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Zh Vopr Neirokhir Im N N Burdenko 2021 ;85(3):104-113

Burdenko National Medical Research Center of Neurosurgery, Moscow, Russia.

Cavernous malformations (CMs) of central nervous system are vascular malformations usually localized in the brain and rarely in the spinal cord. To date, these malformations are well studied. However, some problems of the management of this pathology are still unresolved. 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

No Shinkei Geka 2021 May;49(3):665-676

Department of Neurosurgery, Tohoku University.

In this paper, we outlined the diagnostic and treatment strategies for spinal arteriovenous malformations, focusing on dural and perimedullary arteriovenous fistula(AVF). In many patients with spinal arteriovenous malformations, the symptoms are non-specific. Therefore, we consider it is critical to detect the signal flow voids in the enlarged spinal veins using MRI. Read More

Neuroradiol J 2021 Jun 4:19714009211021776. Epub 2021 Jun 4.

Brain and Spinal Cord Injury Research Center, Tehran University of Medical Sciences, Iran.

Background: Sylvian fissure arteriovenous malformations are rare but important vascular lesions, whose importance lies in both haemorrhage and seizure risk. Although surgery has been recommended as a treatment, the overall estimation of success has not been reported to render outcomes easier to understand in comparison to other treatment modalities.

Objectives: This systematic review of the literature and two cases aims to illustrate the results of surgery as a contemporary treatment option and present a novel anatomical classification system for Sylvian fissure arteriovenous malformations. Read More

World Neurosurg 2021 May 29;152:6-18. Epub 2021 May 29.

International Center for Neurosurgery, Henan Provincial People's Hospital, Zhengzhou, China.

Objective: We sought to examine the conservative treatment of symptomatic spinal cavernomas and evaluate the efficacy and safety of surgical management of spinal cord cavernous malformations.

Methods: This meta-analysis included articles comparing outcomes of conservative treatment and surgical management of spinal cavernomas, published in the full-text form (from 2000 to June 31, 2020). Collected variables included first author name, country, covered study period, publication year, the total number of patients and at follow-up, bleeding, motor weakness, pain, bladder and/or bowel dysfunction neurologic improvement or deterioration after discharge, and the need for reintervention after subtotal surgical resection or hemorrhage. Read More

BMC Pediatr 2021 05 19;21(1):242. Epub 2021 May 19.

Department of Anesthesiology, Rutgers New Jersey Medical School, Newark, NJ, USA.

Background: Spinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations.

Case Presentation: A 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. Read More

J Surg Case Rep 2021 Apr 19;2021(4):rjab122. Epub 2021 Apr 19.

College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

CLOVES syndrome is a condition characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies. The association of cervical arteriovenous fistula with CLOVES syndrome is extremely rare. To the best of our knowledge, only two cases were reported in the literature. Read More

Anaesth Rep 2021 Jan-Jun;9(1):62-66. Epub 2021 Apr 17.

North West School of Anaesthesia Health Education North West Manchester UK.

Klippel-Trénaunay syndrome is a rare congenital disorder affecting the vascular and lymphatic systems. The clinical presentation can vary widely, but the syndrome is broadly characterised by capillary, venous and lymphatic malformations as well as limb hypertrophy. We present the case of a 35-year-old parturient who underwent an emergency caesarean section for suspected fetal distress, and describe the anaesthetic management during the peripartum period. Read More

Neurol Clin 2021 05;39(2):489-512

Division of Neurology, Lahey Hospital and Medical Center, Burlington, MA 01805, USA; Department of Neurology, Tufts University School of Medicine, Boston, MA 02111, USA. Electronic address:

Vascular and infectious causes are rare but important causes of spinal cord injury. High suspicion for these processes is necessary, as symptoms may progress over hours to days, resulting in delayed presentation and diagnosis and worse outcomes. History and clinical examination findings can assist with localization of the affected vascular territory and spinal level, which will assist with focusing spinal imaging. Read More

Neurology 2021 05 15;96(20):958-959. Epub 2021 Apr 15.

From the Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.

Cells 2021 Mar 22;10(3). Epub 2021 Mar 22.

Department of Medical, Surgical and Neurological Sciences, University of Siena, 53100 Siena, Italy.

Cerebral cavernous malformations (CCMs) are vascular lesions that affect predominantly microvasculature in the brain and spinal cord. CCM can occur either in sporadic or familial form, characterized by autosomal dominant inheritance and development of multiple lesions throughout the patient's life. Three genes associated with CCM are known: (krev interaction trapped 1), (encoding a protein named malcavernin), and (programmed cell death 10). Read More

J Neurosurg Spine 2021 Apr 2:1-11. Epub 2021 Apr 2.

1Department of Diagnostic and Interventional Neuroradiology, Hôpital Foch, Suresnes, Hauts-de-Seine, France.

Objective: 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.

Methods: 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. Read More

Rev Neurol (Paris) 2021 May 26;177(5):469-476. Epub 2021 Mar 26.

Diagnostic and interventional neuroradiology, hôpital FOCH, Constitutive Reference Center for Rare Diseases - Spinal Cord Arteriovenous Malformations, Avance Network, "FAVA Multi" Sector, 40, rue Worth, 92151 Suresnes, France.

Spinal Cord Arterio-Venous shunts (SCAVSs) are a rare disease. The aim of this paper is to describe how we classify and consider management of SCAVSs in relation to the location of the shunt focusing mainly on intradural SCAVSs. The anatomical features of the SCAVSs together with data provided by MRI and CT scans allow identification of four types of SCAVSs: paraspinal, epidural, dural and intradural ones. Read More

Neurol India 2021 Jan-Feb;69(1):204-205

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

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

Department of General Internal Medicine, Toyota Regional Medical Center, Toyota, Aichi, Japan.

We present the case of a 47-year-old woman with neurofibromatosis type 1 (NF1) with subarachnoid haemorrhage (SAH) from the left vertebral arteriovenous fistula, along with a review of previous cases. Our patient had a family history of NF1 and presented to the emergency department with a sudden-onset severe headache and neck pain. CT scan showed SAH. Read More

BJR Case Rep 2021 Feb 23;7(1):20200145. Epub 2020 Oct 23.

Department of Radiology, Kasr Al Ainy Hospital, Cairo, Egypt.

Cobb syndrome is a rare vascular disorder characterized by vascular skin lesions distributed in a dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite (metamere). We present a case of a 25-year-old man who presented with a history of right upper limb paresthesia followed by bilateral progressive upper and lower limb weakness and heaviness. Physical examination showed large cutaneous port wine stains on the right side of the chest, the nape, and along the whole right upper limb in a dermatomal distribution, with no corresponding limb hypertrophy or asymmetry. Read More

CNS Neurol Disord Drug Targets 2021 Feb 17. Epub 2021 Feb 17.

Chair of Clinical Pharmacology, Chair of Dermatology and Venereology, Russian National Research Medical University Named After Pirogov N.I., Moscow. Russian Federation.

Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. Read More

Radiologe 2021 Mar 18;61(3):258-262. Epub 2021 Feb 18.

Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, Kirrberger Straße, 66424, Homburg-Saar, Deutschland.

Spinal vascular malformations include various entities, including spinal dural arteriovenous (AV) fistulas, acquired malformations and cavernous hemangiomas, and spinal arteriovenous malformations. AV fistula refers to a spinal cord vascular malformation in which there is a short-circuit connection between a dural artery and a perimedullary vein superficial to the myelon. The condition is relatively rare, initial clinical symptoms are often misinterpreted, and a definitive diagnosis is often delayed by 12 months or more. Read More

BMJ Case Rep 2021 Feb 5;14(2). Epub 2021 Feb 5.

Neurosurgery, Medical College of Wisconsin, Wauwatosa, Wisconsin, USA

Spinal dural arteriovenous fistula (SDAVF) is a rare pathological communication between arterial and venous vessels within the spinal dural sheath. Clinical presentation includes progressive spinal cord symptoms including gait difficulty, sensory disturbances, changes in bowel or bladder function, and sexual dysfunction. These fistulas are most often present in the thoracolumbar region. Read More

Ann Clin Transl Neurol 2021 02 12;8(2):504-522. Epub 2021 Jan 12.

Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

CNS vascular malformation is an umbrella term that encompasses a wide variety of pathologies, with a wide range of therapeutic and diagnostic importance. This range spans lesions with a risk of devastating neurological compromise to lesions with a slow, static or benign course. Advances in neurovascular imaging along with increased utilization of these advances, have resulted in more frequent identification of these lesions. Read More

Neurosurgery 2021 02;88(3):666-673

Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota; Department of Radiology, Mayo Clinic, Rochester, Minnesota.

Background: Spinal epidural arteriovenous fistulas (SEDAVFs) are an increasingly recognized form of spinal vascular malformation and are distinct from spinal dural arteriovenous fistulas (SDAVFs). Differentiating between these 2 entities is important as operative strategies often differ based on angioarchitecture.

Objective: To compare demographic, clinical, anatomic, and imaging findings of SDAVFs and SEDAVFs. Read More

Oper Neurosurg (Hagerstown) 2021 03;20(4):E317-E321

Department of Neurological Surgery, Rutgers-Robert Wood Johnson Medical School, New Brunswick, New Jersey.

Background And Importance: Intramedullary spinal cord cavernous malformations represent 5% to 12% of spinal vascular disease. Most patients present with acute or progressive neurological symptoms, including motor weakness or sensory loss. Surgical resection is the only definitive management and is recommended for symptomatic lesions that are surgically accessible. Read More

Diving Hyperb Med 2020 Dec;50(4):325-331

Neurology Department, Fiona Stanley Hospital, Murdoch, Australia.

Introduction: Spinal cord infarction (SCI) is a potentially devastating disorder presenting with an acute anterior spinal artery syndrome, accounting for an estimated 1% of stroke presentations. Aetiologies include aortic surgical complications, systemic hypotension, fibrocartilaginous embolism and vascular malformations. Diagnosis is clinical combined with restriction on diffusion-weighted magnetic resonance imaging (MRI). Read More

Cureus 2020 Nov 5;12(11):e11351. Epub 2020 Nov 5.

Neurology, Walter Reed National Military Medical Center, Bethesda, USA.

Duplicated origin of the vertebral artery (VA) is an extremely rare normal anatomic variant. While most often considered non-pathological, duplicated origin carries an increased risk of dissection. An association with vascular pathologies such as aneurysms, arteriovenous malformations, and AV fistulas has been suggested. Read More

Vet Pathol 2020 Dec 11:300985820980707. Epub 2020 Dec 11.

Cornell University, Ithaca, NY, USA.

In disease, blood vessel proliferation has many salient roles including in inflammation, when granulation tissue fills superficial defects, or in the recanalization of an occluded blood vessel. Sometimes angiogenesis goes awry-granulation can be exuberant, and plexiform proliferation of vascular components can contribute to pulmonary hypertension. This review focuses on the diverse manifestations of pathologic vascular overgrowth that occur in the brain, spinal cord, and meninges of animals from birth until old age. Read More

Neuroimage 2021 02 20;226:117567. Epub 2020 Nov 20.

INRIA, Université Côte d'Azur, France; INRIA, CEA, Université Paris-Saclay, France.

We aimed to link macro- and microstructure measures of brain white matter obtained from diffusion MRI with effective connectivity measures based on a propagation of cortico-cortical evoked potentials induced with intrasurgical direct electrical stimulation. For this, we compared streamline lengths and log-transformed ratios of streamlines computed from presurgical diffusion-weighted images, and the delays and amplitudes of N1 peaks recorded intrasurgically with electrocorticography electrodes in a pilot study of 9 brain tumor patients. Our results showed positive correlation between these two modalities in the vicinity of the stimulation sites (Pearson coefficient 0. Read More

Childs Nerv Syst 2021 02 30;37(2):645-648. Epub 2020 Oct 30.

Neurosurgery & Interventional Neuroradiology Department, Hygeia Hospital, Erythrou Stavrou 4, Marousi, 15123, Athens, Greece.

Perimedullary arteriovenous fistulae (PMAVFs) (also called type IV spinal cord arteriovenous malformations) are rare lesions. They are located in the subarachnoid space or just under the pia. The shunt occurs between the anterior spinal artery (ASA) and/or posterior spinal artery (PSA) and a network of perimedullary veins. Read More

J Clin Neurosci 2020 Oct 26;80:195-202. Epub 2020 Aug 26.

Department of Neurosurgery, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka 545-8585, Japan. Electronic address:

The objective of this retrospective study is to more fully understand the optimal strategy to manage spinal arteriovenous (AV) shunts. This study included a cohort of 35 patients with a diagnosis of spinal AV shunts who were treated over the past 10 years at a single institute. Angiographic diagnosis of intramedullary AV malformations (IM-AVM), perimedullary AV fistulas (PM-AVF), dural AV fistulas (D-AVF), or epidural AV fistulas (ED-AVF) was carefully made, and the microsurgical or endovascular strategy for them was determined at the interdisciplinary meeting consisting of neurospinal surgeons and endovascular specialists. Read More

World Neurosurg 2021 02 17;146:14. Epub 2020 Oct 17.

Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania, USA. Electronic address:

This case video demonstrates a microsurgical technique for the clipping and obliteration of a Cognard V tentorial dural fistula (Video 1). The patient was a 49-year-old male who presented with progressive upper and lower extremity weakness over 12 months, with associated cervical spinal cord edema. The patient was initially misdiagnosed with transverse myelitis; however, abnormal flow voids on magnetic resonance imaging led to a cerebral angiogram being performed. Read More

Neurosurgery 2021 02;88(3):515-522

Department of Radiology, Mayo Clinic, Rochester, Minnesota.

Background: A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS.

Objective: To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population. Read More