title: Vein of Galen aneurysmal malformation-clinical and angiographic spectrum with management perspective: an institutional experience.
abstract: OBJECTIVE
  Vein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically heterogeneous set of VGAM patients.
  
  
  METHODS
  We retrospectively analyzed cases of VGAM from our departmental archive collected between 1988 and January 2015. Demographic, clinical, therapeutic, and follow-up details were obtained for each patient from the available records.
  
  
  RESULTS
  We identified 36 patients with VGAM including 6 neonates, 18 infants, 7 children aged 2-10 years, and 5 adults. Macrocrania was the commonest presenting feature. Type of fistulae was mural in 14 and choroidal in 18 patients while 4 had a thrombosed sac at presentation. In 3 cases the dilated venous sac had connection with the deep venous system. Bilateral jugular atresia and stenosis were seen in 9 and 6 patients, respectively. Giant venous sac (>4 cm) was significantly correlated with mural type (p=0.0001). Dural arterial recruitment was seen in 4 patients including 3 adults. Among the 23 patients treated by endovascular means, 14 had a good outcome, 5 had a poor outcome, and 4 died. A significant correlation was noted between jugular atresia and poor outcome (p=0.003).
  
  
  CONCLUSIONS
  We encountered a wide range of demographic, clinical, and angiographic features in VGAM. Mural type malformations were associated with giant venous sacs. Good outcome after embolization was seen in selected neonates and in most of the infants, children, and adults. Jugular atresia was significantly associated with poor outcome.
authors.affiliation: Department of Neuroimaging and Interventional Neuroradiology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Himanshu
authors.initials: H
authors.lastname: Agarwal
authors.affiliation: Department of Neuroimaging and Interventional Neuroradiology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Leve Joseph Devarajan
authors.identifier: http://orcid.org/0000-0002-4777-2804
authors.initials: LJ
authors.lastname: Sebastian
authors.affiliation: Department of Neuroimaging and Interventional Neuroradiology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Shailesh B
authors.initials: SB
authors.lastname: Gaikwad
authors.affiliation: Department of Neuroimaging and Interventional Neuroradiology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Ajay
authors.initials: A
authors.lastname: Garg
authors.affiliation: Department of Neuroimaging and Interventional Neuroradiology, CN Centre, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Nalini K
authors.initials: NK
authors.lastname: Mishra
mesh_terms: D000293:Adolescent; D000328:Adult; D019412:Anatomy, Cross-Sectional; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D004621:Embolization, Therapeutic; D057510:Endovascular Procedures / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D054080:Vein of Galen Malformations / Q000000981:diagnostic imaging* / Q000601:surgery*; D017287:Ventriculoperitoneal Shunt; D055815:Young Adult
keywords: Arteriovenous Malformation; Congenital; Intervention; Pediatrics