Factors Contributing to Major Neurological Complications From Vein of Galen Malformation Embolization

Abstract

IMPORTANCEMajor neurological complications from the embolization of vein of Galen malformations (VOGMs) are poorly understood. We provide a detailed analysis of contributors to periprocedural neurological complications and lessons learned.OBJECTIVE To assess the rate of major periprocedural neurological complications following VOGM embolization with major procedural and strategic contributors. DESIGN, SETTING, AND PARTICIPANTSThis retrospective cohort study was conducted at a quarternary referral pediatric hospital (Hospital for Sick Children; Toronto, Ontario, Canada) from January 1999 to December 2018 with a mean clinical follow-up of 44.7 months; all children with VOGM diagnosed and/or treated were eligible (n = 48). Thirty-three patients who underwent endovascular treatment were included.INTERVENTIONS Endovascular staged transarterial embolization performed in 33 patients over 91 sessions. MAIN OUTCOMES AND MEASURESThe primary outcome was the rate of periprocedural neurological complications (occurring within 1 week of embolization). The secondary outcomes were mortality, long-term neurological outcomes, and contributing anatomical and management factors to neurological complications. RESULTSOf 33 patients who underwent embolization (31 boys [64.6%]; 17 girls [35.4%]; median age at first embolization, 4 months [range, 0-29 months]), 10 patients (30.3%) developed major periprocedural neurological complications. Five of these patients died. Univariate logistic regression analyses identified internal cerebral vein drainage to the main venous sac of the VOGM and use of a microcatheter with a distal outer diameter of more than 2.0F as significant predictors of poor neurological outcomes. Lessons learned from our experience include the need to assess the internal cerebral vein drainage pattern on preprocedural magnetic resonance venography, avoidance of excessive embolization into the venous sac, treatment of more distal fistulae before proximal fistulae to avoid a sump effect, and preferably use of smaller (<2.0F outer diameter) microcatheters in neonatal embolization procedures. CONCLUSIONS AND RELEVANCEIn this cohort, 10 patients with VOGM treated with embolization (30.3%) experienced major periprocedural neurological complications, half of whom died. While these outcomes are superior to historic conservative and surgical treatment results, ongoing improvements in treatment and pretreatment diagnostic approaches are needed. Awareness of the lessons learned from our experience can help to avoid similar complications in the future for this vulnerable population.

Scepter mini assisted angiographic cure of a Vein of Galen Malformation with n-butyl cyanoacrylate

“…Endovascular embolization of VOGM is the standard treatment methodology; however, up to one third of patients will suffer major neurologic morbidity or mortality. [2][3][4][5] A recent metanalysis demonstrated that 19% of cases may have technical complications with complete embolization occurring in only 56% of cases. 2 Even with modern endovascular devices, technical complications such as arterial perforation and off target embolization continue to occur.…”

“…2 Even with modern endovascular devices, technical complications such as arterial perforation and off target embolization continue to occur. 3,6,7 This case describes the technical safety of a novel dual lumen balloon microcatheter resulting in the angiographic cure of a VOGM. In the setting of a high flow shunt such as these lesions, a small dual lumen balloon with the ability to tolerate liquid embolic is extremely advantageous.…”

“…Lasjaunias argued for staged treatment and reduction of the shunt, with the goal of a normally developing child. 1,3,8 Immediate cure can lead to either rapid thrombosis of the venous system and venous infarction, or subsequent hemorrhage if an arterial pedicle remains patent in the setting of total venous occlusion. 1,3 In this case, a single injection of n-BCA did lead to angiographic cure.…”

“…1,3,8 Immediate cure can lead to either rapid thrombosis of the venous system and venous infarction, or subsequent hemorrhage if an arterial pedicle remains patent in the setting of total venous occlusion. 1,3 In this case, a single injection of n-BCA did lead to angiographic cure. This is not routinely recommended, but up until this point it would have been very challenging to cure a lesion like this in a single setting via a single pedicle.…”

Vein of Galen Aneurysmal Malformations in Pediatric Patients Submitted to Endovascular Treatment: Report of 3 Cases

“…11 However, some authors concluded that the presence of normal deep venous drainage into the venous sac of the VGAM, excessive embolization of the venous outflow, treatment of more proximal fistulae before treating distal fistulae, and the use of larger microcatheters in neonates can contribute to an unexpected outcome. 12 It is important to consider that despite the technological evolution leading to early diagnosis and more favorable treatments, the prognosis of these patients remains associated with their clinical conditions at birth. 10…”