Perimedullary Arteriovenous Fistula: Ruptured Perimedullary Arteriovenous Fistula Associated with an Incidental Paraspinal Arteriovenous Malformation (AVM) in a New Born; Endovascular Cure by Staged nBCA Embolization

Abstract

A newborn girl displayed sudden onset paresis of both legs at 4 weeks of age. An initial MRI of the spine revealed intramedullary bleeding at the level of the conus medullaris. Emergency surgery to evacuate the spinal cord hematoma was performed, and tortuous dilated intraspinal perimedullary vessels were noted. After surgery, the right leg weakness persisted, but the strength in the left leg improved. Initial catheter angiography workup at 3 months of age revealed a perimedullary arteriovenous fistula with associated varix at the level of the conus medullaris with radiculomedullary arterial feeders from the segmental arteries Th11 left and L4 left as well as a paraspinal arteriovenous malformation located at the lumbosacral junction with arterial feeders from the segmental arteries L4 right and left. Endovascular glue embolization (nBCA/ethiodized oil) of the perimedullary arteriovenous fistula was performed at 8 months (L4 left) and 9 months (Th11 left) of age with complete obliteration of the perimedullary arteriovenous shunt. The paraspinal AVM was successfully treated with glue embolization (nBCA/ethiodized oil) in two sessions at 15 and 18 months. On the last DSA follow-up at 18 months of age, there was no evidence for any recurrent intraspinal arteriovenous shunt, and the anterior spinal artery caliber had normalized. The child can now walk and run with no assistance. Neurogenic bladder issues have, however, been present since the spinal cord bleed. This case illustrates a rare combination of intra- and extraspinal vascular pathologies in a newborn. The clinical benefit of early identification and treatment of the intraspinal arteriovenous fistula at such an age – even after bleeding – is the main topic of this chapter.