General
- AKA spontaneous spinal venous fistula
Presentation
- Early
- Postural headache
- Neck stiffness and tenderness
- Hearing loss
- Late
- Cognitive decline
- Present like frontotemporal dementia
Presumed pathophysiology (Mr Payal)
- Type 3 leaks
- The radicular dural membrane Is thin and there are many periradicular large dilated veins that can ruptured (not sure why ? Increase pressure in vein) and form abnormal connection. Due to the low pressure in veins CSF leaks into the venous system.
- Blood can enter the CSF causing siderosis
CSF venous fistula
- First described in 2014
- Heavily underdiagnosed
- Perhaps account for 1/4ᵗʰ of all SIH cases
- Can be multiple
- Often thoracic, but can be anywhere in the spine
- How many Chiaris are actually CSF VF?
- No UK series published yet
- Neurologists and Neuroradiologists are the key
- MRI Brain can be normal and often is
- Vast majority settle with conservative measures or blood patches
Types
- Type 1 CSF leaks
- Are caused by a dural tear located ventral to the spinal cord (type 1a) or (postero-) lateral to the spinal cord (type 1b).
- Type 2 CSF leaks
- Are associated with simple (type 2a) or complex (type 2b [dural ectasial]) meningeal diverticula.
- Abnormal outpouchings of the common dural sac, the spinal arachnoid, or the nerve root sheath
- Type 3 CSF leaks
- CSF-venous fistulas
- Type 4 CSF leaks
- Are of indeterminate origin.
Investigation
- MRI brain and spine
- Look for chiari 1
- Low pressure chiari
- Sloping brainstem
- Full sella
- Leptomeningeal enchacement
- High pressure chiari
- ICP bolt studies
- Opthalmology review for papilloedema
- Optic nerve sheath dilatation and tortuosity
- Deformed
- SWI
- Look for siderosis of the cerebellum
- CT myelogram in lateral positioning
- Look for contrast leakage
- Can have multiple leakage
Treatment
- Blood patch done at least twice
- Endovascular venous gluing of CSF venous fistula
- Surgically disconnection of CSF venous fistula via a wilsie approach
- Surgically closing ventral or dorsal dural tears type 2
- Ventral
- More complex require intradural closure via mobilising spinal cord using the dentate ligament
- Closure can be done using clips or suture.
- Use a small dural patch + fibrin glue
- Dorsal
- More straightforward closure.