Oropharyngeal dysphagia = when your upper esophageal sphincter, pharynx, larynx, and/or tongue fail to coordinate properly when trying to swallow.

Can occur when one or more of the following nerves is damaged or compressed: facial nerve (CN VII), glossopharyngeal nerve (CN IX), vagus nerve (CN X), and hypoglossal nerve (CN XII).

Now that you know it’s a thing… ask your doctor for a swallow test to confirm it.

Neurosurgeons on this list are ones that have focused much of their practice on Chiari Malformation and Comorbid Conditions. Not everybody will agree with who is on this list and that’s okay. While we do encourage members to share THEIR OWN EXPERIENCES WITH THEIR DOCTORS, we DO NOT ALLOW DOCTOR BASHING in our groups! The doctors that have concerns (some of which are serious/major concerns), amongst patients in our group/site, we have marked with a (!) after their name. It might be worth asking in the group for those that have had negative experiences to share with you about what happened through private message.


ADULTS:

Dr. Petra Klinge – Rhode Island, USA
https://brownneurosurgery.com/our-team/petra-klinge-md-phd/
593 Eddy Street, APC 6, Providence RI, 02903; Ph. 1(401)793-9139 or 1(401)793 9166
Specializes in Tethered Cord and Occult Tethered Cord. *No remote consultations.

Dr. Fraser Henderson – Maryland, USA
http://metropolitanneurosurgery.org/practice/henderson.html
Spine Team Maryland 8116 Good Luck Road, Suite 205 Lanham, MD 20706; Ph. 1(301)654-9390
Specializes in Problems of the Craniocervical Junction (CCI/AAI/BI). Does craniocervical fusions. Insurance might be an issue. *No remote consultations.

Dr. Paolo A. Bolognese (!) – New York, USA
https://nspc.com/physician/paolo-bolognese/
Chiari Neurosurgical Center at NSPC 1991 Marcus Avenue, Suite 108 Lake Success, NY 11042
Ph. 1(516)321-2586; email pbolognese@chiarinsc.com
Specializes in Complex Chiari & Problems of the Craniocervical Junction (CCI/AAI/BI). Does craniocervical fusions. *Known to do video consults for $300. To initiate consultation for 2nd opinion, contact
Jeffrey.Wood@snch.org.

Dr. Gerald Grant – North Carolina, USA
https://med.stanford.edu/profiles/gerald-grant
Duke Children’s Health Center Neurosurgery Clinic, 2301 Erwin Rd, Durham, NC 27710-4699
Ph. 1(919)668-8557 or 1(919)684-5013
Chief of NS. Specializes in Pediatric NS > Chiari & Fusions.

Dr. Anthony L. Capocelli (!) – Arkansas, USA
http://www.orthoarkansas.com/capocelli.php
Ortho Arkansas – Orthopedics & Sports Medicine 10301 Kanis Rd. Little Rock, AR 72205
Ph. 1(501) 604-6900 (800) 264-5633
Neurosurgeon specializing in Chiari. Does craniocervical fusions.

Dr. Dan S. Heffez (!) – Wisconsin, USA
https://www.heffezchiari.com/
Private Practice Milwaukee, WI
Ph. 1(414)955-7188
Neurosurgeon claiming to specialize in Chiari.

Dr. Holly Gilmer (!) – Michigan, USA
https://www2.mhsi.us/doctors/holly-gilmer
Michigan Head and Spine Institute 29275 Northwestern Hwy., Suite 100, Southfield, MI, 48034
Ph. 1(248)784-3667
Neurosurgeon claiming to specialize in Chiari.


PEDIATRICS:

Dr. Douglas L. Brockmeyer – Utah, USA
https://healthcare.utah.edu/fad/mddetail.php?physicianID=u0034828
The University of Utah Health 50 North Medical Drive, Salt Lake City, UT 84132
Ph: 1(801)581-2121 douglas.brockmeyer@hsc.utah.edu
Specializes in Pediatric NS > Chiari & Fusions.

Dr. Richard C. Anderson – New Jersey, USA
https://www.valleyhealth.com/doctors/richard-c-anderson-md
Valley Hospital Health System 1200 East Ridgewood Ave Suite 200, Ridgewood, NJ 07450 Ph: 1(201)327-8600, 1(212)305-0219, Joanna (nurse) 1(973)758-3616 Rca24@cumc.columbia.eds
Specializes in Pediatric NS > Chiari (not sure about fusions).

Dr. Jeffrey Greenfield (!) – New York, USA
https://weillcornell.org/jpgreenfield
Weill Cornell Brain & Spine Center 1305 York Avenue, 9th Floor New York, NY 10021
Ph. 1(212) 746-2363
Specializes in Pediatric NS (but does take adults) > Chiari, some comorbids, does not do fusions, but contracts them out to another surgeon. Known to do remote online for $300 and phone consults for $500.


OTHER SPECIALTIES:

Dr. Wouter Schievink: LEAK SPECIALIST (NS) – California, USA
https://www.cedars-sinai.edu/Patients/Programs-and-Services/Neurosurgery/Centers-and-Programs/Cerebrospinal-Fluid-Leak/
Cedars-Sinai Advanced Health Sciences Pavilion, 127 South San Vicente Blvd. Suite A6600 Los Angeles, CA 90048
Ph. 1(310)423-3277 Wouter.schievink@cshs.org
Neurosurgeon who specializes in leaks. Known to review MRIs for free in patients with low pressure symptoms.

Dr. Ian Carroll: SPINAL LEAKS (EBP, Anesthesia, PM) – California, USA
Stanford 430 Broadway St., Pavilion C, RM 462; MC 6343, Redwood City, California 94063
Ph. 1(650)721-7286 (office) ic38@stanford.edu
Not a neurosurgeon, but his daughter had an Acquired Chiari secondary to a spinal leak, and therefore is VERY PASSIONATE about trying to find leaks in patients dx’d with Chiari if they have low pressure symptoms.

Dr. Kenneth C. Liu: VASCULAR NS – Michigan, USA
https://www.bronsonhealth.com/doctors/kenneth-liu/
Bronson Neuroscience Center – Kalamazoo 601 John St., Suite M-124 Kalamazoo, MI 49007
Ph. 1(269) 341-7500 kenneth.c.liu.md@gmail.com
Specializes in Neuroendovascular Surgery, Venous Hypertension, Intracranial Hypertension


RETIRED/NO LONGER TAKING PATIENTS

Dr. John Oró
https://auroramed.com/service/ccc
Colorado Chiari Institute – The Medical Center of Aurora 1501 S. Potomac Street Aurora, CO 80012
Ph. 1(303)695-2663 ChiariCare@HealthONEcares.com
RETIRED AS SURGEON, but oversees Colorado Chiari Institute

Dr. Faheem A. Sandhu
https://www.medstarhealth.org/doctor/dr-faheem-akram-sandhu-md/
MedStar Georgetown University Hospital – Neurosurgery 10401 Hospital Drive, Suite 101 Clinton, MD 20735
Ph. 1(301) 856-2323
NO LONGER ACCEPTING EDS PATIENTS

It’s hard having a chronic illness that isn’t all that understood.

As patients, we have to fight on absolutely every level!

Before diagnoses, we fight for someone to hear us when:

  • We explain to them that our neck is to weak to hold up our head.
  • We’re trying to hold our heads up with our hands when laying back isn’t an option.
  • Our necks start spasming to the point that we feel like we’ve been internally decapitated.
  • We have to ride in the front seat to try and minimize the car sickness.
  • We suddenly can’t balance to walk.
  • Our eyes start twitching beyond what could ever be considered normal.
  • We aren’t able to do what we could just a short time ago, or even a few hours ago.
  • That we want to scream and cry because of the pain, but we know it will only make it worse.
  • We go to say something and can’t find the right word because it just isn’t in our memory bank at that moment.
  • We spontaneously can’t read because we have double vision, blurred vision, or our eyes wont stop jerking around to focus, yet an hour later we’re fine.
  • We explain that doctors not knowing what’s wrong doesn’t mean that nothing is wrong (even when they say nothing is wrong).

Around diagnoses, we fight to:

  • Process the magnitude of what we’re facing.
  • Learn all we can so we’re prepared for the important decisions before us.
  • Find the right doctors who are knowledgeable and trained in our condition(s).
  • Fight to be stoic when we know that it’s not just our bodies enduring all of this – something is breaking in our souls and we’re fighting to not let it change us for the worse.

When our doctors continue to dismiss our symptoms, we need our friends and families to understand:

  • That we’re still the same wife/husband, mother/father, sister/brother, aunt/uncle, and/or friend that you’ve known and loved for all these years, and we need you now more than ever!
  • That decompression is not a cure! In fact it typically fails to relieve symptoms over the long term nearly 50% of the time when pathological conditions aren’t treated beforehand.

Most Chiarians go to see a surgeon with an expectation of them being knowledgeable in their field. However, while they might be a neurosurgeon, their knowledge of Chiari and its comorbid/pathological conditions might not rank high in their practice. Make the most of your initial appointment by interviewing them and what they really know about Chiari Malformations. Be cautious of inflated success rates. Chiari decompression in general offers a just over a 50% success rate (which means it has a nearly 50% failure rate). Surgeons that claim a 100% (or near 100% success rate) are usually not basing their success on how their patients feel afterward, it is based on if they were successful with the aspects of the surgery:
         Removal of the occipital bone
          Opening the dura and adding the patch/graft
          Laminectomy
          Cauterization/resection of cerebellar tonsils

WE DESERVE BETTER THAN THAT!


HERE IS A LIST OF CHIARI QUESTIONS WE RECOMMEND ASKING AT YOUR FIRST NEUROSURGERY APPOINTMENT:

General Questions:

  • How do you define a Chiari Malformation?
  • What do you believe causes a Chiari malformation?
    • Are all Chiari malformations from a small posterior fossa?
    • Do I have a small posterior fossa? If yes, how big is it? If size is unknown, was my posterior fossa measured? If not, why not? How did you come to the conclusion that I have a small posterior fossa?
    • How common do you believe Acquired Chiari malformations to be?
  • Do you always recommend decompression surgery for all of your patients with herniated cerebellar tonsils? Why/why not?
  • In an average month, how many Chiari decompressions do you perform? How many tethered cord releases? How many craniocervical fusions? What percentage of your practice is spent treating patients with these connective tissue related conditions?
  • Looking at my brain scan, is any part of my “brainstem” herniated (below the posterior fossa)? If so, does that make me a Chiari 1.5?

Intracranial Hypotension (low pressure) Questions:
*Article to help you understand CSF Leaks & Intracranial Hypotension prior to your appointment.
If you have SYMPTOMS OF LOW INTRACRANIAL PRESSURE and/or suspect a cerebrospinal fluid leak, we recommend asking the following questions:

  • S.E.E.P.S.
    • Looking at my brain scan, do you see any Subdural fluid collections?
    • Looking at my brain scan, do you see an Enhancement of pachymeninges?
    • Looking at my brain scan, do you see an Engorgement of my venous structures? Should we do an MRV to make sure?
    • Looking at my brain scan, does my Pituitary appear to be enlarged?
    • Looking at my brain scan, does my brain appear to be Sagging?
  • Looking at my corpus callosum:
    • Does there appear to be a depression?
    • Is there an inferior pointing of the splenium?

If he/she answers affirmatively to any of the above S.E.E.P.S. questions, ask: 

  • What should be done to find/repair a potential leak?
  • Are you aware that it is common for CSF Leaks to not show up on MRI?
  • Are you willing to do a CT Myelogram and/or a digital subtraction myelogram, if I develop symptoms of a leak and none can be found on MRI?
  • Are you aware that it can often take multiple epidural blood patches to try and seal a leak, and sometimes when a blood patch fails to work, a surgical dural repair might be necessary?

Intracranial Hypertension (high pressure) Questions:
*Article to help you understand Intracranial Hypertension prior to your appointment.
If you have SYMPTOMS OF HIGH INTRACRANIAL PRESSURE, we recommend asking the following questions:

  • Looking at my brain scan, do I have cerebrospinal fluid in my sella turcica (Empty Sella Syndrome)?
  • Looking at my brain scan, do you see any evidence of my optic nerves are swollen (papilledema)?
    • If so, should I be referred to a neuro-ophthalmologist?
  • Looking at my brain scan, do my lateral ventricles appear small or flattened?
    • If so, do I need to have my pressures checked?
      • If yes, are you aware of the risks of developing a CSF Leak from a lumbar puncture?
    • What are the symptoms of a CSF Leak, should one develop?
      • What is your plan of action if I should develop these leak symptoms?
      • Are you aware that it is common for CSF Leaks to not show up on MRI?
      • Are you willing to do a CT Myelogram if I develop symptoms of a leak, and none can be found on MRI?
    • Should a leak be found, are you aware that it can often take multiple epidural blood patches to try and seal a leak?

Tethered Cord Questions: 
*Article to help you understand Tethered Cord: Sorry, Coming Soon.
If you have SYMPTOMS OF TETHERED CORD, we recommend asking the following questions:

  • Looking at my brain/cervical scan, does my brainstem appear to be elongated?
  • Looking at my cervical scan, does my spinal cord appear to be stretched?
  • Looking at my lumbar scan, does my conus reach my mid/low L2?
  • Looking at my thoracic and lumbar scan, does my spinal cord appear to be pulling to the back, or one particular side?
    • If so, should we do a prone MRI to see if it has actually adhered to that side?
  • Looking at my lumbar scan, do I appear to have fatty tissue inside the epidermis?
    • If the answer to any of these questions is affirmative, do you suspect that I have a tethered spinal cord?
    • If so, should we plan for a Tethered Cord Release before or soon after decompression surgery, so the likelihood of a failed decompression is reduced?
    • If I have urological issues, can I get a referral for urodynamic testing to rule out any other potential causes of my urological issues?

Craniocervical Instability (CCI) & Atlantoaxial Instability (AAI):
*Article to help you understand CCI & AAI prior to your appointment.
If you have SYMPTOMS OF CRANIOCERVICAL INSTABILITY or SYMPTOMS OF ATLANTOAXIAL INSTABILITY, we recommend asking the following questions:

  • Looking at my brain/cervical scans, what are the measurements of my clivoaxial angle and Grabb-Oakes?
  • Do these measurements meet the diagnostic criteria for Craniocervical Instability?
  • Looking at my flexion and extension imaging, how many millimeters of translation are there between flexion and extension?
  • Does Chamberlain’s Line cross my odontoid? If so, does it cross at a level that would indicate Basilar Invagination?
  • Looking at my rotational imaging, what is the percentage of uncovering of the right and left articular facets on rotation?
  • Do the percentages from my rotational imaging meet the diagnosis criteria for Atlantoaxial Instability?

IF A DIAGNOSIS CRITERIA IS MET IN ANY OF THE ABOVE, WE STRONGLY RECOMMEND THAT YOU WAIT ON DECOMPRESSION AND PURSUE THE TREATMENT OF SAID CONDITION(S) AND THAT OF EHLERS-DANLOS SYNDROME, AS EACH OF THESE CONDITIONS CAN BE PATHOLOGICAL TO AN ACQUIRED CHIARI AND EACH IS A STRONG INDICATOR THAT A CONNECTIVE TISSUE PROBLEM EXISTS. 

*The questions in this article will periodically change as we are able to expand our recommended questions.


*Original version released September 2018, revised 2023.

Tired of your doctors telling you, “If you just exercised, you’d feel better!”?

Well, they may not be all wrong. Just hear me out! The truth is we all just want to feel better and fight to get as much of our lives back as we can. That is never going to be as easy for us as it would be for someone who doesn’t have all of the challenges that we deal with.

Not only is moving necessary for our circulatory systems, and better blood flow means less inflammation, but as Elle Woods says, “Exercise creates endorphins. Endorphins make people happy.” It’s actually true. And whether you’re chronically ill or just sedentary, a slight boost in happiness can change your entire outlook on life. Okay, so I am also not saying go out and pick up CrossFit. Or marathon running. We all know our bodies most likely cannot tolerate that. So how do you exercise safely, while still providing yourself the benefits of movement? Each one of us are different, but my goal is to give you the framework of how to start moving in some way, and then you build on that foundation.

Start small. I want you to remember that for the rest of your life – especially when you are faced with something difficult or are challenging yourself to start moving. Write down a goal (i.e., walk to the kitchen and back, or walk to _____ and back) and put it somewhere that you will see it. Once a day, accomplish that task. Most importantly, listen to your body. If it is day 1 and you accomplish your daily goal and the next day, you cannot move, that goal may be too much. How can you accomplish that daily goal the next day? Do you need to modify what the goal says? Or how you treat your body before or after? Does it involve extra braces, or heat or ice after? The first few weeks will be the hardest. You are re-learning your body and how it moves. Try not to reduce the goal (of course, if symptoms are such that you end up at the hospital, then please – re-evaluate the goal). If you want to walk outside, try to plan a route that will take you by things you enjoy – a friend’s house, a pretty park, etc. If you cannot walk outside, and choose to walk inside (your home, a building, etc.) that is perfectly fine too.

What if you are in a wheelchair or are unable to leave the bed? Well, that is fine too. You can still get your exercise in. You can do leg lifts while lying in bed. Pick a goal to do ___ on each leg. Or if you do not have use of your legs, do upper body movement (just be careful of pulling on your neck too much). Safe upper body movements while laying down include arm lifts (straight out in front of you like you would be a zombie).

Be sure that any movement that you do should be within a pain free range. If you are doing arm lifts and it hurts your shoulders or neck (or instability causes your shoulder to sublux), reduce how high you are lifting your arms. The same principle applies with your legs.

One of our admins is going on a trip soon and they wanted to be able to walk around, so they start walking 1 lap around the block (a small block) each day. After the first day, the pain was pretty bad, so she added some neck bracing, and additional leg braces in order to try it again on day 2. Day 2 she was able to complete without issue! She takes her time and is not concerned with HOW LONG it takes her to complete the walk around the block. She only cares that she DOES it.

That is all you should remind yourself. To pick a small goal and COMPLETE it to the best of your ability. You will be SO proud of yourself for completing that goal and I will be cheering you on and always here for questions and to help!

Ever feel alone in all you’re facing? It’s one thing to read what people are going through, but to see them as they share how they’re going through the same struggles that you are can be game-changing!

Starting in December, the tribe will be hosting online support meetings on the first and third Tuesdays of each month to share and learn about all we’re dealing with.

A valuable resource for patients and their families, looking for support and a sense of direction!

WHEN A DOCTOR TALKS ABOUT THE “MOST SERIOUS” COMPLICATIONS SURROUNDING CHIARI MALFORMATION, THEY USUALLY SPEAK OF PARALYSIS OR DEATH. WHILE BOTH OF THESE ARE FAR LESS COMMON THAN THE ARRAY OF OTHER SYMPTOMS AND COMPLICATIONS, THEY BOTH CAN INVOLVE THAT OF A SYRINX.

The word syrinx (seer-inks), plural syringes  (seer-en-geez), means cavity or cyst. Syringomyelia (seer-ingo-my-el-lee-uh) is when the cyst forms in the spinal cord (myelo usually refers to the spinal cord), and when the cyst is in (or ascends up into) the bulbar region of the brainstem (the medulla oblongata) it is called Syringobulbia (seer-ingo-bulb-e-uh). While these cysts are technically the same cerebrospinal fluid-filled cysts, because they are damaging a different part of the body (each with a completely different function), the location of the syrinx has everything to do with the symptoms that it can cause.

Hydromyelia:
At the center of the spinal cord, there is a hole known as the central canal which runs from the fourth ventricle down the length of the cord. Its role is to carry a microscopic amount of cerebrospinal fluid down the center of the cord from the fourth ventricle. The small amount of fluid in the central canal is usually considered normal. When the central canal has too much cerebrospinal fluid coming in from the fourth ventricle it widens the canal and that abnormal widening that follows the central canal is known as hydromyelia (hydro-my-el-lee-uh) or syringohydromyelia (seer-ingo-hydro-my-el-lee-uh).

The Central Nervous System (CNS) is an amazingly complex network. The brain, brainstem, and spinal cord each play a role in communicating to coordinate everything that happens in our bodies (even when no cognitive thought is required). The messages to and from the brain are sent through the brainstem, down through the spinal cord, and out to our nerves. When that signal is blocked, it can cause interruption from that point down. When it’s thin, it interrupts the signal in the middle (gray matter), as it widens it interrupts the signal more and more, so it’s harder to get signal through the cord at the widest point of the syrinx.

The spinal cord runs through a canal known as the spinal canal. The spinal canal holds cerebrospinal fluid that among other things, nourishes the spinal cord and helps protect it from injury. A syrinx is generally known to be caused by a blockage of cerebrospinal fluid, which is why it is most commonly seen amongst those with Chiari Malformation. When the cerebellar tonsils descend into the spinal canal, like a cork the tonsils block the flow of cerebrospinal fluid. Even when the tonsils fail to descend, they can rest on top of the foramen magnum and still block the flow of cerebrospinal fluid (see Chiari Zero below). There are other conditions that can cause similar blockages as well, so it is not exclusive to Chiari malformations. Anything that causes stenosis (narrowing) of the spinal canal (the cord: canal ratio) can also block cerebrospinal fluid and lead to the formation of a syrinx – conditions such as bulging/herniated discs (from degenerative causes or trauma), spinal cysts/tumors, edema/inflammation of the spinal cord or surrounding membranes (from trauma or conditions like meningitis/arachnoiditis), and/or irregular curvatures of the spine (scoliosis).

Syringomyelia:
Syringomyelia does not usually run the entire length of the spinal cord like the central canal (but it can). Instead, it often seems to spontaneously appear. As the CSF increases in a syrinx, it can either lengthen or widen. The length doesn’t matter much (radiologists note the location because they report what they see and try to be precise). But a neurosurgeon that knows about syringes, knows that what really matters is the diameter of a syrinx (which is where the risk of paralysis can come into play). Symptoms tend to vary based on where the syrinx is located in the spinal cord. The highest point of the syrinx (where the initial disruption starts) and the widest point of the syrinx (location of the greatest disruption) should always be considered because there are different nerves branching from the spinal cord at different intervals. A syrinx interrupts the communication from the top of the syrinx down, so a syrinx that is higher in the spinal cord can have an impact on a larger range of the body. Someone with a syrinx in the lumbar region of the spinal cord will usually have communication problems from the waist down, but someone with a cervical syrinx is likely to have problems from the neck down. A syrinx in the upper thoracic spinal cord (or above) will often include the arms since the peripheral nerves that lead to the arms branch off around the T1 vertebra. The wider the syrinx, the greater the interruption of communication. Therefore, a syrinx should always be gauged by its diameter and not its length. Symptoms generally include (from the syrinx down): muscle weakness, pain, and spasms in legs; pain, tingling, burning of arms; muscle wasting (atrophy); loss of reflexes; loss of pain senses, loss of temperature sensation, numbness, pain, and stiffness in back/shoulders/upper chest (cape-like area); stiffness of muscles; muscle contractions (fasciculations); bowel & bladder dysfunction; scoliosis; paralysis (rare).

Syringobulbia:
Syringobulbia exists when a syrinx forms in the medulla oblongata (the bulbar region of the brainstem) OR when a syrinx in the cervical cord extends upward into the medulla. A syrinx in the medulla can cause medullary issues or in extreme cases, death (since the medulla is responsible for things that happen autonomically (automatically) for survival – such as breathing, heart rate, swallowing, gag reflex, etc.). Dysautonomia is an umbrella term used to describe any dysfunction of the Autonomic Nervous System (ANS), both Sympathetic and Parasympathetic divisions, often accompany any damage to the medulla. When talking about Dysautonomia, many tend to think of Postural Orthostatic Tachycardia Syndrome (POTS), but POTS is but one symptom of Dysautonomia in a long list. The damage from Syringobulbia is not generally isolated to the medulla, but to the cervicomedullary junction (where the cervical spine meets the medulla), it can also affect the cranial nerves causing symptoms such as facial sensory loss (unilateral or bilateral); extraocular muscle palsy; nystagmus; palatal palsy; atrophy of the tongue; dysphonia (vocal cord paralysis); slurred speech; indistinct speech; drooling; tongue fibrillation; oropharyngeal dysphagia; impaired gag reflex; hearing loss; tinnitus (ringing in the ears); alveolar hypoventilation; Sleep-Disordered Breathing (SDB); Central and obstructive sleep apnea; Anhidrosis (inability to sweat normally); Inability to burp (Retrograde Cricopharyngeal Dysfunction – RCP-D).

Common Treatment Options:

Monitoring The Syrinx:
Both Syringomyelia and Syringobulbia tend to be progressive, but in some cases, patients report having no symptoms and imaging proves it to be relatively stable in size. In this case, monitoring is generally recommended. A neurologist or neurosurgeon should carefully monitor these patients to track changes in the diameter of the syrinx (which should include regular imaging) and/or any evolution in related symptoms.

Surgical Treatment Of The Underlying Cause:
For symptomatic patients, or when the syrinx is progressing in diameter, or when the syrinx is so wide in diameter that it is stretching the diameter of the spinal cord from the inside out, treatment is essential. This generally involves treating the cause of the blockage of cerebrospinal fluid. When syrinx exists in a symptomatic patient with Chiari Malformation, a posterior fossa decompression surgery is usually recommended, with the desired result being to re-establish the flow of the cerebrospinal fluid to the spinal canal (so it no longer reroutes to the spinal cord and/or low-lying medulla).

Surgically Draining The Syrinx:
A surgical shunt is commonly used to surgically treat a syrinx when: the underlying cause is unknown or when treating the underlying cause has proven ineffective at reducing the size of the syrinx in a patient that is symptomatic.

When you start to educate yourself on a condition like Chiari, your vocabulary will be challenged. Most of us study with a medical journal article opened in one tab and medical dictionary in the next. Amongst all the medical terminology you will tackle, there are probably a few terms as important to your understanding of Chiari than comorbidities and pathological/etiological cofactors. When two or more conditions tend to co-occur, they are said to be comorbid with one another. It makes no inferences of a causal relationship between the conditions, only that they co-occur. This co-occurrence deduces that a correlation exists, but when the nature of that correlation is not known, they are just said to be comorbidities. When a “causal relationship” is known or suspected, the conditions start being discussed in terms of pathology or etiology, which are similar, but not exactly the same thing.

An etiological cofactor exists when the “root cause” of a condition is known or believed to be known. That “root cause” is the etiological cofactor. When an etiological cofactor can cause a series of events or conditions that can become “direct causes” for other conditions, that series of events creates a pathology. Conditions along the path are called pathological cofactors. Understanding these cofactors is imperative in understanding Chiari and all of the comorbid conditions that accompany it.

ETIOLOGICAL COFACTOR:

Chiari Malformation often seems like a beast that wreaks havoc on our bodies on every level. Indeed it is, but as you can see from the diagram above, it really is not the “root” of everything that is going wrong. There is a bigger beast at work in so many of us, and its name is Ehlers-Danlos. It is not by chance that so many of us with Chiari have so many other conditions in common (especially conditions like Degenerative Disc Disease, arthritis and other connective tissue problems). It is not by chance that so many of us have a history of miscarriage and similar familial histories. It is not by chance that Chiari is more prevalent in females than males. And it is definitely not by chance that Chiari is running in families and they cannot find a definitive genetic link. They cannot find it because they are not looking at the beast hiding in the background.

Ehlers-Danlos Syndromes are a group of inherited disorders involving a genetic mutation in one or more of our bodies’ collagen. Collagen is the most abundant protein, making up 1/3 of the proteins in the human body, affecting our bones, skin, muscles, and connective tissue[1]. Collagen is often described as a “cellular glue” that helps hold the body together. When that glue fails to hold, everything seems to go awry; before and after birth: skulls can under-develop in utero, organs tend to prolapse, and bones begin to shift as joint laxity increases (including the bones/vertebrae at the craniocervical junction). Ehlers-Danlos is a primary “root cause” of Chiari Malformations and a majority of the other problems we have. The list in blue is far from being a complete list of conditions caused by EDS. They are commonly accompanied with Chiari because they can cause or attribute to a Chiari malformation (pathological cofactors).[2]

PATHOLOGICAL COFACTORS:

Cranial Settling occurs when the skull has dropped and the odontoid (C2/axis) enters into the foramen magnum (Basilar Invagination). This drop can further compromise the craniocervical junction and as it pushes everything down, it increases the likelihood of an Acquired Chiari Malformation.

Craniocervical Instability (CCI) & Atlantoaxial Instability (AAI) usually occurs with cranial settling and Basilar Invagination (BI). The settling and/or softening of tissue can cause a shifting of the C2 (resulting in CCI or AAI) and the cerebellar tonsils (which are already inclined to prolapse) simply drop down with each shift affecting ones ability to tilt/rotate their head.[3]

Intracranial Hypertension (IH – High Intracranial Pressure) occurs when your intracranial pressure (ICP) becomes elevated. This elevation can happen for a variety of reasons.

  1. Space Occupying Masses (cysts, tumors or hydrocephalus) take up space inside the skull causing a “mass effect.”
  2. When no mass effect exists, many doctors look no further and give the diagnosis of Idiopathic Intracranial Hypertension.

Because the area of the skull is fixed in an adult cranium and partially fixed in that of a child, the elements inside the fixed space (CSF, blood volume and brain matter) tend to get pushed out wherever they can (the only place that they can escape without breaking through the dura is through the foramen magnum and the brain matter that’s closest to the foramen magnum is the cerebellar tonsils).[4]

Tethered Cord Syndrome occurs when the tissue inside the epidermis adheres to the spinal cord or filum terminale. While this tethering can happen anywhere along the spinal canal, it is most common in the lower lumbar and/or sacral spine. When this adhesion happens it creates a pulling down of the spinal cord and consequently, the brainstem located at the top of the spinal cord and the cerebellar tonsils just get pulled down with it.[5]

Intracranial Hypotension (Low Intracranial Pressure, often involving a CSF Leak) usually involves a cerebrospinal fluid leak or an over-draining shunt, we will highlight the former. Ehlers-Danlos patients tend to have weak dura matter. Tears/holes in the dura can happen anywhere in the dura surrounding the brain or spinal canal and they can happen completely spontaneously (without a known cause). When the leak occurs in the spinal canal, they can create a suctioning effect where cerebrospinal fluid (CSF) is being pulled down and out, causing the intracranial pressure (ICP) to drop. The cerebellar tonsils that are already prone to prolapse (due to EDS) end up getting suctioned downward with the CSF.[6] Cranial leaks often happen when high pressure is left untreated until the high pressure causes a leak in the dura mater. In cranial leaks, fluid usually leaks through the nose or ears (less common), and you can often taste the metallic taste of the cerebrospinal fluid in the back of your throat. While both spinal leaks and cranial leaks can cause low pressure and low-pressure symptoms, and while both can start, stop, and start again spontaneously, there is an increased risk whenever there is an opening where cerebrospinal fluid leaks outside of the human body (if cerebrospinal fluid can make it out of the body, microscopic bacteria can make it inside the same opening where it can enter in the meninges).[7]

Posterior Cranial Fossa Hypoplasia (PCFH) is the only etiological cofactor listed above that is definitely congenital. The role of collagen in bone development has been long-standing, especially its known contribution to certain conditions like Osteogenesis Imperfecta. However, more recent studies are discovering the role collagen plays in congenital posterior fossa anomalies. Posterior Cranial Fossa Hypoplasia is the most commonly “acclaimed” cause of Chiari malformations, but studies show, that even when all of the other causes above are factored out, only approximately 52% of those left (that fail to meet “the diagnosis criteria” for any of the above), have a small posterior fossa.[8]

COMORBIDITIES: 

While all of the conditions listed in the diagram are comorbidities, some are etiological/pathological cofactors of an Acquired Chiari (even though nearly 100% of us are told that our Chiari Malformation is congenital) and others have Chiari Malformation as their etiological/pathological cofactor:

Syringomyelia occurs when cerebrospinal fluid (CSF) is obstructed and a CSF filled cyst/cavity forms inside the spinal cord. This cyst is directly related to the obstruction of cerebrospinal fluid that can be caused by Chiari Malformation, Spinal Stenosis (a narrowing of the spinal canal, spinal cyst/tumor, a herniated disc), or irregular curvature of the spine (scoliosis). When that cyst/cavity extends into the medulla oblongata (the lowest part of the brain stem), it is called Syringobulbia, and it comes with a new set of symptoms consistent with the damage being done to the brain stem. So when Chiari Malformation exists with a syrinx, and there is no stenosis or disc problem in close proximity below it, the Chiari Malformation should be listed as the etiological cofactor for the syrinx. If more than just the Chiari Malformation is believed to be causing the syrinx, each would be more accurately described as a pathological cofactor.

Dysautonomia occurs when damage has been done to the brain stem or Vagus nerve. Whenever either of these is damaged, often from compression at/near the craniocervical junction, the autonomic nervous system can begin to dysfunction.

Confused? If you understand the causal relationships but find yourself wondering if a comorbid condition is an etiological or a pathological cofactor, think of it in terms of a domino effect. Only the first domino is the real etiological cofactor. All of the dominoes in between (on the path) are pathological cofactors. The important thing to remember in this array of medical terminology is that while everything is definitely not Chiari, it almost always shares a connection to it, and that is why so many of us have so many conditions and symptoms that doctors call unrelated! It is imperative in our fight that we know “what” we have and “why” it is happening. With such a broad spectrum of symptoms (like we all have), we must educate ourselves and not just believe the limited knowledge of our doctors.

*Revised November 2019

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References: 

McIntosh, James. “Collagen: What Is It and What Are Its Uses?” Medical News Today, MediLexicon International, 16 June 2017, <www.medicalnewstoday.com/articles/262881.php>.

Quake. “The Chiari Malformation Ehlers-Danlos Connection (Short Version).” Chiari Bridges, 7 Dec. 2017, <www.chiaribridges.org/chiari-malformation-ehlers-danlos-connection-short-version>.

3 Hawkeye. “Overview: Craniocervical Instability and Related Disorders.” Chiari Bridges, 6 Dec. 2017, <www.chiaribridges.org/craniocervical-instability-related-disorders>.

4 Quake. “Brain Under Pressure – Understanding Intracranial Hypertension.” Chiari Bridges, 10 Dec. 2017, <www.chiaribridges.org/brain-pressure-understanding-intracranial-hypertension>.

5 Storm. “The Tethered Cord – Chiari Malformation Connection!” Chiari Bridges, 15 Dec. 2017, <www.chiaribridges.org/tethered-cord-chiari-malformation-connection>.

6 Argent. “Overview: Cerebrospinal Fluid Leaks.” Chiari Bridges, 10 Dec. 2017, <www.chiaribridges.org/cerebrospinal-fluid-leaks>.

7 Pérez, Mario A et al. “Primary Spontaneous Cerebrospinal Fluid Leaks and Idiopathic Intracranial Hypertension” Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society vol. 33,4 (2013): 330-7. doi:10.1097/WNO.0b013e318299c292, <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4040082/>

8 Quake. “Overview: Chiari Malformation.” Chiari Bridges, 6 Dec. 2017, <www.chiaribridges.org/chiari-malformation>.

 

HOMEPAGE: <https://chiaribridges.org/> (go to article)

ABOUT CHIARI (TAB)
  • Understanding Your Head and Neck Pain: <https://chiaribridges.org/understanding-your-head-and-neck-pain/> (go to article)

  • Overview: Chiari Malformation [Revised]: <https://chiaribridges.org/chiari-malformation/> (go to article)
  • What’s In A Name? An Expansive Review of the Name and Definition of Chiari Malformation: <https://chiaribridges.org/whats-in-a-name-chiari-malformation/> (go to article)

  • One Painful Fight – Get Ready To Rumble!!!: <https://chiaribridges.org/painful-fight-get-ready-rumble/> (go to article)

  • The Diagnosis – Round One: <https://chiaribridges.org/diagnosis-round-one/> (go to article)

  • The Important Questions to Ask Your Neurosurgeon [Revised]: <https://chiaribridges.org/important-questions-for-your-neurosurgery-appointment/> (go to article)

  • Overview: Chiari Comorbidities & Etiological/Pathological Cofactors [Revised]: <https://chiaribridges.org/overview-chiari-comorbidities-etiological-pathological-cofactors/> (go to article)

  • Overview: Chiari Treatment Options & Potential Pitfalls: <https://chiaribridges.org/chiari-treatment-options-potential-pitfalls/> (go to article)

  • Overview: Complications Associated With A Chiari Decompression: <https://chiaribridges.org/complications-associated-with-chiari-decompression/> (go to article)

  • A Bruised Mind – Chiari & Depression: <https://chiaribridges.org/bruised-mind-depression/> (go to article)

  • Finding Hope In The Seemingly Hopeless Chiari Fight!: <https://chiaribridges.org/finding-hope-seemingly-hopeless-chiari-fight/> (go to article)
COMORBIDITIES (TAB)
  • Overview: Chiari Comorbidities & Etiological/Pathological Cofactors [Revised]: <https://chiaribridges.org/overview-chiari-comorbidities-etiological-pathological-cofactors/> (go to article)

  • The Chiari Malformation Ehlers-Danlos Connection: <https://chiaribridges.org/chiari-malformation-ehlers-danlos-connection-2/> (go to article)

  • Overview: Craniocervical Instability and Related Disorders: <https://chiaribridges.org/craniocervical-instability-related-disorders/> (go to article)

  • Brain Under Pressure – A Guide to Understanding Intracranial Hypertension:<https://chiaribridges.org/brain-under-pressure-a-guide-to-understanding-intracranial-hypertension/> (go to article)

  • Overview: Cerebrospinal Fluid Leaks: <https://chiaribridges.org/cerebrospinal-fluid-leaks/> (go to article)

  • Spine Pulled Tight – A Guide to Understanding Tethered Cord Syndrome: <https://chiaribridges.org/spine-pulled-tight-a-guide-to-understanding-tethered-cord-syndrome/> (go to article)
WARRIOR STORIES (TAB)
  • Listing of stories: <https://chiaribridges.org/warriors/> (go to article)

  • The Michelle Cole Story – A Chiari Warrior’s Journey: <https://chiaribridges.org/the-michelle-cole-story-a-chiari-warriors-journey/> (go to article)
MISCELLANEOUS
  • Donations: <https://chiaribridges.org/donate/> (go to article)

  • Volunteer: <https://chiaribridges.org/join-the-cb-volunteer-team/> (go to article)

  • Share Your Story: <https://chiaribridges.org/submit-your-story/> (go to article)

  • Submission Agreement: <https://chiaribridges.org/submission-agreement/> (go to article)

  • The Chiarian’s Glossary: <https://chiaribridges.org/glossary/> (go to article)