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Abstract
Background Vein of Galen malformations (VOGM) are congenital vascular malformations. Recent advances in endovascular interventions have drastically improved treatment and clinical outcomes in what was previously an overwhelmingly high-morbidity, high-mortality disease. The high flow shunt pathophysiology is the primary driver of systemic and neurologic sequelae. However, worsened neurologic disease can also result from changes in the malformation angioarchitecture, such as development of jugular bulb stenosis. In this setting, there may be both innumerable arterial feeders that are impractical to embolize, and also obstruction to transvenous access. A combined surgical and endovascular technique may offer an approach to a patient with worsening brain injury and occlusion of the jugular venous outflow. We present a contemporary application of direct puncture via transverse sinus for transvenous embolization of a VOGM.
Clinical Presentation We present two unique cases of complex VOGM malformations in patients who had previously undergone staged endovascular embolization for reduction of flow within the malformation. On follow up, in both cases there was development of severe sigmoid sinus and jugular bulb stenosis, markedly increasing intracranial venous pressures and causing marked clinical deterioration. The stenosis prevented traditional transvenous access and treatment. We describe a direct puncture transverse sinus access via a burr hole approach for endovascular transvenous embolization in both cases with successful radiographic (figures 1 and 2) and neurological outcomes.
Conclusion Originally described in the 1980s by Mickle and Quisling, surgical access for transtorcular embolization was a high risk, high morbidity approach. We have demonstrated successful application of the principles of the original technique, in conjunction with modern endovascular instrumentation, for successful burr hole craniotomy for direct puncture transvenous embolization of the median prosencephalic varix in previously trans arterially embolized VOGM. When there is occlusion or stenosis of distal venous channels limiting traditional endovascular access, this technique should be considered as a safe and effective alternative.
Disclosures S. Rangwala: None. P. See: None. E. Smith: None. D. Orbach: None.