DrSclafani answers some questions

[Cece]

1 - If a patient has a physiological stenosis due to muscle compression of the jugular, rather than placing a stent inside the vein, Dr. Sclafani suggested a pharmacological solution to the muscle. I am imagining botox but it is the only muscle paralyzer that I know. Would paralyzing the muscle be what is needed, or would it be a muscle relaxant?

I was wrong about how botox works. Botox is a muscle relaxant. Specifically, it blocks the acetylcholine receptors of the muscles so they never get the chemical message to contract.

[JohnAm]

DR S

Do You have any numbers on restenosis in azygos for those You have treated? Dr. Zamboni had 4% in his study if I remember correctly. Those that have restenosed azygos, any observable pattern of symptoms?

Acending lumar vein. I read that it has a diameter of approx. 2 mm and wouldn’t that cause it to be an unreliable channel for drainage? Can You say how many mL per minute it drains? Do You see problems with it often?

If the left ALV is hypoplastic or even missing how would the flow in hemiazygos be effected? It seems to connect iliac with lumbar veins and join with the subcostal veins and drain into the hemi. If ALV is missing are You investigating from above (via hemiazygos) to see the extent of what is missing or is this apparent on fluoroscopy with contrast?

As far as I understand there is a communicating vein btw. LRV and left ALV – can You see this perforator if ALV is hypo or missing below?

Is a missing ALV a significant problem in CCSVI?

If there is flow in azygos does that indicate right ALV has patent flow? Do You investigate the right ALV?

Thanks for having the courage and heart to communicate with us.

[drsclafani]

Dr S

In a situation where you are required to use a stent, how do you determine the size and type of stent?


thanks.

Situational circumstances
vessel: Jugular, renal vein, azygous vein or dural sinuses
location in vessel J1, J2 or J3
Indication elastic stenosis, thrombosis, recanalized thrombosis with stenosis,
compliance of vessel
side branches
adjacency to critical vessel, nerve or other structure

Measure cross sectional area of the vein by IVUS. Try for maximum distension by a variety of manuevers for that measurement
Measure length of stent to cover entire narrowed segment plus a little bit.
i prefer self expanding nitinol stents for the veins of CCSVI.

[drsclafani]

DR S

Do You have any numbers on restenosis in azygos for those You have treated? Dr. Zamboni had 4% in his study if I remember correctly. Those that have restenosed azygos, any observable pattern of symptoms?

I havent noticed any restenoses of azygous veins....only missed valvular stenoses in patients who came from other IRs

Acending lumar vein. I read that it has a diameter of approx. 2 mm and wouldn’t that cause it to be an unreliable channel for drainage? Can You say how many mL per minute it drains? Do You see problems with it often?

The diameter of this vein rnges between 2-5 mm, closer to 3 or 4 than 2 mm. There isnt great flow through the alv in general. the lumbars typically drain through the IVC more than alv.
The LALV drains into left iliac vein, into the left renal vein, and into the hemiazygous vein..

If the left ALV is hypoplastic or even missing how would the flow in hemiazygos be effected? It seems to connect iliac with lumbar veins and join with the subcostal veins and drain into the hemi. If ALV is missing are You investigating from above (via hemiazygos) to see the extent of what is missing or is this apparent on fluoroscopy with contrast?

This vein generally peeters out as it reaches the renal vein . It is uncommon for there to be a big connection between hemiazygous and LALV

i think that the lumbar vein hypoplasia might be more significant than the LALV hypoplasia because these are often multiple and require drainage up the vertebral plexus and into the azygous and hemiazygous systems. But the significance of either is unclear to me.

As far as I understand there is a communicating vein btw. LRV and left ALV – can You see this perforator if ALV is hypo or missing below?

probably only if there were a Nutcracker phenomenon.

Is a missing ALV a significant problem in CCSVI?

dont know yet

If there is flow in azygos does that indicate right ALV has patent flow? Do You investigate the right ALV?

Thanks for having the courage and heart to communicate with us.

there should be flow in the azygous vein regardless of the patency of the ascending lumbar vein. The azygous drains the thoracic spine, and the back musculature. The ascending lumber vein drains the lumbar vertebrae and drains into the iliac vein.

I do not investigate the RALV. It would be a very difficult catheterization.

[ttucker3]

Sal

My apologies for a little delay in response. Babysitting two energetic grandsons for the weekend tends to divert one's attention.

My concern for using Botox was from the point of view that its action is described, not only as a muscle relaxant, but also as a muscle paralyzer. After a very quick review of the literature, I perceive I don't have enough bio-chemistry in my background to know what the impact of botox on vein & surrounding tissue compliance might be. There seems to be a paucity of info on the relationship between the two. So my inclination would be to err on the side of caution (ie learn more about it, with the right scientific background) before trying botox as a venous reflux hypertension reduction treatment.

Trev. Tucker

Suggesting Botox injections in muscles near the IJV worry me a bit because I think treatment wants to increase the compliance of the veins and surrounding tissue, increase their ability to expand and thereby absorb some of the over-pressure associated with reflux. This increased expansion down lower in the veins should reduce the pressure forced higher into the venule region.

Trev. Tucker

trev
please clarify. I dont understand what you mean. if the muscles are relaxed, that should increase the compliance. also if, (i am not asserting that this is the case) the spastic muscles are causing compression and obstruction of the J2 segment, then relaxation of those muscles should increase luminal diameter and antegrade flow.

So speak to me more about your idea

S

[mo_en]

Dear Doctor,
The dural sinuses from which IJVs emerge are connected inside the brain, is that right? Why some online sources (ie wikipedia) say that the left internal jugular is the natural continuation of the straight sinus while the right is of the superior sagittal sinus? What does this mean in terms of blood flow? Is it possible (or common) that the left and right transverse sinuses do not communicate and the jugulars receive blood from totally different parts of the brain?

[Cece]

I havent noticed any restenoses of azygous veins....only missed valvular stenoses in patients who came from other IRs

Have you had patients where the jugulars remained patent from a previous procedure and only the azygous needed treatment? It would be interesting to know what effect treating the azygous has, without being confounded by jugulars treated at the same time.

[THEGREEKFROMTHED]

1 - If a patient has a physiological stenosis due to muscle compression of the jugular, rather than placing a stent inside the vein, Dr. Sclafani suggested a pharmacological solution to the muscle. I am imagining botox but it is the only muscle paralyzer that I know. Would paralyzing the muscle be what is needed, or would it be a muscle relaxant?

I was wrong about how botox works. Botox is a muscle relaxant. Specifically, it blocks the acetylcholine receptors of the muscles so they never get the chemical message to contract.

I don't know what Botox would do in the neck but I received injections in the legs and believe me it's paralyzing alright. I can't even hold myself up! I'm actually looking forward to the leg spasms to come back so I can use the walker. so I would be cautious about pumping it in a neck muscle!

[CD]

Special care would have to be avoiding the Voice box, Esophagus and/ or Thyroid gland. Another powerful thought.
CD

That would be my concern. What if it was a muscle paralyzer to my above comment body parts? A disaster waiting to happen.
CD

Edit to add: Thank you Dr Sclafani for your recent reply to me. Still checking, will keep you in the loop.
CD (Brooklyn born, and I loved it!)

[drsclafani]

Special care would have to be avoiding the Voice box, Esophagus and/ or Thyroid gland. Another powerful thought.
CD

That would be my concern. What if it was a muscle paralyzer to my above comment body parts? A disaster waiting to happen.
CD

Botulinum toxin treatment of spasmodic torticollis.
T J Anderson, J Rivest, R Stell, M J Steiger, H Cohen, P D Thompson, and C D Marsden
University Department of Clinical Neurology, National Hospital, Queen Square, London.
Abstract
We reviewed the efficacy and adverse effects of repeated botulinum toxin injections into hyperactive neck muscles of 107 successive patients with spasmodic torticollis. They received 510 injection treatments over a median period of 15 months (range 3-42 months). One patient failed to benefit at all, but 101 (95%) patients reported considerable (moderate or excellent) benefit from at least one treatment. On a global subjective response rating, 93% of 429 treatments resulted in some improvement and 76% in moderate or excellent improvement. Pain reduction followed 89% of 190 treatments with moderate or excellent reduction after 66%. Median duration of benefit was 9 weeks. All torticollis types responded equally well and injections into two (or more) involved neck muscles were more effective than injection into a single muscle. The most frequent adverse effect was dysphagia, occurring after 44% of all treatments, but this was severe after only 2%. Antibodies to botulinum toxin were detected in the serum of three out of the five patients in whom loss of treatment efficacy occurred. We conclude that botulinum toxin treatment is the most effective available therapy for spasmodic torticollis and practical advice is provided for anyone wishing to set up the technique.

Efficacy and safety of botulinum type A toxin (Dysport) in cervical dystonia: Results of the first US randomized, double-blind, placebo-controlled study
Daniel Truong MD1,*, Drake D. Duane MD2, Joseph Jankovic MD3, Carlos Singer MD4, Lauren C. Seeberger MD5, Cynthia L. Comella MD6, Mark F. Lew MD7, Robert L. Rodnitzky MD8, Fabio O. Danisi MD9, James P. Sutton MD10, P. David Charles MD11, Robert A. Hauser MD12, Geoffrey L. Sheean MD13
Article first published online: 25 FEB 2005
DOI: 10.1002/mds.20403
Copyright © 2005 Movement Disorder Society
Issue
Movement Disorders
Volume 20, Issue 7, pages 783–791, July 2005
Abstract
Botulinum toxin type A (Dysport) has been shown in European studies to be a safe and effective treatment for cervical dystonia. This multicenter, double-blind, randomized, controlled trial assessed the safety and efficacy of Dysport in cervical dystonia patients in the United States. Eighty patients were randomly assigned to receive one treatment with Dysport (500 units) or placebo. Participants were followed up for 4 to 20 weeks, until they needed further treatment. They were assessed at baseline and weeks 2, 4, 8, 12, 16, and 20 after treatment. Dysport was significantly more efficacious than placebo at weeks 4, 8, and 12 as assessed by the Toronto Western Spasmodic Torticollis Rating Scale (10-point vs. 3.8-point reduction in total score, respectively, at week 4; P ≤ 0.013). Of participants in the Dysport group, 38% showed positive treatment response, compared to 16% in the placebo group (95% confidence interval, 0.02–0.41). The median duration of response to Dysport was 18.5 weeks. Side effects were generally similar in the two treatment groups; only blurred vision and weakness occurred significantly more often with Dysport. No participants in the Dysport group converted from negative to positive antibodies after treatment. These results confirm previous reports that Dysport (500 units) is safe, effective, and well-tolerated in patients with cervical dystonia. © 2005 Movement Disorder Society

it seems pretty safe

[THEGREEKFROMTHED]

Special care would have to be avoiding the Voice box, Esophagus and/ or Thyroid gland. Another powerful thought.
CD

That would be my concern. What if it was a muscle paralyzer to my above comment body parts? A disaster waiting to happen.
CD

Botulinum toxin treatment of spasmodic torticollis.
T J Anderson, J Rivest, R Stell, M J Steiger, H Cohen, P D Thompson, and C D Marsden
University Department of Clinical Neurology, National Hospital, Queen Square, London.
Abstract
We reviewed the efficacy and adverse effects of repeated botulinum toxin injections into hyperactive neck muscles of 107 successive patients with spasmodic torticollis. They received 510 injection treatments over a median period of 15 months (range 3-42 months). One patient failed to benefit at all, but 101 (95%) patients reported considerable (moderate or excellent) benefit from at least one treatment. On a global subjective response rating, 93% of 429 treatments resulted in some improvement and 76% in moderate or excellent improvement. Pain reduction followed 89% of 190 treatments with moderate or excellent reduction after 66%. Median duration of benefit was 9 weeks. All torticollis types responded equally well and injections into two (or more) involved neck muscles were more effective than injection into a single muscle. The most frequent adverse effect was dysphagia, occurring after 44% of all treatments, but this was severe after only 2%. Antibodies to botulinum toxin were detected in the serum of three out of the five patients in whom loss of treatment efficacy occurred. We conclude that botulinum toxin treatment is the most effective available therapy for spasmodic torticollis and practical advice is provided for anyone wishing to set up the technique.

Efficacy and safety of botulinum type A toxin (Dysport) in cervical dystonia: Results of the first US randomized, double-blind, placebo-controlled study
Daniel Truong MD1,*, Drake D. Duane MD2, Joseph Jankovic MD3, Carlos Singer MD4, Lauren C. Seeberger MD5, Cynthia L. Comella MD6, Mark F. Lew MD7, Robert L. Rodnitzky MD8, Fabio O. Danisi MD9, James P. Sutton MD10, P. David Charles MD11, Robert A. Hauser MD12, Geoffrey L. Sheean MD13
Article first published online: 25 FEB 2005
DOI: 10.1002/mds.20403
Copyright © 2005 Movement Disorder Society
Issue
Movement Disorders
Volume 20, Issue 7, pages 783–791, July 2005
Abstract
Botulinum toxin type A (Dysport) has been shown in European studies to be a safe and effective treatment for cervical dystonia. This multicenter, double-blind, randomized, controlled trial assessed the safety and efficacy of Dysport in cervical dystonia patients in the United States. Eighty patients were randomly assigned to receive one treatment with Dysport (500 units) or placebo. Participants were followed up for 4 to 20 weeks, until they needed further treatment. They were assessed at baseline and weeks 2, 4, 8, 12, 16, and 20 after treatment. Dysport was significantly more efficacious than placebo at weeks 4, 8, and 12 as assessed by the Toronto Western Spasmodic Torticollis Rating Scale (10-point vs. 3.8-point reduction in total score, respectively, at week 4; P ≤ 0.013). Of participants in the Dysport group, 38% showed positive treatment response, compared to 16% in the placebo group (95% confidence interval, 0.02–0.41). The median duration of response to Dysport was 18.5 weeks. Side effects were generally similar in the two treatment groups; only blurred vision and weakness occurred significantly more often with Dysport. No participants in the Dysport group converted from negative to positive antibodies after treatment. These results confirm previous reports that Dysport (500 units) is safe, effective, and well-tolerated in patients with cervical dystonia. © 2005 Movement Disorder Society

it seems pretty safe

Its very safe. Effective and reversible obviously as it loses efficacy but its action(temp weakness) must be considered

[doria]

Dr Sclafani, you worked on both my jugular veins back in March and I have recently developed laryngospasm. Could it be related? I've learned that it is usually due to an injured nerve. Do you come in contact with the larnyx during ballooning the jugulars? Thanks, Dori

[Cece]

A link to a very in-depth answer from Dr. Sclafani over on his Facebook page. Question was whether MRV would be useful as a nonoperative imaging tool in assessment of upper jugular narrowings.

I didn't know that the condylar emissary veins were part of the fetal circulatory system and were supposed to atrophy when the jugular developed as part of the adult system!

Maybe the next paper can be on the condylar emissary veins and the light they shed on the origins of CCSVI, if that is not yet part of the dialogue? The enigma was stated as such: patients with MS tend to have either very large condylar emissary veins, as I did, or very small ones. My understanding was that patients with large condylar emissary veins, going through the bony canals of the skull that could only have enlarged during fetal development, must have had significant outflow obstructions even as a fetus. But what could the very small or nonexistant condylar emissary veins signify? Could these patients have a defect in their ability to grow collaterals, thus their condylar emissary veins did not expand? If very small conydlar veins are atypical, it must signify something. A defect in the ability to grow collaterals, coupled with an outflow obstruction, would make a bad situation worse.

It was very interesting to read how he would look at the condylar emissary veins to distinguish between a hypoplastic upper jugular narrowing and a recanalized thrombosis upper jugular narrowing.

[drsclafani]

A link to a very in-depth answer from Dr. Sclafani over on his Facebook page. Question was whether MRV would be useful as a nonoperative imaging tool in assessment of upper jugular narrowings.

I didn't know that the condylar emissary veins were part of the fetal circulatory system and were supposed to atrophy when the jugular developed as part of the adult system!

Maybe the next paper can be on the condylar emissary veins and the light they shed on the origins of CCSVI, if that is not yet part of the dialogue? The enigma was stated as such: patients with MS tend to have either very large condylar emissary veins, as I did, or very small ones. My understanding was that patients with large condylar emissary veins, going through the bony canals of the skull that could only have enlarged during fetal development, must have had significant outflow obstructions even as a fetus. But what could the very small or nonexistant condylar emissary veins signify? Could these patients have a defect in their ability to grow collaterals, thus their condylar emissary veins did not expand? If very small conydlar veins are atypical, it must signify something. A defect in the ability to grow collaterals, coupled with an outflow obstruction, would make a bad situation worse.

It was very interesting to read how he would look at the condylar emissary veins to distinguish between a hypoplastic upper jugular narrowing and a recanalized thrombosis upper jugular narrowing.

Cece
large emissary veins does not prove hypoplasia of J3. obstruction of the more common J1 valve stenoses can also provide a good reason for the persistence of the condular veins.

so my analysis of the condylar veins as discussed was related to trying to differentiate a narrowing of the J3 segment caused hypoplasia from a narrowing caused by a stenosis.

[NZer1]

Dr. is it likely that flow rates from all exiting veins (at the point of exit) is the key rather than looking for slow flow areas once the flow has exited. If it isn't getting in or through then what comes out is "so what".
The brain internal flow is known to be different in PwMS, the reason is not.

In my mind looking at this as a plumber would, you have to know what you are starting with before you can expect or improve the outflow?

After that time there would be reason to look at back flows etc.

If it ain't going in, it ain't coming out and when it does it has issues as well?

Regards Nigel