Pediatric high-flow, cervical spinal, macro-arteriovenous fistula, treated with the endovascular cotton candy glue injection technique

Abstract

A 3-year-old boy presented to our hospital with progressive neurological deficits. Spinal magnetic resonance imaging (MRI) revealed a perimedullary macro-arteriovenous fistula (PMAVF) resulting in a large venous pouch within the parenchyma of the lower cervical spinal cord. Transarterial varix embolization of the fistula from the venous side was performed using N-butyl cyanoacrylate with tantalum powder. Postembolization angiography confirmed obliteration of the fistula, and MRI revealed thrombosis and reduction in size of the venous component. The patient's clinical symptoms were reduced dramatically following the intervention, and no neurological complications occurred due to the treatment. The prognosis of spinal PMAVF depends primarily on the presence of medullar signs and symptoms and on time to treatment. The volume of the venous pouch after the intervention was markedly reduced, and complete clinical recovery was obtained. Urgent endovascular intervention, as seen in this case, is considered first-line therapy and aims to decrease the risk of neurological sequela.

Single hole, high-flow, spinal cord peri-medullary arteriovenous fistula (PMAVF) in a child, treated with transarterial embolization: A case report

“…There are various classifications of spinal vascular malformations. The most common classification by Rosenblum divides spinal vascular malformations into four categories, which are type I (Dural arteriovenous fistula (AVF), type II (Glomus arteriovenous malformation (AVM)), type III (Juvenile AVM), and type IV (Peri-medullary AVF) [4] , [5] , [6] ]. Type I is the most common, accounting for 80%-85% of all spinal vascular malformations, while type IV is rare [4] .…”

“…In this case, because of the high flow of fistula, the transition was marked by the formation of a large venous pouch. PMAVF in the ventral area of the spinal cord is generally subpial and is supplied by the anterior spinal artery, whereas if it is subarachnoid, it is generally supplied by the posterolateral spinal artery [5] . This case was unique, because the large fistula was supplied by only 1 feeder artery, which was anterior radiculomedullary artery, and drain into a large venous pouch.…”

“…On the other hand, macrofistula PMAVF (types IVb and IVc) are more common in children and are associated with genetic syndromes such as Hereditary Haemorrhagic Telangiectasia (HHT), or less commonly Klippel Trenaunay-Weber or Cobb's syndrome [2] . HHT is an autosomal dominant disease caused by mutations in the endoglin or novel kinase gene, with a broad clinical manifestation that includes cutaneous telangiectasia, recurrent epistaxis, and arteriovenous malformations involving various organs, such as lungs, gastrointestinal tract, liver, genitourinary tract, brain, and spinal cord [5 , 8] . In this case, we did not perform genetic testing for HHT because the patient did not present with classic HHT clinical manifestations and had no family history.…”

“…Proximal feeder artery occlusion increases the risk of recanalization, while occlusion of the venous drainage at an overly distal site will increase the risk of bleeding [3] . There is a case report showing the effectiveness of venous pouch embolization using a cotton candy-like injection technique [5] .…”

Clinical features and outcomes of perimedullary arteriovenous fistulas: comparison between micro- and macro-type lesions

“…Previous clinical evidence has indicated that lesion size and blood flow may be related to the spectrum of pathophysiological mechanisms of PMAVFs (eg, venous congestion, bleeding or spinal cord compression), which can result in diverse clinical characteristics 2 3 6. Additionally, when developing treatment strategies for specific PMAVF patients, these parameters are also important considerations 4–8. Therefore, the optimal classification of PMAVFs should take the lesion size and blood flow into account 9–11.…”