1. CCSVI Richard F. Neville, MD Professor of Surgery
Chief, Division of Vascular Surgery
George Washington University

2. Chronic Cerebrospinal Venous Insufficiency (CCSVI)
Syndrome characterized by stenoses of the internal jugular or azygous veins with collateral formation

3. CCSVI Theory Prof Paolo Zamboni 2009
MRI – insufficient drainage by increased mean transit time
Venograpy – truncular venous malformations
Patterns
Type A (30%)
Proximal Azygous
One IJV
Type B (38%)
Both IJVs
Type C (14%)
Normal azygous
Type D (18%)
Proximal azygous\
Lumbar azygous
50% IJV

4. Cerebrospinal Venous System
Deep cerebral venous system
Extracranial and extravertebral venous stenosis
DMCV, deep middle cerebral veins;
TS, transversal sinus
CV, condylar veins
VV, vertebral veins
IJV, internal jugular vein
VP, vertebral plexus
SVC, superior vena cava
AZY, azygous vein
Zamboni P, Galeotti R. The chronic cerebrospinal venous insufficiency syndrome. Phlebology 2010;25:

5. Chronic Cerebrospinal Venous Insufficiency (CCSVI)
Theory
Insufficient drainage of the cerebral circulation
Reduced cerebral blood flow
Increased mean transit time in MRI perfusion studies
Truncal venous malformations
Annulus
Malformed valves/Congenital septae or webs
Hypoplasia
Twisting

6. CCSVI and MS Not contradictory with the autoimmune theory
High rate of cerebral venous reflux in MS
Extracranial reflux transmitted to the deep cerebral system in 50% of MS cases
Histology in MS
Iron stores encircling the vein wall
Hemosiderin deposits in macrophages
Pericapillary fibrin cuffs
No CCSVI with Alzheimer’s, Parkinson’s, Amyotrophic lateral sclerosis
Cerebral venous hemodynamics in normal patients
Laminar
Mono-directional
Low velocity
CCSVI prevalance in MS
Reported to be 56 – 100%

7. Truncal venous malformations
Parallel between truncular venous malformation in the jugular veins and in other venous segments.
(a) Membranous obstruction of the SVC in primary Budd-Chiari syndrome
(b) The same lesion in observed in the IJV
c) Truncular stenosis of the left IJV d) Truncular stenosis of the external iliac vein
Lee BB, Laredo J, Neville RF. Embryological background of truncular venous malformation as the cause of chronic CCSVI. Intl Angio 2010;29(2):95-107

8. Duplex Ultrasound evaluation
Reflux in cervical veins
Flow direction in intracranial veins
B mode images
IJ area < 0.4cm2
Asymmetry of IJVs
Blocked outflow in cervical veins in all postural positions
Cross sectional area does not widen in supine position

9. Dynamic US testing IJV cross-sectional area
Left: increased cross-sectional area (CSA) measured in the IJVs of healthy controls passing from the sitting to the supine posture.
Right: flat or even negative CSA variation measured in the majority of CCSVI-MS patients.
Zamboni P, Galeotti R. The chronic cerebrospinal venous insufficiency syndrome. Phlebology 2010;25:

10. Venographic images
Left: annulus of the IJV between the venous valve (VV) and the brachio cephalic trunk (BCT)
Right: twisting of the AZY just below the arch with dilation and reflux toward the spine. AZY, azygous vein

11. Treatment of CCSVI (a) Stenosis of the right IJV
(b) The balloon inflated during the PTA
(c) Postoperative result IJV

12. Case controlled study raises doubts
21 relapsing-remitting MS
20 controls
MRI (phase-contrast)
3/21 IJV stenosis
No difference in IJV outflow
No difference in IJV reflux
Sundstrom P, et al. Ann Neurol 2010;68:

13. Study
Multiple sclerosis and Chronic Cerebrospinal Venous Insufficiency, IRB#

14. Study Duplex US screening n=100 Positive Zamboni criteria n=57
Venograms n=48
IVUS on all studies
Treated n=32
Angioplasty only
Large, low pressure balloons
Prolonged inflation times

15. Imaging Data Duplex ultrasound Venography Reflux
R IJV 57% (reflux time 0.55 – 1.70 secs
L IJV 62% (reflux time 0.68 – 2.25 secs)
Unilateral reflux 52%
Bilateral reflux 43%
Stenosis
R IJV 0
L IJV 1 thrombosis
Venography
Any abnormality 73%
R IJV stenosis 29%
L IJV stenosis 33%
Azygous stenosis 10%

16. L IJ stenosis

17. R IJV valve, Left IJV stenosis

18. R IJV, L IJ with valve

19. Treatment Data Angioplasty 32 (66.7%) R IJV angioplasty 9 (28.1%)
L IJV angioplasty 13 (40.6%)
Azygous angioplasty 4 (12.5%)

20. Right IJV stenosis with angioplasty

21. Left IJV stenosis - angioplasty

22. L IJ stenosis with angioplasty

23. MW – Azygous PTV

24. Summary (no Conclusions)
Initial observations of central venous system in MS patients
No controls
Seemingly, a large number of venous abnormalities
IVUS important
Identify valves
Discern pathology
Questionable results without adjunct techniques beyond angioplasty alone
Questionable outcomes