Chiari Connection International (CCI) --- The Best In Chiari Resources Worldwide!!

Do you recommend the Medic Alert after decompression surgery?

November 2007
Dr. John Oro:

I have not recommended a Medic Alert Bracelet after decompression surgery. There are currently two young athletes in the news, both having undergone Chiari decompression surgeries, that are back to contact sports. One is playing college football, the other is still in high school playing soccer, baseball and basketball. Although the risks are not zero, for those with an adequate decompression, I have not found a specific warning to be needed.

Dr. Heiss:

I do not recommend a Medic Alert Bracelet for the surgery itself.

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Do you recommend the Medic Alert bracelet for any other condition related to Chiari?

November 2007
Dr. Heiss:

Yes. Listing conditions such as sleep apnea, ataxia (unsteadiness when walking), chronic pain (requiring narcotic treatment), depression, hydrocephalus (presence of a shunt, especially if the pressure is adjustable) and arthritis might be helpful to emergency care providers.

Prof. Stoodley:

I don't think a bracelet is necessary for an uncomplicated Chiari treated with posterior fossa decompression. A bracelet would be recommended for hydrocephalus treated with a shunt or for a complex syrinx involving the upper cervical cord or brain stem.

Dr. Kula:

There is Medic Alert and also American Medical ID:
American Medical ID
Medic Alert

It is pretty much up to the patient and to a degree the anticipated degree to which they may anticipate problems. The primary issue is the risk of neck manipulation should the patient be found unconscious.

The alert could indicate "Chiari I malformation - Avoid hyperextension of the neck." This is, however, generally the policy in emergency treatment following head/neck injury anyway, so the caveat may not add much reassurance. In the case of a need for emergency incubation in the field, the warning may trigger extra caution, but it may also generate extra restraint possibly delaying necessary treatment.

Each patient should probably discuss this individually with their neurosurgeon. The use of an alert may be more indicated in patients with fusions, such as "Chiari I malformation - Caution: Craniocervical fusion to C5 in place." This would at least prepare a medic for an understanding of observed resistance to head/neck movement.

The appropriate use of an alert will be dependent on a number of factors:

1) The presence of a possible fusion
2) The presence of possible joint hypermobility or Ehlers-Danlos problems
3) Prior history of head/neck injury
4) The presence of a syrinx or significant degenerative disc disease

Again, this is all best addressed with the most knowledgeable neurosurgeon or neurologist at hand.

Dr. Frim:

In general, I have only recommended the bracelets for things that help someone if they are brought to an outside ER in a state where they cannot tell their medical history (i.e., after a high speed MVA unconscious, etc.). SO, if there is an implanted shunt, or another implant, that is a must. For people without post-op symptoms, etc., it is probably not needed. If there are ongoing symptoms, etc. then may not is Ok depending on the symptoms.

Misao Nishikawa:

I think that it is not necessary the patients after decompression surgery to put MA bracelet, excepting special cases which have severe symptoms or signs by compression for brain stem and instability. We don't have the standard guideline about it, so far.

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Thoughts On Minimally Invasive Decompressions

January 2008
Paolo Bolognese, M.D.:

These kind of surgeries are currently being pushed by a number of pediatric neurosurgeons, who are concerned about the incidence of complications from the standard CMI decompression surgery, their main fear is the CSF leakage.

CSF leakages occur more often with less experienced surgeons, and this is the MAIN parameter to keep in mind. When one specific surgeon is picked, his chances of CSF leakage will increase as he opens the skull, then the dura, then the arachnoid (which is the last water-proof barrier holding the CSF). Opening the skull alone like in the minimally invasive surgeries does not have a zero risk of CSF leakage, because of an anatomical trap called "cranial lacunae".

To give an idea how much the surgeon (and not the specific kind of surgery) is the major determinant for CSF leakage, read the following stats:

- most of the top national CMI experts average an 8% incidence of CSF leakage, when the dura is open

- less expert neurosurgeons can have an incidence of CSF leakage ranging from 20 to 45%, with the same kind of surgery (= opening the dura)

- the chance to have a CSF leakage with a minimally invasive procedure is around 1-2%

- the incidence of CSF leakage at TCI (where not only we open the dura, but the arachnoid as well, and where we have a high awareness about potential cranial lacunae) is only 0.3%

Minimally invasive surgeries tend also to be minimally effective. The reality is that these surgeries work well only when the following criteria are met:

- the posterior fossa is small, but not very small
- the tonsillar herniation is minimal
- the tonsils are not compressing the brainstem, as mass occupying lesions
- the tonsils are not laterally herniated
- a large syrinx is not present
- brainstem symptoms are minimal or absent

Bottom line: Minimally invasive decompressions work well only with minimal forms of Chiari I; with other forms of Chiari, they tend to provide incomplete and often short-lived clinical improvements

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Why do many of us suffer from insomnia that doesn't necessarily revolve around pain?

March 2008

Michael J. Rosner, M.D.:

Best estimate: In Chiari, the brainstem is dysfunctional due to compression, distortion, abnormal and high velocity CSF flow, distorted input from the periphery, high intracranial pressure, and more. The brainstem is crucial in the establishment of diurnal rhythms, habituation (suppression) of distracting stimuli which include internal events. It helps suppress thought and allows drowsiness to evolve to sleep, dreams, and normal patterns. Many Chiari patients complain of intrusive thoughts, of their minds racing, etc: abnormal sleep. The respiratory center of the medulla may be dysfunctional leading to sleep apnea of central, peripheral or mixed types: unrefreshing sleep. Many other distruptive events can occur in the face of a misbehaving brainstem including excessive hypnagogic jerks, panic-like attacks, nocturia due to a neurogenic bladder….

Misao Nishikawa, M.D.:

We don't have scientific evidence which directly suggest connection between insomnia and CM/SM.

The cases of insomnia should be explained, if the insomnia is induced by sleep disorder (central sleep apnea etc.), that may have relationship among them. Excepting psychological factors, we can expect some relationship between insomnia and CM/SM, according the rouse is controlled by the reticular formation in brain stem.

John D. Heiss, M.D.:

General measures to get a good night sleep include 1) eating earlier in the evening so your stomach is not so full when you go to sleep and 2) avoiding TV and work just before bedtime. Try reading a book or a magazine instead so your thinking can shut down smoothly at bedtime. Sleep apnea (periodic closing of the breathing passages during the dream stage of sleep that results in partially waking up) can be a reason for insomnia, daytime sleepiness, and fatigue. Snoring, obesity, and Chiari I can be associated with sleep apnea. You can ask your primary care physician, an ENT doctor, or a neurologist who specializes in sleep disorders if they think that you may have sleep apnea.

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What causes the sensation of "internal vibration" that so many of us experience?

March 2008

Paolo Bolognese, MD:

It probably comes from the mechanical distorsion of the posterior columns of the spinal cord (Goll and Burdach), which carry information for fine tactile sensation, fine proprioception, position and vibration sense. They carry the sensory information which allows us to feel the vibration of a tuning fork. The posterior columns pass in front of the cerebella tonsils on their way up to the thalamus.

Michael J. Rosner, M.D.:

Best estimate: The brainstem is a relay and 'data-processing' center for incoming stimuli from the entire body-both external sensations (heat, cold, touch, vibration, pain, etc., etc.). If the brainstem misclassifies a light touch as burning pain, then this is what the person perceives. If the normal movement of the GI contents, circulation or other stimuli are misclassified as 'vibration' then this is what one might perceive. There are specific spinal cord pathways which project though the brainstem which carry vibration: I suspect it is a 'misclassification' or 'short-circuiting' of normal sensations that lead to this internal vibration perceived by many with Chiari.

Misao Nishikawa, M.D.:

I think that there are two causes:

1. Vascular lesion : blood flow insufficiency may induce the "internal vibration" in balance organ of inner ear. We always see the compression to anterior inferior cerebellar artery (AICA), which is feeding artery for the inner ear, and posterior inferior cerebellar artery (PICA) by downward displaced brain stem and tonsils during operation.

2. Brain stem (neural) lesion: the compression to the neural structure of the brain stem, which includes balance center (pons-medulla oblongata - cerebellum), may induce that.

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Have you seen a reduction in abdominal symptoms and pain? Diarrhea, constipation, stomach pain with patients having CCI or Decompression surgery? Or is it just in the patients who have had TC surgery that the reduction of these symptoms is seen?

March 2008
Paolo Bolognese, M.D.:

In patients with CCI or CMI, the GI symptoms are due to an involvement of the vagus nerve, or cranial nerve X, which is located in the inferior part of the brainstem.

Michael J. Rosner, M.D.:

Yes. This table contains no tethered cord patients. It is derived from questionnaires completed at regular intervals by my Chiari patients. '0' is 'no problem'. '1' is 'mild', '2' moderate and '3' a 'severe' problem (in the eyes of the patient). The values are averages + standard deviations; the values in square brackets are medians and are the easiest to interpret quickly. Most patients with Chiari do not view their GI complaints as 'severe' though some do. However, most do improve over the course of a year or two. If they do not resolve completely, the complaints are usually more easily managed. (The 'P' value gives the probability that these changes occurred by chance: they did not.)

	Pre-Op N = 217	12 mo Post-Op N = 147	P value
Abdominal Cramps	1.0 + 1.0 [1.0]	0.5 + 0.8 [0.0]	P = 1.7E-05
GERD	1.1 + 1.1 [1.0]	0.8 + 1.0 [0.0]	P = 0.0015
Dysphagia	1.2 + 1.0 [1.0]	0.7 + 0.9 [0.0]	P = 0.00026
Diarrhea	1.0 + 1.1 [1.0]	0.5 + 0.8 [0.0]	P = 7.3E-09
Constipation	1.2 + 1.1 [1.0]	0.7 + 1.0 [0.0]	P = 0.000003
Nausea	1.3 + 1.1 [1.0]	0.7 + 0.9 [0.0]	P = 5.9E-06
Hoarseness	1.0 + 1.0 [1.0]	0.6 + 0.9 [0.0]	P = 0.0012

As you can see, most of these symptoms occur to at least a mild degree in those with Chiari; most significantly improve by one year after surgery.

Misao Nishikawa, M.D.:

We have some cases in which abdominal symptoms and pain have improved after decompression surgery. In those cases, the autonomous nervous function in the brain stem, cervical and thoracic might improved by decompression.

John D. Heiss, M.D.:

I have not recognized a reduction in abdominal symptoms or stomach pain after craniocervical decompression surgery. After TC surgery improvement in these symptoms can occur because of improvement in bowel and bladder function.

Many of the symptoms that are mentioned may result from drug treatment rather than from Chiari I malformation: 1) diarrhea can come from antibiotics; 2) constipation can arise from narcotics; and 3) stomach pain can occur from gastric irritation from the use of steroidal or non-steroidal anti-inflammatory medication (or stress).

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How significant a finding is a sacral dimple and/or small dermal sinus in the diagnosis of a tethered cord?

March 2008
Paolo Bolognese, M.D.:

It is a warning sign for tethered cord

Michael J. Rosner, M.D.:

It is significant enough to raise the question of a cauda equine abnormality in a patient with neurological or musculoskeletal symptoms and signs.

Misao Nishikawa, M.D.:

Sacral dimple and/or small dermal sinus are not always suggest TCS, this is merely one of signs, which suggest connection between neural axis and ectoderm. I cant definitely say, because we don't know the dominator. In our series, there are about 8-9% who have spina bifida occulta.

John D. Heiss, M.D.:

The frequency of sacral dimples is much higher than that of tethered cord syndrome, so most people with sacral dimples do not have tethered cord. The diagnosis of tethered cord syndrome is made by a neurologist or neurosurgeon on the basis of 1) a history of bladder dysfunction and lower extremity dysfunction, 2) findings on neurological examination of dysfunction of the lower part of the spinal cord, and 3) MRI findings that the end of the spinal cord (conus medullaris) lies below (inferior to) its normal location or is associated with adjacent abnormal structures.

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Are PVCs ( Premature Ventricular Contractions) considered to be a potential symptom of CM/SM?

March 2008
Paolo Bolognese, M.D.:

SVT's are the most frequent arrhytmias for CMI. PVC's are high on the list. SM are not responsible for arrhythmias, unless the syrinx cavity extends to the brainstem (syringobulbia).

Michael J. Rosner, M.D.:

No. However, they may occur with other cardiac dysautonomic symptoms which might be caused or amplified by Chiari.. As an isolated symptom, I would be unconcerned.

Misao Nishikawa, M.D.:

Ventricular contraction is controlled by supra atrial (SA) node and atrial ventricular (AV) node electronically. Premature ventricular contraction (PVC) is electronical activity of ventricle, which is deviation from this control. So PVCs is not directory suffered from CM/SM. However we know arrhythmia (bradycardia) are caused by compression (autonomous nerve insufficiency) for brain stem and/or vagus nerve etc.

John D. Heiss, M.D.:

PVC's can occur in anyone and in most patients with CM/SM they are unrelated to CM/SM. Infrequently there are signs of dysfunction of the brainstem or the vagus nerves in patients with Chiari malformations and cardiac irregularity may arise in this context. Cardiologists are experts in diagnosing the cause of PVC's and their treatment.

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What, exactly, are the signs/symptoms of post surgical infection to look for? What should patients watch for, report, or not worry about?

March 2008
Paolo Bolognese, M.D.:

The ancient Romans have a list: tumor, rubor, calor, and functio laesa

--- tumor = the lesion is swollen
--- rubor = it is red
--- calor = it is hot
--- functio laesa = you cannot use that area of your body without extreme discomfort

therefore, watch for the all of the above. Blood testing can show increase of the white cell count in the serum; ESR can go up as well. When pus shows up, it is the ultimate giveaway.

Michael J. Rosner, M.D.:

Progressive pain and tenderness, swelling, redness, drainage of cloudy, purulent (pus) fluid, fever, stiff neck: The problem is that to some degree, most normally healing wounds have some of these characteristics. Normal wounds do not drain frank pus, but slightly cloudy, yellow fluid is not too uncommon. However, the 'normal 'pattern' is for these characteristics to gradually improve over time. Usually this improvement is clear cut by a week after surgery, though pain may well persist for many weeks. When in doubt, call your surgeon.

Misao Nishikawa, M.D.:

The signs of post surgical infections are wound swelling, redness, hot and dirty (pus) discharge from the wound. We never see meningitis or severe infection after TCS surgery (SFT), only 3 cases (0.9%) with wound infection in our series.

John D. Heiss, M.D.:

The most frequent sign of infection is persistent fever (maximum daily temperature exceeding 101.5 degrees Fahrenheit or 38.5 degrees Celsius). Other signs of infection of the wound are redness, swelling, and pain in the area of the surgery. Infection within the CSF and meninges may cause headache and neck stiffness.

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Many with a Syrinx in the t-spine complain of taking a deep breath. It's similar to the "MS Hug", or a girdle sensation. For someone with MS, it's thought that this sensation can be a sign of a new exacerbation, or in the case of a pre-existing spinal lesion, an increase in either ambient or body core temperature. For those with SM, could this be a indication of an increase in the size of the Syrinx?

March 2008
Paolo Bolognese, M.D.:

It could be an indication of an increase in the size (or in the internal pressure) of a syrinx.

Michael J. Rosner, M.D.:

Doubtful that it represents a 'marker' for progression of the syrinx: However, It suggests to me that there is continued dysfunction of the sensory systems including pain. If new complaints arise or old ones return, I restudy my patients looking for inadequate decompression of the Chiari, or co-existent cervical stenosis or disc disease. Thoracic kyphosis from weak axial muscles can also lead to similar symptoms.

Misao Nishikawa, M.D.:

We should think that new symtopms suggest the enlargement of syrinx, it is necessary to do particular neurological evaluations if those symptoms are reliable or valid.

John D. Heiss, M.D.:

No. Normal-sized breaths come from the diaphragm, which receives its nerve supply (the phrenic nerves) from the cervical spinal cord (in the neck). Large breaths also call into play the accessory muscles of breathing that receive their nerve supply from the thoracic spinal cord. So, a large breath (but not a small breath) requires proper function of the thoracic part of the spinal cord. When there is dysfunction of the thoracic spinal cord from syringomyelia, it may be recognized only when you take a deep breath.

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For those with SM, could this be a indication of an increase in the size of the syrinx? Would a course of IV Methyl Prednisone also be helpful for a SMer experiencing this problem?

March 2008

Paolo Bolognese, MD:

I would not recommend it, since it would "cover" the process for a while, preventing the physician to address its cause

Michael J. Rosner, M.D.:

Methyl-prednisolone and other glucocorticoids are great for most of what hurts, but they are non-specific. If restudy yields no treatable problem, then a short course of steroids may break the cycle until something sets it off again.

Misao Nishikawa, M.D.:

I think that methyl predonisone is effective for that situation similary it is effective in multiple sclerosis (MS). However methyl predonisone is not essential treatment for the cases with SM, we should resolve the essential cause of SM.

John D. Heiss, M.D.:

No. It would not help the problem. In MS there are inflammatory cells that attack the substance of the spinal cord; anti-inflammatory steroids like methylprednisolone or prednisone can reduce the amount of inflammation and the amount of spinal cord dysfunction. In syringomyelia, the dysfunction is not from acute inflammation but from chronic spinal cord injury. Anti-inflammatory steroids do not improve chronic spinal cord injury.

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Minimally Invasive Decompressions

March 2008

Paolo Bolognese, M.D.:

These kind of surgeries are currently being pushed by a number of pediatric neurosurgeons, who are concerned about the incidence of complications from the standard CMI decompression surgery, their main fear is the CSF leakage.

CSF leakages occur more often with less experienced surgeons, and this is the MAIN parameter to keep in mind. When one specific surgeon is picked, his chances of CSF leakage will increase as he opens the skull, then the dura, then the arachnoid (which is the last water-proof barrier holding the CSF). Opening the skull alone like in the minimally invasive surgeries does not have a zero risk of CSF leakage, because of an anatomical trap called "cranial lacunae".

To give an idea how much the surgeon (and not the specific kind of surgery) is the major determinant for CSF leakage, read the following stats:

- most of the top national CMI experts average an 8% incidence of CSF leakage, when the dura is open

- less expert neurosurgeons can have an incidence of CSF leakage ranging from 20 to 45%, with the same kind of surgery (= opening the dura)

- the chance to have a CSF leakage with a minimally invasive procedure is around 1-2%

- the incidence of CSF leakage at TCI (where not only we open the dura, but the arachnoid as well, and where we have a high awareness about potential cranial lacunae) is only 0.3%

Minimally invasive surgeries tend also to be minimally effective. The reality is that these surgeries work well only when the following criteria are met:

- the posterior fossa is small, but not very small
- the tonsillar herniation is minimal
- the tonsils are not compressing the brainstem, as mass occupying lesions
- the tonsils are not laterally herniated
- a large syrinx is not present
- brainstem symptoms are minimal or absent

Bottom line: Minimally invasive decompressions work well only with minimal forms of Chiari I; with other forms of Chiari, they tend to provide incomplete and often short-lived clinical improvements

John Heiss, M.D.:

If minimally invasive decompressive surgery for treatment of Chiari I can relieve the obstruction in the CSF pathways as well as conventional surgery can, then minimally invasive surgery may be preferable to conventional surgery if other considerations such as duration of surgery and complication rate are similar in either treatment. The minimally-invasive part of the surgery allows a shorter skin incision but the extent of bone removal should be the same in either procedure. Minimally-invasive surgery for Chiari I is now being performed at several medical centers but I have not seen any studies comparing the long-term results of standard surgery and minimally-invasive surgery. Long-term results are important because Chiari I and syringomyelia can progress slowly and long-term follow-up with MRI and neurological examination is necessary to detect if treatment has been successful in resolving the syrinx. During the present period in which there is little information on the long-term results of minimally-invasive treatment some patients will be interested in being treated at centers offering minimally-invasive decompressive surgery for Chiari I and syringomyelia. If these centers carefully analyze the outcome of minimally-invasive treatment with respect to resolution of the syrinx, pain, weakness, sensory loss, etc. and compare their results with standard treatment, it will provide later patients with pertinent information about the advantages and disadvantages of minimally-invasive treatment versus standard treatment.

Please remember that syringomyelia is caused by tissue that obstructs the CSF pathways. In the case of syringomyelia associated with Chiari I malformation, the cerebella tonsils obstruct the CSF pathways. In other types of syringomyelia, the obstruction is usually caused by scar tissue that remains after inflammation or trauma. Surgery that opens the CSF pathways is the most reliable way to permanently relieve syringomyelia, and the primary surgical objective is therefore to effectively remove the obstruction. I recommend surgical procedures that effectively open CSF pathways.

Miseo Nishikawa, M.D.:

The "Minimally- invasive surgery" is trend in neurosurgical field, now. I think that the "Minimally- invasive surgery" is necessary and minimum surgery. So far in the present, we don't have the quantitative indicator to decide what is necessary and minimum for CCI patients. We have been challenging to have the "Minimally-invasive surgery" in each case, using color doppler ultrasonography during operation and morphometric analysis before surgery. "Minimally-invasive surgery" must have same results and success rate as conventional surgery, being minimal invasion to the patient. We believe that our surgery has been getting better and in near future we will report those results.

David M Frim, M.D.:

I don't know of any side-to side comparisons. I did our first endoscopic Chiari exploration a couple of weeks ago and it went smoothly, but I am not sure if the access will be adequate for all situations or for complications. I think in time we will know all of these answers.

Professor Marcus Stoodley:

My concern with "minimally invasive" surgery for Chiari is that there is a significant risk of insufficient decompression.

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Do you believe that Chiari and/or SM possess an autoimmunity factor?

March 2008

Paolo Bolognese, M.D.:

CMI is a mesenchimal disorder, as well as many autoimmune disorders; in CMI pts, rheumatoid factor and ANA titers are frequently moderately elevated; at the moment we cannot say anything more.

Professor Marcus Stoodley:

I don't think there is any evidence for this.

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Is there any relationship between CM and pineal cysts? How do you recommend that these be treated?

March 2008

Paolo Bolognese, M.D.:

In our experience, we did not see any higher incidence of pineal cysts in CMI patients, when compared to the non-CMI population.

Professor Marcus Stoodley:

Small (< 1cm) cysts are very common and I have seen them in patients who also have Chiari malformations. I doubt that there is any connection and they should not be treated unless they are symptomatic in their own right.

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What do you think about Cold laser therapy?

March 2008

Paolo Bolognese, M.D.:

I used it in Europe in the mid 80's with good results for pain and decubiti; properyl used, it is another useful tool for CMI patients, but it cannot help with severe pain.

Professor Marcus Stoodley:

I have no experience with this. It may be helpful for pain in some patients. It will not affect a Chiari malformation or syrinx at all.

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How is cerebellar ptosis diagnosed and treated?

March 2008

Paolo Bolognese, M.D.:

It is easily seen on MRI's. In our experience, its presence is almost always linkable to Tethered Cord

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We often have list members trying to differentiate between potentially low pressure and high pressure headaches. What do you feel are the most common symptoms that would help to classify these?

March 2008

Paolo Bolognese, M.D.:

High pressure headaches: whole head or Suboccipital pain, described as a sensation of pressure, often pulsatile, exacerbated by exercise, relieved by resting; the pt sleeps with the head up.

Low pressure headaches: the pain is referred to the vertex, frontally, and behind the eyes; the pt is pale, nauseous, and sickly; standing up makes it worse, while laying flat makes it better; the pt sleeps in a flat position.

Professor Marcus Stoodley:

The main differentiating feature is that low pressure headaches are worse with upright posture and are relieved with lying down.

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