EPT VADIC .pdf
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Title: Engelman Domino Pressure Theory on Chiari Malformation associated with EDS
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Engelman Domino Pressure Theory on Vertebral Artery Dissection Induced Chiari : (EDPT / VADIC)
By Gary Engelman, BSN, RN
Chiari malformation is thought to be a congenital condition, however there are also cases of
acquired Chiari malformation. Incidental MRI findings show many asymptomatic individuals
with this malformation. There are also those with Chiari Zero (without tonsillar herniation),
who present with identical symptoms of those with the other forms of Chiari malformation
(with tonsillar herniation). The author theorizes that Acquired Chiari malformation is caused
by a primary Vertebral Artery Dissection (VAD) causing high Intracranial Pressure (ICP), and
Pseudotomor Cerebri (PS), also known as Idiopathic Intracranial Hypertension (IIH). This
cascade of events will in turn cause an increase in Cerebral Spinal Fluid (CSF) pressure, thus
decreasing Cerebral Spinal Fluid (CSF) flow. The triad of events will displace the Cerebellum
Tonsils downward into the Foramen Magnum opening and downward into the Spinal Fluid
Canal (SFC), thus giving the Physical finding on MRI of Tonsillar herniation and / or Cerebellar
crowding and possibly, decreased CSF flow, as evidenced by CINE flow studies. Diffusion
studies will demonstrate Cerebellar hyper intensities in those with Cerebral Ischemia
secondary to involvement of the Posterior Inferior cerebral Artery (PICA) (Fig. 1).
Figure 1. Diffusion study showing hyper-intensity signal, right hemisphere of cerebellum.
Those with Primary Vertebral Artery (PVA) involvement and absent PICA involvement may
have adequate posterior Cerebellar perfusion from crossover blood flow down the basilar
artery and into the PICA from the unaffected Vertebral Artery. Autonomic Dysfunction (i.e.
POTS, Horner’s Syndrome (Fig. 4), Hypothalmic Pituitary Adrenal (HPA) dysfunction, etc.) will
result in many symptoms due to Brainstem Medullary Ischemia from the affected Vertebral
Artery. Compression of Vagus nerve (Cranial nerve X) appears to play a big role in Autonomic
Dysfunction. Empty Sella Syndrome (Fig. 5) may or may not be present on MRI as well, as the
Pituitary gland endures the high pressure associated with the above phenomenon. Other
findings on MRI have been associated with either Ethmoid, Maxillary Sinusitis (or both), on the
same side as the affected Vertebral Artery. SPECT imaging will confirm what is seen on
diffusion studies, once again looking at blood flow. The Authors SPECT report is consistent
with decreased blood flow in the right cerebellar hemisphere (Fig. 6).
Figure 2. Right PICA Artery picking up flow from the Left Vertebral Artery. (Depends on
severity of dissection).
Figure 3. Coronal view of MRI showing Ethmoid Sinusitis, (Right Side, same as VAD).
Hypoplastic Artery measuring 2mm can also be seen.
Figure 4. Horner’s Syndrome. Caused by interruption of sympathetic nerve supply to the eye.
Figure 5. Empty Sella Syndrome shown above. Comparison of Sagittal views from 2010 and
2014 shows progressive flattening of Pituitary gland to a full Empty Sella. Low Testosterone of
160 is highly correlated of a dysfunctional pituitary gland. Phenomenon is documented as a
cause from IIH and Pseudotumor Cerebri.
Figure 6 above: SPECT report emphasizing decreased blood flow to the area of the right
The Author conducted a survey question to those with EDS and/or Chiari malformation in his
Facebook Group with approximately 1800 members. Of those members, 236 members
answered the question of whether or not something stressful had happened to them just prior
to the onset of their symptoms. 180 members or (76%) answered affirmatively to this
question. The Author had just lost his young brother unexpectedly and remembers crying very
hard (creating similar pressure as a sneeze), synonymous with a cause of IIH, and high ICP
through both high pressure exerted in the Arterial system and pressure exerted through the
middle ear into the brain itself. Others reported the onset of symptoms after a vaginal
delivery, car accident, sports injury, and other emotional and physical traumas.
Another factor involved in the Authors case, but also seen on the imaging of others with VAD,
is that Tortuosities co-exist and may complicate matters more. The Author has several Arterial
Tortuosities throughout his major arterial supply. There are two kinks seen on his imaging
which under normal circumstances would not be a problem and continue to provide blood
flow around the loops. However, in the case of EDS, the Arterial walls lose their strength and
with added dissection issues, they can collapse or fold in on themselves around the looped
areas (Fig. 7 and 8).
Figure 7 above: Kink is seen below basilar artery, pinching off blood flow. Kink is above the PICA artery, thus
corrective blood flow from the Left Vertebral Artery will not occur.
Figure 8 above: Coronal view of the Tortuosity around C2-C3. Kink is seen again here and
correlates with Angiogram in upper right hand corner. Bending the neck forward creates a
total loss of blood flow as if one were to bend a garden hose in half to halt water flow.
There are many PubMed articles on the subject of VAD available on the internet. Many of
these articles report similar types of findings suggesting the cause of VAD. Examples in the
literature suggesting a spontaneous VAD are: Injuries to the Arteries by way of (EDS, Marfan’s
syndrome, other genetic conditions that weaken the arterial wall, and perhaps an infectious
agent), Traumatic Injuries arising from (traffic collision, strangulation, head trauma, sports
injuries, coughing, sneezing, vomiting, straining bowel movement, vaginal delivery, neck
manipulation through chiropractic adjustment, etc.). The author believes that the above
causes and many others (too many to list) are the direct cause and, if you will, the 1st domino
to fall in the cascade of events which follow as described above, thus the Author’s Domino
Theory. The author also theorizes that those born with Chiari malformation had a physical
problem with the closing of the cranial skull bones during vaginal delivery such as the use of
forceps or pressure exerted on the cranium itself from the pushing and squeezing of child
birth. Because the process of Vaginal Delivery itself can be a cause of Spontaneous (VAD) to
the mother herself, the author concludes this would be the reason why women with Chiari
malformation outnumber men by a ratio of 3 to 1.
The Monro-Kellie hypothesis:
The pressure-volume relationship between ICP, volume of CSF, blood, and brain tissue, and
cerebral perfusion pressure (CPP) is known as the Monro-Kellie doctrine or the Monro-Kellie
The Monro-Kellie hypothesis states that the cranial compartment is incompressible, and the
volume inside the cranium is a fixed volume. The cranium and its constituents (blood, CSF, and
brain tissue) create a state of volume equilibrium, such that any increase in volume of one of
the cranial constituents must be compensated by a decrease in volume of another.
The principal buffers for increased volumes include CSF and, to a lesser extent, blood volume.
These buffers respond to increases in volume of the remaining intracranial constituents. For
example, an increase in lesion volume (e.g. epidural hematoma) will be compensated by the
downward displacement of CSF and venous blood. These compensatory mechanisms are able
to maintain a normal ICP for any change in volume less than approximately 100–120 mL.
The above theory describes some of the physiological changes that are thought to occur in the
Author’s theory. So, in the instance of VAD, when Arterial flow is reduced, to compensate, CSF
production would increase. Over time, this imbalance would seem fit to cause the increased
ICP. As this pressure increases, the environment is such that Cerebellar herniation can occur,
Cerebellar crowding, Empty Sella, and so on. The net pressure effect on the Nerve structures
in the brain can cause things like Trigeminal Neuralgia, Occipital Neuralgia, a Swooshing
sensation in the ear, Blurred vision, and so on. Lastly, at a tissue level, we will see symptoms
such as Brain fog, Short term memory loss, Cognitive deficits, and so on. At a chemical level,
we will see many other problems: Low Dopamine (Parkinsonian like tremors), Serotonin,
Norepinephrine, DHEA, Aldosterone, Cortisol, Insulin, and so on. Many of these chemicals will
affect our overall moods, anxiety, adrenal function, gastrointestinal system, sleep
So, why do some feel better after Surgical Decompression, but then seem to decline, many
reporting they are worse off than before surgery. The Author has yet another theory here. In
the short term, a few things are happening. First off, most people get large amounts of the
Steroid drug Decadron during the surgical decompression surgery. Decadron, not only helps
prevent post-operative swelling, but can also have a net effect on the inflammation one is
already experiencing prior to surgery. VAD in itself produces a very high level of inflammation
in the body. This is noted in the literature and the author has severely elevated IL6 since being
ill. IL6 is very non-specific as are all of the inflammatory cytokines. Nevertheless, if one feels
ongoing pain, he/she can assume there is an associated inflammation in the body, as
inflammation is the cause of pain.
For example: If you jump and land incorrectly on your ankle, a few things will happen. Right
away, the major twist will break open tiny blood vessels and capillaries. This will eventually
result in the bruising effect. But the nerves that become severed or compressed send signals
back up to the brain signaling this injury, thus alerting the chemical cytokine messengers to
send out the troops. In this instance, many inflammatory chemicals rush to the site of injury.
In addition, we will have platelet aggregation to stop any bleeding that may be occurring
under the skin. The eventual swelling that occurs at the site of injury comes from fluid sources
all over the body. The end result is nerve compression at the site of injury. So long as there is
compression on the nerves, a never ending viscous cycle of signals will go back and forth to the
brain producing the effect of pain at the site of injury. As inflammation goes down, these
signals lessen and thus, so does the pain. This feedback system is essential to allow tissues
and blood vessels to heal and to prevent further injury to occur at the site of injury.
So over the next few weeks, the body has increased its own production of steroids as well to
help battle the surgical wounds. In addition, many are mineral depleted at the time of surgery
and many receive tons of IV fluids which help restore hydration status and a bit of sodium
depletion perhaps. Post operatively, many are also on narcotics which may block previous
pain if the patient was not previously on them. They are usually also placed on NSAIDS as well
to help with inflammation, which again, they probably were not on. Additionally, the high CSF
pressure is probably greatly reduced from the surgical procedure itself depending on what was
done. The Author would suspect that those that receive CSF shunts would have a higher
correlation of post-operative symptom relief then those that did not receive one. Assuming
the patient does not receive a CSF shunt, post-operatively, the CSF pressure will once again
build over time, once again producing similar effects that the Author theorizes in the
beginning of this paper. The difference being that post-operatively, the stress that has been
placed on the body from the surgery itself has perhaps added to original stress of the VAD and
perhaps has worsened this condition resulted in new symptomology or the worsening of
Similarly, if the Author is correct, this would coincide with some of the study results outlined
by Dr. Bejjani in his article titled below relating to failed Decompression Surgery:
Treatment Options After Failed Decompression Surgery:
A neurosurgeon from the University of Pittsburgh believes that some cases of failed Chiari
decompression surgery may be due to a partial misdiagnosis and co-existence of another
condition, idiopathic intracranial hypertension (see Side Bar). Dr. Ghassan Bejjani along with
neuro-opthamologist, Dr. Kimberly Cockerham, and their colleagues reported on 6 cases of
failed decompression surgery in the February, 2003 issue of the European journal Acta
Bejjani's study looked at Chiari patients whose primary symptoms returned after
decompression surgery. The six cases included 5 women and 1 man ranging in age from 19 to
43 years. Each patient had a Chiari malformation verified by MRI and neurological symptoms
consistent with Chairi and severe enough to warrant intervention. As to be expected, the most
common symptom was headache along with neck pain, visual disturbances, and weakness and
numbness in arms and legs. None of the six had a syrinx.
The patients all underwent similar decompression surgeries involving a suboccipital
craniectomy, C1 laminectomy, and duraplasty. For all six patients there was some symptom
relief initially, but symptoms began to return 1 to 9 months later. The patients underwent
follow-up MRI's, including cine-MRI to look at CSF flow. The MRI's were reviewed by a
neuroradiologist, who did not know the purpose of the study or the clinical reports of the
patients, to assess whether there was adequate decompression. The radiologist looked at
whether the tonsils had moved up or down after surgery, the shape of the tonsils, and
whether there was CSF flow behind the tonsils. There was very little movement of the tonsils
as compared to before surgery, but in 4 of the patients the tonsils were more rounded in
shape. In addition, there was CSF flow behind the tonsil in all 6 patients. While not conclusive,
these findings are suggestive that surgically, the decompression was adequate and should
have relieved the symptoms.
In addition to the radiological review, 4 of the 6 patients underwent an eye exam by Dr.
Cockerham. All four patients showed signs of elevated intracranial pressure (ICP). Specifically,
the patients lacked what are known as spontaneous venous pulsations (SVPs). In most people,
the veins in the retina (in the back of the eye), fluctuate in size periodically. In a person with
elevated ICP, these fluctuations do not occur. Besides the absence of SVP's, two of the patients
showed additional signs of elevated ICP.
Following the MRI's and eye exams, all six patients underwent a lumbar puncture to both
measure their ICP and relieve it if it was elevated. Most of the patients were near or above the
pressure level considered normal and all patients reported a temporary (about one week)
improvement in symptoms following the lumbar punctures. Because of this, four patients
chose to have ventriculo-peritoneal shunts placed in an effort to drain CSF and lower their ICP.
Two patients chose to undergo periodic lumbar punctures along with taking acetazolamide, a
drug which can lower ICP. Following these treatments, all patients reported significant
improvement in their symptoms and were stable at least 16 months later.
Why did CSF drainage - using shunts or lumbar punctures - work where decompression surgery
failed? The researchers cite several possibilities, including surgical scarring disrupting CSF flow
and inadequate decompression, but speculate that at least some of the patients also suffered
from idiopathic intracranial hypertension - a condition where ICP is abnormally high for
unknown reasons. Bejjani points out that in the case of surgical scarring, the lumbar punctures
should not have relieved symptoms as the procedure is below the level of CSF blockage. While
it is difficult to define an adequate decompression, the MRI's for most patients in this study
showed signs that are typical of an adequate decompression.
In support of the idiopathic intracranial hypertension (IIH) theory, Bejjani points out that IIH
symptoms, as reported, are similar to Chiari and could be difficult to distinguish. In addition,
the eye exams and elevated pressure readings after surgery are suggestive of IIH. Adding to
this theory is that some of the patients were overweight, a predisposing factor for IIH. Perhaps
the strongest piece of evidence that supports the IIH theory however, is that the patients
responded to treatments designed to drain cerebrospinal fluid and didn't respond to the
An association between IIH and Chiari has been identified and discussed previously in the
medical literature, but the exact nature of the relationship is not known. Does a Chiari
malformation cause elevated ICP, or can sustained high pressure actually cause the cerebellar
tonsils to herniate? Much more research will be required to sort out the link between the two
Failed decompression surgery can be devastating to a patient and happens much too
frequently. While there are likely many reasons why surgeries fail - as reported elsewhere in
this issue - for one group of patients, even if it’s a small group, the reason may be because of a
second, often treatable, condition.
Co-morbid conditions associated with VAD:
Postural Orthostatic Tachycardia Syndrome (POTS) is a condition of the Autonomic Nervous
System (ANS) that is often seen in both EDS and Chiari malformation. By definition, POTS is
defined by an increase in heart rate by 30 beats per minute when going from supine to the
upright position. Many with this condition will have a significant drop in blood pressure upon
standing to the point of passing out, while others will have the exact opposite and a large spike
in blood pressure. Then there are the ones like the Author who don’t have much change at all.
So begs the question what is going on here? While some of the ANS Doctors try and treat the
condition with drugs that raise blood pressure, increase fluid retention, and lower heart rate,
the Author believes that this condition is actually an attempt by the Central Nervous System
(CNS) to correct itself to maintain Homeostasis. If we recall the Monro-Kellie hypothesis (Fig.
9) from above, we know that a shift in one fluid causes a shift in another, mainly blood
pressure and CSF production. The Author theorizes that by attempting to correct these ANS
problems, a further imbalance is being created, thus creating further symptoms for the already
sick patient. Let’s look at a few examples that utilize this theory:
Fig. 9 below