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.
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:- Suboccipital bone was appropriately decompressed. ✔️
- Dura was opened and dura patch was successfully inserted. ✔️
- Lamina was successfully removed from the C1 (and sometimes the C2 as well). ✔️
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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>.
I was what you would consider a “typical developing child” growing up, I did not have any health issues and was able to enjoy much of my childhood. My journey to finding answers in regard my health began at 15 years old, when I began rapidly losing my vision in my left eye. I dealt with severe headaches and the doctors struggled to draw a connection to my declining vision. I went from 20/20 vision to 20/400 in my right eye and 0 vision in my left. I was considered legally blind. I had to relearn how to navigate life with very little vision.
Fast forward a few years, I was told that a second decompression surgery was required, which I agreed to. It resulted in a rip in my dural patch causing a cerebral spinal fluid leak at the surgical site. After these two decompression and a CSF leak repair surgery, my vision had improved significantly yet I was worse off symptom wise than I was when I initially began noticing changes in my body. Sadly, I was told from my specialists that there was nothing more they could do for me. They referred me to the headache/face pain clinic. After many failed attempts at managing my pain with medications, my doctor mentioned that my symptoms resembled a spinal fluid leak and that there is a doctor who is navigating research and I should be evaluated.
contrast, CT , digital subtraction myelogram, MR myelogram, and the list goes on. After a few months of investigating we were able to confirm that I suffer from spontaneous intracranial hypotension, meaning that I have multiple leaks or suspicious areas in my spine, that happened spontaneously (without known trauma). My doctor mentioned that my Chiari diagnosis is what is classified as an Acquired Chiari Malformation.
After several hours I regained consciousness, family and doctors surrounded me trying to explain what they knew about baby Eric. They explained Eric and I would have to be immediately flown out to a larger center where they could properly care for my baby. Eric couldn’t support his own airway, he couldn’t suck, swallow, or move due to hypertonic muscles. He was having several seizures, all pointed to brain damage. At this point they had no idea what was wrong and could not conclude whether my baby would live. I recall saying I wanted to go see my baby and trying to get up out of the hospital bed, but the nurses told me I couldn’t see him, because I had just gone through a major surgery and he was too sick; we both had to rest for transport. When the nurses told me that, I flipped, telling them that come hell or high water, I was going to see him. I got as far as swinging my legs over the bed before I vomited everywhere. The nurses finally clued in that I was going with or without their help, so they laid me back down and wheeled me to the NICU to see him. I couldn’t even see my baby for he was so small and leads encased his body where he layed. They had his chin strapped up as every time it fell backward it would close off his airway leading to oxygen desaturation. They didn’t have the resources to intubate a baby at this hospital, so they decided to fly us out to Vancouver Children’s Hospital.
begging, pleading to God for your child to be healed. After 3 weeks they finally let me have skin to skin cuddles and started teaching us how to care for him. This isn’t the way it’s supposed to go; you’re supposed to go to the hospital, have a baby and take them home, right? Instead you walk around numb, you don’t remember the last time you ate, or showered, you just feel like you’re having an out of body experience, as if you’re watching this happen to someone else. You’re scared to leave their side for even a minute because what if it’s that minute that he leaves this world and you’re not there?
After about a month we had a meeting, and it was then that the doctors told us we had a failure to thrive baby, his cells didn’t migrate to the right place at the right time, that his cerebellum and brainstem were severely underdeveloped. The statistics 19 years ago was that one out of one million babies were born like this and they had yet to find out what caused the illness. Our baby couldn’t regrow the parts of his brain that didn’t develop. He had gestational arrest at 32 weeks, he had scoliosis, spina bifida occulta, epilepsy and severe brain damage. Our child would never move on his own, suck or swallow or be able to interact and that he would most likely die of aspiration pneumonia. He would live his life in hospital more than out. I have worked with special needs adults with this type of quality of life, I could play the tape to the end, and I knew hanging on to him would only be for selfish reasons. I understood that my baby was in pain, I could see in his beautiful blue eyes, as I said before, a mother always knows. It was at this time Eric’s father and I decided to sign a” Do Not Resuscitate” order. It was without a doubt the single hardest thing I have ever done.
Within 2 weeks, it was clear this child was a strong fighter and wasn’t ready to give up quite yet. We had another meeting and it was decided we would take him home. We wanted his big sister to have time with him and show him what a home was like. We took a 2-week crash course on neonatal nursing. We had to learn how to do his lung physio, how to suction him, and how to work a feeding pump and so much more. Eric was brought home February 10,1999. We did his 24-hour care until March 9th when he took a turn for the worse. He was diagnosed with aspiration pneumonia. This left me in a panic; I wasn’t ready to let him go, and I wanted him sent back to Children’s Hospital and be treated. We had an amazing Doctor who came to the hospital and took me for a walk to discuss why we made the DNR code and why we made the right choice for our son. I took my son home that day knowing we were running on borrowed time. His breathing became very shallow, he turned blue from lack of oxygen and on March 11, 1999 at 3:15 am our darling boy went home to be with his creator. The year following is a fog, I 
remember very little. I was deeply depressed but I knew I had to keep moving forward for my daughter, she needed me. I know she was hurting too but I was so consumed with my own grief, that I couldn’t reach out to her, I couldn’t handle both her grief and mine. My daughter and I have had to take a lot of time since to heal together. With that being said, the pain of losing a child is not something that you can run away from or attempt to forget; I relive my sons small time on this earth every single day.
I had a rough journey with these conditions and I am sharing it to help show how complex we can be and how much we need the medical community to step it up a notch (or ten)! I grew up in Denmark, where I lived when I was diagnosed and had my first surgeries.
Years passed by with various periods of severe pain flares, flares that felt nothing like the pains I had before. But no doctor was really believing me. I had a spontaneous collapsed lung a couple of times in this period and ended up with surgery for this (I woke up under this surgery which later also turned out to have a significance). At 25, one day I had a sudden and severe onset of symptoms – a pain in the back of my head feeling like two stabbing knives. This did not resolve and after several attempts with various medicines, that I only got sicker from, I finally saw a new rheumatologist whom again treated me with harsh accusations of laziness and psychological imbalance. I can assure you he was the one bringing on my tears that day, despite the extra severe pain I had been in for weeks. I was placed in the care of the hospital physios and after a while, it became clear to them that there was something really wrong and they got me to see another rheumatologist, who in turn took their word and referred me for an MRI. I had only just turned 26 when I was diagnosed with Chiari 1 Malformation and Syringomyelia – in my full spine.
I did a bit of internet research as information in Danish was very sparse and realized there was a certain number of bad outcomes due to something called Ehlers-Danlos Syndrome, Retroflexed Odontoid and Basilar Invagination. I asked my neurosurgeon, who was supposed to be the best in Denmark at this point, about these things – he claimed I didn’t have any. However, he did agree to refer me for Ehlers-Danlos evaluation. Here I was told I did not have that either. However, I was bordering on a similar connective tissue disorder called Marfan Syndrome, which they still could not diagnose me with due to my heart and eyes not being faulty. I had my first decompression surgery in December 2006. It was rough. I reacted badly to anesthetics and to the morphine and I also lost a lot of spinal fluid. I could not raise myself up the first month which I thought was normal. Slowly, I got better, and imaging showed my syrinx shrinking. Two and a half years later, though, I started experiencing dizziness and nausea and though my first surgeon didn’t believe me, imaging finally showed a big collection of fluid outside my spinal cord originating from a hole in the duraplasty used to close after tonsillar cauterization at my first surgery. I tried talking to the surgeon about concerns of Klippel-Feil and instability, that I had read about, but they would not hear of it and said that for now they would just focus on this issue. So, this was repaired, and I moved to Spain with my boyfriend at the time. I was placed on a disability pension from Denmark and that enrolled me in the Spanish public healthcare. I did, however, in the meantime follow up on my concerns and contacted a specialist, who had written about the Klippel-Feil and Chiari connection, and he straight away stated I had some severe issues with my odontoid and needed it removed and my neck fused to my skull. My first meeting with a surgeon in Spanish health care came up and he just looked at my imaging two minutes then stated my problems were way bigger than Chiari and Syringomyelia. He also diagnosed a severe retroflexed odontoid and Basilar Invagination – so severe he had a hard time understanding how I could breathe, let alone walk. But given my reasonably good condition, he opted to postpone these surgeries as they are big and not without risks.
A couple of years of enjoying the benefits the climate change gave me (and likely putting my head in the sand) went by but then I could no longer ignore the fact that I was getting worse. I was in a rough period with other matters of life, so it took a while before I realized I couldn’t escape the changes in my body. I started losing weight amongst other things and after a quick detour of fear of stomach cancer, I finally realized that everything that was going on was related to my brainstem compression. So, I went back to the neurosurgeon. He ordered some testing but before it could be done, I ended up admitted urgently after I stopped breathing one night. From here started a roller coaster. I didn’t feel right about their suggestions and the surgeon that was going to operate didn’t feel very secure himself even. I ended up getting transferred to a private hospital in Barcelona that calls themselves a “Chiari Institute.” Had I known what I do now, I would never have paid the fee for a filum release, but the doctor claimed this was what I needed and well… It was worth a shot in this urgent situation. He then sent me home, claiming I was cured. I didn’t feel right and breathing through the night was still a problem, so I started sending my imaging to experts around the world and working on getting referred to another hospital in Spain’s public health with higher expertise. All these experts claimed I wouldn’t have long to live unless I had this odontoid approached.
October 2016 I finally had a partial odontoidectomy and a fusion, which beyond doubt saved my life. It was a rough ride, for both me and the surgeons. They had to deal with complications related to my anatomy, to the mess the first surgeon in Denmark had left – he had indeed damaged my muscles more than I ever knew – and to the problems relating to the soft tissue. I do know they did a great job, but due to all the mistakes, how complicated my case was and is, I am unfortunately not done. I have ongoing issues and though some of these could have been avoided with the right approach from the beginning, some are just the way it is with these conditions.
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!
INCURABLE DISEASES. THE GENERAL ASSEMBLY OF THE UNITED NATIONS HAS GONE ON RECORD STATING THAT, “UNTREATED PAIN IS TANTAMOUNT TO TORTURE OR CRUEL, INHUMAN OR DEGRADING TREATMENT OR PUNISHMENT.” 
“Opioid Crisis” is a new buzz term being overused by national news and government officials to evoke a negative feeling about a drug that was created for helping people. The definition of “crisis” by Merriam-Webster dictionary is partially defined as “an unstable or crucial time or state of affairs in which a decisive change is impending; especially: one with the distinct possibility of a highly undesirable outcome.”
There is also a negative stigma attached to having to take pain meds. Many chronic pain patients take prescription opioids to continue meaningful work. This could be a huge problem because they are afraid to speak out, afraid of losing their job, or at least bringing suspicion upon themselves from employers. Many patients are afraid that if those around them know they take pain medication, they will be seen as addicts. But it is time to brush off the stigma and the fear and to stand up for our rights to access proper and effective medical treatment. If we don’t, we may lose access permanently and keeping our jobs, social lives, and other important activities and relationships will not be possible due to disability from pain.
Doctors that want to be doctors are now caught up wasting time in government “guidelines” or laws and pressure from insurance companies that do not want to cover pain control prescribed by our doctors. Many doctors who have continued to prescribe despite the current hostile regulatory environment have been threatened, raided, and even arrested by the DEA.
Empowering With Knowledge! 
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! 
Zona McGee was blessed with a vibrant and beautiful little boy on July 2nd, 1993. She named him Ryan Andrew and fell in love with his sweet face the second she first held him. He looked just like her! Her husband, Kevin, and daughter Crystal, eight-years old at the time, were over the moon as well.
Ryan was a good student and took a local job after graduating from high school. He loved working and earning his own money. By all accounts, Ryan was a completely normal, functioning, and on-target, young adult. He was doing what most recent high school grads do – living life, having fun, and trying to decide what profession he might go into.
Over the next few months, Ryan was rushed to the E.R. on several occasions due to cyclic, intractable, vomiting along with severe head and neck pain. During these dozen-plus visits to the E.R., he was turned away multiple times and labeled “drug-seeking, weak, and dramatic.” This was a slap in the face, as Ryan was advised to blindly have the surgery to prevent him from returning to the E.R. This painful irony was not lost on Zona, and it only added to her trauma and confusion.
On the last visit to this particular hospital, the chief neurosurgeon refused to treat him neurologically and ordered a psychiatric evaluation instead. After speaking with Ryan, the psychiatrist said, “I do not believe you are crazy, but you are a bit of a wimp.”