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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.
Authors: Kirill Orlov; Anton Gorbatykh; Vadim Berestov; Timur Shayakhmetov; Dmitry Kislitsin; Pavel Seleznev; Nikolay Strelnikov Journal: Childs Nerv Syst Date: 2017-07-08 Impact factor: 1.475