Journal of the neurological sciences

1878-58834162020Sep15Journal of the neurological sciencesJ Neurol SciGalenic Pial Arteriovenous Fistulas in Adults.11701411701410.1016/j.jns.2020.117014S0022-510X(20)30351-8Vein 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.We 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.Our 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).GPAVFs 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.Copyright © 2020 Elsevier B.V. All rights reserved.GhaliMichael George ZakiMGZDepartment of Neurological Surgery Karolinska Institutet, Nobels väg 6, Solna and Alfred Nobels Allé 8, Huddinge SE-171 77, Stockholm, Sweden; Centrum för traumaforskning Karolinska Institutet, Nobels väg 6, Solna and Alfred Nobels Allé 8, Huddinge SE-171 77, Stockholm, Sweden. Electronic address: michaelgeorgezakighali@karolinskainstitute.org.ArboreliusUlf PUPDepartment of Neurological Surgery Karolinska Institutet, Nobels väg 6, Solna and Alfred Nobels Allé 8, Huddinge SE-171 77, Stockholm, Sweden; Centrum för traumaforskning Karolinska Institutet, Nobels väg 6, Solna and Alfred Nobels Allé 8, Huddinge SE-171 77, Stockholm, Sweden.VeznedarogluErolEDirector of the Drexel Neurosciences Institute, Robert A. Groff Chairman in Neurosurgery, Global Neurosciences Institute, Mercerville, NJ 08619, United States of America.SpetzlerRobert FRFDepartment of Neurological Surgery, Barrow Neurological Institute, 350 W Thomas Rd, Phoenix, AZ 85013, Phoenix, AZ 85013, United States of America.YaşargilM GaziMGDepartment of Neurosurgery, Department of Neurophysiology, University Hospital Zurich, Rämistrasse 100, 8091 Zurich, Switzerland.MarchenkoVitaliyVInstitut Fiziologii imeni O O Bogomolca Nacional'na akademia nauk Ukraini, Bogomoletz str. 4, Kiev 01024, Ukraine.engJournal Article20200705