Background and purpose 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.
- Arteriovenous Malformation
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Contributors HA: acquisition, analysis and interpretation of data; drafting the work and revising it critically for important intellectual content. LJDS: conception and design of the work, analysis and interpretation of data; drafting the work and revising it critically for important intellectual content. SBG: design of the work and revising it critically for important intellectual content. AG: design of the work and revising it critically for important intellectual content. NKM: conception of the work, analysis and interpretation of data; drafting the work and revising it critically for important intellectual content. All authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Competing interests None declared.
Ethics approval The study was approved by the Ethics Committee for Post Graduate Research, All India Institute of Medical Sciences.
Provenance and peer review Not commissioned; externally peer reviewed.