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  • Overview: Cerebrospinal Fluid Leaks

    Overview: Cerebrospinal Fluid Leaks

    Cerebrospinal fluid (CSF) is the clear, colorless liquid that surrounds the brain and spinal cord and is contained within a lining called the dura. The cerebrospinal fluid protects and cushions the brain and central nervous system. Among other functions, this fluid provides buoyancy to the brain, allowing it to float and weigh less, thus reducing the pressure at the base of the brain. A cerebrospinal fluid (CSF) leak occurs when there is a tear or hole in the dura that then allows this fluid to escape[1]. When leaks occur, the overall volume and pressure within the skull drops, and the cushioning and buoyancy effect is reduced, causing the brain to slump. In many cases, this leads to a condition known as intracranial hypotension and a vast range of symptoms.

    The main symptom of a CSF leak is a headache that is worse when upright and improves when lying down horizontally. This is sometimes called a “positional” or “orthostatic” headache. However, not all positional headaches can be attributed to a CSF leak, and not all CSF leak headaches are positional. This is particularly the case in the chronic (vs acute) phase of CSF leaks, where the “positional” or “orthostatic” characteristic of symptoms may become more constant, lessen, or disappear entirely, including headache. Symptoms often worsen as the day goes on. Other leak symptoms can include, but are not limited to: nausea, vomiting, neck pain or stiffness, heaviness of head, pain between the shoulder blades, feeling of pressure within the head, changes in hearing (muffled or underwater sensation), tinnitus (ringing, buzzing, or pulsatile), feeling of liquid in the ears, sense of imbalance, sensitivity to light, sensitivity to sound, pain or numbness in the arms, changes in cognition (“brain fog,” memory loss, or loss of concentration), dizziness or vertigo, scalp sensitivity or tingling sensation within the scalp, visual changes (blurring, double vision, visual field defects), pain behind the eyes or when moving eyes, facial numbness or pain, sinus pressure, temporomandibular joint pain and stiffness, and subdural hematoma[2]. Cranial leak specific symptoms can vary even more and can include: fluid discharge from ears, nose (usually only one side) and to back of throat often reported as salty or metallic tasting, recurring or chronic meningitis, loss of sense of smell, change in hearing or ringing in the ears, and less frequently cognitive changes. Rare signs or complications of CSF leaks can include: quadriplegia, dementia (often mimicking Frontotemporal Lobe Dementia), Parkinsonism, other movement disorders, ataxia (unsteady gait), hypersomnolence, stupor, coma, stroke (hemorrhagic or ischemic), and even death.

    CSF leaks are often very hard to locate, if ever. Approximately 50% of leaks cannot be found on imaging. Imaging and other tests used to attempt to find leaks are often read as “normal” even when there is a leak present. Other times, especially (but not always) in the case of chronic leaks, the positional symptoms either lessen or go away altogether, including the headache. Many who are leaking are not even aware that they are leaking. Leaks are often misdiagnosed as well[3]. Some of those common misdiagnoses are Postural Orthostatic Tachycardia Syndrome (POTS), migraines, sinus headaches, Meniere’s Disease, Chronic Fatigue Syndrome, Parkinson’s Disease (sometimes other neurodegenerative diseases), Fibromyalgia, Ehlers-Danlos Syndrome, Tarlov Cyst, Chiari Malformation, Cervical Spine Disease, cervicogenic headache, tension headache, and Sinusitis. To make diagnosis even more complex and elusive, CSF leaks can and do often occur along with any of these disorders and perhaps several simultaneously. A leak can cause an acquired Chiari malformation or coexist and complicate an existing congenital Chiari malformation[4]. Some patients have had unnecessary decompression surgeries when the underlying, sole cause was a leak all along.

    Leaks can be caused by:

    1. Medical procedures (also called iatrogenic leaks) for various diagnostic or therapeutic reasons such as lumbar punctures to collect fluid for analysis if meningitis is suspected, lumbar puncture for injection of contrast (myelography), spinal anesthesia, epidural injections, epidural steroid injections, prior skull base or spinal surgery, CSF shunt over-drainage, prior sinonasal surgery, and chiropractic or other spinal manipulations.
    2. Traumatic injuries such as brachial plexus injuries falls, sports injuries, motor vehicle accidents, roller coaster rides, and other whiplash injuries.
    3. Spontaneous leaks that occur with minimal or no clear cause. Sometimes spontaneous leaks may be associated with some sort of spinal pathologies such as calcified disk material or bone spurs. These leaks are usually ventral (or in front of the spinal cord).

    There is growing evidence suggesting that a significant number of spontaneous CSF leaks occur as the result of a preexisting weakness in the dura[5]. Heritable Disorders of Connective Tissues (HDCT’s) such as Marfan Syndrome, Ehlers-Danlos Syndrome (both classical and hypermobility type), autosomal dominant Polycystic Kidney Disease, and other HDCT’s predispose patients to CSF leaks. One leak expert estimates that “slightly less than 100% of patients with spontaneous CSF leaks have an underlying connective tissue disorder.”[6] The dura is made out of connective tissue and patients with HDCT’s have thinner dura mater, that is more susceptible to tears and leaks. HDTC patients are more prone to spinal conditions such as perineural cysts, meningeal diverticula, and other HDCT defects such as aneurysms and dilatations. Oftentimes, a CSF leak is the first sign of an underlying HDCT.

    Lumbar punctures (LP’s or spinal taps) should be avoided in patients with Chiari Malformation and/or in patients with HDCT’s[7]. There is a risk of causing a herniation of the cerebellar tonsils or making an existing herniation worse from the pull-down mechanism involved in lumbar punctures. Unfortunately, lumbar punctures are not always avoidable and sometimes very necessary, especially in cases to rule out life-threatening viral or bacterial conditions such as meningitis, subarachnoid hemorrhage, encephalitis, or syphilis. In these cases, measures can be taken to minimize LP risks such as using a certain needle type and size, limit the number of cc’s collected (by spontaneous drip ONLY), and, of course, always done under fluoroscopy by a competent physician[8]. Additionally, it is important to be aware that patients with HDCT’s are at greater risk for the dura to fail to heal following an LP. Patient’s should be aware of post-dural puncture headache (PDPH) symptoms and speak with their physicians if they suspect a leak following an LP.

    The procedures and tests used to diagnose leaks will vary between patients and certain criteria are used to diagnose leaks[9]. Some of these tests and procedures might be: endoscopic exam and fluid collection and Beta-2-Trasnferrin testing (cranial leaks), Cisternography (including radioisotope cisternogram), Magnetic Resonance Imaging (MRI) including Magnetic Resonance (MR) myelography, Dynamic CT myelography, Digital Subtraction myelography, and Intrathecal saline infusion-enhanced myelography, a lumbar puncture to collect and test fluid and measure opening pressure. This imaging often includes both brain and spinal imaging. The normal opening pressure is not uncommon and does not rule out a leak. High pressure can also occur while leaking. Pre-existing intracranial hypertension can be related to the development of spontaneous spinal CSF leaks. Some reports suggest that spontaneous cerebrospinal fluid (CSF) leaks are strongly associated with idiopathic intracranial hypertension (IIH). There are 5 main findings on imaging that doctors look for, however, the absence of these findings does not rule out a CSF leak. The mnemonic SEEPS is used for most of these findings: subdural fluid collection, enhancement of pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain[10]. Other imaging findings that might be seen are small ventricles, cisterns might have less fluid, optic chasm might flatten over pituitary, pituitary might enlarge, empty sella, fluid in front of the pons, or pons might become flatter than normal. Repeat imaging is often necessary.

    Treatment of leaks can either be medical or surgical. Conservative treatment is often recommended, if possible. This can include bed rest and avoidance of coughing, sneezing, straining, bending, twisting, and lifting, increased fluid intake and caffeine, the use of an abdominal binder, and sometimes steroids are recommended. Others may have a lumbar drain placed in the low back to decrease the pressure of the CSF around the area of the leak in an attempt to allow this area to heal. Some patients may need an epidural blood patch. Where a blood patch is not successful, a fibrin glue patch may be tried. About 30-40% of leaks occur at multiple sites, especially in those with an HDCT. Multisite patches may be required. Higher volumes of blood may be needed in order to reach where it needs to go, and/or the position of the needle may need to vary from the standard placement (transparietal or lateral placement)[11]. It is not uncommon for several patches to be tried. Many doctors make the mistake that if an EBP fails, there was no leak as well. Sometimes when more conservative and less invasive treatments have failed, neurosurgery may be necessary. Surgical repairs vary and are tailored according to the type and location of the leak. Sometimes in a select set of patients, other procedures have been used including epidural saline infusions through an implanted epidural catheter or lumbar dural reduction surgery. A condition known as rebound intracranial hypertension (RHP) may occur following any of these treatments[12]. Usually, but not always, there is a different pattern to the headache where one feels worse when horizontal and better when upright. Sometimes, acetazolamide (Diamox) or a similar medication is prescribed to help treat RHP.

    Leaks are poorly recognized, poorly understood, under-researched, understudied, often misdiagnosed, can complicate existing conditions, are difficult to find, mimic many other disorders (including Chiari Malformation), and can be comprised of a vast array of symptoms. Most doctors are familiar with the symptoms of a leak in the acute phase. Very few doctors are familiar with how long-term, chronic CSF leaks “present” in regard to headaches and other leak symptoms and often miss the more subtle symptoms of chronic leaks. Like Chiari and other related disorders, no two patients with CSF leaks have the same symptoms and often experience misdiagnosis, delayed diagnosis, are disbelieved concerning their symptoms of the severity thereof, and all too often dismissed to suffer excruciating pain, decline, and debility. Educating yourself as much as possible about CSF leaks will help guide and empower you and those around you who may have existing, suspected or potential future complications that may arise due to CSF leaks.

    CSF Leak Symptoms List: https://dev.chiaribridges.org/glossary/symptoms-of-intracranial-hypotension/

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    References:

    1 “CSF Leak: A Curable Cause of Headache.” A Non-Profit Hospital in Los Angeles, <www.cedars-sinai.edu/Patients/Programs-and-Services/Neurosurgery/Centers-and-Programs/Cerebrospinal-Fluid-Leak/CSF-Leak-A-Curable-Cause-of-Headache.aspx>.

    2 “Cerebrospinal Fluid (CSF) Leak.” A Non-Profit Hospital in Los Angeles, <www.cedars-sinai.edu/Patients/Health-Conditions/Cerebrospinal-Fluid-CSF-Leak.aspx>.

    3 Schievink, Wouter l. “Misdiagnosis of Spontaneous Intracranial Hypotension.” Archives of Neurology, American Medical Association, 1 Dec. 2003, <www.jamanetwork.com/journals/jamaneurology/fullarticle/785098>.

    4 Haider, Ali S., et al. “Spontaneous Intracranial Hypotension Presenting as a ‘Pseudo-Chiari 1.”Cureus, Cureus, 16 Feb. 2017, <www.ncbi.nlm.nih.gov/pmc/articles/PMC5354398/>.

    5 Reinstein, Eyal, et al. “Connective Tissue Spectrum Abnormalities Associated with Spontaneous Cerebrospinal Fluid Leaks: a Prospective Study.” European Journal of Human Genetics, Nature Publishing Group, Apr. 2013, <www.ncbi.nlm.nih.gov/pmc/articles/PMC3598315/>.

    6 Schievink, Wouter l. “Q & A With Dr. Schievink.” Spinal CSF Leak Foundation, 14 July 2014, <www.spinalcsfleak.org/q-a-with-dr-schievink/>.

    7 Erbay, Sami H., et al. “Is Lumbar Puncture Contraindicated in Patients with Chiari I Malformation?” American Journal of Neuroradiology, American Journal of Neuroradiology, 1 Apr. 2005, <www.ajnr.org/content/26/4/985>.

    8 Matas, Sandro Luiz de Andrade. “Why Should We Use Atraumatic Needles in Lumbar Puncture?” Arquivos De Neuro-Psiquiatria, <www.scielo.br/pdf/anp/v71n9B/0004-282X-anp-71-09b-681.pdf>.

    9 Schievink, W. l., et al. “Diagnostic Criteria for Spontaneous Spinal CSF Leaks and Intracranial Hypotension.” American Journal of Neuroradiology, American Journal of Neuroradiology, 1 May 2008, <www.ajnr.org/content/29/5/853>.

    10 Schievink, Wouter l. “Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension.” JAMA, American Medical Association, 17 May 2006, <www.jamanetwork.com/journals/jama/fullarticle/202849>.

    11 Griauzde, J., et al. “Large-Volume Blood Patch to Multiple Sites in the Epidural Space through a Single-Catheter Access Site for Treatment of Spontaneous Intracranial Hypotension.”American Journal of Neuroradiology, American Journal of Neuroradiology, 30 Apr. 2014, <www.ajnr.org/content/early/2014/04/30/ajnr.A3945>.

    12 Kranz, P. G., et al. “Rebound Intracranial Hypertension: A Complication of Epidural Blood Patching for Intracranial Hypotension.” American Journal of Neuroradiology, American Journal of Neuroradiology, 1 June 2014, <www.ajnr.org/content/35/6/1237>.

  • 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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  • The Christopher Ellington Story – A Chiari Warrior’s Journey [Updated]

    The Christopher Ellington Story – A Chiari Warrior’s Journey [Updated]

    My introduction to Chiari malformation I (CM1) begins in 1994. I had been married about 7 months and we had just celebrated our first Christmas together as newlyweds. Shortly after the new year, I developed a bad headache that eventually evolved into losing my eyesight in one eye. I went to the eye doctor, who immediately sent me to the hospital. I was diagnosed with Pseudotumor Cerebri and Papilledema, which are known to often accompany Chiari (co-morbid conditions). At the time, we were told that it was likely due to a virus. I had five failed lumbar punctures and finally a successful sixth in radiology, was given Diamox, and the problems went away. Nothing was ever said about Chiari or an abnormal MRI. I also had no idea that I could or should get a copy of the MRI from the hospital, so I could keep my own records. I wasn’t even given any reason on why it might be necessary. I trusted my doctors and they helped resolve the problem with my sight. Little did I realize that it was only the beginning and I was in for the fight of my life!

    From 1994 until 2005, I had few further significant issues. I continued to have headaches which I treated with Excedrin and ringing in my ears (tinnitus), which was generally attributed to the aspirin in the Excedrin and sinus issues. In January of 2005, I started having jaw pain. I saw a number of dentists and doctors who couldn’t figure out the problem. I eventually ended up seeing a doctor specializing in pain management who indicated he thought it was Trigeminal Neuralgia (which is another co-morbid disorder of Chiari, but its connection wasn’t made known to us). I was sent to a neurologist, who pointed out I had a 2cm (20mm) herniation, which he said wasn’t enough to worry about, but it was a Chiari malformation. Again, he was the doctor, so we just trusted that it wasn’t something to worry about. The pain worsened:  it started lasting for longer periods of time and increased in both intensity and frequency, so I went to the ER on the advice of the neurologist for pain relief. When I arrived, the neurosurgical group that I had an appointment with had left word for me to go to their office, so I could see them. Since my appointment wasn’t for another several weeks, we were thrilled to get in so fast. We were also told I had Trigeminal Neuralgia and that Chiari wasn’t related and that Chiari couldn’t kill you; although they informed me that I would need surgery. I was scheduled for a decompression surgery almost immediately and thought I was cured. The neurosurgeons indicated that a 2cm (20mm) herniation was quite significant and that I was “in good hands.” The decompression was somewhat successful in that it resolved some of my symptoms, but the relief was short lived.

    I stopped taking the Gabapentin for Trigeminal Neuralgia but began having trouble with balance issues and nobody was sure why. I was told that the Chiari had nothing to do with any of this and that I was “just lucky” that all my conditions were minor. My surgeon considered Microvascular Decompression (MVD), but said I was “too tight” and the surgery wouldn’t be a good idea. In January 2007 and November 2007, I had rhizotomies performed to deaden the nerve. The rhizotomy only worked for about six months before the pain returned. I had a repeat rhizotomy in November 2007 and that has been successful to date. I was still having a lot of symptoms (that I now know to be Chiari symptoms) but they continued to assume them to be due to the Pseudotumor Cerebri, even though I didn’t have my pressure checked or any sign of a papilledema. In June 2008, I had a Ventriculoperitoneal (VP) shunt put in. It was ligated (tied off) in July 2010 since I had lost weight and the symptoms had switched from what was presumed to be high pressure to low pressure symptoms. Due to my new low-pressure symptoms, they tried several blood patches in an attempt to repair what was assumed to be a leak, even though it was never found on any of the testing. Initially, the blood patches worked well, but over time the blood patches were less effective, and I started getting headaches again.  Eventually, the doctors gave up on blood patches as they weren’t helping the headaches and finally, I stopped getting headaches (which we later found out wasn’t uncommon for those who have had long-term CSF leaks.

    Despite my lack of headaches, I started having neurodegenerative problems: trouble maintaining consciousness, hypersomnolence, severe balance problems, bouts with confusion and cognitive changes. My neurologist became convinced that I needed a second decompression. He discussed my case with my neurosurgeon who initially didn’t think that another decompression would help. He was convinced to perform the surgery and I had the decompression in January 2012. The decompression was very successful for 23 days. After that, I declined rapidly. Over the next year, I developed multiple lung infections, which they presumed to be from my history as a smoker, but in reality, it was due to dysphagia. In January 2013, I was admitted to the hospital for yet another lung infection and by May, I was given a feeding tube. My wife was brought into a meeting with my neurologist where he said I needed to go to a nursing home and that my death could be imminent. I went to the nursing home and did better than they expected. Instead of dying at the nursing home, I improved and was released to go home a month later. I continued to improve enough to have the feeding tube removed that July.

    Once home, my condition continued to decline. My wife continued researching and we decided to go and see a Chiari specialist since her research indicated it was the only real way to proceed, especially with a difficult case. In June 2014, we met with one such expert who was able to explain why the first two decompressions failed. Ehlers-Danlos Syndrome and Craniocervical Instability were two of the missing pieces to my puzzle. It was an eye-opening experience and finally, my issues made sense. We discussed several options for surgery, and eventually settled on a date, October 30, 2014. I had my third decompression and this time, a spinal fusion. It was a wild success! Many of my symptoms were resolved immediately. While it was not a cure, it certainly helped me substantially in restoring many functions and my quality of life.

    However, in September 2015, I went into a coma while hospitalized for a reduced state of awareness (which coincided with weaning off the Decadron) and the local doctors said it was another failed decompression. In October 2015, I had yet another MRI. While going over the radiologist report, my wife noticed something none of my doctors mentioned, it stated that I had severe Intracranial Hypotension. My wife sent my MRI images to a leak expert and my NY neurosurgeon for further assessment.

    In January 2016, I was in another hospital across the country having imaging for the known CSF leak. After the testing was done, a leak was found in my lumbar spine and a location they felt was suspicious on my thoracic spine (where I had old stress fractures and incredibly thin dura, believed to have been causing leaks). There were also bone fragments next to the fractures, so the doctor double reinforced my spine in that area and performed a dural reduction surgery in parts of my thoracic and lumbar spine. I got better until August 2016, when I started experiencing symptoms of a CSF leak. Upon consultation, we decided a blood patch would be the place to start and it resolved my symptoms quickly.  As of August 2018, the blood patch has continued to keep me from leaking and no leak symptoms have occurred.  While I’m going to be at risk for leaks, and likely leak periodically due to Ehlers-Danlos Syndrome, I will take the results I’ve seen thus far.  It’s also interesting to note that while I’ve had a number of unsuccessful patches, this time the post-patch recovery protocol was different in that I laid flat for 2 hours post patch, then 3 days lying flat except to go to the bathroom and eat.  I’m convinced that this protocol helped immensely, as the weight of the CSF Is much greater when upright thus increasing the chance of a leak.  Also, a blood patch doesn’t end the leak repair, it merely starts it.  Once the clotting effect has finished, the next stage occurs, which includes tissue growth to repair the opening.  With Ehlers-Danlos Syndrome, this process is often diminished and requires longer than the normal recovery time, which is why I believe many of us have had failed patches.

    I am still continuing to heal, and likely will never be returned to my old self.  I have been discharged from physical therapy as of December 2017, however I still exercise every day for at least an hour.  I know that this is a key part of my healing; there have been a few occasions when I was unable to do my exercises for a few days I notice difficulty in doing my daily activities.  In addition, I will not likely be able to work as a paid employee ever again, however, I have been able to provide some help to a variety of people.  I enjoy helping others and this works well as if I have a less than optimal day, I can just let anyone who needs my help know that I won’t be available to help them.

    My recovery hasn’t been without trouble, as I returned to the hospital once, in the spring of 2018, for what was presumed to be a gall bladder problem.  Since I’ve suffered issues with kidney stones, it’s not surprising to me that I have a lot of gall stones.  I suspect it has to do with some of the gastrointestinal issues that hEDS brings, but there is nothing definitive.  After 2 days of pretty significant pain, the pain subsided and nothing more came of it.

    Overall, there have been a number of positive outcomes and I wouldn’t change the decision to have my third decompression & fusion.  This has granted me the ability to lead a life, which while not “normal,” is fulfilling.

    I am not, and never will be, completely healed. Many of my symptoms have resolved to the point where I can tolerate them and at times, don’t even notice them. While there is no cure for Chiari or the Ehlers-Danlos Syndrome causing it all, there is more healing possible. They are difficult disorders for many reasons and one of the biggest issues is the way it presents itself; for each person, it can be entirely different, making the diagnosis very difficult. I will always have to be monitored for leaks each time the symptoms present themselves, but for now, I find myself thankful to be alive and so very lucky to have the support I do, especially from my wife, my hero!

    *Updated August 2018

  • The Diagnosis – Round One

    The Diagnosis – Round One

    One of the biggest hurdles a Chiari patient may face is that of simply being diagnosed. Some studies cite an average of 5 years between the onset of symptoms significant enough for a patient to seek medical care and the patient receiving an accurate diagnosis of Chiari Malformation. Sadly, however, online support groups and message boards are peppered with the stories of patients who went undiagnosed, or more often, misdiagnosed, for decades. Patients are frequently misdiagnosed with conditions such as Fibromyalgia, Multiple Sclerosis, Chronic Fatigue Syndrome, Chronic Migraine, and various autoimmune disorders. Even more disturbing is the fact that in a study by Dr. Thomas Milhorat of over 300 patients diagnosed with Chiari, 59% had been diagnosed with a psychosomatic illness.[1] Diagnoses such as Hypochondriasis, Somatoform Disorder, Conversion Disorder, and a tricky little term some doctors use to disguise the fact that they believe their patient’s symptoms are “all in their head,” called ‘Functional Somatic Syndrome’ (which by definition does not necessarily imply a psychosomatic cause but are often interpreted by other medical professionals in that manner) are frequently written in patient’s medical records to account for their very real, medically explainable symptoms.[2] This often further undermines a patient’s efforts to find a diagnosis when they decide to try a new doctor, with their defiled medical records in tow, often without even realizing what has been written in their charts or what it actually means in ‘doctor speak.’

    Diagnosis of Chiari Malformation is based upon the presence of Chiari symptoms, such as an occipital headache that is brought about or worsened by Valsalva maneuvers, neurological symptoms such as poor balance, numbness or tingling in the arms, weakness in the legs, etc. combined with the “gold standard” of imaging studies for Chiari, an MRI of the brain.[3] The MRI should show a tonsillar herniation that inhibits the normal flow of CSF fluid out of the Fourth ventricle of the brain and through the foramen magnum. When normal flow is in question, a CINE MRI may be conducted to determine how the CSF is flowing through the brain and upper spinal canal. There, are, however, controversial cases sometimes referred to as “Chiari Zero,” where no herniation exists, but interruption of flow and symptoms are present.[4]

    So, with all these wonderful tests available, why is it so difficult to get a diagnosis? Well, first of all, most primary care physicians and neurologists alike are under-educated or outright miseducated about Chiari Malformation. It has always been presumed to be a relatively rare disorder, and with over 7,000 rare diseases known to modern medicine, medical schools cannot give a thorough education to medical students on everyone. However, since the advent and now fairly commonplace use of the MRI, it is becoming apparent that Chiari is not quite as rare as it was once thought to be. When a primary care physician is stumped by a patient’s complaints of headaches and neurological symptoms, it is only natural to refer that patient to a neurologist for evaluation. But even many neurologists are grossly uninformed about Chiari. Many patients, even with an MRI that shows a herniation, are told that their many varied symptoms can’t possibly be due to the herniation of their brain, even with numerous studies available which show otherwise. Some patients have even been told by neurologists and even neurosurgeons that “Chiari doesn’t cause headaches [pain].” In fact, most symptomatic patients experience severe headaches, nerve pain, and pain from related disorders such as Syringomyelia.

    Furthermore, radiologists across the U.S. are using vastly different criteria from one another to determine whether a patient’s herniation constitutes a Chiari Malformation. Some are using criteria of a 3-5mm herniation, others a 7 mm herniation, and some are failing to look for a herniation altogether. The latest research, in fact, suggests that the size of the herniation does not matter at all. Some people with large herniations show no symptoms, while others with quite small herniations experience severe symptoms. Experts are now focusing more on whether the herniation blocks the normal flow of cerebrospinal fluid (CSF) in determining whether a patient may benefit from surgical intervention.

    It is important to address the situation of “Incidental Findings” of Chiari Malformation. It is true that sometimes while having an MRI for an unrelated matter, a completely asymptomatic patient is found to have a Chiari Malformation. Unless this type of patient were to become symptomatic later, no treatment is necessary in these situations. However, if you look at radiology reports with a claim of ‘incidental finding of Chiari Malformation,’ you are likely to see that the reason mentioned for the imaging being done was to look for a cause of headaches or other known symptoms of Chiari. In these cases, these are absolutely not ‘Incidental Findings.’ Again, this is an example of the lack of education about Chiari.

    For patients, the best piece of advice one can give on your road to diagnosis of your symptoms, whether  due to Chiari or something else, is to trust yourself and never give up seeking answers. If you believe your symptoms are due to Chiari or that your MRI may have been misread, get a second opinion from a neurosurgeon that specializes in treating Chiari Malformation.

     

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    References:

    1 Milhorat, T H, et al. “Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients.” Neurosurgery., U.S. National Library of Medicine, May 1999, <www.ncbi.nlm.nih.gov/pubmed/10232534>.

    2 Mayou, Richard, and Andrew Farmer. “Functional Somatic Symptoms and Syndromes.” BMJ : British Medical Journal, BMJ, 3 Aug. 2002, <www.ncbi.nlm.nih.gov/pmc/articles/PMC1123778/>.

    3 “The Chiari I Malformation.” Chiari I | Chiari & Syringomyelia Foundation (CSF), <csfinfo.org/education/physician-information/chiari-i/>.

    4 Hofkes, S K, et al. “Differentiation between Symptomatic Chiari I Malformation and Asymptomatic Tonsilar Ectopia by Using Cerebrospinal Fluid Flow Imaging: Initial Estimate of Imaging Accuracy.” Radiology., U.S. National Library of Medicine, Nov. 2007, <www.ncbi.nlm.nih.gov/pubmed/17890352>.

  • Overview: Chiari Treatment Options & Potential Pitfalls

    Overview: Chiari Treatment Options & Potential Pitfalls

    Once diagnosed, you will usually be referred to a specialist (not a Chiari Specialist, but an everyday, run-of-the-mill neurologist or neurosurgeon). They tend to come in one of two types: Either they are very passive and just want to wait and see how bad it gets, or they are very pro-surgery and while they will still usually give you a 50% chance of helping your symptoms, they will tell you how decompression surgery really is your best option. Both are problematic.

    FOR THE ASYMPTOMATIC CHIARIAN:
    Without a doubt, the passive approach is by far the best plan of action for the asymptomatic Chiari patient. Approximately 30% of those with a Chiari 1 malformation can go their entire lives without having symptoms (in fact, many remain undiagnosed and don’t even know they have it because they have no symptoms).[1] The Chiari is often discovered while looking for something else entirely and is therefore considered an “Incidental Finding.” While there is a chance that the Chiarian will become symptomatic one day, the risks of surgical complications exceed the potential benefit on something that has not and might not ever affect their life.

    FOR THE SYMPTOMATIC CHIARIAN:
    If the Chiarian is symptomatic, however, “Decompression Surgery is the only treatment available [at this time] to correct functional disturbances or halt the progression of damage to the central nervous system. Most individuals who have surgery see a reduction in their symptoms and/or prolonged periods of relative stability. More than one surgery may be needed to treat the condition.”[2] Despite the reasons for haste however, we do recommend slowing down and making sure that adequate testing is done to ensure that the Chiari is a “Congenital Chiari” formed only by a small posterior fossa, with no other etiological/pathological co-factors that could make it an “Acquired Chiari Malformation.” If not identified and addressed, these etiological/pathological co-factors can cause complications and even lead to a failed decompression surgery. (Note: most are told at diagnosis that it is a “congenital defect.” However, that is usually a presumption on their part, stemming from a lack of knowledge of Chiari and its comorbidities, and them giving too much credence to the paragraph or two on Chiari malformations in their medical school textbooks. That is not the reality that many are dealing with; so, testing is imperative!)

    TESTING:
    If you have been diagnosed with a Chiari malformation or a tonsillar ectopia (regardless of the size of tonsillar descent), you should have the following tests/images done.

    1. A sleep study to check for Sleep Apnea.

    • Central Sleep Apnea (CSA) is more common with Chiari, especially when there has been damage to the brainstem or Vagus Nerve.

    • Obstructive Sleep Apnea (OSA) is commonly linked with obesity in the general population, it is also very common amongst those with Connective Tissue Disorders (such as Ehler’s-Danlos Syndrome).

    • Both CSA and OSA can be present in the Chiarian with a Connective Tissue Disorder. When both are causing apnea, it becomes known as “Complex Sleep Apnea.”

    • Sleep Apnea, regardless of the type, is a common “killer” amongst Chiarians.


    2. A brain and full spine MRI (upright recommended) with and without contrast.

    What they should be looking for in these MRIs:

    a) Do you have a syrinx (Syringomyelia or Syringobulbia)?

    • If you have either, that is proof that the blockage of CSF is significant enough to cause these potentially serious complications.

    b) Is any part of your brainstem below the foramen magnum?

    • If it is, you have a variant of Type 1, known as a Chiari 1.5[3] or Type 2, both indicate that there is something else going on causing the brainstem to herniate along with the cerebellar tonsils. 

    c) Do you have a cyst/tumor causing increased intracranial pressure that is pushing the cerebellar tonsils down?

    • If you have either, and they are operable, there is a chance that surgically removing it could decrease the intracranial pressure and allow the cerebellar tonsils (and brainstem if it is below the foramen magnum at all) to go to proper position. Furthermore, if the cyst/tumor is not addressed before, during, or soon after decompression surgery, the chances of your tonsils herniating again after decompression are high.[4]

    d) Do you have Hydrocephalus causing increased intracranial pressure that is pushing the cerebellar tonsils down?

    • If you do, there is a chance that the placement of a Ventriculoperitoneal Shunt could decrease the intracranial pressure enough to allow the cerebellar tonsils (and brainstem if it is below the foramen magnum at all) to go to their proper position. Furthermore, if the Hydrocephalus is not addressed before, during, or soon after decompression surgery, the chances of your tonsils herniating again after decompression are high.[5]

    e) Do you have signs/symptoms of Idiopathic Intracranial Hypertension (often shows as excessive fluid above the lateral ventricles, with small, “slit-like” lateral ventricles; usually accompanied by Papilledema and/or Empty Sella Syndrome), which can cause enough cranial pressure that it can push your cerebellar tonsils down?

    • If you do, there is a chance that a prescription for Diamox or Topamax, or the surgical placement of a Ventriculoperitoneal Shunt, could reduce the pressure enough to allow the cerebellar tonsils (and brainstem if it is below the foramen magnum at all) to go into proper position. Studies exist both in favor of shunting first and doing the decompression first; there are documented cases where unresolved IH has led to failed decompression surgeries, while other cases attribute a Chiari decompression as being that which resolved IH symptoms.[6/7]

    f) Do you have signs/symptoms of Tethered Cord Syndrome that could be pulling on the spine from below?

    • If you do, there is a chance that a less invasive surgery known as a Tethered Cord Release, could stop the downward pulling of the spinal cord and allow the cerebellar tonsils (and brainstem if it is below the foramen magnum at all) to go into proper position. Furthermore, if the Tethered Cord is not addressed before, during, or soon after decompression surgery, the chances of your tonsils herniating again after decompression are high. (Note: not all Tethered Cords are easily visible by MRI, when they are not visible, it is called Occult Tethered Cord (OTC) and surgery on a OTC remains controversial.)[8]

    g) Do you have signs/symptoms of Intracranial Hypotension (CSF Leak) that could be pulling or creating a suctioning effect from below?

    • If you do, there is a chance that a less invasive blood patch or a Dural Tear Repair Surgery, could stop the downward pulling/suctioning effect on the spinal cord and allow the cerebellar tonsils (and brainstem if it is below the foramen magnum at all) to go into proper position. Furthermore, if the leak(s) are not addressed before, during, or soon after decompression surgery, the chances of your tonsils herniating again after decompression are high. (Note: not all leaks are easily visible by MRI.)[9]

    h) Do you have signs of Spina Bifida (Myelomeningocele, Meningocele, or Spina Bifida Occulta)?

    • While it is important to know if any of these exist, a Myelomeningocele would put you at an increased risk of having a Chiari Type 2. While all Chiari Malformations with a Myelomeningocele do not meet the standards for Type 2, 90% of those with a Myelomeningocele have a Chiari Type 2. Most neurosurgeons will not perform a decompression on someone with a Myelomeningocele because of the risk of further complications.[10]

    i) Do you have signs/symptoms of disc degeneration problems and/or stenosis, especially in the cervical spine where it can damage the Vagus Nerve?

    • If you do, these could be another sign of a connective tissues disorder. If you have a syrinx above a herniated/bulging disc, there is a good chance that the disc is attributing to it. Herniated/bulging discs in the cervical spine can be quite troublesome. They can usually be addressed by a second procedure called an Anterior Cervical Discectomy and Fusion (ACDF), but if there are multiple cervical problems and Craniocervical Instability, some surgeons will opt to do a fusion at the time of decompression.[11]

    j) Do you have signs and symptoms of a cervicomedullary syndrome[12], which are often the result of Craniocervical Instability (CCI), Atlantoaxial Instability (AAI), or both. (CCI and AAI can be confirmed with: An upright c-spine MRI with flexion, extension, and neutral views or a 3D cervical CT with rotational views.)

    • CCI/AAI can produce many of the same symptoms as Chiari 1 malformation, including occipital or craniocervical junction headaches made worse by Valsalva maneuvers. It has been theorized that CCI/AAI may also be capable of causing or worsening a herniation of the cerebellar tonsils, and it has been demonstrated that CCI can cause ventral brainstem compression and deformative stress injury to the brainstem.[13] Failure to diagnose and treat CCI before or along with decompression has also been linked to decompression failure. CCI and AAI, while rare in the general population, are relatively common in patients with HDCT’s (Heritable Disorders of Connective Tissue), such as Ehlers-Danlos Syndrome. CCI, when identified to be the root cause of significant symptoms, can be treated conservatively, and in the short term, with the use of a hard-cervical collar, physical therapy and, possibly, traction. More permanent treatment in the form of a fusion and stabilization surgery (fusing the occiput to C1, C2, and sometimes additional vertebrae).[12/13]

    3. A preliminary check for connective tissue disorder (especially Ehler’s-Danlos Syndrome). If preliminary results indicate the presence of a connective tissue disorder, genetic testing should be done to rule out more serious types.


    IF NONE OF THESE PROBLEMS APPEAR TO EXIST: 
    Some doctors and patients prefer to wait and just treat the symptoms medically. That is your choice to make and you should never let anyone try to bully you or manipulate you into having surgery or not; although it is important to know that statistics show that those who have had a shorter duration of onset of symptoms and surgery tend to have a better surgical outcome.[14]

    Statistics show that 80% of decompressed patients report some relief from some of their symptoms; headaches are the most commonly reported symptom to gain relief (81%).

    Most surgeons will give a 50% chance of relieving each symptom individually, with the exception of pain associated with damaged nerves.

    While some surgeons prefer a conservative approach to surgery such as a “bone only” decompression or a “Minimally Invasive Subpial Tonsillectomy,”[15] a full decompression usually consists of:

    ♦ Craniectomy: removal of part of the sub-occipital skull.

    ♦ Laminectomy of C1 (and sometimes C2): removal of the lamina from the vertebra.

    ♦ Duraplasty: opening of the dura (the outermost membrane enveloping the brain and spinal cord) and patching it so there is more room.

    ♦ Cerebellar Tonsillectomy: removal/cauterization of the cerebellar tonsils.


    IS DECOMPRESSION SURGERY A CURE FOR A CHIARI MALFORMATION? 
    There is a fundamental problem with the question and what many neurologists and neurosurgeons believe about decompression surgery. For the patient, the symptoms are synonymous with the condition. If we continue to struggle living our lives because of these symptoms, having a doctor that refuses to validate that struggle, can add insult to injury. Decompression surgery should NEVER be considered a cure to the symptoms of a Chiari malformation, it is merely the only treatment available [at this time] to correct functional disturbances or halt the progression of damage to the central nervous system. The likelihood of continued symptoms is almost absolute, Decompression is only a means of reestablishing the flow of CSF between the brain and spinal canal (which is imperative). Once flow is restored, there should be some relief from many of the symptoms. Complications such as Syringomyelia should be reduced or resolved, so the possibility of paralysis is significantly reduced. With as bad as the pain and symptoms are, it really can get worse if left untreated. Finding a specialist that is not only experienced with decompression surgeries, but who fully understands the correlation and treatment of etiological/pathological co-factors and co-morbid conditions, substantially increases the likelihood of a positive surgical outcome.

     

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    References:

    1 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>.

    2 “Chiari Malformation Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, June 2017, <www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Chiari-Malformation-Fact-Sheet>.

    3 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/>.

    4 Wang, J, et al. “Acquired Chiari Malformation and Syringomyelia Secondary to Space-Occupying Lesions: A Systematic Review.” World Neurosurgery., U.S. National Library of Medicine, Feb. 2017, <www.ncbi.nlm.nih.gov/pubmed/27894943>.

    5 Graham, A, et al. “An Unusual Cause of Neck Pain: Acquired Chiari Malformation Leading to Brainstem Herniation and Death.” The Journal of Emergency Medicine., U.S. National Library of Medicine, Dec. 2012, <www.ncbi.nlm.nih.gov/pubmed/21215551>.

    6 Fagan, L H, et al. “The Chiari Pseudotumor Cerebri Syndrome: Symptom Recurrence after Decompressive Surgery for Chiari Malformation Type I.” Pediatric Neurosurgery., U.S. National Library of Medicine, 2006, <www.ncbi.nlm.nih.gov/pubmed/16357496>.

    7 Park, Michael S., et al. “Coexistent Chiari Malformation and Idiopathic Intracranial Hypertension: Which Should Be Treated First?- Case Report and Review.” JSM Neurosurg Spine, vol. 2, no. 3, ser. 1025, 20 Mar. 2014. 1025, <www.jscimedcentral.com/Neurosurgery/neurosurgery-2-1025.pdf>.

    8 Milhorat, T H, et al. “Association of Chiari Malformation Type I and Tethered Cord Syndrome: Preliminary Results of Sectioning Filum Terminale.” Surgical Neurology., U.S. National Library of Medicine, July 2009, <www.ncbi.nlm.nih.gov/pubmed/19559924>.

    9 Atkinson, J L, et al. “Acquired Chiari I Malformation Secondary to Spontaneous Spinal Cerebrospinal Fluid Leakage and Chronic Intracranial Hypotension Syndrome in Seven Cases.” Journal of Neurosurgery., U.S. National Library of Medicine, Feb. 1998, <www.ncbi.nlm.nih.gov/pubmed/9452230>.

    10 Vandertop, William P., et al. Surgical Decompression for Symptomatic Chiari II Malformation in Neonates with Myelomeningocele. Oct. 1992, <www.thejns.org/doi/abs/10.3171/jns.1992.77.4.0541>.

    11 Dahdaleh, Nader S., and Arnold H. Menezes. Incomplete Lateral Medullary Syndrome in a Patient with Chiari Malformation Type I Presenting with Combined Trigeminal and Vagal Nerve Dysfunction. 2008, <www.thejns.org/doi/pdf/10.3171/PED.2008.2.10.250>.

    12 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>.

    13 Henderson, FC, et al. “Deformative Stress Associated with an Abnormal Clivo-Axial Angle: A Finite Element Analysis.” Surgical Neurology International, 16 July 2010, <www.europepmc.org/articles/PMC2940090/>.

    14 Ma, J, et al. “Cerebellar Tonsillectomy with Suboccipital Decompression and Duraplasty by Small Incision for Chiari I Malformation (with Syringomyelia): Long Term Follow-up of 76 Surgically Treated Cases.” Turkish Neurosurgery., U.S. National Library of Medicine, 2012, <www.ncbi.nlm.nih.gov/pubmed/22664992>.

    15 Beecher, Jeffrey S., et al. “Minimally Invasive Subpial Tonsillectomy for Chiari I Decompression.” Acta Neurochirurgica, Springer Vienna, 5 July 2016, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4980444/>.

  • Overview: Craniocervical Instability and Related Disorders

    Overview: Craniocervical Instability and Related Disorders

    Craniocervical Instability and related pathologies of the craniocervical junction are an important topic for anyone diagnosed with Chiari 1 malformation. “Complex Chiari,” or the presence of craniovertebral abnormalities or instability in addition to the presence of cerebellar tonsillar herniation, is present in approximately one fourth of all cases of Chiari 1 malformation[1]. These cases usually involve the presence of a genetic connective tissue disorder and are thought by experts to be the cause of most Chiari decompression failures[2]. When the doctor and patient alike are not knowledgeable about these conditions and the additional symptoms that often accompany them, these more complex cases are often treated with a standard decompression, which can actually weaken the stability of the craniocervical junction more, and result in an increase of symptoms rather than a clinical improvement. Understanding what signs and symptoms to look for that may indicate that your Chiari is more complex, is vital in receiving the appropriate treatment the first time. This is especially important considering that, according to Chiari expert Paolo Bolognese, M.D., “[with revision surgeries], the results are not as good as if you had done the posterior decompression well the first time.”[3]

    Punjabi and White define instability as the “loss of the ability of the spine under physiological loads to maintain relationships between vertebrae in such a way that there is no damage or subsequent irritation of the spinal cord, (brain stem) or nerve roots, and in addition that there is development of deformity or incapacitating pain due to structural changes.”[4] This means that the ligaments and muscles that normally hold the spine together, are too weak or damaged to handle the normal range of motion and weight of anatomic structures. For example, in Craniocervical Instability, the neck is not strong enough to support the normal weight of the head, without elements of the spine moving in such a way that it causes pain or damage to the nervous system (spinal cord, brain stem, and even cranial nerves). The result is that the bones that make up the lower skull and upper spine get pushed out of their normal anatomic location and begin to impinge on or cause stretching of these parts of the nervous system.

    Craniocervical Instability can result from or be exacerbated by a trauma, such as a severe whiplash injury. However, many cases of CCI are associated with some sort of connective tissue disorder, such as a heritable disorder of connective tissue (HDCT, like Ehlers-Danlos Syndrome or Marfan’s), or an autoimmune condition that affects the connective tissue (such as Rheumatoid Arthritis), or a few other rarer conditions that affect the integrity of bony structures in the skull and spine. Instability can result either from lax ligaments and other connective tissues, soft bones (also seen in HDCTs) or from something like pannus formation, where repeated rubbing together of the joints causes a build-up of granulated tissue around bony structures and changes the way certain bones lie in relationship to one another[5]. Craniocervical Instability can also result as a complication of Chiari decompression surgery, when too much bone is removed from the skull, resulting in the instability of the skull on the top of the spine[6].

    In the patient community, the term “CCI” is often used in reference to both Craniocervical Instability and Atlantoaxial Instability (AAI). CCI is often used to refer to the commonly seen combination of issues with the craniocervical junction, that include the instability of the joints where the skull meets the C1 vertebrae (which is true CCI), the instability of the joints between C1 and C2 (true AAI), a retroflexed odontoid, pannus formation, and a kyphotic clivo-axial angle (which are all forms of basilar impression/invagination). But CCI really should refer to the movement of the skull with respect to the spine. This sliding is referred to as “translation” and is measured on dynamic imaging in millimeters. The pathological threshold for the degree of translation of the basion with respect to the odontoid process between flexion and extension is 2mm, and any amount of translation greater than 1mm is capable of producing symptoms7. Likewise, at the C1-C2 joint, instability in the form of AAI can cause an excessive uncovering of the joint facets. Facets are the surfaces of the vertebrae that articulate with next vertebra. An uncovering of the facets that exceeds 20% is considered pathological.

    The occipito-atlantic joint allows for about half of the cervical spine’s ability to flex and extend (tilt forward and backward). Likewise, the atlantoaxial joint [the articulation between C1 (atlas) and C2 (axis)] accounts for about half of the cervical spine’s ability to rotate the head. Because of this, these vertebrae lack the same amount of stability as the remainder of the spine, and ligaments are largely responsible for their stability[8]. Therefore, ligamentous laxity, as seen in connective tissue disorders, make these areas of the spine particularly prone to pathologic instability. Symptoms of AAI may include visual changes, syncope (fainting) or near-syncopal episodes, dizziness, nausea, facial pain, difficulty swallowing, choking, respiratory issues, and upper cervical tenderness. These symptoms will usually improve with the use of a neck brace[9]. For patients with connective tissue disorders, as are seen in 12-20% of patients diagnosed with Chiari, dynamic imaging is very important in identifying potential instability. The ideal tests to diagnose CCI and AAI are an upright MRI with flexion and extension and a 3D CT with rotational views, respectively[10]. It is important to note that ventral brain stem compression may not be seen on traditional supine MR imaging, while it may be very evident on dynamic imaging.

    Basilar Invagination and Basilar Impression are also often seen with instability. They are almost identical to one another, and refer to upward displacement of the bones of the spine. However, technically, Basilar Invagination is caused by this deformation with normal bone, while Basilar Impression results from softening of bone[11]. For our purposes, this distinction is less important, but we will discuss any displacement in terms of “Basilar Invagination,” or “BI” for short. Forms of Basilar Invagination now include the prolapse of the odontoid process through the foramen magnum (the original condition described by the term), cranial settling, a kyphotic clivo-axial angle, and a retroflexed odontoid[12]. The kyphotic clivo-axial angle is an important and relatively easy measurement to indicate potential deformative stress on the brain stem. The clivus is a wedge-shaped bone that normally lies above and ventral to the top of spine. When it lies more horizontally, it creates a sharp angle that results in a bending of the brainstem. The odontoid peg (also called the odontoid process or the dens) is the part of the C2 vertebrae, or Axis, that the skull pivots upon, so named because of its tooth-like shape. A retroflexed odontoid occurs when the odontoid is bent backwards, often compressing the front of the brain stem. Other important measurements involving ventral brain stem compression for a kyphotic clivo-axial angle and/or retroflexed odontoid include the Grabb-Oakes and Harris measurements.

    • The clivoaxial angle is measured by drawing a line along the posterior (back, or when lying more horizontal, the top) side of the lower clivus and intersecting that line with a line drawn on the posterior side of the axis.  If the angle created is less than 135°, it is considered pathological.  Like instability, a kyphotic clivoaxial angle is often seen in patients with connective tissue disorders and degenerative rheumatoid disease[13].  See figure 1 below.
    Left – Clivoaxial Angle (CXA). Right – Grabb-Oakes measurement.

    For the Grabb-Oakes measurement, a line is drawn from the basion (the midpoint of the anterior margin of the foramen magnum) to the inferior posterior C2. A perpendicular line is then drawn from the center of this line to the dura of the brain stem. A Grabb-Oakes measurement greater than 9 mm denotes a form of basilar invagination. This is a very helpful measurement for determining how much a retroflexed odontoid is compressing the brain stem. See Figure 2 above.

    The Harris measurement is the distance between the basion and the Posterior Axial Line. This distance should not be more than 12 mm. A measurement of more than 12 mm also denotes instability. This measurement can also be used to measure the translation between flexion and extension in dynamic imaging[14]. See Figure 3.

    Harris measurement

    Symptoms of ventral brain stem compression can occur with various types of BI and instability. They may be referred to together as a “cervicomedullary syndrome” and may include[15]:

    • A heavy headache (often referred to as feeling like a “bobblehead” or feeling like the head is a “bowling ball”)
    • A Chiari-type pressure headache aggravated by Valsalva maneuvers (because these conditions, like Chiari, can also cause flow issues)
    • Dysautonomia (including tachycardia, heat intolerance, orthostatic intolerance, syncope (fainting), polydipsia (extreme thirst), delayed gastric emptying, chronic fatigue)
    • Neck pain (often severe)
    • Central or mixed sleep apnea
    • Facial pain or numbness – Occasionally, including Trigeminal Neuralgia
    • Balance and coordination impairment
    • Muscle weakness
    • Dizziness and vertigo
    • Vision problems, including double vision and downward nystagmus
    • Reduced gag reflex and dysphagia (difficulty swallowing)
    • Tinnitus (ringing in the ears) and hearing loss
    • Nausea and vomiting
    • Paralysis
    • In more severe cases, non-epiform seizures have also been documented

    In addition to producing significant pain and neurological symptoms, the compression and kinking of the brain stem can cause significant injury to the brain stem neurons by stretching the axons of the nerves to the point that they break and recoil, producing what are called “axon retraction bulbs” that can be seen on microscopic examination of the cells. The stress placed on the brain stem by both compressing and stretching simultaneously is much greater than the mere sum of these two mechanisms. Interestingly, during the flexion of the normal spine, it stretches 17% of its length. Research has shown that the axon of a giant squid fails when stretched to 20% if its length. Therefore, the normal motion of the human neck brings us very close to injuring our brain stem. Consequently, it only takes a slight alteration of our normal anatomy to cause injury to these delicate nervous tissues[16].

    Treatment of Craniocervical Instability typically begins with more conservative medical management, such as neck bracing, activity limitation, physical therapy (including isometrics, sagittal balance, core strengthening and cardio), and pain management. Other causes of symptoms such as co-morbid conditions, multiple sclerosis, dystrophy, mitochondrial disorders, vitamin deficiencies and Lyme disease should be ruled out or treated. However, surgical intervention via a craniocervical fusion is indicated when the following criteria are met:

    Severe headache or neck pain >7/10
    AND cervicomedullary syndrome
    AND neurological deficits referable to the craniocervical junction
    AND radiological findings indicative of instability

    Surgeons and patients alike should consider surgery after medical management has been maximized and the patient has shown a positive response to neck bracing[10].

    Various specific surgical techniques are applied in craniocervical fusions. A more common technique is the open reduction and fusion stabilization procedure. This procedure involves stabilizing the head with screws, making an incision that exposes the occiput through C2, and fixing plates to the occiput which attach to the C1 and C2 (and sometimes C3) vertebrae with rods. A newer technique adapted by Dr. Paolo Bolognese is using a less invasive Chiari decompression surgery known as MIST (minimally invasive subpial tonsillectomy) along with a fusion using screws placed in the occipital condyles. Although the dura is opened to partially resect the tonsils, a duraplasty is not done. Advantages of this procedure include a smaller incision, smaller hardware, less bone removal, and a thicker bone for which to insert screws in the occiput. Risks of both methods include vertebral artery injury, and a slightly increased risk for segmental instability below the fusion, along with the standard risks of any surgical procedure, such as bleeding, infection, and complications from anesthesia. Both procedures demand the hands of a very skilled and experienced surgeon because vital structures lie in and around the area. Using condylar screws may increase the risk of injury to major vessels, particularly in the hands of a less experienced surgeon. An important cranial nerve also lies just deep to the occipital condyles, making precise screw placement extremely important. This may limit the procedure in becoming more widely used, but the resulting fusion may be stronger, despite the reduced amount of hardware[17].

    In the presence of a retroflexed odontoid, an open reduction, fusion and stabilization procedure may be enough to relieve ventral brain stem compression. However, in more severe cases, a further surgery called an odontoidectomy may be needed to remove the odontoid process. This surgery may be done through the mouth or the nose[18].

    Every Chiari patient should be aware of hereditary connective tissue disorders and the signs and symptoms of Craniocervical Instability and Basilar Invagination. Many neurosurgeons do not evaluate their patients for these conditions prior to performing Chiari decompression surgery, often resulting in the need for revision surgeries and poor results. Being an educated patient can help you ask the right questions and insist on the proper evaluation and testing to avoid the pitfalls that many other patients have faced.

    References:

    1 Bolognese, Paolo A, director. Surgical Techniques for Chiari Malformations. YouTube, American Syringomyelia Chiari Alliance Project, 16 Mar. 2015, <www.youtu.be/KfYmJnB6sPQ>.

    2 Bolognese, Paolo A. “Modern Classification and Subclassification of Chiari Malformations.”YouTube, American Syringomyelia Chiari Alliance Project, 16 Mar. 2015, <www.youtu.be/ZQ9ZmquN-M0>.

    3 Bolognese, Paolo A. “2016 ASAP CM/SM Conference – ‘Complex Posterior Fossa’ – Bolognese.” YouTube, American Syringomyelia Chiari Alliance Project, 7 Dec. 2016, <www.youtu.be/3jKH_DHadO8>.

    4 Augustus A. White III, Manohar M. Panjabi, et al. “Clinical Biomechanics of the Spine.” By Augustus A. White III, <www.leomed.at/listhoscan/white_90.pdf>

    5 The Pain Relief Foundation, The Pain Relief Foundation, <www.thepainrelieffoundation.com/craniocervical-instability/>.

    6 Bolognese, Paolo. Complex Posterior Fossa revisions. YouTube. December 7, 2016. <www.youtu.be/3jKH_DHadO8>.

    7 Menezes, Arnold H. “Craniovertebral Junction Anomalies: Diagnosis and Management.”Seminars in Pediatric Neurology, vol. 4, no. 3, Sept. 1997, <www.sempedneurjnl.com/article/S1071-9091(97)80038-1/fulltext>.

    8 Yang, Sun Y., et al. “A Review of the Diagnosis and Treatment of Atlantoaxial Dislocations.”Global Spine Journal, Georg Thieme Verlag KG, Aug. 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4111952/>.

    9 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, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

    10 Henderson, Sr. , Fraser C. “Neurological Management of Hereditary Disoders of Hypermobility Connective Tissue Disorders.” Ehlers-Danlos Society Annual Conference 2015. Ehlers-Danlos Society Annual Conference 2015, 14 Aug. 2015, Baltimore, <www.ehlers-danlos.com/2015-annual-conference-files/Henderson_0.pdf>.

    11 Hain, Timothy C. “Basilar Invagination, Basilar Impression and Atlantoaxial Subluxation.”Basilar Invagination, Basilar Impression and Atlantoaxial Subluxation, 19 Apr. 2013, <https://dizziness-and-balance.com/disorders/central/cerebellar/basilar_invagination.htm>.

    12 Kim, Louis J., et al. “JNS JOURNAL OF Neurosurgery OFFICIAL JOURNALS OF THE AANS since 1944.” Treatment of Basilar Invagination Associated with Chiari I Malformations in the Pediatric Population: Cervical Reduction and Posterior Occipitocervical Fusion | Journal of Neurosurgery: Pediatrics, Vol 101, No 2, Nov. 2004, <www.thejns.org/doi/abs/10.3171/ped.2004.101.2.0189?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dpubmed>.

    13 Henderson, Sr. , Fraser C. “Cranio-Cervical Instability in Patients with Hypermobility Connective Disorders.” OMICS International, OMICS International, 18 Apr. 2016, <www.omicsgroup.org/journals/craniocervical-instability-in-patients-with-hypermobility-connective-disorders-2165-7939-1000299.php?aid=71754#11>.

    14 Bono, C M, et al. “Measurement Techniques for Upper Cervical Spine Injuries: Consensus Statement of the Spine Trauma Study Group.” Spine., U.S. National Library of Medicine, 1 Mar. 2007, <www.ncbi.nlm.nih.gov/pubmed/17334296>.

    15 Henderson, Sr. , Fraser C. “Diagnosis and Treatment of Craniocervical Instability in the Chiari Patient.” Chiari and Syringomyelia Foundation Educational Lecture. 20 July 2011, Greater Metropolitan Washington Area, Greater Metropolitan Washington Area, <www.youtu.be/U33T8JFXvk0>.

    16 Henderson, F C, et al. “Neuropathology of the Brainstem and Spinal Cord in End Stage Rheumatoid Arthritis: Implications for Treatment.” Annals of the Rheumatic Diseases, U.S. National Library of Medicine, Sept. 1993, <www.ncbi.nlm.nih.gov/pmc/articles/PMC1005138/>.

    17 Bolognese, Paolo A. “Surgical Techniques for Chiari Malformations.” YouTube, American Syringomyelia Chiari Alliance Project, 16 Mar. 2015, <www.youtube.com/watch?v=KfYmJnB6sPQ>.

    18 Hwang, Steven W., et al. “C1–C2 Arthrodesis after Transoral Odontoidectomy and Suboccipital Craniectomy for Ventral Brain Stem Compression in Chiari I Patients.”European Spine Journal, Springer-Verlag, Sept. 2008, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2527411/>.

  • Suicide Prevention List – International & By State

    Suicide Prevention List – International & By State

    International list of suicide hotline websites
    https://en.m.wikipedia.org/wiki/List_of_suicide_crisis_lines

    http://www.crisistextline.org

    United States Suicide Hotline numbers links
    http://www.suicide.org/suicide-hotlines.html

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

     

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