro arteriovenous fistulas (MAVFs), 92 pial micro arteriovenous fistulas (mAVFs), 33 superficial pial niduses, and 69 intramedullary niduses. Thirteen sulcal shunts (either fistulas or niduses) were considered subtypes of pial lesions. Among the 21 multiple cases, 11 were monomyelomeric while 10 were multimyelomeric. Pial lesions, either fistulas or niduses, were dominantly vascularized by pial arteries (anterior or posterior depending on the localization of the shunt) and occasionally (except for MAVFs) by transmedullary arteries. Pial niduses occasionally extended into the funiculus by recruiting intrinsic veins or by extension of the nidus itself inside the white matter. Intramedullary niduses were always vascularized by both centrifugal and centripetal feeders, respectively, from sulcal arteries (SAs) and pial arteries. Sulcal lesions are pial lesions located within the ventral median sulcus and vascularized by SAs and veins. Single or multiple ID-SAVSs can be part of various syndromes such as hereditary hemorrhagic telangiectasia, Parkes-Weber, RASA1, CLOVES, and spinal arteriovenous metameric syndromes. Histogenetic analyses revealed a specific distribution of each ID-SAVS in the 5 histogenetic units of the spinal cord: intramedullary niduses were found almost equally from cervical to thoracic units, while MAVFs and mAVFs were mostly found from thoracic to postcrural ones. Pial niduses showed intermediate features between intramedullary and fistulous lesions and were mostly distributed from brachial to crural segments. Precise analysis of the anatomical disposition of ID-SAVSs in relation to functional histogenetic units allows a better understanding of these lesions and improved therapeutic management.

Role of endovascular embolisation for curative treatment of intracranial non-Galenic pial arteriovenous fistula

Background and purposeThe safety and effectiveness of endovascular treatment for non-Galenic pial arteriovenous fistula (NGPAVF) is inadequately known. The aim of this study is to explore the role of endovascular...

De Novo Pial Arteriovenous Fistula Associated With Cerebral Infarction and Venous Hypertension: Report of 2 Cases Suggesting a “Second Hit Theory”

ABSTRACT BACKGROUND AND IMPORTANCE The etiology of de novo pial arteriovenous fistula (AVF) is unknown. We found 2 cases of de novo pial AVF, which appeared after cerebral infarction and which was associated with venous hypertension secondary to venous sinus thrombosis with a dural AVF (dAVF). Additional angiogenic stimuli (second hit) were considered as one of the mechanisms of de novo pial AVF. CLINICAL PRESENTATION A 63-yr-old male was admitted to our hospital due to an intraventricular hemorrhage. He had a history of cerebral infarction 2 yr before. Angiography demonstrated multiple dAVFs with bilateral occlusion of the distal transverse sinus associated with prominent retrograde cortical venous drainage. A pial AVF was found at the border of his previous cerebral infarction. Both lesions were successfully treated using endovascular technique. A second case involved a 47-yr-old female who was admitted to our hospital due to venous infarction also associated with sinus thrombosis. De novo pial AVF at the border of the venous infarction and dAVF at the transverse sigmoid junction were demonstrated on angiography 6 mo later. CONCLUSION We speculate that venous hypertension associated with additional angiogenic stimuli (second hit) due to brain ischemia and/or brain injury related to infarction caused de novo pial AVF in these 2 cases.

Endovascular Treatment of Intracranial Pial Arteriovenous Fistula in Paediatric Patient

Pial arteriovenous fistula (AVFs) are a rare, although clinically significant, vascular anomaly affecting the pediatric population, consisting of a high-flow connection between one or more pial arteries and the venous. In children, the clinical presentation varies according to age, can present with high output cardiac failure, macrocrania, neurodevelopmental delay, seizures, venous infarctions due to venous stenosis or hemorrhage secondary to venous thrombosis. The treatment, is a multimodal approach, most commonly endovascular, is effective in the obliteration of AVF. A case is presented of a previously healthy infant boy with neurological deficit arising from pAVF of the middle and posterior cerebral artery. The pAVF was successfully treated with endovascular occlusion.

Letter to the Editor Regarding: “Spontaneous Resolution of Dural and Pial Arteriovenous Fistulae Arising After Superficial Temporal Artery to Middle Cerebral Artery Bypass for Moyamoya Disease”

Letter to the Editor Regarding: “Spontaneous Resolution of Dural and Pial Arteriovenous Fistulae Arising After Superficial Temporal Artery to Middle Cerebral Artery Bypass for Moyamoya Disease”

In Reply to the Letter to the Editor Regarding “Spontaneous Resolution of Dural and Pial Arteriovenous Fistulae Arising After Superficial Temporal Artery to Middle Cerebral Artery Bypass for Moyamoya Disease”

In Reply to the Letter to the Editor Regarding “Spontaneous Resolution of Dural and Pial Arteriovenous Fistulae Arising After Superficial Temporal Artery to Middle Cerebral Artery Bypass for Moyamoya Disease”

Pial arteriovenous fistula in adult: a case report and literature review

Pial arteriovenous fistula in adult: a case report and literature review

Acquired Pial and Dural Arteriovenous Fistulae Draining into the Sylvian Veins after Craniotomy for Hematoma Evacuation: A Case Report

Acquired Pial and Dural Arteriovenous Fistulae Draining into the Sylvian Veins after Craniotomy for Hematoma Evacuation: A Case Report

Surgical management of a large pediatric temporal pial arteriovenous fistula: Case report

Surgical management of a large pediatric temporal pial arteriovenous fistula: Case report

外頚動脈系からも流入が認められた開頭術後pial AVFの1例

外頚動脈系からも流入が認められた開頭術後pial AVFの1例

Chapter 11 - Cerebral arteriovenous fistulae

Dural arteriovenous fistulae (dAVFs) are diverse, complex lesions that share the common feature of arteriovenous shunting without an intervening nidus. In this chapter, the ensuing discussion is organized by dAVF location, followed by further consideration of less common, distinct types of dAVFs-carotid cavernous fistulae, pial arteriovenous fistulae, and vein of Galen malformations. For each lesion type, epidemiology, clinical presentation, imaging findings, classification considerations, and treatment options are discussed.

Microsurgical Resection of a Spinal Cord Pial Arteriovenous Fistula: 2-Dimensional Operative Video

We present a patient who was diagnosed 20 yr prior to current presentation with a spinal arteriovenous malformation. This patient had a 10-yr history of worsening back pain (and underwent lumbar fusion), urinary dysfunction leading to 3-yr dependence on intermittent catheterization, lower extremity paresthesias and pain, and progressive weakness with multiple falls, leading to walker then wheelchair dependence for mobility. Magnetic resonance studies showed extensive thoracic cord expansion and edema with enlarged spinal cord surface veins and flow voids extending from spinal levels T6 to the conus medullaris. Partial embolization at an outside institution elicited transient symptom improvement. Repeated spinal angiogram demonstrated persistent T10 pial arteriovenous fistula (AVF) supplied by the posterior spinal artery arising from the right T11 segmental artery as well as by the anterior spinal artery from the left T10 segmental artery. Because additional embolization carried significant risk, we planned open surgery with fistula resection. Informed consent for the surgery and video recording was obtained. The patient was placed in the prone position, and a radial artery access was obtained for intraoperative angiogram. Following a posterior T9-T11 laminectomy and dural opening, a pial dissection was performed to expose the AVF. Intraoperative indocyanine green angiography was used to assist in identifying the feeders and major drainage of the AVF. Post-AVF resection, a formal intraoperative radial access spinal angiogram demonstrated complete resection of the lesion with no residual shunt or early venous drainage. The patient improved significantly and, on last follow-up, is ambulating without any assistive devices.

Galenic Pial Arteriovenous Fistulas in Adults

BackgroundVein of Galen aneurysmal malformations (VOGMs) are pial arteriovenous fistulas possessing Galenic venous drainage most commonly presenting during the neonatal period and infancy, with initial discovery during adulthood quite rare. Objectives and methodsWe conducted a literature survey of the PubMed database in order to identify Galenic pial arteriovenous fistulas (GPAVFs) with major manifestation or initial presentation during adulthood. Inclusionary criteria included pial AVFs with Galenic drainage with major manifestation or initial presentation at, or older than, 18 years. Exclusionary criteria included exclusive pediatric onset of symptomatology attributable to GPAVFs without a new onset major presentation during adulthood, exclusive or major dural arterial supply, arteriovenous malformations with Galenic drainage, developmental venous anomalies with Galenic drainage, isolated varices or anomalies of the vein of Galen, and any lesions with uncertainty regarding true GPAVF nature. ResultsOur search generated 1589 articles. Excluding duplicates, 26 cases met criteria for evaluation. Mean age was 34.1 +/− 2.53 years. Clinical presentations of GPAVFs among adults included headache, intracranial hemorrhage, seizures, and focal neurologic deficits. Management strategies included observation (n = 5), emergent ventriculostomy or Torkildsen shunt (n = 3), cerebrospinal fluid diversion via ventriculoperitoneal shunting (n = 4), microsurgical obliteration or thrombectomy (n = 4), transarterial and/or transvenous embolotherapeutic obliteration (n = 7), and concurrent embolotherapy and radiosurgical irradiation (n = 1). ConclusionsGPAVFs in adults often present with symptomatology of mild severity and may be effectively managed conservatively, though occasionally present catastrophically or may be treated via cerebrospinal fluid diversion, microsurgical obliteration, or endovascular embolization. Severity sufficient to require emergent intervention portended a poor outcome.

Pial arteriovenous fistula: A clinical and neuro-interventional experience of outcomes in a rare entity

Purpose:Pial arteriovenous fistulae (PAVF) are rare intracranial vascular malformations, predominantly seen in children and distinct from arteriovenous malformations and dural arteriovenous fistulae. PAVF often leads to high morbidity and mortality. The aim of our study was to describe the clinical features and endovascular management of PAVF at various intracranial locations; to analyze the use of liquid embolic agents and coils alone or in combination in the treatment of PAVF and to analyze the outcome of embolization.Materials and Methods:Retrospective review of diagnostic angiography and neurointerventional database of our institution identified a cohort of 15 patients with non-galenic PAVF from 2008 to 2014 out of 6750 patients. Fourteen patients were treated endovascularly with coils and liquid embolic materials in combination or alone. Patients were followed up for evaluation of prognosis.Results:Age of the patients ranged from 3 to 37 years. Most patients were male and most common presentation was headache followed by seizure. Most common location of fistula was frontal lobe. The most common type was single artery single hole fistula with venous varix. Satisfactory obliteration was seen in all cases. One patient developed intraparenchymal hematoma on the first post procedural day and outcome was poor.Conclusions:PAVF are rare intracranial vascular malformations which can effectively be managed endovascularly with liquid embolic, coils alone, or in combination. Complete occlusion of the fistula can be achieved in most cases in a single sitting with a reasonable morbidity related to the procedure, compared with the natural history of this disease.

Typical subdural contrast effusion secondary to endovascular treatment of a pediatric pial arteriovenous fistula.

Subdural contrast effusion secondary to endovascular treatment is exceptionally rare and might be mistaken as subdural hematoma because of similar hyperattenuation on computer tomography. The authors present the case of a 13-month-old girl with a history of increased head circumference and developmental retardation. Cerebral digital subtraction angiography showed a high-flow pial arteriovenous fistula fed by multiple arteries on the right cerebellar surface, with occlusion of the right sigmoid sinus and severe stenosis of the left sigmoid sinus. Staged endovascular treatments were performed to eliminate the fistula. Follow-up head computer tomography scans performed 3 h after both procedures demonstrated typical high-density subdural effusion with computer tomography attenuation value similar to hemorrhage. These effusions did not aggravate the condition and disappeared spontaneously 32 h after the first treatment and 29 h after the second, respectively.

Four-dimensional digital subtraction angiography for exploration of spinal cord vascular malformations: preliminary experience

BackgroundThe precise understanding of the angioarchitecture of spinal vascular malformations (SVMs) is often difficult to reach with conventional digital subtraction angiography (DSA). The purpose of our study was to evaluate...