Abstract

Objective: Congenital intracranial pial arteriovenous fistula (PAVF) is a rare cerebral vascular pathology characterized by a direct shunt between one or more pial feeding arteries and a cortical draining vein. Transarterial endovascular embolization (TAE) is widely considered first line therapy. Curative TAE may not be achievable in the multihole variant due to the potential to harbor innumerable small feeding arteries. Transvenous embolization (TVE) may be considered to target the final common outlet of the lesion. Here, we present a series of four patients with complex multi-hole congenital PAVF treated with staged TAE followed by TVE.Methods: A retrospective review was conducted on patients who underwent treatment for congenital, multi-hole PAVFs treated by a combined TAE/TVE approach at our institution since 2013.Results: We identified four patients with multi-hole PAVF treated by a combined TAE/TVE. Median age was 5.2 (0-14.7) years. Median follow-up of 8 (1-15) months by catheter angiography and 38 (23-53) months by MRI/MRA was obtained. TVE achieved complete occlusion in three patients that proved durable on radiographic follow-up and demonstrated excellent clinical outcomes with a modified Rankin Score (mRS) of 0 or 1. Complete occlusion of the draining vein was not achieved by TVE in one case. This patient is graded as pediatric mRS=5 three years post-procedure.Conclusions: With thorough technical considerations, our series indicates that TVE of multi-hole PAVF that are refractory to TAE is feasible and effective in arresting the consequences of chronic, high-flow AV shunting produced by this pathology.