I would like to know treatment options for vertebrobasilar
dolichoectasia with a moderate mass effect on the medulla.  No
apparent aneurysm is apparent.  I would like to see articles from
current literature and commentary.

---

Clarification of Question by rmclb-ga on 11 Sep 2005 18:33 PDT

This condition is also associated with headaches, although eye
problems could be another source of these headaches.  What does the
literature and show about the probability of vertebrobasilar
dolichoectasia causing the headaches?

This is not a fusiform aneurysm.  The vertebrobasilar dolichoectasia
deforms the left anterolateral aspect of the medulla.

I am fully aware, although I am being specific, that I am not asking
for medical advice.  I am just asking for theoretical commentary on
what is presented.

Hi rmclb-ga, and thanks for your question.  My sincere condolences on
your unfortunate condition.  As you state, this answer is not a
substitute for medical advice or direct medical evaluation.

The literature on vertebrobasilar dolichoectasia (VBD) is somewhat
limited, and I will try to focus on the most relevant and
scientifically meaningful reports.  To keep things organized, I have
placed the full references at the bottom of this answer and will
reference them with the text, e.g., [Ref #1].  I discuss the possible
association with headaches within the body of the answer.  There is a
summary at the end.

____________________

First, let me give some background information on VBD.  Dolichoectasia
refers to elongation and distention.  For example, in some people, the
descending aorta weaves back and forth as it descends.  This is
referred to as ectasia.

Here is a page that describes VBD, as well as the major secondary effects:
http://rad.usuhs.mil/medpix/radpix.html?mode=single&comebackto=mode%3Dgeo_browse&recnum=1402

It is also helpful to have a visual picture of the vertebrobasilar
arterial anatomy.  Here is a good diagram (viewed from below and just
behind the brain):
http://www.healthopedia.com/pictures/vertebrobasilar-circulatory-disorder.html

Here is a more detailed diagram showing the arterial anatomy along the
brainstem, along with the Cranial Nerves, which is very useful for
understanding the discussion below.  The discussion of stroke on this
page is not particularly relevant to this topic, however.
http://www.tchain.com/otoneurology/disorders/central/tia.html

____________________

Drs. Rabb and Barnwell at the Oregon Health Sciences University (OHSU)
[Ref #2] reported two cases of subarachnoid hemorrhage resulting from
VBD.  Their introductory paragraph nicely summarizes the current
thinking on VBD:

"Vertebrobasilar dolichoectasia (VBDE) is an uncommon entity that
afflicts less than 0.05% of the population (1-3). Traditionally, VBDE
has been regarded as atherosclerotic in nature, much like aneurysms of
the peripheral vascular system. However, recently, Mizutani and Aruga
(8) suggested that some cases represent a dissecting process. Symptoms
resulting from this structural abnormality are generally thought to
fall into two groups: 1) those resulting from compression of adjacent
cranial nerves and the brain stem, and 2) ischemic events (1,4,5,7,9).
Although subarachnoid hemorrhage has been reported to occur in
association with anterior circulation fusiform aneurysms and in rare
cases of posterior circulation dolichoectasia, the concept that VBDE
may hemorrhage has not widely been recognized (1,6,7).

In recent years, the aggressiveness with which cerebrovascular
ischemic events are managed has increased, and anticoagulation and
thrombolysis are being used with increasing frequency. If a patient
sustaining a cerebral infarct were also observed to harbor a vascular
entity known to hemorrhage, one should carefully weigh the potential
for exacerbating a possible intracranial hemorrhage before instituting
anticoagulation or thrombolysis. We report two cases of patients with
VBDE who presented with ischemic events, were anticoagulated, and
subsequently suffered massive fatal subarachnoid hemorrhage."

Interestingly, one of these patients presented only with headaches. 
More worrisome is that, as in the cases reported in [Ref #2], is that
VBD can result rupture or leakage with resultant subarachnoid
hemorrhage.  Although VBD is not the classic basilar aneurysm, the
symptoms associated with ruptured VBD would likely be similar, i.e.,
severe headache and mental status changes.  As the authors state, that
VBD lesions can hemorrhage is an important point to consider when
weighing treatment options.  Also, a portion of the neurosurgery
literature describes certain types of VBD as fusiform aneurysms.  The
typical basilar aneurysm is sacular and occurs at the top of the
basilar artery.  See, for example, this abstract:
http://www.neurosurgery-online.com/pt/re/neurosurg/abstract.00006123-200411000-00033.htm;jsessionid=Dq292NPkJrfTd07OZYXjyABEqsKejzJjmYz0X1tqgzWYx40sofpU!514292530!-949856145!9001!-1

____________________


There isn't a great deal of literature on VBD, as I mentioned above,
primarily due to the rarity of the condition.  The authors of [Ref #2]
summarize the situation:

"The rarity with which VBDE is noted accounts for the paucity of
information in the literature concerning its natural history. As
stated previously, the primary presenting symptoms associated with
VBDE have been generally thought to be either ischemic or related to
compression of cranial nerves. It has been suggested that patients
with markedly ectatic arteries are more likely to develop ischemic
symptoms than those with purely elongated arteries (11). None of their
patients experienced hemorrhage. They point out that once the
diagnosis has been made using CT scans, angiography may not be
warranted to further confirm the diagnosis."

Goyal, et al., [Ref #3] also give a good summary in their introductory paragraph:

"Intracranial arterial dolichoectasia is characterized by enlargement,
tortuosity or elongation of major arteries at the base of the brain.
It usually involves distal vertebral arteries, basilar artery or
distal internal carotid artery. Vertebrobasilar dolichoectasia (VBDE)
is relatively rare. It generally manifests as compression of cranial
nerves or the brainstem, ischemic stroke or transient ischemic attacks
(TIA) and rarely as intracerebral hemorrhage.[1],[2] "

Within their discussion [Ref #3], the authors describe the current
thinking on how VBD may come about and other conditions with which it
can be associated:

"The most common site of dolichoectasia is the vertebrobasilar system.
The basilar artery is judged elongated if at any point along its
course, it lies lateral to the margin of the clivus or dorsum sellae
or if its bifurcation is above the plane of the suprasellar cistern,
and the artery is considered ectatic if the diameter of the artery is
greater than 4.5 mm. The factors responsible for the development of
dolichoectasia are unclear. The degeneration of the vascular wall due
to atherosclerosis alone or in association with arterial hypertension
is suggested as the pathogenetic factor. However, others consider VBDE
to be a congenital vascular anomaly on the basis of histological
observations of defects in the internal elastic lamina and thinning of
the media secondary to smooth muscle atrophy.[3] Also, VBDE is
suggested to be a component of generalized ectasia of the intracranial
vessels[4] and is associated with ectasia in other vascular
territories and with aneurysm of the abdominal aorta.[4] Rarely, it
has been documented in Fabry's disease.[5]"

==================
Headaches and VBD
==================

A fairly recent article [Ref #1] by Passero, et al., looks at exactly
the situation you describe.  Their study included patients
specifically with compression of the brain stem, including the pons
and medulla (as in your case), divided out into subgroups by which
portion of the brain stem was affected (i.e. medulla, pons, or
midbrain).  Here is an excerpt of their paper regarding their
conclusions:

"Neurovascular compression of the brain-stem, even with severe
distortion, is seldom associated with overt clinical signs, whereas
subclinical dysfunctions are relatively frequent. The central pathways
of the BR [blink reflex] and the corticospinal pathways are more
susceptible to compression than acoustic and sensory pathways. BR, MEP
[motor evoked potentials] and BAEP [brain-stem auditory evoked
potentials] data provide a functional evaluation of the brain-stem and
some cranial nerves, which is lacking in imaging studies. Functional
investigations may be useful in the long-term management of these
patients, since VBD may be progressive and surgical correction may be
required at some stage."

These findings do not describe a mechanism by which VBD might cause
the types of headaches you describe, although other patients with VBD
do have headaches that can be directly or secondarily attributed to
VBD.  Of note, VBD is often not associated with easily elicited
clinical signs or symptoms.

That being said, the site mentioned above,
http://rad.usuhs.mil/medpix/radpix.html?mode=single&comebackto=mode%3Dgeo_browse&recnum=1402
in their discussion of secondary effects of VBD mentions that the
clinical symptoms that do become apparent in VBD are the result of
compression of the Cranial Nerves that lie along the course of the
vertebrobasilar arterial system.  At this point, I suggest referring
to the diagram previously mentioned showing the arterial anatomy along
with the Cranial Nerves:
http://www.tchain.com/otoneurology/disorders/central/tia.html

____________________

The order in which VBD typically involves the Cranial Nerves (CN) is
as follows: VII, V, III, VIII, and VI.  As one might expect, based on
this information, "Trigeminal neuralgia associated with hemifacial
spasm is one commonly encountered combination," as the USUHS site
above mentions.  Trigeminal Neuralgia is a very debilitating condition
with severe facial pain (There have been patients who have tried to
commit suicide.).  Involvement of CN VII typically results in
hemifacial spasm or paresis, while involvement of CN V results in
trigeminal neuralgia.  You can read more about trigeminal neuralgia
here, at eMedicine:
http://www.emedicine.com/NEURO/topic593.htm

Widening of the vertebrobasilar arterial system can result in slow
blood flow, clot formation and/or ischemia of the brainstem (including
the medulla).  The USUHS site discusses this:

"Ischemia may be caused secondary to direct mass effect and/or
hemodynamic changes from marked stasis of flow (more common with
larger basilar artery diameters). These patients present with
nystagmus, vertigo, dysarthria, ataxia, hemiparesis and/or seizures.
They frequently have a rapidly progressive downhill clinical course."

Insufficient blood flow through the vertebrobasilar system is termed
vertebrobasilar arterial insufficiency, and can result in a wide
variety of brainstem and other strokes.

____________________

Here are some useful resources describing the functions of the Cranial Nerves:

In diagram form from the classic Netter text:
http://mywebpages.comcast.net/epollak/PSY255_pix/cranial_nerves_netter.JPG

A detailed Cranial Nerve resource with excellent diagrams:
http://info.med.yale.edu/caim/cnerves/

Although it's called "neuroscience for kids," neuroscience is
sufficiently complicated that this is likely a very good place to
start.  This site includes simple text descriptions of the primary
functions of the Cranial Nerves:
http://faculty.washington.edu/chudler/cranial.html

========
Treatment
========
Rabb and Barnwell [Ref #2] nicely summarize the VBD treatment dilemma:

"Once the diagnosis of VBDE has been made, the decision regarding what
treatment should be instituted is formidable. The rather grim natural
history of the underlying disease process precludes satisfactory
attempts at anything other than palliative treatment. It is unlikely
that in the near future we will witness any definitive surgical repair
of the lesion itself. Cases of cranial neuropathies may respond to
microvascular decompressive procedures, and patients with
hydrocephalus often are helped by cerebrospinal fluid diversion."

By cerebrospinal fluid (CSF) diversion, the authors mean, for example,
a ventriculoperitoneal shunt - a tube with a pressure valve going from
within the CSF filled spaces within the brain down to the abdomen to
decompress the ventricles of the brain and relieve the pressure.

As the authors state, there are currently no good complete surgical or
endovascular procedures for complete repair of VBD.  Part of the
reason for this is the difficult location of the vertebro-basilar
arterial system.  It is very difficult to gain surgical access to the
brain stem.  Because VBD can cause compression of one or more cranial
nerves, one treatment option is to use microneurosurgical techniques
to decompress individual cranial nerves as they become involved.  Such
a procedure is a major undertaking with a significant risk of side
effects, which must be weighed against the symptoms being treated.


Another group in Japan [Ref 4] describe two cases where an ectatic
vertebral artery compressed the medulla.  In both of these cases, the
neurosurgeons were able to successfully detach the vertebral artery
from the medulla and anchor it to the adjacent dura mater (the tough
outer covering of the brain).  The authors go on to discuss other
reports that describe vascular decompression of the medulla for this
type of condition:

"There are few reports describing a tortuous vertebral artery
compressing the medulla oblongata and causing neurological deficits,
such as pyramidal tract signs or lower cranial nerve deficits. In
1985, Kim et al. (5) reported the first such case. Their patient
responded well to vascular decompression. Kobayashi et al. (6)
reported a patient with compression of the medulla oblongata by the
vertebral arteries bilaterally associated with a Chiari malformation,
in which the symptoms improved after vascular decompression of the
medulla oblongata. Except for the patient reported by Tanaka et al.
(10), in whom the involved artery was an excessively long basilar
artery, the vessel responsible is usually a tortuous vertebral artery,
but never one with a ?megadolicho? shape."

Making the decision to go to surgery is a difficult one:
"It is not easy to determine when surgery is indicated. For hemifacial
spasm or trigeminal neuralgia, surgical microvascular decompression is
indicated by the clinical status, even when no apparent offending
vessels are observed on MRI scans or magnetic resonance angiograms. On
the other hand, in symptomatic patients in whom compression of the
medulla oblongata by the vertebral artery is suspected, a definite
diagnosis should be made with the aid of MRI as well as with a precise
neurological examination. In our two patients, the deformity of an
apparently compressed medulla oblongata was visible on the MRI scans."

The bottom line seems to be that if a patient is quite symptomatic and
the vertebral artery seems to be the offending vessel via compression
of the medulla, microsurgical vascular decompression may be an option.
 Surgery is more difficult if the basilar artery is primarily involved
along its course after the junction of the right and left vertebral
arteries.


There are several types of surgery that might be performed for
vascular decompression of the medulla.  Hongo, et al., [Ref #4]
summarize these nicely:

"There are several possible methods of surgical vascular
decompression. Simply inserting a prosthesis between the brainstem and
the offending vessel can usually decompress the typical hemifacial
spasm or trigeminal neuralgia. However, with compression of the
medulla oblongata by the vertebral artery, the offending artery may be
thick and quite difficult to decompress by this method. Fukushima (3)
and Kondo (7) proposed lifting and anchoring the offending vessel to
the nearby dura mater with a variety of materials. In our cases, the
tortuous vertebral artery was shifted and anchored to the dura mater
with a Teflon sheet in Patient 1 and Gore-Tex in Patient 2, without
inserting a prosthesis. Good vascular decompression was achieved,
which improved the neurological deficits. The vertebral artery should
be shifted carefully, to avoid damaging small branches or perforating
arteries from the vertebral artery entering the medulla oblongata. We
previously reported a patient treated by sectioning the tortuous
vertebral artery to produce vascular decompression of the medulla
oblongata (4). This is the ultimate way to achieve vascular
decompression, but indications for this approach may be quite limited.
Shifting the vessel and anchoring it to the dura mater is a more
appropriate choice."


Dr. Ubogu, et al., [Ref #7] at Case Western advocate early surgical intervention:

"Earlier surgical intervention is of paramount importance
and might have resulted in fewer sequelae from this vas-
cular anomaly. The mild improvements noted in this case
indicate that early recognition and surgical intervention in
the setting of symptomatic VA dolichoectasia may be ben-
eficial in reversing neurological deficits and improving out-
comes."

=============

So, to summarize, VBD is difficult or impossible to treat with
definitive surgery because there are multiple small blood vessels
supplying the brainstem from the basilar artery.  Tearing these
vessels would result in brainstem stroke.  There are multiple means by
which VBD causes symptoms, including ischemia, direct pressure (for
example, against the medulla or Cranial Nerves), or hemorrhage. 
Current surgical techniques aim to provide symptomatic relief by
decompressing the brainstem and/or providing improved blood flow
through the vertebrobasilar arterial system.  There is not enough
experience yet with endovascular (e.g. treatment from within the
artery) and wrapping (reinforcement of the area of dilatation with
Gore-Tex or other device) to determine if these methods are
efficacious in the long term.

VBD can cause headaches through multiple mechanisms and evaluation of
your specific condition would be required to determine if VBD is in
fact the causative agent.

I hope this information is useful.  Best of luck with your treatment
and recovery.  If you do opt for surgery, I highly recommend finding a
skilled neurosurgeon with training in skull base surgery.

Please feel free to request any clarification.

Best,

               -welte-ga

______________________________________

=========
References
=========

[Ref #1]
Passero S. Rossi S. Giannini F. Nuti D. Brain-stem compression in
vertebrobasilar dolichoectasia. A multimodal electrophysiological
study. [Journal Article] Clinical Neurophysiology. 112(8):1531-9, 2001
Aug.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11459694&query_hl=1
The full text reprint can be requested from Dr. Passero at this address:
passero@unisi.it
_____

[Ref #2]
Rabb CH. Barnwell SL. Catastrophic subarachnoid hemorrhage resulting
from ruptured vertebrobasilar dolichoectasia: case report. [Case
Reports. Journal Article] Neurosurgery. 42(2):379-82, 1998 Feb.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9482190&query_hl=3
You can request the full text from Dr. Barnwell at OHSU:
http://www.ohsu.edu/neurosurgery/faculty/slb.shtml
http://www.ohsu.edu/dotter/barnwells.htm
barnwesl@ohsu.edu
_____

[Ref #3]
Panda S, Goyal V, Gupta V, Singh S, Srivastava T, Padma MV, Behari M.
Vertebrobasilar dolichoectasia presenting as lower cranial nerve
palsy. Neurol India 2004;52:279-279

Panda S, Goyal V, Gupta V, Singh S, Srivastava T, Padma MV, Behari M.
Vertebrobasilar dolichoectasia presenting as lower cranial nerve
palsy. Neurol India [serial online] 2004 [cited 2005 Sep
15];52:279-279. Available from:
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2004;volume=52;issue=2;spage=279;epage=279;aulast=Panda

Reprint of article can be requested from Dr. Goyal:
drvinaygoyal@hotmail.com
_____

[Ref #4]
Hongo K. Nakagawa H. Morota N. Isobe M. Vascular compression of the
medulla oblongata by the vertebral artery: report of two cases. [Case
Reports. Journal Article] Neurosurgery. 45(4):907-10, 1999 Oct.

You can request a reprint from Dr. Hongo (he's the Chairman of the
Dept. of Neurosurgery at Shinshu University in Japan):
kakizawa@hsp.md.shinshu-u.ac.jp
http://dept.md.shinshu-u.ac.jp/i-noge/index.html
http://www.neurosurgerytoday.org/findaneuro/details.aspx?PersonId=15055
_____

[Ref #5]
Dziewasa R. Freund M. Ludemann P. Muller M. Ritter M. Droste DW.
Stogbauer F. Treatment options in vertebrobasilar dolichoectasia--case
report and review of the literature. [Case Reports. Journal Article]
European Neurology. 49(4):245-7, 2003.
Reprint available from Dr. Dziewasa:
dziewas@uni-muenster.de
_____

[Ref #6]
Stefano Passero, and Giuseppe Filosomi.  Posterior Circulation
Infarcts in Patients With Vertebrobasilar Dolichoectasia.  Stroke 29:
653-659

The full text of this article is freely available here:
http://stroke.ahajournals.org/cgi/content/full/29/3/653
_____

[Ref #7]
Ubogu EE. Chase CM. Verrees MA. Metzger AK. Zaidat OO.
Cervicomedullary junction compression caused by vertebral artery
dolichoectasia and requiring surgical treatment. Case report. [Case
Reports. Journal Article] Journal of Neurosurgery. 96(1):140-3, 2002
Jan.
Full text is here:
http://64.233.161.104/search?q=cache:zXD94fD6ye4J:www.thejns-net.org/jns/issues/v96n1/pdf/n0960140.pdf+vertebrobasilar+dolichoectasia+treatment&hl=en

You can request a reprint of the article from Dr. Ubogu:
eru.bugzy@excite.com
_____

[Ref #8]
Cheung RT. Mak W. Two chinese patients with vertebrobasilar
dolichoectasia. [Case Reports. Letter] Stroke. 29(10):2213-5, 1998
Oct.
Full text is available free here:
http://stroke.ahajournals.org/cgi/content/full/29/10/2213

This is a letter to the editor of the journal Stroke and further
discusses some of the mechanisms by which a variable presentation of
VBD arises.


==========
Bibliography
==========
Current literature on the topic, including those references cited above...

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=====
Search terms using Google, and the Ovid and PubMed medical literature databases:

vertebrobasilar dolichoectasia

vertebrobasilar dolichoectasia treatment

corticospinal tract

cranial nerves [images]

Another useful technique is to look up the references of a
particularly useful or authoritative paper (the way literature
searches were done in the past!).

---

Request for Answer Clarification by rmclb-ga on 16 Sep 2005 19:08 PDT

Many thanks for what looks like a useful answer. There's a lot in it
and I will consider it carefully.

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Clarification of Answer by welte-ga on 17 Sep 2005 07:41 PDT

Hi rmclb-ga,  Thanks for the high rating and kind remarks.  Please let
me know if you would like any further info on any of the papers within
the Bibliography that I did not directly reference within the body of
my answer.

Best,

        -welte-ga

Well worth $200.  The research and compilation of information was
obviously the result time, effort and expertise that produced useful
information on an arcane subject.