CCSVI Richard F. Neville, MD Professor of Surgery

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Presentation transcript:

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

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

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

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;25:269-279

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

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%

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

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

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;25:269-279

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

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

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;68:255-259

Study Multiple sclerosis and Chronic Cerebrospinal Venous Insufficiency, IRB# 2010-186

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

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%

L IJ stenosis

R IJV valve, Left IJV stenosis

R IJV, L IJ with valve

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

Right IJV stenosis with angioplasty

Left IJV stenosis - angioplasty

L IJ stenosis with angioplasty

MW – Azygous PTV

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