DrSclafani answers some questions

[Cece]

I went looking for a good image of the external jugulars and this article has the best one yet of all the veins. Look for the interior jugulars, the subclavian, and vertebrals too. Hope it's fine to share this here, as part of our course materials.

http://radiographics.rsna.org/content/2 ... l.pdf+html

[Nunzio]

Tim, it is detected from time to time. I do not treat it if there is no symptoms from it.

Dear Dr. Sclafani,
I am afraid we are falling in the same frame of mind as most neurologists which is " Why fix a vein if the patient has no symptoms ( except MS)?"
So let me see if I can make the point.
As you know, Zamboni/Galeotti always enter trough the left side when they perform a CCSVI treatment.
The reason is that they can check for restriction of the iliac vein as it enter the Inferior Vena Cava.
In that case the blockage will force the venous flow to use the Iliac-lumbar vein as a collateral.
See picture below:

The blood flow that is going through the collateral will eventually drain in the Azygous vein overloading it and not allowing proper drainage of the spinal column.
Now the patient might have well compensated the restricted flow through the collaterals and not show any signs of May-Thurner syndrome, but still the collateral is overloading the Azygous vein.
Dr Galeotti found and treated this problem in about 5% of CCSVI patients.
He also checks and treats a similar % of patients with narrowing of the renal vein for the same reason.
You can see the full PDF presentation in Italian here:http://www.ospfe.it/attach/smcferrara/d ... tti%20.pdf
So, in conclusion I Think IR should follow Zamboni/Galeotti lead until additional reserch come up with better technique.

Nunzio
if the azygos vein is obstructed, then increased flow in that area because of renal vein and iliac stenosis is potentially deliterious. However if there is no obstruction of the azygos, then i think flow will not be overwhelming. Remember that left iliac stenosis is well compensated by using the cross pelvic collaterals into the inferior vena cava

Also, i did not find any mention of treatment of the may thurner sydrome during liberation in their paper.

We need more information

Dear Dr Sclafani,
thank you for your answer.
I must agree with you that if the azygous vein is not involved than probably there is no need to consider treatment of those other veins.
In TMrox case however we know he has azygous involment because they ballooned it so further investigation of the ilac vein is warranted.
I have seen a picture of a ballooned renal vein in the PDF presentation above and I am implying they do the same for the iliac vein but a direct question to Dr. Galeotti will help clarifying the situation.
I also found a very interesting paper from 1976 that is very similar in finding to what Dr. Zamboni/Galeotti do:

Cavo-spinal phlebography in myelopathies. Stenoses of internal jugular and azygos veins, venous compressions and thromboses.

Acta Radiol Suppl. 1976;347:415-7.
Leriche H, Aubin ML, Aboulker J.
Abstract

Increased intraspinal venous pressure, resulting according to ABOULKER in numerous spastic paraplegias and quadriplegias is due to multiple venous abnormalities demonstrated by cavo-spinal phlebography. The most frequent are stenoses of the internal jugular veins, the left renal, the left iliac veins, the azygos veins and compressions of the innominate venous trunks. These abnormalities cause a permanent stasis in the intraspinal plexuses through excessive supply or insufficient drainage. Out of 80 patients, 60 per cent had at least 2 abnormalities, 38 per cent at least 3 abnormalities.

[Cece]

Nunzio, is there access to the full article? The abstract mentions pressure not reflux and I don't know what it means by permanent stasis in the intraspinal plexuses, but the part about having at least 2 abnormalities is eerily familiar. Great find.

Interesting, it showed up in the references of this paper by Zamboni:
http://jnnp.bmj.com/content/80/4/392.full

[drsclafani]

Dr. S, what is your take on this? Could endolethin-1 be tied to stenosis?

-----------
Increased endothelin-1 plasma levels in patients with multiple sclerosis.

Haufschild T, Shaw SG, Kesselring J, Flammer J.

University Eye Clinic, Basel, Switzerland.
Abstract

OBJECTIVE: We tested the hypothesis that the plasma level of endothelin-1 (ET-1) is increased in patients with multiple sclerosis (MS). The peptide ET-1 is one of the most potent known vasoconstrictors. An increased level of endothelin could explain some of the vascular symptoms of these patients.

MATERIALS AND METHODS: A specific radioimmunoassay was used to determine ET-1 plasma levels. Twenty patients with MS were compared to 20 age- and sex-pair-matched healthy subjects.

RESULTS: The plasma ET-1 levels were, on average, 224% higher in the patients with MS than in the controls (p < 0.005). The mean ET-1 levels (mean +/- standard deviation [SD]) were 3.5 +/- 0.83 pg/mL (min 2.13, max 5.37 pg/mL) in patients with MS and 1.56 +/- 0.3 pg/mL (min 0.9, max 2.13 pg/mL) in healthy volunteers. Neither the different forms nor stages of MS had an influence on the results. The ET-1 level was also not correlated with the duration of the disease.

CONCLUSIONS: The plasma ET-1 level is markedly and significantly increased in patients with MS. Neither the cause of such an increase nor the pathogenetic role is known.

it is believed that the stenosis is caused by a genetic mutation that leads to malformation of veins and valves. Endothelin-1 causes vasoconstriction (usually increased contraction of the muscle of arteries leading to narrowing of the arteries.
so while both may be correct, one affects the blood inflow and one affects the blood outflow. I do not think that this can explain the constrictions of the veins

[drsclafani]

Dr. S, what is your take on this? Could endolethin-1 be tied to stenosis?

-----------
Increased endothelin-1 plasma levels in patients with multiple sclerosis.

Haufschild T, Shaw SG, Kesselring J, Flammer J.

University Eye Clinic, Basel, Switzerland.
Abstract

OBJECTIVE: We tested the hypothesis that the plasma level of endothelin-1 (ET-1) is increased in patients with multiple sclerosis (MS). The peptide ET-1 is one of the most potent known vasoconstrictors. An increased level of endothelin could explain some of the vascular symptoms of these patients.

MATERIALS AND METHODS: A specific radioimmunoassay was used to determine ET-1 plasma levels. Twenty patients with MS were compared to 20 age- and sex-pair-matched healthy subjects.

RESULTS: The plasma ET-1 levels were, on average, 224% higher in the patients with MS than in the controls (p < 0.005). The mean ET-1 levels (mean +/- standard deviation [SD]) were 3.5 +/- 0.83 pg/mL (min 2.13, max 5.37 pg/mL) in patients with MS and 1.56 +/- 0.3 pg/mL (min 0.9, max 2.13 pg/mL) in healthy volunteers. Neither the different forms nor stages of MS had an influence on the results. The ET-1 level was also not correlated with the duration of the disease.

CONCLUSIONS: The plasma ET-1 level is markedly and significantly increased in patients with MS. Neither the cause of such an increase nor the pathogenetic role is known.

Sounds like finding endothelin-1 plasma levels is a blood test...I wonder how expensive it is to do? Might be an useful marker for "MS" symptoms if a correlation could be established.

Donnchadh

maybe i am frugal, but i think that tests that do not lead to change in diagnosis, a refinement of diagnosis, measure progress of disease or define indications to treat, are not worth doing. they eat up the health care dollars without significant effect on the outcomes..

This coming from a radiologist??

s

[drsclafani]

Bringing this over from the bottom of the previous page in hope Dr. S will see it. Thx

i think that the next step is to determine whether there is or is not improvement in the Doppler results. If the Doppler is the same, i think another opinion is valuable. if the doppler shows eradication of the ccsvi criteria, then perhaps the brain damage is too great. but of course this decision is between patient and doctor

The figure that was posted in the forum the other day, that Dr. Sinan was finding and correcting azygous issues in 95% of patients, is startling if it can be verified.

Some treating physician recently described the sinan balloon inflation technique of discovering azygos vein malformation as Radiologic Braille. I didnt like the pergorative term but lets face it, we need to validate this. No one has ever done this in normal adults

While I agree with what Dr. Sclafani said above about the Doppler findings for the jugulars, the azygous cannot be imaged outside of a catheter venogram, and the azygous is an area in which the different docs have different techniques and expertise.

the doppler measures hemodynamic effects of abnormal venous return from the spine and brain. Abnormal doppler has been shown to be associated with azygos flow. Not to say that the doppler images the veins of the spinal cord anatomy. rather it measures the hemodynamics.

These are difficult decisions for people. They have spent considerable money, gotten disappointing outcomes and need to have some reason to double their financial hit without guarantee of improvement at this point. That is what i mean about the decision between patient and doctor

[drsclafani]

Dr Sclafani,

On the report of my Doppler, it says "Collateral circulation via left lobe of the thyroid gland was noted".

Is it something that can be the cause of thyroid gland disorders? I would like to know what you think about that.

Thank you very much.

There have been some preliminary data that suggest that patients may have an inflammatory thyroiditis associated with CCSVI. Since there is venous insufficiency in the thyroid gland, it is not surprising that this might happen

more work needs be done about this...like everything else!

[drbart]

For information, Dr. Sinan reported that 95-96% of his patients have abnormal valves or webs in the azygous. He said this at 42:00 to 42:40 into the presentation at

You can link directly to a time spot in a YT video. Dr Sinan starts talking about azygos at 41:45, so the link is

[drsclafani]

Dear Dr. Sclafani,

Here is my situation... I've done the procedure in July the 8th to correct stenosis in the LIJ and RIJ and three in the azygos. Dr. Martins Pisco put a stent in my left jugular and did angio in the right and in the azygos. I felt some small improvements right afterwards but they resumed one week after. In September, I experienced a small relapse and did a course of steroids. I have talked to Dr. Pisco about this and he thinks that there might have been some restenosis, probably in teh right jugular. I have agreed to go back later for a new procedure in which he'd probably put a stent in my RIJ.

What's your opinion on this? I understand you oppose stenting but in what grounds?

There has yet to be a publication on the long term effects of stents in the jugular vein. I know of one soon to be publication that shows that stents do not migrate, but thromboses seem to be occuring more so from stenting than balloon angioplasty. My major concern is that in the upright position (greater than 50% of the teim) there is little flow through the jugular vein. A blood vessel with no flow and a foreign body seems like a set up for thrombosis. But time will tell.......thus i choose to have someone other clinician and their patients make that analysis. For me, i will just redilate as often as necessary.

I think that part of the reason for the relapse is not restenosis but inadequate angioplasty that never creates sufficient stretch to tear the abnormal collagen and valves.

The rate of restenosis in jugulars has been quite high and many people are flaring as they renarrow. Is there a better alternative?


Thanks for your attention.

Spiff

yes actually

1. better technique of angioplasty
2. use of less stents
3. conservativism (less ballooning) toward high narrowings
4. aggresiveness (larger diameter balloons) toward lower narrowings
5. vigilant surveillance by ultrasound at one, three, six , twelve and eighteen months
6. liberal repeat angiography to try to avoid relapse once surveillance shows recurrent ccsvi BEFORE relapse

[drsclafani]

Based on the four CCSVI patterns described by Dr. Zamboni, only 14% had no azygous involvment.

Type A pattern (30%) is characterized by significant stenosis
of the proximal AZY or of one of the two IJVs, with a
compensatory contralateral IJV that appears with an
ample cross-sectional area; it was observed in 10 of 35
RR patients, in five of 20 SP patients, but never in PP
patients.

Type B pattern (38%) is characterized by significant stenoses
of both IJVs and the proximal azygous; it was
observed in 19 of 35 RR patients, in nine of 20 SP
patients, and in one of 10 PP patients.

Type C pattern (14%) is characterized by bilateral stenosis
in both IJVs, with a normal AZY system; it was observed
in four of 35 RR patients, in five of 20 SP
patients, but never in PP patients.

Type D pattern (18%) is characterized by the multilevel
involvement of the AZY and lumbar systems. Association
with the IJVs was observed in approximately 50%
and caused an additional obstruction in these patients.
It was observed in two of 35 RR patients, in one of 20
SP patients, and in nine of 10 PP patients.

A prospective open-label study of endovascular treatment of chronic cerebrospinal venous insufficiency
Paolo Zamboni, MD,a Roberto Galeotti, MD,a Erica Menegatti, RVT,a Anna Maria Malagoni, MD,a Sergio Gianesini, MD,a Ilaria Bartolomei, MD,b Francesco Mascoli, MD,a and Fabrizio Salvi, MD,b Ferrara and Bologna, Italy

JOURNAL OF VASCULAR SURGERY
Volume 50, Number 6

Dr. S. What does proximal mean? I understand it means where it starts ... so where is the proximal part of the azygous?

this is a very confusion terminology. For reference proximal is closer to and distal is further away. With vessels we tend to talk about a reference of the heart. Thus the lowest portion of the jugular vein as it enters the chest is the proximal portion. The part of the jugular vein that is at the skull is dista. Same goes with the azygos.....the closer to the heart, the more proximal.So the part of the azygos vein that is closest to the superior vena cava is more proximal and the veins farthest away from the superior vena cava are more distal

I dont like those terms.....i like to call them central and peripheral

[drsclafani]

Based on the four CCSVI patterns described by Dr. Zamboni, only 14% had no azygous involvment.

Type A pattern (30%) is characterized by significant stenosis
of the proximal AZY or of one of the two IJVs, with a
compensatory contralateral IJV that appears with an
ample cross-sectional area; it was observed in 10 of 35
RR patients, in five of 20 SP patients, but never in PP
patients.

That first category has an "or" in it, so it could mean jugular or azygous. I'd divide that category in half and add an additional 15% to your 14% figure, making 29% with no azygous involvement, but with the caveat that my math is monkey math.

lets not be pergorative toward other primates, please.

Besides Dr. Sclafani and Dr. Sinan, do any of the other docs seem like azygous experts? It's ok to pm me rather than post here.

you forgot robbie and paolo. but lets be clear, i am no expert. I am a concerned observer, a humble plumber trying to fix the pipes

ConstableComfortable, the external jugulars drain the face, not the brain, so if there is reflux I don't think it would damage the brain like IJV reflux.

i sort of agree. Although when the secondary pathways of the highway are also backed up, it does slow the main highway

[1eye]

one eye
what dr z found was the there was a 38% reduction in NEW LESIONS.
Some neurologists say that that is the natural way of things: that new lesions progressively reduce with time and that exacerbations are reduced with time as the disease goes into the progressive form

but nonethelss, it is compelling data

So, over one year a cohort of 65 patients should have 38% fewer gadolinium-enhancing lesions, just by the expected natural history of the disease? Hard to believe. I think in us progressive patients there are supposed to be fewer lesions. But a) I don't believe it, and b) I think the percentage reduction would have been even higher if you *only* included the progressive patients.

"A prospective open-label study of endovascular
treatment of chronic cerebrospinal venous
insufficiency"
Paolo Zamboni, MD,
Roberto Galeotti, MD,
Erica Menegatti, RVT,
Anna Maria Malagoni, MD,
Sergio Gianesini, MD,
Ilaria Bartolomei, MD,
Francesco Mascoli, MD,
Fabrizio Salvi, MD,

"MRI outcome measure: The rate of patients with
active gadolinium-enhanced lesions at MRI decreased
significantly from 50% to 12% (P .0001; Table IV."

These are definitely gadolinium-enhancing lesions.

[eric593]

Dear Dr. Sclafani,

Here is my situation... I've done the procedure in July the 8th to correct stenosis in the LIJ and RIJ and three in the azygos. Dr. Martins Pisco put a stent in my left jugular and did angio in the right and in the azygos. I felt some small improvements right afterwards but they resumed one week after. In September, I experienced a small relapse and did a course of steroids. I have talked to Dr. Pisco about this and he thinks that there might have been some restenosis, probably in teh right jugular. I have agreed to go back later for a new procedure in which he'd probably put a stent in my RIJ.

What's your opinion on this? I understand you oppose stenting but in what grounds?

There has yet to be a publication on the long term effects of stents in the jugular vein. I know of one soon to be publication that shows that stents do not migrate, but thromboses seem to be occuring more so from stenting than balloon angioplasty. My major concern is that in the upright position (greater than 50% of the teim) there is little flow through the jugular vein. A blood vessel with no flow and a foreign body seems like a set up for thrombosis. But time will tell.......thus i choose to have someone other clinician and their patients make that analysis. For me, i will just redilate as often as necessary.

I think that part of the reason for the relapse is not restenosis but inadequate angioplasty that never creates sufficient stretch to tear the abnormal collagen and valves.

The rate of restenosis in jugulars has been quite high and many people are flaring as they renarrow. Is there a better alternative?


Thanks for your attention.

Spiff

yes actually

1. better technique of angioplasty
2. use of less stents
3. conservativism (less ballooning) toward high narrowings
4. aggresiveness (larger diameter balloons) toward lower narrowings
5. vigilant surveillance by ultrasound at one, three, six , twelve and eighteen months
6. liberal repeat angiography to try to avoid relapse once surveillance shows recurrent ccsvi BEFORE relapse

But what is a doctor to do if it is clear that the vein is not helped by ballooning, or even worse, looks like it is narrower or collapses at the time of initial angio? What option is available except a stent, I wonder? Try a balloon that is wider than the diameter of the healthy parts of the IJV and hope it doesn't tear? Leave it in worse shape? Or...???

If stents aren't an option, what is the best course when faced with an even narrower vein than it was to begin with as the result of ballooning?

[spiff1970]

Dear Dr. Sclafani,

Here is my situation... I've done the procedure in July the 8th to correct stenosis in the LIJ and RIJ and three in the azygos. Dr. Martins Pisco put a stent in my left jugular and did angio in the right and in the azygos. I felt some small improvements right afterwards but they resumed one week after. In September, I experienced a small relapse and did a course of steroids. I have talked to Dr. Pisco about this and he thinks that there might have been some restenosis, probably in teh right jugular. I have agreed to go back later for a new procedure in which he'd probably put a stent in my RIJ.

What's your opinion on this? I understand you oppose stenting but in what grounds?

There has yet to be a publication on the long term effects of stents in the jugular vein. I know of one soon to be publication that shows that stents do not migrate, but thromboses seem to be occuring more so from stenting than balloon angioplasty. My major concern is that in the upright position (greater than 50% of the teim) there is little flow through the jugular vein. A blood vessel with no flow and a foreign body seems like a set up for thrombosis. But time will tell.......thus i choose to have someone other clinician and their patients make that analysis. For me, i will just redilate as often as necessary.

I think that part of the reason for the relapse is not restenosis but inadequate angioplasty that never creates sufficient stretch to tear the abnormal collagen and valves.

The rate of restenosis in jugulars has been quite high and many people are flaring as they renarrow. Is there a better alternative?


Thanks for your attention.

Spiff

yes actually

1. better technique of angioplasty
2. use of less stents
3. conservativism (less ballooning) toward high narrowings
4. aggresiveness (larger diameter balloons) toward lower narrowings
5. vigilant surveillance by ultrasound at one, three, six , twelve and eighteen months
6. liberal repeat angiography to try to avoid relapse once surveillance shows recurrent ccsvi BEFORE relapse

But what is a doctor to do if it is clear that the vein is not helped by ballooning, or even worse, looks like it is narrower or collapses at the time of initial angio? What option is available except a stent, I wonder? Try a balloon that is wider than the diameter of the healthy parts of the IJV and hope it doesn't tear? Leave it in worse shape? Or...???

If stents aren't an option, what is the best course when faced with an even narrower vein than it was to begin with as the result of ballooning?

Can restenosing veins actually become narrower than before angio? I did actually experienced my first relapse in almost five years! Another patient also had a bad relapse after the treatment. I wonder if that is the reason.

[Cece]

Some treating physician recently described the sinan balloon inflation technique of discovering azygos vein malformation as Radiologic Braille. I didnt like the pergorative term but lets face it, we need to validate this. No one has ever done this in normal adults

It took me a while to understand it was pejorative. Braille is an invention that allows people with a disability (any people with disabilities around here?) to get information where they otherwise wouldn't! So when it comes to the azygous, if feeling around in the dark is the way to get information, it works for me. It just has to be determined if it's accurate information.

And, what, Dr. Sclafani is doing something no one has ever done before? We have gotten to know you well enough to not be shocked.

the doppler measures hemodynamic effects of abnormal venous return from the spine and brain. Abnormal doppler has been shown to be associated with azygos flow. Not to say that the doppler images the veins of the spinal cord anatomy. rather it measures the hemodynamics.

I hate feeling like I am wrong rather often here but I appreciate being set right. Thanks for your patience.

These are difficult decisions for people. They have spent considerable money, gotten disappointing outcomes and need to have some reason to double their financial hit without guarantee of improvement at this point. That is what i mean about the decision between patient and doctor

That's an extremely good point.