Although I'd love to take credit for the Budd-Chiari analogy, it was a world-reknowned venous specialist, Dr. B. B. Lee of Georgetown University who connected this. I heard him speak in Bologna. He has since written a paper outlining the similarities of truncular venous malformations in Budd-Chiari and CCSVI-- I have linked this paper many times, but will add parts of it here, because I keep referring to it....
http://www.fondazionehilarescere.org/pd ... 8-ANGY.pdf
Truncular VM lesions therefore, present either as a hypoplastic or hyperplastic lesion causing obstruction or dilatation (e.g. internal jugular vein aneurysm; azygos vein stenosis), depending on the various defective conditions of the vessel trunk 25-28 (Figure 2 A-D). Intraluminal defects within the vein (e.g. vein webs or membrane) result in stenosis or obstruction.29, 30 More infrequently, truncular VM lesions may also present as a persistent fetal remnant vein that has failed to involute or regress normally (e.g. marginal/sciatic/lat- eral embryonic veins) 31, 32 (Figure 3 A, B).
Truncular VM lesions present with more serious hemodynamic consequences in general due to the direct involvement of the lesion with the truncal venous system (e.g. avalvulosis, marginal veins, popliteal vein aneurysm, IVC steno- sis/occlusion), compared to extratruncular lesions.
As a consequence, chronic venous congestion and hypertension due to venous reflux or occlusion results in more tissue and organ damage along the venous capacitance; membraneous/focal/segmental stenosis of suprahepatic inferior vena cava, known as Primary Budd-Chiari Syndrome has such a profound impact on the liver resulting in severe portal hypertension due to hepatic venous outlet obstruction in addition to chronic venous insufficiency affecting the lower extremities 33, 34 (Figure 4 A-C).
The cerebrospinal venous circulation is not exempt from truncular venous malformations. Cerebrospinal VMs also carry the potential risk of long term of chronic venous hypertension to the brain resulting in various clinical conditions/illnesses such as chronic cerebrospinal venous insufficiency (CCVI) .35-38
An example of CCVI, internal jugular vein valve incompetence (IJVVI) 39 has been postulated to be a cause of transient global amnesia (TGA).40-43 IJVVI is diagnosed when retrograde jugular vein flow is detected by extracranial duplex ultrasound during Valsalva maneuver.
It is believed that IJVVI may produce transient mesiotemporal ischemia by venous congestion. This mechanism requires a patent venous path-
way from the affected IJV through the transverse sinus, confluence, straight sinus, and vein of Galen into the basal vein of Rosenthal and into the internal cerebral veins.
As included in UIP Consensus on the Venous Malformations, 2009 44 further data supporting the role of CCVI in brain function, is found in patients with multiple sclerosis. Truncular VM lesions causing stenosis along the internal jugular, innominate, superior vena cava, and azygos vein system, have been suspected to contribute to the development or exacerbation of multiple sclerosis 45-49 (Figure 5).
Therefore, proper understanding of the evolutional as well as involutional process of embryonic development of the venous system 50-53 is essential for the appropriate management of these truncular VM lesions as a potential cause of var- ious conditions involving chronic cerebrospinal venous hypertension/insufficiency......
This is the best known intrinsic defect to cause varying degree of stenosis and obstruction together with another condition of venectasia and aneurysm causing venous dilatation. Such anomalous condition of the suprahepatic IVC with membraneous obstruction of vena cava (MOVC) along the proximal terminal segment of IVC gives clinically significant hemodynamic impact not only to the lower extremity to cause chronic venous hypertension, but also to the liver resulting in hepatic venous outlet obstruction resulting in portal hypertension.
In view of such profound impact of a relatively simple truncular venous malformation such as a venous web at the hepatic venous outlet causing portal hypertension, a similar condition involv- ing to the head and neck venous system may cause CCSVI and may be involved in the development or exacerbation of multiple sclerosis.
Yes, I know the word "may" is used, I realize that this is an "hypothesis", and I know what that means. But what the top venous specialist in the world is saying (not me on a website), what all of the venous specialists that met in September '09 to discuss truncular venous malformations in MS, is that this NEEDS TO BE STUDIED. Because what IRs are finding in CCSVI patients like my husband...webs, malformed veins, missing veins, inverted valves, etc. ARE EXACTLY WHAT THEY FIND IN OTHER VENOUS DISEASES. And these could very well be creating MS.
I really don't know how else to say this...if someone wants to debate, please link to research papers we can all read. Not opinion---
Husband dx RRMS 3/07
dx dual jugular vein stenosis (CCSVI) 4/09
dual stents placed 5/09
CCSVI in MS