Chiari Bridges

Category: General Information

  • The Cameron Hallihan Story – A Chiari Warrior’s Journey

    The Cameron Hallihan Story – A Chiari Warrior’s Journey

    My son’s story started right from the moment he was born. I knew as soon as he tried to breastfeed that something was wrong. At that time, I didn’t know exactly what I was getting into. I figured he was aspirating like my first child. I took my brand-new baby home and tried to feed him for four weeks. Breast, bottle, syringe, you name it, we tried it. At four weeks he couldn’t keep weight on or eat. We spent a few hospital admissions trying to figure out the problem with many, many extensive tests. They inserted an NG tube to feed him and we thought wow what a relief! They assured me he just had GERD and it would get better with age. Let me tell you nothing got better.

    When he was seven months old, he began vomiting anything and everything. My baby cried for the first eight months of his life and screamed blood-curdling screams while hooked to the feeding pump. I thought it was reflux but looking back I should have known better. Tylenol would help. (How would Tylenol help reflux?)

    They continued to look because I was a squeaky wheel making weekly visits to the children’s emergency department, telling anyone who would listen that something was wrong with our baby. Our son’s doctors thought we were crazy and that we, “could not cope as parents.” We were admitted to the hospital for a few tests and in walks a lady introducing herself as a physiatrist. I was thrown off but wasn’t surprised. She talked to me for an hour. I remember asking her if she was there because they think I’m crazy. “They think I have Munchausen’s, don’t they?” I asked. “Maybe,” she replied. At the end of the conversation, she told me that I needed to keep advocating for my child. She understood the situation and knew I wasn’t crazy, and she too was convinced that the doctors were overlooking something. It felt good to have someone finally believe me, but all that I could do was hope that it would result in them finally looking into what was really going on with our son.

    Finally, a doctor agreed to do an MRI and there it was. On New Year’s Eve, at the age of thirteen months, we found the source of the problem – our son had a Chiari malformation and pressure was building in his brain because part of his brain had dropped out of the skull and was blocking the flow of cerebrospinal fluid. They recommended an emergent decompression surgery and surgery was scheduled for January 5th. When the day that the diagnosis came, the doctor who had thought that I was crazy the entire time, came in and apologized.

    At my insistence, they did a sleep study that showed that our son suffered from central sleep apnea. The sleep study showed he never ever made it to a deep sleep before awakening. The same doctor who refused to believe that our son was anything other than a typical baby awakening often was being humbled by the results. His initial surgery was bumped, and on January 11, 2019, our baby went in for brain surgery (a posterior fossa decompression with duraplasty).

    Cameron had a very hard recovery. After decompression, he seemed hyperactive and unresponsive to my presence (almost catatonic). I work at a hospital and it seemed like he was acting much like babies born with narcotic addictions. I asked if it could possibly be related to the morphine he was on and once again, I felt as though my concerns were falling on deaf ears. Finally, three days after decompression, they considered it and after switching him from morphine to Dilaudid (hydromorphone), I had my son back and he was diagnosed with an allergy to morphine causing a paradoxical reaction. He still hadn’t walked after surgery and I waited by his bedside holding on to hope. Finally, on day five, he walked. By day six his swallowing was amazing, and he could be fed without vomiting! He finally could sleep through an entire night without awakening. Things were finally looking up!

    Less than a week after returning from the hospital, he started acting as he had prior to surgery. I took him in, and they diagnosed him with a post-operative leak known as a pseudomeningocele. They assured me that he would be fine if I could just “get him to slow down and rest.” Two days later I couldn’t handle seeing him in such pain again, so I took him back. They decided to take him back into surgery to repair the leak.

    This time, we knew going in that morphine was not an option. We talked with the anesthesiologist right before surgery and he ensured us that Cameron WOULD NOT be given morphine. After the leak surgery I went to the recovery room and the moment I set eyes on my baby I knew he was given morphine. I asked and they denied it, saying he was only given Tylenol. Finally, on the ward, the doctors looked and affirmed that he was indeed given morphine and they apologized.

    Morphine wasn’t his only allergy. We already knew that he had a dairy allergy requiring him to have a special formula and we were given an epinephrine injector due to an allergic reaction he had to pumpkin. The hospital was made aware of all his known allergies. However, during his recovery, my son started vomiting uncontrollably because they gave him the wrong formula in his feeding tube. While the nurse scurried to clean up the vomit, he was oblivious to the fact that our son was going into anaphylaxis. I took the epi-pen out of my purse and saved our child’s life right there in the hospital. From that day forward our son would go into anaphylaxis over everything and anything several times a week without explanation. I pushed for additional testing and again was treated like an irrational, crazy mom. They did the tests to make me stop bugging them, and the tests came back positive for Mastocytosis.

    We fought a thirteen-month fight for our son, that no family should have to fight. It wasn’t just the medical problems that took the toll on us, but the incompetence and neglect from those we were trusting to help us. It was financially, emotionally and physically exhausting. Our eldest son, Dominic, who is just shy of three years older than Cameron, was passed around our family while we were in and out of the hospital begging for the medical professionals that we trusted to help us find answers. It took a toll on our relationships, with one another and with friends and family, because everyone had an opinion on everything we were doing. As a family, Cameron’s dad and I underwent counseling to heal from all the emotional turmoil we had gone through. It changed us, and we had to learn to interact and trust again.

    Today, Cameron is a little firecracker, full of energy and spunk. He is always on the move. Even on Cameron’s hardest days it nearly slows him down. He loves his sleep. Cameron still struggles with his mast cell day-to-day and is on a few different medications to stabilize it.

    For anyone out there still looking for answers, don’t give up and don’t give in. You can do this!

  • Palliative Care: An Essential for EDS & Chiari Families

    Palliative Care: An Essential for EDS & Chiari Families

    When I was first diagnosed with Chiari Malformation, I believed everything that my neurosurgeon told me. I was originally diagnosed with a Chiari 1 Malformation. I was told that it was congenital and due to my mother either using drugs or not getting proper prenatal care, which was crushing to hear, but not all that unlikely since I was born in the early 1970s. Little did I know, that these assumptions weren’t based on my findings, but on what textbooks have said since before the advent of the MRI in the early 1980s. Sadly, the percentage of Chiari patients that are given these same faulty assumptions remains near 100%. It didn’t take much time after my decompression surgery for me to realize, that it wasn’t quite as easy or cut-and-dry as it had been made out to be. When you have a connective tissue disorder, that is the root cause behind your Chiari diagnosis, the risk of postoperative complications is high, especially when pathological conditions went undiagnosed and untreated when the decompression was done. The difficulties of having a genetic mutation that can cause problems throughout your entire body, can make a patient seem like a hypochondriac, and that is exactly what most doctors think when they have a subpar understanding of Ehlers-Danlos Syndromes. When something goes wrong structurally, it can cause a cascading effect that manifests throughout the body. We go into the Chiari fight understanding that something is wrong with our brain and neck, but when it crosses over to problems with the autonomic nervous system (for instance), we don’t realize that the continued compression on our brainstem is why our hearts are now beating so fast, why we are now so short of breath, or why we are having fainting spells. We find ourselves wishing for someone, or a panel of experts to help us navigate through it all. While it is still far from the panel that we really need, Palliative Care can offer help with some essentials.

    What is Palliative Care?

    Palliative Care (pronounced “pal-lee-uh-tiv” care) is a subspecialty of medical care, where an interdisciplinary team of professionals (both medical and social) are committed to helping provide “relief from symptoms and stress” for patients with serious, life-altering illnesses, and their families. [1] Palliative Care is “supportive care,” by professionals committed to you as a patient with a serious illness! Your Palliative Care Team generally consists of a Palliative Doctor, a nurse, pharmacist, social worker, nutritionist, and a chaplain [2] (all as needed). Together, they will seek to:

    • Ease symptoms and/or help control pain to relieve suffering
    • Help improve your quality of life
    • Help coordinate with your care team
    • Assist with stress, fears, anxiety, and/or depression in the patient, caregiver, and/or family
    • Help you create a plan for end-of-life care (directives)

     

    Benefits to Palliative Care:

    • Both Chiari Malformations and Ehlers-Danlos Syndromes are relatively complex. Both conditions can spur a vast array of symptoms. One of the objectives of your Palliative Team is to help you find a way to alleviate symptoms and/or help you with coping mechanisms through them.
    • Because Palliative Care is designed for patients with “serious” medical conditions, being accepted as Palliative Care patient, means that they recognize the seriousness of our condition. For those of us that have spent years/decades with our symptoms being dismissed, this alone is no small thing.
    • The CDC Guideline for Prescribing Opioids, as well as many of the state laws regarding the same, have exemptions for palliative patients (along with cancer and end-of-life patients). Doctors can still decide to discontinue prescriptions, taper your prescriptions, or require an “opioid contract,” but it will be 100% by their choice, and not due to CDC Guidelines and laws. Although, you may have to tell them that. [4]

     

    Misconceptions Surrounding Palliative Care:

    • While it is outlined in the U.S. Department of Heath & Human Services (NIH) under the “National Institute on Aging” (NIA), there is NO AGE CRITERIA to qualify as a Palliative Care patient. [1]
    • While Palliative Care is frequently listed beside Hospice Care and/or End-of-Life Care, there is no national requirement for a patient to be in an end-of-life situation (or even an expected early demise). [1] Hospice care falls under palliative care, but palliative care expands beyond the scope of just hospice. Palliative care may begin at any stage of a serious illness. [2]
    • You can continue treatments (even curative treatments) with your personal doctor/specialist, while receiving palliative care. There is no “incurable condition” requirement for a serious condition under palliative care. [2][3] In fact, a patient can qualify and receive Palliative Care whether their illness is curable, chronic or life-threatening. [2]
    • You do not need to wait until your condition reaches a certain level of severity, you can start with Palliative Care at any stage of your illness. In fact, Palliative Care works best when it begins as early as possible in the illness, as some symptoms may be avoidable or more manageable if addressed early.
    • Palliative Care does not replace or override your pain management doctor, or any of our doctors for that matter. Instead, we give them permission to discuss our case with our medical professionals, so they can help coordinate our care, especially in regard to our pain/suffering and quality of life care.
    • Your Palliative Care Team will not consist of the medical specialists that we specifically need, but they can help navigate you to the type of doctor you may need and discuss these recommendations with your doctors.

     

    Problems Surrounding Palliative Care:

    • Primary Care Doctors (who are supposed to be offering referrals to Palliative Care for their patients with serious medical conditions) often fail to fully understand the spectrum of Palliative Care. Because of their faulty understanding, most of us are never offered Palliative Care, and when we request it, we are often told that it is equivalent to hospice care or set aside for hospice patients, and therefore they believe that we do not qualify. THEY ARE WRONG!
    • Our medical professionals often fail to recognize just how “serious” of a condition and life-altering Chiari and EDS really are (on us as patients and on our families).
    • Not all insurance companies cover Palliative Care. Because some insurance companies fail to see the seriousness of our conditions, qualifying may be difficult. Private insurance and HMOs are more likely to offer Palliative Care. Different states have different policies regarding the level of Palliative Care (if any) they offer. [2] If cost concerns are an issue, the social worker on your Palliative Team can assist you in ways that might help you qualify. [3]

     

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    References:

    1. “What Are Palliative Care and Hospice Care?” U.S. Department of Health and Human Services, National Institute on Aging, 17 May, 2017. <https://www.nia.nih.gov/health/what-are-palliative-care-and-hospice-care>

    2. “Frequently Asked Questions About Hospice and Palliative Care.” American Academy of Hospice and Palliative Medicine. <http://palliativedoctors.org/faq>

    3. “Handout for Patients and Families.” Center to Advance Palliative Care. <https://getpalliativecare.org/handouts-for-patients-and-families/>

    4. “CDC Guideline for Prescribing Opioids for Chronic Pain.” Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Unintentional Injury Prevention. 29 Aug, 2017 <https://www.cdc.gov/drugoverdose/prescribing/guideline.html>

  • Finding Hope In The Seemingly Hopeless Chiari Fight!

    Despite the pain we face on a daily basis, that our doctors so often ignore…

    Despite the anger that builds towards a medical system that is relatively
    clueless about our conditions…

    Despite the frustrations we face when those we love fail to understand what we’re going through…

    … Despite it all, hope remains!

    Purple-flower-HOPE_AS185193152.jpeg
    “They tried to bury us. They didn’t know we were seeds.”

    There is little easy about the Chiari fight (and that of its co-morbid conditions), but it is easy to lose hope! The thing about that however, is if you look at the complexities of our bodies and the conditions we face, one thing is for sure, we weren’t created for easy! Coping with conditions like Chiari Malformations, Ehlers-Danlos Syndromes, and their many ugly friends can be a very daunting task that we all have to face. There are many challenges, including chronic pain that can be severe, fatigue that can be debilitating, the fight with doctors for proper diagnosis and treatment, the fight with loved ones to be understood and supported, and the general lack of awareness of these conditions in the public. It is easy to lose sight of the light at the end of the tunnel. But, even with all the hardships we face, there is a lot to still be hopeful about.

    Education & Research 
    One of the most frustrating aspects of dealing with Chiari and its comorbidities is the lack of education within the medical community. There is little more disconcerting than walking into a doctor’s appointment and being asked to spell your condition, so the doctor can Google it. These appointments often end in tears because the doctor has absolutely no clue about our condition, but this is improving. Just ten years ago, few of the doctors we encountered had heard of Chiari Malformation, we had to explain it to almost every doctor we came across, regardless of their specialty. This is happening far less now. While there are still many misconceptions that medical professionals tend to have about these conditions, many have a general level of familiarity now. That means we are making real progress and the future is looking brighter. In a recent webinar on Chiari, Complex Chiari and Craniocervical Instability, Complex Chiari expert, Dr. Paolo Bolognese shared the following:

    In reference to the association between EDS and Complex Chiari, Dr. Bolognese shared that he stumbled upon the association in 2002 during office visits, when he saw a half dozen patients in a 2-week period diagnosed with both. After consulting colleagues and experts in genetics and connective tissue disorders, Dr. Bolognese and his colleague, Dr. Milhorat, conducted a pilot study of the association and published their findings in a major neurosurgical journal in 2006. These findings were met with much skepticism among even the world’s top Chiari experts. In 2017, however, at the XXIX ASAP Conference on CM1, Dr. Bolognese submitted a questionnaire to these top experts. 63 experts from 4 countries responded. 77.5% agreed that there is an association between EDS and CM1. 99.5% agreed about the existence of Complex Chiari. On this fight to get other experts to see the connection, Dr. Bolognese said, “Science does not have a linear behavior. It goes in accelerations, stops, accelerations, stops, some steps backwards, some steps forward. So, it took 12 years to convince most of the leading experts in our field. It will take 12 more years to convince the ‘regular’ neurosurgeons. It will take 12 more years to convince primary care physicians.”

    While this delay in translating research and scientific discovery into medical practice is frustrating for us as patients, there are also some great reasons to be hopeful. First, there are doctors who have dedicated their careers to helping us find answers and improving our quality of life in the meantime. These doctors are willing to put their professional reputations on the line and face the critics to make improvements in our treatment outcomes. Second, the experts are being convinced, and as more experts come to believe in what we, the patient community, already know to be true, it is more likely that younger generations of doctors and researchers will want to get involved in these areas of research. While many of us are suffering and fighting for care by physicians who fully understand the complexities of our conditions, progress is being made that will likely revolutionize the future of patient care.

    In addition to this little snapshot into the experts’ world, anyone who has spent any time in online support groups over the last decade can tell you that patients are getting diagnosed sooner, tested for comorbid conditions more often, and are having better surgical outcomes because they are better informed and are asking all the right questions of their doctors. This trend is likely to continue as patients now have better access to pertinent research information through a wide variety of educational materials offered online by Chiari and EDS non-profit organizations. It is finally OUR time and each of us can do our part to make the most of it, by educating ourselves and sharing information with others, as well as supporting the organizations that make this information available to us.

    Support
    Thanks to the worldwide availability of the internet and social media, support is easier to access and is more abundant than ever before. Patients and caregivers alike can now find a wide variety of support groups for these conditions on a variety of social media platforms. Groups range from small and intimate, to large and very active, and there is a fit for practically any personality. There are groups for women only, men only, teens only and parents only. There are groups focused on laughter and those focused on hobbies like gaming or crafting. Some groups focus on support, while others focus on information, and some do an excellent job at both. There are international groups where you can interact with other patients from around the world, and there are local groups where you can potentially meet people in your own town or surrounding area. There are also numerous in person support group meetings where you can meet fellow patients, attend educational lectures, participate in awareness activities, and much more. Interacting with others who are enduring similar struggles is not only an opportunity to feel less alone and more validated, it is also an opportunity to help others and even potentially form lifelong friendships.  

    Awareness
    The patient community has made great strides in raising awareness for Chiari and related disorders. Between individuals sharing awareness materials on social media, patients reaching out to local media to share their stories and organizations hosting awareness walks, much needed attention and information is getting out to the public. While we still have a long way to go in making Chiari a household name, we are making progress toward it. You can join in these efforts by getting intentional about awareness! What has helped you, might help others!

    Creating Our Own Hope
    We have been a neglected community for far too long. We have been ignored, dismissed, and sometimes even abused. We have had doctors laugh in our faces, call us crazy, and even accuse us of making things up. It often leaves us feeling hopeless, like there’s nowhere left to turn, but we are standing up and demanding better care. We are demanding that our healthcare professionals educate themselves and study the most recent research. We are demanding that our voices be heard and that we are taken seriously. Although there has been some progress, our fight is far from over. It is just beginning, and we refuse to sink into the shadows because we are mighty warriors who will fight for what we need! When necessary, we will create our own hope! As the old Mexican proverb goes, “They tried to bury us. They didn’t know we were seeds.” Our community is growing every single day and there is a need now, more than ever, to stand up not only for ourselves, but those among us who are vulnerable and need us to do it for them. Please, whether you are a patient yourself, a caregiver, a spouse, family member or friend to someone who is suffering, find your inner warrior and join us in this fight!

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  • Overview: Chiari Malformation [Revised]

    Overview: Chiari Malformation [Revised]

    CHIARI MALFORMATIONS (PRONOUNCED: KEE-AH-REE) ARE STRUCTURAL DEFECTS IN WHICH THE CEREBELLUM, THE HIND PART OF THE BRAIN, DESCENDS BELOW THE FORAMEN MAGNUM INTO THE SPINAL CANAL.

    While Arnold Chiari Malformation (Type 2) was first identified in the late 19th century by the Austrian pathologist Hans Chiari, much of the current medical knowledge has developed since 1985 with the expanded use of Magnetic Resonance Imaging (MRI). The number of patients diagnosed with Chiari malformations continues to increase, and with that increase Chiari Malformation is getting some of the attention the condition has always demanded.

    Chiari malformations (“CMs”) are neurological disorders in which the cerebellum extends out of the skull and into the spinal canal, which in turn blocks the flow of cerebrospinal fluid, puts pressure on the brainstem and spine, and may result in varying degrees of nerve compression. Once thought to occur in 1 in 1000 people, it is now believed to be much more frequent of an occurrence. A 2016 pediatric study found it to occur in 1 in 100 children[1]. However, since the most common type (Type 1) tends to become symptomatic during late teens and early adulthood, it is likely to be much more common when adults are factored in. Females are more likely than males to have a Chiari Malformation (at a ratio of 3:1), and significantly higher amongst those with both Chiari Malformation and Ehlers-Danlos Syndrome (9:1)[2]. We affectionately refer to those that live with this condition, including the attendant pain and frequent disregard from the medical community, as Chiarians (regardless of whether they have had surgical intervention or not).

    While some Chiarians are symptomatic throughout their lifetime, the vast majority of Chiarians (those with Type 1) develop symptoms in their late teens or early adulthood. Those symptoms can range from mild to crippling, and can become severe enough to cause paralysis (often associated with syringomyelia) or death.


    WHAT CAUSES A CHIARI MALFORMATION?

    Multiple factors have been identified which can either cause or attribute to Chiari malformations. Although they too were once thought to be rare, Acquired Chiari malformations are now being diagnosed in increasing numbers. A brief overview of what each of these labels entail, together with a summary of the different types of CM’s, is provided below:

    • Congenital Chiari, is believed to be caused by a posterior cranial fossa hypoplasia (PCFH)[3], which can also be caused by a connective tissue disorder such as Ehlers-Danlos.[4] While the cerebellum continued to grow in utero, the posterior skull failed to grow proportionately. Problems resulting from this size discrepancy continue and eventually the overcrowding of the hindbrain squeezes the cerebral tonsils downward into the opening of the spinal canal (cranial constriction). While the herniation of the cerebellar tonsil(s) can take place during gestation or after birth, because the cause is 100% congenital, and the process most likely began in utero, it is usually considered a Congenital Chiari Malformation when the only pathology found is a small posterior cranial fossa. In one large study, they found those with a Chiari Malformation and no associated etiological/pathological co-factors, with only slightly over 52% having a small PCF. When other co-factors were present, the number of Chiarians found with a small PCF plummeted, and therefore it should be considered acquired until proven otherwise.[5]

    • Acquired Chiari can have one or more possible pathological co-factors; any of which can result in the descent of the cerebellar tonsils. Many Chiarians often mistakenly conceptualize an Acquired Chiari Malformation as being brought on only by trauma; however, “acquired” is an antonym for “congenital,” so an Acquired Chiari Malformation in medical terms is one that a person was not born with. While this can include Acquired Chiari malformations resulting from trauma, it can also include Acquired Chiari malformations resulting from a variety of other medical conditions:
      • Heritable Disorders of Connective Tissue (HDCTs), most commonly Ehlers-Danlos Syndromes (EDS), make the tonsils more prone to prolapse below the foramen magnum.
      • Multiple conditions are known to create a pushing/pulling effect that can result in a tonsillar herniation. These conditions include: Intracranial Hypertension (IH), Atlantoaxial Instability and Craniocervical Instability (AAI/CCI), Tethered Cord Syndrome (TCS), and Intracranial Hypotension (cerebrospinal fluid leaks), Hydrocephalus, and a variety of cysts and brain tumors.[2]

      Special care should be taken when any of these co-morbid conditions exist in conjunction with a Chiari Malformation. Before the consideration of decompression surgery, a plan should be developed which addresses each possible comorbid condition before decompression. This can reduce the likelihood of complications and/or a failed decompression surgery.

    SEVEN TYPES OF CHIARI MALFORMATIONS WORTH DISCUSSING (asterisks “*” indicate commonly known types)

    Chiari Zero:  The lower part of the cerebellum (the cerebral tonsils) are blocking the foramen magnum, but are not descended through. Because of the cerebellum’s position, it blocks the flow of cerebrospinal fluid and all the effects of that blockage are comparable to Type 1.

    Diagnosis Requirements: Symptomology; MRI showing no herniation but the low-lying tonsils that are pressing against the top of the foramen magnum; MRI showing a syrinx (despite the name, Chiari Zero is classified under Syringomyelia and not Chiari Malformation – so a syrinx is technically required for diagnoses). [6][7]

    Treatment Options: With few symptoms, non-surgical treatments might be recommended. When a syrinx is present, a decompression is often recommended before the syrinx has a chance to further develop and cause additional damage to the spine. However, even when a syrinx is present, all pathological cofactors should be explored and addressed prior to decompression surgery.

    Chiari 0.5: In cases of Chiari 0.5, the lower part of the cerebellum (the cerebral tonsils) are descended through the foramen magnum, but descends < 5mm (which is the measurement that some doctors use to define Chiari). Usually labeled “tonsillar ectopia” on radiology reports, the symptoms and effects of the obstruction are generally the same as those experienced with Type 1 or Chiari 1.5.[3]

    Diagnosis Requirements: Symptomology; MRI showing a herniation of < 5mm, unless already properly diagnosed with a Type 1 or Chiari 1.5; presence of a syrinx is not “required” for diagnosis, but as with Chiari Zero, it illustrates that it is causing a problem obstructing the flow of cerebrospinal fluid and may be relevant when deciding between various courses of treatment.

    Treatment Options: The same as Type 1 or Chiari 1.5, respectively.

    *Chiari Malformation Type 1: The most common type of Chiari Malformation, Type 1 is diagnosed when the cerebral tonsils descend below the foramen magnum. Medical professionals unfamiliar with current research surrounding Chiari Zero and Chiari 0.5 (and the symptomology surrounding the blockage of cerebrospinal fluid), believe that a tonsillar herniation of less than 5mm is simply a tonsillar ectopia and only diagnose a Chiari Malformation when the descent is > 5mm. However, the 5mm requirement is controversial, and many doctors now base their diagnoses not solely on measurements, but rather on symptomology and a combination of other factors, including cine MRI’s, a patient’s symptoms, and other relevant factors.[6] Many people with a Chiari Zero, Chiari 0.5, or Type 1 can be asymptomatic for a lifetime: one large study found that approximately 30% of those with a CM measuring between 5-10mm were asymptomatic.[8] If symptoms develop, they often present in adolescence or early adulthood. Anecdotal evidence supports the proposition that once symptoms start, the symptoms often progress rapidly until the damage is stopped surgically.

    Diagnosis Requirements: Symptomology; MRI indicating at least one herniated tonsil (without the brainstem descending as well).[9]

    Treatment Options: Prior to surgery any/all comorbidities should be explored and treated especially if you are found to have a normal sized posterior fossa. However, if you have classic Chiari 1 Malformation with a small posterior fossa, the risks of surgery should be weighed against the severity of symptoms and the impact that symptoms are having on the patient’s quality of life. It is often recommended to treat mild symptoms with medication, with surgical options typically reserved for cases in which symptoms cause more serious medical and quality of life problems. However, symptoms do tend to progress, and studies have shown a correlation between successful decompression surgery and the amount of time between the onset of any symptoms and surgical intervention[10]. See “Decompression Surgery” below.

    Chiari 1.5: This type of CM (often referred to as a “Complex Chiari”) is often acquired as opposed to congenital.  Chiari 1.5 should be the diagnosis when the tonsil(s) and all/part of the lower brainstem (the medulla oblongata) has descended past the foramen magnum. This is usually indicative of another comorbid condition pushing the brainstem downward from above or pulling downward from below.[5][11][12]

    Diagnosis Requirements: symptomology; MRI indicating at least one herniated tonsil AND a downward displacement of all/part of the brainstem; without the other radiological findings associated with Type 2.

    Treatment Options: Treatment options can vary significantly from patient to patient depending on the cause of the Chiari 1.5. While a variety of medical options might initially be used to treat symptoms, it is extremely important that all possible causes and/or comorbidities are thoroughly investigated and treated prior to the consideration of decompression surgery. Failure to identify and treat any such conditions can increase the likelihood of a failed decompression and further complications such as brain slumping, increased cervical instability, etc.

    *Chiari Malformation Type 2 (also known as Arnold Chiari Malformation): Type 2 involves a herniation of the cerebellar tonsils and lower part of the brainstem (the medulla oblongata). Unlike in Chiari 1.5, in Type 2 the fourth ventricle is usually herniated, all/part of the cerebellar vermis (the tissue connecting both halves of the cerebellum) is missing or herniated, the corpus callosum (nerve fibers connecting both hemispheres of the brain) is fully/partially absent (agenesis), and it is almost always accompanied by a myelomeningocele (the most serious form of Spina Bifida, a congenital neural tube defect where the spinal canal does not close properly).[13][14][15]

    Diagnosis Requirements: While a myelomeningocele is usually evident and diagnosed at birth, a brain MRI should confirm the radiological aspects of Type 2.

    Treatment Options: Myelomeningocele is usually treated surgically at birth. If other related problems develop, such as hydrocephalus and/or tethered cord, they are often dealt with surgically as they become problematic. While some with Type 2 are decompressed, anecdotal evidence reflects a general trend of an increased failure rate with decompression surgeries as compared to those with Type 1. Because of this, some neurosurgeons choose not to decompress those with Type 2.

    *Chiari Malformation Type 3: Type 3 is a serious type of Chiari Malformation involving herniated cerebellar tonsils, brainstem, and fourth ventricle. However, in most cases of Type 3, a sac forms out of the back of the skull (encephalocele) that contains brain matter from the cerebellum and the meninges. Type 3 causes severe neurological problems that are evident at birth and has a high infant mortality rate.[16][17]

     *Chiari Malformation Type 4: Type 4 is the most severe type of Chiari Malformation, but does not involve a hindbrain herniation (and therefore arguments have been made that it is not a Chiari Malformation). Instead, it consists of an undeveloped or underdeveloped cerebellum. Most infants born with Type 4 die in infancy.[16][17]


    S    URGICAL INTERVENTION 

    Decompression surgery is currently the only available means of attempting to stop the progression of symptoms of a congenital chiari (with no other pathological cofactors), but decompression is not a cure (not even close). Statistics show that up to 69% of decompressed patients find some measure of relief from surgery (usually headaches)[18]. Most neurosurgeons will give only a 50% chance of helping each individual symptom. Some of the symptoms are irreversible once they develop. Recent studies show that there is a correlation between early surgical intervention and positive post-surgical outcomes.[19] However, we cannot over emphasize the importance of your doctors taking time to find, diagnose, and treat co-morbid conditions BEFORE decompression surgery. If they are not willing to consider comorbidities, they are probably not the doctor for you!

     

     

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    *Original version released January 2018, revised October 2018.

     

    References:

    1     Eltorai, Ibrahim M. “Rare Diseases and Syndromes of the Spinal Cord” Cham: Springer International Publishing: Imprint: Springer, 2016. Page 43, 15.2, <www.springer.com/us/book/9783319451466>.

    2 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    3 Sekula, Raymond F, et al. “Dimensions of the Posterior Fossa in Patients Symptomatic for Chiari I Malformation but without Cerebellar Tonsillar Descent.” Cerebrospinal Fluid Research, BioMed Central, 2005, <www.ncbi.nlm.nih.gov/pmc/articles/PMC1343586>.

    4 Stagi, Stefano, et al. “The Ever-Expanding Conundrum of Primary Osteoporosis: Aetiopathogenesis, Diagnosis, and Treatment.” Italian Journal of Pediatrics, BioMed Central, 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4064514>.

    5 Milhorat, Thomas H., et al. “Mechanisms of Cerebellar Tonsil Herniation in Patients with Chiari Malformations as Guide to Clinical Management.” Acta Neurochirurgica, Springer Vienna, July 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2887504>.

    6 Isik, N, et al. “A New Entity: Chiari Zero Malformation and Its Surgical Method.” Turkish Neurosurgery., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/21534216>.

    7 “JNS JOURNAL OF Neurosurgery OFFICIAL JOURNALS OF THE AANS since 1944.” The Resolution of Syringohydromyelia without Hindbrain Herniation after Posterior Fossa Decompression | Journal of Neurosurgery, Vol 89, No 2, <www.thejns.org/doi/abs/10.3171/jns.1998.89.2.0212?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dpubmed>.

    8 Elster, A D, and M Y Chen. “Chiari I Malformations: Clinical and Radiologic Reappraisal.”Radiology., U.S. National Library of Medicine, May 1992, <www.ncbi.nlm.nih.gov/pubmed/1561334>.

    9 Wilson, Eugene. “Chiari.” CEDSA Home, <www.cedsa.org/index.php/59-quick-reference/73-chiari.html>.

    10 Hindawi. “Surgical Management of Patients with Chiari I Malformation.” International Journal of Pediatrics, Hindawi, 28 June 2012, <www.hindawi.com/journals/ijpedi/2012/640127>.

    11 Kim, In-Kyeong, et al. “Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation.”Journal of Korean Neurosurgical Society, The Korean Neurosurgical Society, Oct. 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2982921>.

    12 Malik, Amita, et al. Chiari 1.5: A Lesser Known Entity. Annals of Indian Academy of Neurology, <www.annalsofian.org/article.asp?issn=0972-2327;year=2015;volume=18;issue=4;spage=449;epage=450;aulast=Malik>.

    13 Wolpert, Samuel M, et al. “Chiari II Malformation: MR Imaging.” American Journal of Roentgenology, <www.ajronline.org/doi/pdf/10.2214/ajr.149.5.1033>.

    14 Yumer, M H, et al. “Chiari Type II Malformation: a Case Report and Review of Literature.”Folia Medica., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/16918056>.

    15 Kim, Irene. “Chiari II Decompression in Patients with Myelomeningocele in the National Spina Bifida Patient Registry (NSBPR).” <http://spinabifidaassociation.org/sbworldcongress/wp-content/uploads/sites/10/2017/04/B.4-Kim-Neurosurgery.pdf>.

    16 “Chiari Malformation Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Chiari-Malformation-Fact-Sheet>.

    17 “Chiari Malformations.” NORD (National Organization for Rare Disorders), <www.rarediseases.org/rare-diseases/chiari-malformations>.

    18 14 Aliaga, L, et al. “A Novel Scoring System for Assessing Chiari Malformation Type I Treatment Outcomes.” Neurosurgery., U.S. National Library of Medicine, Mar. 2012, <www.ncbi.nlm.nih.gov/pubmed/21849925>.

    19  Siasios, John, et al. “Surgical Management of Patients with Chiari I Malformation” International Journal of Pediatrics, Article ID 640127, Hindawi, 2012, <www.hindawi.com/journals/ijpedi/2012/640127>.

  • What’s In A Name? An Expansive Review of the Name and Definition of Chiari Malformation

    What’s In A Name? An Expansive Review of the Name and Definition of Chiari Malformation

    THE DEFINITION OF A CHIARI MALFORMATION HAS BEEN LONG DEBATED. IT REALLY IS NO WONDER THAT PATIENTS AND MEDICAL PROFESSIONALS ALIKE ARE CONFUSED. THEN, WITH US FULLY UNDERSTANDING ALL SIDES OF THE DEBATE, WE DEFINED A CHIARI MALFORMATION AS STRUCTURAL DEFECTS IN WHICH THE CEREBELLUM, THE HIND PART OF THE BRAIN, DESCENDS BELOW THE FORAMEN MAGNUM INTO THE SPINAL CANAL. THIS DEBATE IS BEING ANALYZED THIS YEAR, AS CERTAIN ORGANIZATIONS ARE BRAVING TO ATTEMPT TO BRING DOCTORS ALL UNDER ONE UNIFORM DEFINITION AND DIAGNOSTIC CRITERIA. THEREFORE, AMIDST ALL THE CONFUSION AND DEBATE, WE WANTED TO EXPLAIN THE FACTORS INVOLVED, AND WHY WE WENT WITH THE DEFINITION THAT WE DID, AND WHY ONE STANDARD IS SO IMPORTANT!

    To better facilitate our explanation, we will call all associated terms by their specific medical names:

    Tonsillar Ectopia (TE) = tonsillar herniation of any size
    Posterior Fossa Hypoplasia (PFH) = an underdeveloped posterior fossa

    Chiari Malformation Vs. Arnold Chiari Malformation

    The most common type of Chiari is Type 1 (which includes a Chiari 1.5, where the brainstem is also below the foramen magnum). Many people use the term “Chiari Malformation” when diagnosed with Type 1, while others cling to the name “Arnold Chiari Malformation” with the same diagnosis. Is there a difference? The name “Chiari Malformation” came from Hans Chiari, an Austrian pathologist, who first discovered the malformation in the late 19th century.[1, 2] Julius Arnold, a German pathologist, later expanded on Chiari Type 2, and Type 2 took on his name “Arnold Chiari Malformation.” Therefore, technically speaking, a Chiari Malformation and an Arnold Chiari Malformation are not the same; Arnold Chiari Malformation is specific to Chiari Type 2 (which usually includes a myelomeningocele, the most serious form of Spina Bifida). However, they are used interchangeably by many, even by medical professionals and the misnomer is of little consequence one way or the other.[3]

    Chiari Malformation = Posterior Fossa Hypoplasia Theory

    Many ascribe to the theory that a Chiari Malformation ONLY consists of a posterior fossa hypoplasia (which means that the back of the skull is malformed, and therefore the cranial area (space) at the rear is too small). They believe that a tonsillar ectopia is only a symptom, and a Chiari Malformation can exist with or without an accompanying ectopia. This argument is not without merit, because much of what was initially being looked at by Hans Chiari were deformities in the posterior skull upon postmortem examination (so there wasn’t soft tissue to analyze). He originally attributed much to hydrocephalus, but expanded his research into the pons, medulla oblongata, and cerebellum (which can all be attributed to intracranial pressure as a pathology of a “tonsillar ectopia”). To ascribe to this belief would also mean that “Acquired Chiari Malformations” cannot exist, as one doesn’t “acquire” a small posterior fossa. And that would also mean that Chiari Type 2, Type 3 and Type 4 technically would not be a Chiari Malformation at all either, since their definitions do not require a posterior fossa hypoplasia. Perhaps type 3, which has an opening at the back of the skull, but no “small posterior fossa” is even implied in the definitions.

    But to look at the full history of what became known as a Chiari Malformation, we can begin by looking at the research of a German pathologist, named Theodor Langhans. In his research in 1881 (a decade before Hans Chiari conducted his research on what became known as a Chiari Malformation), while looking at syringomyelia (“a cavity created in the spinal cord”), he noted a “change in the cerebellar cavity.” Upon dissection of the cerebellum, he described the cerebellar tonsils as “two symmetrical pyramidal tumors,” pushing the brainstem forward.[4] In fact, the other noted researchers: Nicholas Tulp (1593–1674), John Cleland (1835–1925), and Julius Arnold (1835–1915), all centered on the hindbrain hernia [herniation] without speculation as to its etiology/pathology. It is said that “many of the English translations of Chiari’s work contain inaccuracies.” But note that Chiari’s first paper was on “ectopia of cerebellar tissue,” and that he went on to define Type 1 as showing, “elongation of the tonsils and medial parts of the inferior lobes of the cerebellum into cone shaped projections, which accompany the medulla oblongata into the spinal canal.”[5] Which sounds like what is now known to be a Chiari 1.5. Much later, in 1938, at a time when the posterior fossa decompression became the popular surgical treatment for a Chiari Malformation, a Chiari 2 patient “underwent posterior fossa exploration with the authors not considering hindbrain herniation in their differential. Penfield and Coburn later stated that: ‘In retrospect it seems that we should have suspected the Arnold-Chiari malformation. Instead, a suboccipital craniotomy was carried out…” So even the early neurosurgeons seeking to perfect their surgical treatment felt that it was a mistake to concentrate on the posterior fossa and not take into account etiologies of the hindbrain herniation. That mistake is still going on 80 years later.[6]

    The biggest problem that they are going to have with strictly defining a Chiari Malformation as a small posterior fossa resides in the fact that the diagnosis criteria for a Chiari Malformation only consists of ONE MEASUREMENT, the length of the tonsillar ectopia (how far the tonsils herniate below the foramen magnum). Generally, there are no measurements of the posterior fossa taken when radiologists make the initial diagnoses. Furthermore, most neurosurgeons see the radiology reports, and depending on symptomology, they make the decision to decompress or not to decompress without ever measuring the size of the posterior fossa. Most never look for (and often do not know about) etiological/pathological cofactors that could have been causing the tonsillar prolapse in the first place.

    Where does this assumption leave us?
    Unfortunately it leaves most of us with failed decompressions, fighting with our neurosurgeons that “something is still wrong.” These neurosurgeons look at their post-operative checklist and see that they successfully did everything surgically required in their out-of-date textbooks:
    1. Suboccipital bone was appropriately decompressed. ✔️
    2. Dura was opened and dura patch was successfully inserted. ✔️
    3. Lamina was successfully removed from the C1 (and sometimes the C2 as well). ✔️
    They did all that was required of them based on the diagnoses presented! They don’t have time (or don’t care) to look beyond that, so once again, the idea of our continued symptoms are thought of as being psychosomatic.
     
    While we applaud the efforts of those seeking to get a measure of consistency in how Chiari is defined, the truth remains that until the diagnosis criteria is changed as well, we are being diagnosed with Chiari Malformation based on our tonsillar herniation; it is presumed to be congenital; we are being surgically treated as though it is congenital, and we are ending up with failed decompressions. This confusion is beyond unacceptable, it’s reprehensible!
    When it is all redefined, hopefully we will have a well defined diagnosis criteria, or it is all irrelevant. And the many that really did acquire what was assumed to be “congenital” who are now being told that they do not have Chiari Malformation at all, will be able to get lawyers for “an improper diagnosis” that lead to the incorrect brain surgery being done. There are surgeons coming around and finally seeing that there is merit to these studies that have been done since the late 1990s, that have shown a pushing/pulling effect that can cause the tonsillar ectopia that gets us diagnosed with a Chiari Malformation, and we applaud them for having the integrity to stand up and get it right. That’s exactly what we need and deserve!
    If you were diagnosed with a Chiari Malformation and want to know how all of this might be affecting you, we encourage you first to find your initial radiology reports, and see if there were measurements taken of the posterior fossa. And then wait with that information… wait and see what changes are actually made to the definition. While you are waiting learn. Learn everything you can about every etiological/pathological cofactor, and every comorbidity. If it is “officially” redefined as a small posterior fossa, we will have to work together as a community (like we always do) to help lawyers see how we have been getting lost in the shuffle, year after year. If it’s not officially changed and Chiari continues to be defined as a structural defect involving the cerebellar tonsils, we will have to continue in our fight to make these cofactors of Acquired Chiari Malformation known!

    [wpedon id=”4396″ align=”center”]

    References:

    1 Tubbs, et al. “Hans Chiari (1851–1916).” Journal of Neurology, Pioneers in Neurology, Springer Berlin Heidelberg, 26 Mar. 2010, <https://link.springer.com/article/10.1007/s00415-010-5529-0>.

    2 “Hans Chiari.” Whonamedit – Dictionary of Medical Eponyms, <www.whonamedit.com/doctor.cfm/1123.html>.

    3 Tubbs, R. Shane, and W. Jerry Oakes. The Chiari Malformations: A Historical Context . 2013, <https://pdfs.semanticscholar.org/79dd/127d31820d612600c0b032225437295d86c3.pdf>.

    4 Mortazavi, M M, et al. “The First Description of Chiari I Malformation with Intuitive Correlation between Tonsillar Ectopia and Syringomyelia.” Advances in Pediatrics., U.S. National Library of Medicine, Mar. 2011, <https://www.ncbi.nlm.nih.gov/pubmed/21361763>.

    5 Pearce, J M S. “Arnold Chiari, or ‘Cruveilhier Cleland Chiari’ Malformation.” Journal of Neurology, Neurosurgery & Psychiatry, BMJ Publishing Group Ltd, 1 Jan. 2000, <https://jnnp.bmj.com/content/68/1/13>.

    6 Mortazavi, Martin M., et al. “The First Posterior Fossa Decompression for Chiari Malformation: the Contributions of Cornelis Joachimus Van Houweninge Graftdijk and a Review of the Infancy of ‘Chiari Decompression.’” SpringerLink, Springer, Dordrecht, 6 Apr. 2011, <https://link.springer.com/article/10.1007%2Fs00381-011-1421-1>.

  • Submit An Article For Publication Consideration

    Have you written something that you think would be of value to the Chiari community? Consider publishing it with us! It might be exactly what someone else needs to hear for them to make it through their next mile of the fight!

    Not all of our pieces are technical pieces. We want articles that are encouraging, uplifting, and even those that might be seen as controversial. We feel it is both necessary and prudent in our fight to talk blatantly about what Chiarians go through at the hands of our medical professionals and insurance companies alike (in a general sense), but because we don’t want anyone to get “blacklisted” by those they must do business with, we have certain protocols in place to help protect the identities of authors that do such controversial pieces. Please see our Author Submission Agreement and ensure that you are agreeable to the terms before submitting your article for consideration. We are very particular about violating copyright laws, so the signed agreement is required before publication.

    We have limited the character count to 10,000 characters including spaces (that is just over 2 full, typewritten pages). Anything more than that lessens the chances of it being read by our readers. (If you desire to go over that, it will take approval from our editor. Submit what you have with the limit and when she contacts you about your story, you can request what you would like to add that makes it exceed the limit.) So think carefully about what you want to share and start writing!

    [ninja_form id=12]

  • Our Vision

    Our Vision

    Like most of us, when my symptoms started, I felt very alone. I was blessed to have family and friends that helped me through and cheered me on, but there was a part of the fight that only those directly going through it can understand. At that time, online support groups were far from what they are now. The support groups have definitely improved, but there is still so much more that we need. We need knowledge of the studies that are already out there! We need hope for the future! We need training in self-advocacy! We need help in trying to get our bodies and minds as healthy as we possibly can, because like it or not, things can get much worse than they already are. It was from our desire to help meet those needs, that the Chiari Bridges vision began!

    Empowering With Knowledge!
    Chiari Bridges aims to challenge everything we have been taught about Chiari and its comorbid conditions, and write about them in a way that makes them easier to understand. Although, because we know how hard it can be to get our doctors to listen to us, we will do everything we can to supply the studies behind the facts that we write about (that is why our citations are in red, so they are not overlooked). Knowledge is power! Once we as a community have a better understanding of all that is likely to be going on in our bodies, we can arm ourselves with the studies and take them to our doctors and show them the standard of professional knowledge that we expect! The bar has to be raised, for our radiologists and doctors alike, and they are not going to raise the bar for themselves. We have to inspire them to do that and we are going to raise the roof until they do!

    Raising Our Bar!
    One of the biggest problems we see amongst Chiarians is that our broken bodies have degraded our spirits and our confidence. It is time for us to learn to believe in ourselves once again! The doctors that we were taught to respect, got their knowledge by studying. And many have dropped the ball in their studying, yet they continue to operate on our brains, answer our questions with wrong answers, and deny our symptoms, knowing that they are not knowledgeable at all of our condition and its comorbidities. They like to “act” like we are crazy enough to think that we could wrap our brains around medical concepts that their narcissistic disorders incline them to believe that only medical professionals could understand. Well, they truly have underestimated the reality of all that our big brains can truly accomplish! We cannot let their ineptitude become ours, because it is our brains on the line, not theirs! We plan on creating a venue to share about our doctors, the good and the bad, in a way that decreases the potential for legal liability. We have a right to share our experiences – we just need to do it in the right way.

    Encouraging Hopes and Dreams!
    When it comes to conditions such as ours, the first things to come under attack is our hopes and dreams for the future. Even when we start out being hopeful (often because our doctors understate the significance of what has happened to our bodies), by the time we are decompressed and we find that we are still symptomatic, those hopes and dreams quickly diminish. I have been quoted as saying, “If Chiari has stolen your dreams, dream again!” I still believe that and have been in a constant state of having to practice it. We may not be what we originally wanted to be, but if we continue to see all that we can do and not just what we cannot do, we can dare to dream again! We are all multifaceted human beings with broad gifts and talents. We might not be the athletes we once aspired to be, but that says nothing of our strength. We all have the potential to change the world around us! You might be an artist that hasn’t practiced your art in years – start practicing again! You might have always thought about writing books, but because of your diminished hope for the future, you haven’t written in years – pick up a pen and start writing again! The only way to ever know your true potential is to try and try again! Dare to dream again! 

    Helping Chiarians Thrive!
    While I cannot go into much detail on this topic right now, we do have plans in the works to help encourage Chiarians to gain back a sense of control and live out their passions. Some have already started, as we aim to encourage help with self-advocacy, health and fitness, political lobbying awareness, and the like. But we also plan on encouraging Chiarians to start those businesses that have been on their hearts for so long (not multi-level marketing ploys either, but authentic, heart-inspired, creative businesses), and helping get the word out that it is Chiari owned. We as a community can accomplish so much more, but we need to work together to do it, and start applauding one another instead of tearing one another down! We all need a new chapter in our lives, where we overcome and succeed in the things planted in our hearts! 

    Passion and Motivation to Create Change in Our Community!
    When we first embarked on building this bridge, I realized that my vision was much bigger than my ability to do it alone. Therefore, I sought out certain people within the community that were passionate about specific aspects that can help our community as a whole. Some loved the idea and still serve on the Chiari Bridges team. Some are on the team and just starting to give birth to all that is in their hearts to accomplish. (I absolutely love fresh ideas and fanning the flame until they happen!) Not everyone loved the idea though. Some saw it as a threat to their own projects. They mocked me and one called me “&*%@ing crazy” (she had no idea how right she was). Instead of taking us up on the offer, she made every attempt to try to stop us, and she and her friends have been reporting every image that we use and claiming it as their images. (Good luck with that!) If wanting to create change makes me crazy, I will gladly wear the crown! Let them talk and let’s do this anyway! While they are busy gossiping, we see an increase in Chiarians losing their battles every year. Last year, two of my good friends ended their fight. That’s two too many! We have no desire to compete with anyone or any other non-profits, especially the ones that serve to try and benefit (and not exploit) our community (that childishness has only served to hurt our community). We actually want to cheer on those organizations, individuals and groups in what they are doing and do all we can to help generate support in their efforts. There really is enough need in our community for them to do what is in their hearts to do and still be active and true to what is in our hearts to do. If you have passion and motivation, and just need an outlet or help doing it, tell us about it. It takes a village working together to bring about change!

    Oh, and by the way, our vision for change is global! Our bar is not low. We aim to change the world and want to connect with those who desire the same!

    Michelle Cole
    President, Chiari Bridges, Inc.

     

    [wpedon id=”4396″ align=”center”]

  • The Chiari Malformation Ehlers-Danlos Connection

    The Chiari Malformation Ehlers-Danlos Connection

    CHIARI (KEE-AR-EE) MALFORMATIONS ARE FAR FROM RARE, THEY ARE JUST RARELY UNDERSTOOD, EVEN BY MOST MEDICAL PROFESSIONALS. A CHIARI MALFORMATION EXISTS WHEN THE LOWEST PART OF THE HIND BRAIN (THE CEREBELLAR TONSILS) PROLAPSES INTO THE HOLE AT THE BOTTOM OF THE SKULL (FORAMEN MAGNUM), ENTERS THE SPINAL CANAL AND OBSTRUCTS THE FLOW OF CEREBROSPINAL FLUID (CSF), PUTS PRESSURE ON THE BRAIN STEM AND SPINE, AND MAY RESULT IN VARYING DEGREES OF NERVE COMPRESSION.

    PREVALENCE OF CHIARI: Once thought to occur in 1 in 1000 people, it is now believed to be much more frequent of an occurrence. A 2016 pediatric study found it to occur in 1 in 100 children[1]. Since Chiari Malformation Type 1, the most common type, tends to become symptomatic during late teens and early adulthood, it is likely to be much more common when adults are factored in.

    THE CONNECTION: Chiari malformations were originally believed to be caused by a posterior fossa hypoplasia (small area inside the back of the skull) and doctors speculated that lack of maternal prenatal care or drug abuse caused the deformity. However, as studies continue, they are finding that many with this hind brain herniation have connective tissue disorders, such as Ehlers-Danlos Syndromes. Ehlers-Danlos Syndromes involve a mutation in one of the collagen genes. Collagen is a protein that is often described as a “cellular glue” that helps hold the body together. When that glue fails to hold, everything seems to go awry; specifically, as related to Acquired Chiari Malformations: organs tend to prolapse, and bones begin to shift as joint laxity increases (including the bones/vertebrae at the craniocervical junction). They are finding that these acquired Chiari malformations are far more common than originally thought. There are many pathological co-factors that can cause or attribute to the formation of a Chiari Malformation, and most can be linked to these Heritable Disorders of Connective Tissues (HDCTs), including a posterior fossa hypoplasia. In one large study, they found those with a Chiari malformation and no associated co-factors, with only slightly over 52% having a small Posterior Cranial Fossa (PCF). When other co-factors were present, the number of Chiarians found with a small PCF plummeted and therefore it is should be considered acquired until proven otherwise.[2]

    DIAGNOSES: A decade ago, it took 10-20 years from the onset of symptoms to be diagnosed and now it takes an average of 1-2 years, because medical professionals are slowly beginning to look for it. Magnetic Resonance Imaging (MRI) remains the best tool for diagnosis. Some medical professionals believe that a tonsillar herniation of less than 5mm is simply a tonsillar ectopia and only diagnose a Chiari malformation when the descent is > 5mm. However, the 5mm requirement is controversial and many doctors now base their diagnoses not solely on measurements, but rather on symptomology and a combination of other factors (including Cine MRI, a patient’s symptoms, and other relevant factors). Due to the prevalence of connective tissue issues, gravity often proves to be a significant factor and should be taken into account by use of an upright MRI whenever possible.[3] EDS should be diagnosed by a geneticist before surgery is considered.

    TREATMENT OPTIONS: There is no cure for a Chiari malformation, but there are treatment options. When symptoms are minimal and not life-altering, pain management is usually offered. However, it is important to know that while medications may ease some symptoms, Chiari symptoms tend to be progressive. Decompression surgery is the only treatment available to attempt to halt the progression of the damage being done to your Central Nervous System (CNS). The most common reason that decompression surgeries fail, is undiagnosed co-morbid conditions, especially those that can be etiological/pathological co-factors. More than one surgery might be necessary to successfully treat a Chiari malformation and any/all co-morbid conditions and there is a correlation between early surgical intervention and positive surgical outcomes[4].

    [wpedon id=”4396″ align=”center”]

    References:

    1 Eltorai, Ibrahim M. “Rare Diseases and Syndromes of the Spinal Cord” Cham: Springer International Publishing: Imprint: Springer, 2016. Page 43, 15.2, <www.springer.com/us/book/9783319451466>.

    2 Milhorat, Thomas H., et al. “Mechanisms of Cerebellar Tonsil Herniation in Patients with Chiari Malformations as Guide to Clinical Management.” Acta Neurochirurgica, Springer Vienna, July 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2887504>.

    3 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    4 Siasios, John, et al. “Surgical Management of Patients with Chiari I Malformation” International Journal of Pediatrics, Article ID 640127, Hindawi, 2012, <https://www.hindawi.com/journals/ijpedi/2012/640127>.

  • Overview: Complications Associated With A Chiari Decompression

    Overview: Complications Associated With A Chiari Decompression

    From Intracranial Hypertension (formerly known as Pseudotumor Cerebri), Hydrocephalus, Tethered Cord Syndrome, to conditions related to the presence of a connective tissue disorder, such as Ehler’s-Danlos Syndrome, the primary reason for post-decompression complications seen in the Chiari Patient Community continues to be largely related to undiagnosed and untreated comorbid conditions. Time and time again, we see decompression failure, or a recurrence of symptoms after decompression, because there are other underlying conditions that need to be addressed. For this reason, we strongly recommend that patients get evaluated for the possibility of these known comorbid conditions before undergoing decompression surgery, unless circumstances require emergency surgery. (More information about the testing we recommend can be found in “The Treatments” article). Potential complications of decompression surgery may vary, depending upon the specific technique used, such as whether a duraplasty is performed, and how much bone is removed during a suboccipital craniectomy. The most common complications are infection, CSF leak, and Pseudomeningocele in adult patients.


    INFECTIONS

    Surgical site infections:
    A surgical site infection is a risk of any surgery. While hospitals and surgical staff strive to maintain a sterile environment, hospitals are known for harboring pathogens, including many that are antibiotic resistant. Patient factors, including diabetes, age, being overweight, and being a smoker can also increase a patient’s risk of developing a post-operative infection.[1] Antibiotics are typically given post-surgically (and sometimes before surgery) in order to reduce the risk of infection. Some infections require wound revision surgery, to remove pus and infected tissue in order to improve healing.


    MENINGITIS

    Meningitis is an additional surgical risk when the dura is opened during a decompression. It is characterized by inflammation of the meninges, the linings of the brain. There are three main types of meningitis: aseptic, bacterial and chemical.

    CSF Meningitis/Blood (left); CSF Meningitis (middle); CSF (right)

    ♦ Aseptic Meningitis is by far the most common type, and is generally less severe than the bacterial type. Most cases of aseptic meningitis are caused by viruses, but may rarely be fungal, autoimmune, parasitic or drug-induced.[2] The treatment for aseptic meningitis is usually supportive care.[3] Chemical meningitis is also a risk any time surgery or other procedures or treatments are performed on the brain or spine.[4]

    ♦ Bacterial Meningitis is much more serious and can be life threatening. Three types of bacteria cause most cases: streptococcus pneumoniae, Group B streptococcus, and Neisseria meningitidis. Typical treatment includes antibiotics and supportive care.


    CSF LEAKS

    Duraplasty leak:
    Post-decompression CSF leaks are a risk of decompression surgery whenever the dura is opened. The risk of a CSF leak dramatically increases with the presence of untreated hydrocephalus[5], intracranial hypertension (IH)[6], and connective tissue disorders, such as Ehlers-Danlos Syndrome. Nationwide statistics indicate that the risk of a CSF leak post-duraplasty is 10-15%. However, some surgeons report a significantly lower incidence of CSF leaks in their patients.[7] The use of biologic glue to seal the dural suture line has greatly reduced the incidence of post-duraplasty CSF leaks. A common sign of a leak is clear fluid leaking from the incision site.

    Pseudomeningocele:
    A pseudomeningocele is a type of CSF leak, where the leak creates a pocket in the muscles in the back of the neck. It is one of the most common complications of duraplasty. While some surgeons have managed to keep the incidence of pseudomeningocele low in their patients, some report an incidence as high as 18%.[8] A study at Vanderbilt University at 2013 showed that the development of a pseudomeningocele after decompression significantly reduced the benefit of decompression at one-year post-op on pain, disability and quality of life.[9] A smaller pseudomeningocele may re-absorb on its own. However, with large and persistent PM’s, the duraplasty may adhere to the cerebellar tonsils, blocking flow and making a revision surgery more difficult. There is much debate among neurosurgeons as to whether doing routine duraplasty as part of decompression outweighs the risks. Some argue that duraplasty increases the risk of complications, while others say that failure to perform duraplasty often results in inadequate decompression, reduced benefit, and the need for additional surgeries. Some experts argue that duraplasty using the patient’s own pericranial tissue and using water-tight sutures and biologic glue minimizes the risk of a leak and makes routine duraplasty the best option for most patients. A squishy pocket of fluid is often seen near the base of the skull and a PM can be confirmed and monitored with an MRI. In some cases, a surgeon may try draining the pocket of fluid with a needle and syringe.


    BLEEDING AND ANESTHESIA-RELATED COMPLICATIONS

    Excessive Blood Loss:
    Excessive blood loss is a risk of any major surgery, but can be minimized by a careful surgical technique. Patients with connective tissue disorders may have an increased risk of bleeding complications, due to fragile blood vessels, particularly with vascular EDS or vascular crossover symptoms. Cessation of blood-thinning medications, such as warfarin, aspirin and NSAIDS also reduces the risk of bleeding.

    Anesthesia Risks:
    While risks of general anesthesia are quite low, the risk may be higher if you or someone related to you has had previous adverse interaction to general anesthesia. Some EDS patients are also prone to anesthesia issues, such as requiring more anesthesia or ineffectiveness of local anesthetics. Therefore, it is important to inform your anesthesiologist of your pertinent medical history.[10]


    BONY REGROWTH

    Regrowth of the bone removed during decompression is a risk associated with the pediatric patient population, particularly patients under the age of 2. Surgeons have reported as much as a 50% incidence of bony regrowth in patients under the age of 5, and as much as 80% in patients under age 2. Regrowth of bone may result in the need for future surgery.[5]


    CRANIOCERVICAL INSTABILITY

    While Craniocervical Instability is not uncommon among those with connective tissue disorders, it is pretty rare in the general population. However, aggressive bone removal during decompression surgery can create an unstable craniocervical junction. It is important to discuss with your surgeon how much bone they plan to remove, and the risks and benefits of laminectomy, particularly if you also have a connective tissue disorder, which increases your risk for developing instability.


    CEREBELLAR SLUMPING (PTOSIS)

    Cerebellar slumping (aka cerebellar ptosis) occurs as a result of too much bone being removed around the foramen magnum that there is no longer enough bone to support the weight of the cerebellum. The brain slumps downward toward the spine, re-herniating the cerebellar tonsils, and often compressing the cerebellum itself against the back of the skull and brain stem. This can often result in worse symptoms than the patient had before decompression. Surgical techniques have been developed to revise the decompression and provide more support to the cerebellum.[11]


    OCCIPITAL NEURALGIA

    Occipital neuralgia is nerve pain, often accompanied by numbness and/or tingling, of the occipital nerve in the back of the head. It can be caused by compression of or damage to the occipital nerve. While the presence of a Chiari malformation itself can cause compression of the cranial nerves, including the occipital nerve, decompression surgery can also cause occipital neuralgia. This can be due to compression of the nerve from the use of retractors to hold apart musculature during surgery, or the build-up of scar tissue around the nerve. More conservative treatment of occipital neuralgia may include medications, such as lidocaine patches and medication that target nerve pain, physical therapy, cutaneous nerve stimulators, and nerve root blocks. Severe and persisting occipital neuralgia may require surgical decompression of the nerve or occipital neurectomy, surgical removal of the occipital nerve.[12]


    SCAR TISSUE AND ADHESIONS

    Like with occipital neuralgia and pseudomeningocele, the development of scar tissue and adhesions can cause symptoms to return or failure to relieve symptoms after a decompression surgery. Adhesions and scar tissue can develop wherever tissue is cut, including the dural graft, cauterized tonsils and the skin incision. Scar tissue and adhesion can inhibit or block CSF flow and often require revision surgery to remove the scar tissue. A careful selection of the graft material used for a duraplasty may reduce the risk of developing adhesions and scar tissue.[13] 


    DECOMPRESSION FAILURE

    While perhaps technically not a complication, the failure rate of decompression surgery to alleviate symptoms deserves a mention here. While proper complications can often result in the failure of a decompression to relieve symptoms, or in fact, may make them worse than before decompression, even complication-free decompressions surgeries have a high rate of failure, as much as 40%, depending upon the study. Some reasons for decompression failure in the absence of the above-listed complications include failure to diagnose and treat comorbid conditions that may be causing symptoms, an inadequate decompression (failure to create enough space by removing bone and performing a duraplasty), and some or all of the symptoms being due to another cause, such as migraines. In cases of an inadequate decompression, a more aggressive decompression revision surgery may provide relief. In cases where a comorbid condition exists, that condition must be diagnosed and treated. However, there are still a small percentage of patients who do not get relief, even with further decompression and other treatments. The reason for this is not clearly understood.[5]

     

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    References:

    1 Torpy, Janet M. “Postoperative Infections.” JAMA, American Medical Association, 23 June 2010, <www.jamanetwork.com/journals/jama/fullarticle/186132>.

    2 Ramachandran, Tarakad S. “Aseptic Meningitis Treatment & Management.” Aseptic Meningitis Treatment & Management: Approach Considerations, Medical Care, Prevention, 22 Aug. 2017, <www.emedicine.medscape.com/article/1169489-treatment>.

    3 “Meningitis.” Meningitis | Brain & Spine Foundation, <www.brainandspine.org.uk/meningitis>.

    4 McDaniels, Edison. “Chiari Decompression Surgery.” Neurosurgery101-TheBlog, 3 Apr. 2013, <www.surgeonwriter.com/chiari-4/>.

    5 Trumble, Eric. “Chiari Overview & Surgical Issues.” Chiari and Syringomyelia Foundation, Chiari and Syringomyelia Foundation, 13 Oct. 2015, <www.csfinfo.org/videos/physician-lecture-videos/csf-lectures-archive/chiari-overview-surgical-issues/>.

    6 “Chiari Surgery.” Chiari Surgery | Mayfield Chiari Center, 10 Dec. 2017, <www.mayfieldchiaricenter.com/chiari_surgery.php>.

    8 “Pseudomeningocele Following Chiari Surgery Decreases Quality of Life.” CHIARI MEDICINE, 17 May 2015, <www.chiarimedicine.com/blog/2015/5/17/pseudomenigocele-following-chiari-surgery-decreases-quality-of-life>.

    9 Parker, S. L., et al. “Effect of Symptomatic Pseudomeningocele on Improvement in Pain, Disability, and Quality of Life Following Suboccipital Decompression for Adult Chiari Malformation Type I.” Journal of Neurosurgery., U.S. National Library of Medicine, Nov. 2013, <www.ncbi.nlm.nih.gov/pubmed/24010974>.

    10 “The Risks of Anesthesia and How to Prevent Them.” WebMD, WebMD, 2016, <www.webmd.com/a-to-z-guides/anesthesia-risks-what-patients-should-know>.

    11 “Surgical Technique Alleviates Cerebellar Slumping.” Cerebellar Slumping, 31 May 2007, <www.conquerchiari.org/articles/surgery/techniques/cerebellar-slumping.html>.

    12 Mueller, Diane. “Occipital Neuralgia and Chiari Malformation.” CHIARI MEDICINE, 4 Apr. 2013, <www.chiarimedicine.com/blog/2013/4/4/occipital-neuralgia-and-chiari-malformation>.

    13 Attenello, Frank J., et al. Suboccipital Decompression for Chiari I Malformation: Outcome Comparison of Duraplasty with Expanded Polytetrafluoroethylene Dural Substitute versus Pericranial Autograft. 4 Sept. 2008, <www.link.springer.com/article/10.1007/s00381-008-0700-y>.

  • One Painful Fight – Get Ready To Rumble!!!

    One Painful Fight – Get Ready To Rumble!!!

    “Make it stop!”

    “I can’t take this pain anymore!”

    How many times have you, or your loved one, cried out these very words?

    Pain from a Chiari headache can be brought on from the simplest of things – a sneeze, a cough, laughter, or bearing down when going to the bathroom. We never know when the headache is going to strike, how long it will last, or when it will end. We are unable to describe the intensity of the pain to others, and when asked to rate our pain on a scale of 1 – 10, we want to scream, “14!” The radiating, crushing pain of the headaches robs us of our ability to function for days on end. And depending on the extent of damage the Chiari has caused you may also have the burning, stabbing, and shooting involved with neuropathic pain and neuralgia. The frosting on the pain cake, perhaps? With the many co-morbid disorders that go hand in hand with Chiari, such as Ehlers-Danlos Syndrome, Intracranial Hypertension, Hydrocephalus, and Tethered Cord Syndrome, is it any wonder we cry out, “Make it stop!”?

    Once finally diagnosed, whether you feel like you are losing your mind, or rejoicing over finally having answers, I guarantee you, this emotional roller coaster will still prove to be the ride of your life! Even if they say your Chiari was an “incidental finding,” I can almost guarantee that when you look back in hindsight, you will see that some of the signs of Chiari or its comorbid conditions were always there and you thought they were just “normal.” If you have a history (or familial history) of headaches made worse when you cough, sneeze, laugh, flinch, strain, or bend over; neck pain; stomach aches; ankle/knee/hip/elbow problems; hypermobile joints; dislocations; scoliosis; bulging/herniated discs; miscarriages; aneurysms; numbness; muscle wasting (atrophy); vision loss; double vision; or unexplained muscle/eye spasms (and the list could go on); then they are all probably related to Chiari and its comorbidities. If you just got a new diagnosis of “Chiari Malformation,” you might feel fear, with a sigh of relief to finally have a diagnosis to go with the many symptoms you have complained about for years. Whatever you are feeling right now, be sure to take the time to breathe and take care of you, because the battle is an accumulation of long and tiring rounds and odds are, Chiari Malformation is not the only problem you have going on.

    By a wide margin, the hardest part of our fight is dealing with doctors. One would think it would be the never-ending pain, but when your doctors do not believe you, ridicule you, or outright verbally abuse you, it not only adds insult to injury, but it probably illustrates reasons that your doctor(s) just might be the ones that need counseling. As patients, we are paying for them to help us with our medical problems; what we get instead is usually a referral to a therapist because of their ineptitude. The absurd part of this circle of insanity is that when we make ourselves more knowledgeable about our condition(s), because we have no other choice, a doctor that understand the Hippocratic Oath would respond by making themselves more knowledgeable as well, so they can help their patients. Yet, what is far too common are doctors that do not want to know the results of studies, who are complacent with their fifteen minutes of Chiari education, and think if they talk a good game, patients can be manipulated into thinking maybe it’s all in their minds.

    In a distant second, would have to be the heart-wrenching feeling we get when our loved ones put more stock in the opinions of our morally bankrupt doctors, even once we have shared study after study and article after article showing you that our doctors are wrong. What we go through, feeling like our bodies have betrayed us, knowing that our doctors have betrayed us (even if it is because they do not know any better), we need you in our corner. This is the fight of our lives, for our lives, and we should never have to do it alone! The energetic, feisty, loving person that you have loved so much over the years is still inside of us! When your brain falls into your spinal canal and your connective tissue is wasting away, there is no measure of motivation that is going to help fix it! We need help with our battle, someone in our corner! We don’t need judgement or motivational speeches; we need love and understanding – and an occasional shoulder to cry on. We need you to help us stay grounded and remind us that despite the pain and brokenness, we still have value in this world! Without a shadow of a doubt, we really need you!

    What our bodies go through from head to toe really can only be understood by remembering the importance of your Central Nervous System in everything that you do. Every nerve in your body passes through the Foramen Magnum, where the Cerebellar Tonsils invaded, and in most cases, every single one of those nerves are damaged to some degree (and some of that damage is permanent). The headaches we get when we cough, sneeze, heave (or do anything that causes a bounce or even a slight movement of our skulls), can often only be compared to the pain of childbearing – rendering the pain rating scales useless for not having the angry face reaching around and trying to rip his skull out from the base. The neck pain consists of spasms (either continuous or intermittent). For most of us with HDCT, Craniocervical Instability makes our heads constantly feel like they weigh far too much for our weak necks to support (often described as a ‘bobblehead” feeling). Some might consider the pins and needles or ice pick stabbing pains behind our eyes, in our heads (neuralgia) or in our extremities (peripheral neuropathy) to be a crushing reality of life despite surgery.

    Finally, this lack of awareness, education and research makes it even more difficult for those of us who find ourselves no longer able to work. The Social Security Administration has yet to add Chiari Malformation to its list of impairments which would automatically qualify for disability benefits (apparently, they do not know the importance of the Central Nervous System either). Although benefits can still be fought for and awarded, the fight becomes far more difficult than it needs to be. Chiarians often must fight for years, filing appeal after appeal, to prove that their reality is the reality.

    Is their hope? Yes!

    Through it all, and despite all the challenges that face us, we are many, and together, we are powerful! We can change all of this by becoming our own advocates and by raising awareness and dollars for research and awareness information that strives to educate the medical professionals that are treating us. This is our war to fight and being the warriors that we are, we must rise to the occasion – because LOSING IS NOT AN OPTION!

     

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