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Childs Nerv Syst 2021 Jun 18. Epub 2021 Jun 18.

Graduated in Medicine, CESMAC University Center, Maceió, Alagoas, Brazil.

Introduction: Neural crest and mesoderm cell dysfunction of certain metameric level result in vascular malformations, i.e., cerebrofacial arteriovenous metameric syndrome (CAMS) and cerebrofacial venous metameric syndrome (CVMS). Read More

Brain Nerve 2021 Jun;73(6):685-696

Department of Neurology, Anjo Kosei Hospital.

Spinal dural arteriovenous fistulas (SDAVF) are rare and most commonly affect men aged >50 years. Patients with SDAVF develop an abnormal vascular dural shunt between the dural branch of a segmental artery and a subdural radicular vein that drains the perimedullary venous system, leading to venous hypertension and secondary congestive myelopathy. Most SDAVFs are located in the thoracolumbar region, and usually patients present with slowly progressive paraparesis and urinary disturbances. Read More

World Neurosurg 2021 Jun 2. Epub 2021 Jun 2.

Division of Neuroradiology, JDMI, University Health Network, Department of Medical Imaging, University of Toronto, Toronto, Ontario, CANADA; Division of Neurosurgery, University Health Network, Ontario, CANADA.

Background: Focal brain edema in unruptured brain arteriovenous malformations (AVMs) is rare and associated with venous outflow abnormalities and aneurysm growth. These patients have an increased rate of progressive neurological symptoms, as well as a potentially increased risk of haemorrhage. In this study, we aim to assess in further detail the relationship between perifocal edema and enhancement of the vessel wall in symptomatic patients with an unruptured brain AVM. Read More

Kyobu Geka 2021 Jun;74(6):477-480

Department of Thoracic Surgery, Japanese Red Cross Osaka Hospital, Osaka, Japan.

A 68-year-old man was referred to our hospital for further examination for chest abnormal shadow in the right lung field. He had a past history of subarachnoid hemorrhage. He was diagnosed with pulmonary arteriovenous malformation (PAVM) and recommended early treatment, but he didnot accept immediate surgical treatment. Read More

Arch Argent Pediatr 2021 06;119(3):152-161

Coordinación de Diagnóstico por Imágenes. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan, Ciudad Autónoma de Buenos Aires.

Introduction: High-flow vascular malformations of the brain are uncommon in pediatrics.

Objective: The objective of this study is to establish the differences among these pathologies and group them by age at onset, clinical manifestations, and angioarchitecture.

Population And Method: This was a retrospective and observational study. Read More

BMC Neurol 2021 May 20;21(1):204. Epub 2021 May 20.

Department of Neurosurgery, Timone University Hospital, APHM, 264 rue Saint-Pierre, 13005, Marseille, France.

Background: Since the nineteenth century, a great variety of crossed brainstem syndromes (CBS) have been described in the medical literature. A CBS typically combines ipsilateral cranial nerves deficits to contralateral long tracts involvement such as hemiparesis or hemianesthesia. Classical CBS seem in fact not to be so clear-cut entities with up to 20% of patients showing different or unnamed combinations of crossed symptoms. Read More

J Neurosurg 2021 May 7:1-10. Epub 2021 May 7.

2Division of Neuroradiology, Department of Medical Imaging, and.

Objective: The aim of this study was to 1) compare the safety and efficacy of acute targeted embolization of angiographic weak points in ruptured brain arteriovenous malformations (bAVMs) versus delayed treatment, and 2) explore the angioarchitectural changes that follow this intervention.

Methods: The authors conducted a retrospective analysis of a prospectively acquired database of ruptured bAVMs. Three hundred sixteen patients with ruptured bAVMs who presented to the hospital within 48 hours of ictus were included in the analysis. Read More

J Stroke Cerebrovasc Dis 2021 Jul 17;30(7):105798. Epub 2021 Apr 17.

Department of Neuroradiology, AUSL Romagna, M. Bufalini Hospital, Cesena, Italy. Electronic address:

A 71-year-old man, with a pial micro-arteriovenous malformation (pAVM) draining into the confluence of the vein of Trolard and the vein of Labbé was surgically removed, sparing these cortical veins. 4-months MR and angiographic controls showed a de novo dural arteriovenous fistula (dAVF) draining into the previously spared cortical veins. It was removed using intraoperative motor evoked potentials (MEP). Read More

J Clin Neurosci 2021 May 4;87:17-19. Epub 2021 Mar 4.

Royal Brisbane and Women Hospital, Queensland, Australia. Electronic address:

Arteriovenous malformations (AVMs) are a rare cause of cerebrovascular abnormality with incidence of about 1 in 100,000 people per year and point prevalence of about 0.2%. AVMs are associated with serious complications such as intracranial haemorrhage (2-4% a year, 16% and 29% at 10 and 20 years after diagnosis), seizures (10-30%), focal neurologic deficits, and headaches. Read More

No Shinkei Geka 2021 Mar;49(2):362-367

Department of Radiology, Faculty of Medicine, Oita University.

Dural arteriovenous fistulas(dAVFs), which are arteriovenous shunts between the dural/epidural artery and dural vein and/or dural venous sinus, can cause various symptoms, and the risk of aggressive symptoms such as cerebral hemorrhage and venous infarction mainly depends on venous drainage patterns in patients. Patients with dAVFs with cortical venous reflux have a high risk of aggressive symptoms due to cerebral venous congestion or varix rupture, and they often develop brain edema and/or hemorrhage. In some cases, patients with dAVFs may have CT and MRI findings similar to those of patients with brain tumors. Read More

No Shinkei Geka 2021 Mar;49(2):271-277

Department of Diagnostic Radiology, Shinrakuen Hospital.

Brain calcification can be either physiological or pathological. Pathological calcification occurs due to a wide spectrum of causes, including congenital disorders, infections, endocrine/metabolic diseases, cerebrovascular diseases, and neoplasms. The patient's age, localization of the calcification, and association with other imaging findings are useful for the correct diagnosis. Read More

BMJ Case Rep 2021 Mar 22;14(3). Epub 2021 Mar 22.

Department of Neurological Surgery, University Hospitals, Cleveland, Ohio, USA.

Carotid-cavernous fistulas (CCFs) are abnormal connections between arteries and the cavernous sinuses. Traditionally, the Barrow Classification system has been used to characterise these fistulas based on their arterial supply from the internal carotid artery (ICA), external carotid artery (ECA) or both. We present a unique case of a patient with a complex CCF with arterial feeders from dural branches of the ECA, ICA and vertebral artery (VA), which, to our knowledge, has not been reported in the literature. Read More

BMC Infect Dis 2021 Mar 19;21(1):277. Epub 2021 Mar 19.

Department of Infectious Diseases, University Hospital Puerta de Hierro Majadahonda, Madrid, Spain.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease associated with neurological complications, including cerebral abscesses (CA). They tend to be unique, supratentorial and lobar. While the surgical intervention is a rule of thumb when treating and diagnosing the etiology of these lesions, this is not always possible due to dangerous or inaccessible locations. Read More

J Stroke Cerebrovasc Dis 2021 May 25;30(5):105676. Epub 2021 Feb 25.

Department of Neurosurgery, University of Kentucky, Lexington, KY, USA; Department of Neurology, University of Kentucky, Lexington, KY, USA; Department of Radiology, University of Kentucky, Lexington, KY, USA; Department of Neuroscience, University of Kentucky, Lexington, KY, USA. Electronic address:

Introduction: Dural arteriovenous fistulae (DAVF) are intracranial vascular abnormalities encountered in neurosurgery practice. Treatment options are microsurgical disconnection, endovascular embolization and/or radiosurgery. Past studies have reported the efficacy, safety, and predictors of success of radiosurgery. Read More

BMJ Case Rep 2021 Feb 22;14(2). Epub 2021 Feb 22.

Department of Pediatrics, University of Minnesota Medical Center, Minneapolis, Minnesota, USA.

Acta Neurochir (Wien) 2021 04 10;163(4):1181-1189. Epub 2021 Feb 10.

Department of Neurosurgery, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Background: Pure arterial malformations (PAMs) are rare vascular lesions characterized by dilated, tortuous arterial loops without venous shunting. The natural history of PAMs remains unclear. We report the mid-term radiological and longer-term clinical outcomes of the largest series of patients with PAM. Read More

Am J Hematol 2021 05 11;96(5):640-642. Epub 2021 Feb 11.

Department of Internal Medicine, General Hospital of Šibenik-Knin County, Šibenik, Croatia.

JCI Insight 2021 Mar 8;6(5). Epub 2021 Mar 8.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver, National Institute of Child Health and Human Development, NIH, Bethesda, Maryland, USA.

Mutations in EPAS1, encoding hypoxia-inducible factor-2α (HIF-2α), were previously identified in a syndrome of multiple paragangliomas, somatostatinoma, and polycythemia. HIF-2α, when dimerized with HIF-1β, acts as an angiogenic transcription factor. Patients referred to the NIH for new, recurrent, and/or metastatic paraganglioma or pheochromocytoma were confirmed for EPAS1 gain-of-function mutation; imaging was evaluated for vascular malformations. Read More

Interv Neuroradiol 2021 Jan 11:1591019920988205. Epub 2021 Jan 11.

Department of Neurosurgery, Yale University School of Medicine, New Haven, USA.

Intracranial high-resolution vessel wall MRI (VW-MRI) is an imaging paradigm that is useful in site-of-rupture identification in patients presenting with spontaneous subarachnoid hemorrhage and multiple intracranial aneurysms. Only a handful of case reports describe its potential utility in the evaluation of more complex brain vascular malformations. We report for the first time three patients with ruptured cranial dural arteriovenous fistulas (dAVFs) that were evaluated with high-resolution VW-MRI. Read More

Clin Neurol Neurosurg 2021 02 18;201:106443. Epub 2020 Dec 18.

Department of Neurosurgery, Faculty of Medicine, Gazi University, Ankara, Turkey.

Background: Cerebral developmental venous anomalies (DVAs) are frequently diagnosed incidentally owing to the advances in neuroimaging. They are regarded as clinically insignificant due to their supposed quiescent existence which the authors aimed to contradict in this paper.

Aim: In the aim of constituting a better understanding of clinical presentation of DVAs and making an estimation regarding the probability of resulting in a hemorrhage, the authors presented their experiences with a case series of DVAs. Read More

BMJ Case Rep 2020 Dec 21;13(12). Epub 2020 Dec 21.

Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Post-traumatic direct carotid-cavernous fistulas may develop in patients with a closed head injury. The classical presentation is the Dandy's triad-chemosis, pulsatile proptosis and orbital bruit. Associated findings may include orbital pain, dilated episcleral corkscrew vessels, vision deficit and cranial nerve palsies. Read More

AJNR Am J Neuroradiol 2021 01 17;42(2):397-401. Epub 2020 Dec 17.

Imaging (M.M., R.S.P., V.S.W., F.G.M.), Cedars-Sinai Medical Center, Los Angeles, California.

Background And Purpose: Spinal CSF-venous fistulas are increasingly recognized as the cause of spontaneous intracranial hypotension. Here, we describe the challenges in the care of patients with CSF-venous fistulas who are morbidly or super obese.

Materials And Methods: A review was undertaken of all patients with spontaneous intracranial hypotension and a body mass index of >40 who underwent digital subtraction myelography in the lateral decubitus position to look for CSF-venous fistulas. Read More

J Magn Reson Imaging 2020 Dec 11. Epub 2020 Dec 11.

Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio, USA.

Arterial spin labeling (ASL) is a powerful noncontrast magnetic resonance imaging (MRI) technique that enables quantitative evaluation of brain perfusion. To optimize the clinical and research utilization of ASL, radiologists and physicists must understand the technical considerations and age-related variations in normal and disease states. We discuss advanced applications of ASL across the lifespan, with example cases from children and adults covering a wide variety of pathologies. Read More

Eur J Radiol 2021 Jan 1;134:109455. Epub 2020 Dec 1.

School of Medicine, National Yang Ming University, Taipei, Taiwan; Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan. Electronic address:

Purpose: To investigate the reproducibility of quantitative digital subtraction angiography (QDSA) measurements and their associations with brain arteriovenous malformation (BAVM) hemorrhage.

Methods: From 2011-2019, 37 patients with BAVMs who had undergone both diagnostic and stereotactic DSA were divided into hemorrhagic and nonhemorrhagic groups. QDSA analysis was performed on the 2 DSA exams. Read More

Indian J Radiol Imaging 2020 Jul-Sep;30(3):286-293. Epub 2020 Oct 15.

Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India.

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. Read More

Rozhl Chir 2020 ;99(10):467-471

Pulmonary arteriovenous malformation (PAVM) is formed by abnormal connections between pulmonary arteries and veins that bypass the pulmonary capillaries and transport deoxygenated blood through pulmonary veins to the left heart. This causes insufficient oxygenation of blood in the lungs. This condition remains symptomless for a long period of time. Read More

J Neurointerv Surg 2021 Mar 16;13(3):278-283. Epub 2020 Nov 16.

Diagnostic and Interventional Neuroradiology Department, Hôpital Foch, Suresnes, France.

Background: Torcular dural sinus malformations (tDSMs) with arteriovenous shunts are rare congenital intracranial vascular malformations that carry a high rate of neurologic impairment and death in the neonatal, infant and young pediatric population. Their impact on brain venous drainage, especially the deep venous system, is one of the key factors in the clinical prognosis and natural history of the disease. We describe our therapeutic strategy for tDSMs, disconnecting the reflux into the deep venous system by performing an endovascular straight sinus occlusion. Read More

North Clin Istanb 2020 11;7(5):508-511. Epub 2020 Aug 11.

Departmental Faculty of Medicine and Surgery, Unit of Diagnostic Imaging and Interventional Radiology, Università Campus Bio-Medico di Roma, Rome, Italy.

The Cerebro-Facial Venous Metameric Syndrome is characterized by ipsilateral venous/lymphatic anomalies involving simultaneously the brain and the face with a metameric distribution. This case report to describe a case of Cerebro-Facial Venous Metameric Syndrome presenting with a lump of the medial canthus. This was a case report a 24-year-old woman with a history of a mild headache, complained of a sporadic (at least once a month) serous leakage from the left eye and a small cutaneous protuberance in the left medial canthus, without focal neurological symptoms. Read More

Stroke 2020 12 2;51(12):e355-e358. Epub 2020 Nov 2.

Department of Radiology (S.E.O., J.H.), University of California, San Diego.

AJNR Am J Neuroradiol 2020 12 29;41(12):2358-2363. Epub 2020 Oct 29.

From the Department of Neuroradiology (L.G., O.N., G.B.), Sainte-Anne University Hospital, Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1266, Paris, France

Background And Purpose: Whether architectural characteristics of ruptured brain AVMs vary across the life span is unknown. We aimed to identify angioarchitectural features associated with brain AVMs ruptured early in life.

Materials And Methods: Patients with ruptured brain AVMs referred to 2 distinct academic centers between 2000 and 2018 were pooled and retrospectively analyzed. Read More