Very rare. Congenital (malformative) or post-traumatic origin. Unlike arteriovenous perineuraxial malformations, the nidus is not located into the spinal canal but outside the dura mater, although its venous drainage is usually via the epidural venous network.

According to the Stuttgart's classification, one can distinguish:

·       isolated forms

- acquired: with intra- or extraspinal drainage

- traumatic: with intra- or extraspinal drainage

- congenital without identified genetic cause: with intra- or extraspinal drainage

·       related forms

-         with a metameric link: with intra- or extraspinal drainage: see neurofibromatosis, Osler-Rendu-Weber disease

-          systemic dysplasia of genetic origin: with intra- or extrarachidian drainage: see Cobb, CLOVES or Klippel-Trenaunay syndromes

These fistulas are usually located at the thoracic level and can extend to the paraspinal muscles, intervertebral foramens, the paravertebral region or even the spinal canal.

Clinical signs:

-        either due to the presence of the arteriovenous fistula: pulsatile mass, cardiac failure, paravertebral murmur

-          either related to the venous dilation downstream of the malformation with signs of compression: neurological signs (compression of a root or of the spinal cord), sometimes erosion of the vertebral body

Diagnosis: angio-MRI and angiography

Treatment: repetitive embolizations.

Anesthetic implications: 

echocardiography (cardiac function), check hemostasis (local DIC with increased D-dimers level ?)

References : 

-        Farhat N, Despreschins B, Otto B, Ramaekers V, Seghaye M-C.
Paraspinal arterio-venous fistula in children: two more cases of an exceptional malformation.
Clinics and Practice 2015; 5: 707 (p37-9)

-        Wendl CM, Aquilar-Perez M, Feller S. et al.
Paraspinal arteriovenous malformations: Stuttgart classification based on experience and review of literature.
Br J Radiol 2018; 91: 20170337

Updated: September 2019