Internal jugular vein duplication: new malformation

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Internal jugular vein duplication: new malformation

Postby frodo » Tue Nov 01, 2011 3:06 pm

This is an anecdotal report but interesting, nevertheless.

Internal jugular vein duplication: a further truncular malformation in a patient with multiple sclerosis.

Abstract

Different internal jugular vein (IJV) abnormalities can be found in patients with multiple sclerosis (MS): stenoses, complete occlusion, distortions and intraluminal structures, such as membranes, webs and inverted valves. IJV duplication is a very rare phenomenon. We report a case of right IJV duplication as an incidental finding during IJV morphological and haemodynamic assessment in a patient with MS.

A 55-year-old female patient was admitted to our Institute for IJV and vertebral veins morphological and haemodynamic assessment. During the last seven years she had been treated for MS. Colour Doppler ultrasonography in our patient did not reveal IJV or vertebral veins stenoses or abnormal valves, but instead right IJV duplication. This finding was confirmed using multislice computed tomography angiography and by selective phlebography. In conclusion, to our knowledge, a case of IJV duplication in a patient with MS has not been described yet. This further venous malformation can be assessed by the means of Doppler ultrasounds.

http://www.ncbi.nlm.nih.gov/pubmed/22037279
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Re: Internal jugular vein duplication: new malformation

Postby Cece » Tue Nov 01, 2011 3:52 pm

Interesting. This was in Serbia. It says that no IJV stenoses or abnormal valves were found, so I am guessing the patient was not treated. Has anyone read the full paper? I am wondering if it is a full duplication of separate IJV veins or if they are sharing a common wall. Fenestration of the common wall was once suggested as a treatment for IJV duplication.

My guess, if the patient was not treated at all, is that there may have been an additional missed IJV stenosis in the other IJV or an azygous stenosis. I am used to CCSVI abnormalities coming in multiples. A duplication on the right seems like something the body could compensate for by using the left jugular for more of the flow.

The way a duplication can affect the hemodynamics is if the second IJV fills up and then presses on the first IJV, cutting off flow in that IJV.
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Re: Internal jugular vein duplication: new malformation

Postby frodo » Tue Nov 01, 2011 4:06 pm

Cece wrote:Interesting. This was in Serbia. It says that no IJV stenoses or abnormal valves were found, so I am guessing the patient was not treated. Has anyone read the full paper? I am wondering if it is a full duplication of separate IJV veins or if they are sharing a common wall. Fenestration of the common wall was once suggested as a treatment for IJV duplication.

My guess, if the patient was not treated at all, is that there may have been an additional missed IJV stenosis in the other IJV or an azygous stenosis. I am used to CCSVI abnormalities coming in multiples. A duplication on the right seems like something the body could compensate for by using the left jugular for more of the flow.

The way a duplication can affect the hemodynamics is if the second IJV fills up and then presses on the first IJV, cutting off flow in that IJV.


It is not clear either but I suppose the duplicated vein has its own valve. Your explanation about how hemodynamics is affected sounds convincing.
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Re: Internal jugular vein duplication: new malformation

Postby Cece » Tue Nov 01, 2011 4:39 pm

Technically it was Dr. Sclafani's explanation.... :)
He compared the duplication to a windsock, that distends and presses on the jugular wall. He has seen this on IVUS.
chronic-cerebrospinal-venous-insufficiency-ccsvi-f40/topic10680-4305.html#p148742
chronic-cerebrospinal-venous-insufficiency-ccsvi-f40/topic10680-4320.html#p148796
What Dr. Sclafani is describing is "an incomplete duplication of 206 cm of the jugular vein centrally. it shares a common wall with the true jugular vein. and an orifice with the sublcavian/inominate." It was not the same as a fully duplicated IJV, as described and imaged in this article: http://www.ispub.com/journal/the_intern ... ature.html

Still not sure which sort of duplication is seen in this patient. My guess would be that it is the windsock version that Dr. Sclafani described. A full duplication, as in the linked article, might actually increase outflow rather than limit it.
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Re: Internal jugular vein duplication: new malformation

Postby drsclafani » Tue Nov 01, 2011 9:58 pm

Cece wrote:Technically it was Dr. Sclafani's explanation.... :)
He compared the duplication to a windsock, that distends and presses on the jugular wall. He has seen this on IVUS.
chronic-cerebrospinal-venous-insufficiency-ccsvi-f40/topic10680-4305.html#p148742
chronic-cerebrospinal-venous-insufficiency-ccsvi-f40/topic10680-4320.html#p148796
What Dr. Sclafani is describing is "an incomplete duplication of 206 cm of the jugular vein centrally. it shares a common wall with the true jugular vein. and an orifice with the sublcavian/inominate." It was not the same as a fully duplicated IJV, as described and imaged in this article: http://www.ispub.com/journal/the_intern ... ature.html

Still not sure which sort of duplication is seen in this patient. My guess would be that it is the windsock version that Dr. Sclafani described. A full duplication, as in the linked article, might actually increase outflow rather than limit it.


i would hold off on accepting this diagnosis without a full read.

the more likely thing is either a vertical septum dividing the vein in two but only one of the two iJV would be able to go through the jugular canal into the dural sinus.....or this is an external jugular branch draining into the jugular vein.

this last scenario is the reason that i always get at least a guidewire to go through the jugular canal and into the dural sinus. This proves jugular vein....i have never seen two neg veins paralleling each other with BOTH veins connecting with the dural sinus

i doubt this is a true complete duplication
Salvatore JA Sclafani MD
Patient contact: ccsviliberation@gmail.com
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Re: Internal jugular vein duplication: new malformation

Postby Cece » Tue Nov 01, 2011 10:39 pm

I took the article at its word that it was a duplication! You are right, those are other possibilities.

There are a small number of publications on IJV duplications, and none of them mention the patient as being an MS patient. Most have the veins staying separate after they split but here is one where the two IJVs merged back into one IJV: http://www.ajnr.org/content/25/8/1433.full
The appearance of an internal jugular vein on CTA images is characteristic. The vein begins its course with a single lumen (A, B). It then bifurcates forming two distinct lumena (C). As the vein continues caudally, it once again becomes a single vessel (D, E). Panel F shows the fenestrated internal jugular vein reformatted in a single sagittal, oblique plane.
The internal jugular vein is the great collecting vein of the cranium, face, and anterior neck. It is the continuation of the sigmoid sinus beginning at the jugular foramen, where it drains most of the venous blood from the brain as well as CSF. The internal jugular vein continues inferiorly from the skull base until it joins the subclavian vein to form the brachiocephalic vein.

Fenestration of the internal jugular vein is uncommon. There have been five previously published reports of seven cases of a fenestrated internal jugular vein (2–6). One study estimated that fenestration of the internal jugular vein occurs in as much as 0.4% of the population (2).
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Re: Internal jugular vein duplication: new malformation

Postby Cece » Tue Nov 01, 2011 10:55 pm

http://www.ijps.org/article.asp?issn=09 ... last=Paolo
Duplication of the internal jugular vein is a rare congenital anomaly. The vein divides into two branches that separately enter the subclavian vein. Almost always it involves the upper third of the vein.[2],[3] In our case, the duplication was 2 cm below the base of the skull.

Duplication of the internal jugular vein is usually reported in association with phlebectasia, which is a soft non-pulsatile cervical swelling that increases in size during Valsalva maneuver. [4] In our case, no aneurysm or phlebectasia was observed.

Would a duplication of this sort slow down the outflow? I don't think it would. I don't think this sort of duplication is relevant to CCSVI.
A duplication that started 2 cm below the base of the skull would be below the dural sinuses.

There's a really clear cadaver image of a short (2.7 cm) area of IJV fenestration in this one:
http://www.ijav.org/2011/ijav_2011_144-146.pdf

Clarifying the terminology:
Fenestrations or duplications of the IJV are rare variations,
but there is an important difference between fenestration
and duplication. Downie et al. (10) suggested
that the term ‘duplication’ should be used for those cases
where the branches of the anomalous vessel, regardless
of artery or vein, remain separate along the whole
length of their normal course and that the term ‘fenestration’
should be used for those cases where branched
vessels rejoin a single normal vessel.

http://www.google.com/url?sa=t&rct=j&q= ... LNZ7jESqsQ
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