General
- AKA
- Type 1 Borden
- Extradural (paraspinal) vascular malformations
Number
- Rare (or, more correctly, rarely diagnosed)
Location
- Spinal AVF reside in the epidural space within the spinal canal
Aetiology
- Unknown
- Spontaneous
- Complication of trauma
- NF 1
Connection
Fistula between the
- Epidural (Ventral) arterial arcade ↔
- Also known as the retrocorporeal arcade, since it is located behind the vertebral body
- Epidural venous plexus
Normal
- The aorta gives off paired segmental arteries (A), with a dorsal branch (B) penetrating the neural foramen to supply structures inside the spinal canal, such as the “epidural arterial arcade” (C).
- These are normally seen as diamond-shaped vessels; they provide a robust anastomosis between left and right segmental arteries.
- On the venous side, the ventral epidural venous plexus “D” is extensively connected up and down along the spinal canal, and communicates via multiple foraminal arteries (E) with the paraspinal veins (large vertical blue cylinders).
- The spinal bridging veins “F” connect the intradural spinal surface veins (G, H) with the extradural veins of the epidural venous plexus and foraminal veins.
- The bridging veins are the critical conduit between intradural and extradural venous systems.
Asymptomatic Extradural fistula
Symptomatic extradural fistula
- Reflux of venous outflow into the spinal veins, as seen in the diagram below.
- For whatever unknown reason, some epidural fistulas either do not have sufficient venous outflow, or this outflow becomes occluded, as seen in the figure
- Here, the fistula opens into a “venous pouch”, which has insufficient outside connections via the foraminal vein (E) for the degree of venous congestion. Because the epidural venous plexus is also connected to spinal veins (G) via the bridging vein (F), spinal congestion and its associated clinical sequela can follow.
- This pouch is usually seen on MRI as a clue into the possibility of a extradural fistula, and also to identify its level.
Clinical features
Asymptomatic
- See figure above
- Most patients
Symptomatic
- Very rare
- Due to the
- Venous anatomy: many connections between components of the epidural plexus and with paraspinal veins outside the spinal canal via the foraminal veins (F). → spinal cord surface veins (G,H), remain unaffected despite their potential connection with the epidural plexus.
- Extradural fistulas very occasionally on spinal angiography are incidentally diagnosed
- Due to (see figure above)
- Reflux of venous outflow into the spinal veins, as seen in the diagram below.
- Because the pathophysiology (cord venous congestion) is the same as for the spinal dural fistula, the clinical presentation is similar also — progressive myelopathy, bowel-bladder issues, sensory changes, etc.
- Hemorrhage has NEVER been described.
- Most epidural hematomas are probably spontaneous venous hemorrhages rather than spinal extradural (epidural) fistulas
- Compression of nerve roots and by venous engorgement
- Vascular steal phenomenon: blood supply shunted away from the spinal cord and contributing to myelopathic symptoms.
- EDH can occur in paeds
- 3 – 18% of EDH occurs in children due to extradural vascular malformation
Radiology
Treatment
- Surgery > endovascular treatment
- Obliterate the connection