Chiari Malformation

“My Brain Is Too Big for My Head”

By Ruth Werner
[Critical Thinking]

Key Point 

• Chiari malformation involves a herniation of part of the brain into the spinal cord, which may involve severe and chronic headaches, hydrocephalus, weakness, dizziness, and much more.

 

In March 2022, episode 208 of the “I Have a Client Who . . .” Pathology Conversations with Ruth Werner podcast included a client who had repeatedly been denied massage because they had a diagnosed Chiari malformation—the massage therapists at their clinic had been too nervous to work with them. The contributor was perplexed, wanting to know why this might be so, and if any real risk was present. Since then, I’ve had the opportunity to chat with several people with Chiari malformation and learned some fascinating things that are relevant for massage therapists.

Anatomy Review

To understand the importance of Chiari malformation, we must do some anatomy review to refresh our knowledge of the functions and locations of a few key structures. 

The cerebellum, or “little brain,” has several functions. It regulates coordination between prime mover muscles and their antagonists, helps maintain balance, controls eye movement, and sets muscle tone and posture. The brain stem, which has several parts, carries out important autonomic functions. It helps control respiratory rate, blood pressure, swallowing, and sleep. The brain stem and cerebellum, along with the rest of the central nervous system, rely on the free flow of cerebrospinal fluid (CSF). When the cerebellum and brain stem are impaired, or if CSF can’t move freely, some or all these vital functions may be limited or lost. 

The foramen magnum, or “big hole,” in the occiput is the passageway from the spinal cord into the skull. For most people, that big hole is the right size and in the right place. The cerebellum and the brain stem are tucked inside the back of the skull. The occiput doesn’t slip off the atlas; the supporting ligaments around C1 keep everything in position, and the myodural bridge connects the suboccipital triangle muscles to the dura mater in such a way as to allow both stability and freedom of movement for the head.

But what happens if all those structures are not in exactly the right relationship with each other? What happens if some parts of the cerebellum are pushed down into the spinal canal and the flow of CSF is blocked? The repercussions of such a structural anomaly range from being trivial to severe and extreme. This is Chiari malformation, and it affects about one in every 1,000 live births in the US, which amounts to about 3,700 new cases every year.1 Research suggests about 2.5 million people in the US have been diagnosed with the most common type of Chiari malformation, and many more may have it without knowing. 

Chiari malformation can have a profound impact on the quality of a person’s life. Does massage therapy have a role to play for people with this condition? After we gather some information, we will put this question through a critical thinking process to figure out what might be possible. 

What Is Chiari Malformation?

Chiari malformation describes a situation where structures that should be encased within the skull are pushed through the foramen magnum into the area that should be fully occupied by the spinal cord. This can affect the cerebellum, the brain stem, the fourth ventricle of the brain, and the upper part of the spinal cord. 

Four types of Chiari malformation have been labeled: types I through IV. These don’t occur on a spectrum where type I might progress to type II and so on. Rather, they are four distinct structural anomalies. Types III and IV are rare, and they are described as “incompatible with life;” in other words, they are a cause of infant mortality. Type II, which is also called Arnold–Chiari syndrome, can be serious, and is usually associated with a neural tube defect and spina bifida. And type I, or Chiari I, is the most common form, and the focus of this discussion. 

In Chiari I, the cerebellar tonsils, a pair of small extensions of the cerebellum, descend below the foramen magnum. An official diagnosis requires they protrude at least 5 millimeters into the spinal cord. This is sometimes called congenital tonsillar herniation, or tonsillar descent. Mechanical pressure here may affect cerebellar, brain stem, and upper spinal cord function. A blockage in the flow of CSF can also cause symptoms, especially when the brain accumulates fluid to cause hydrocephalus, or a syrinx (a cyst filled with CSF), forms in the spinal cord; this is called syringomyelia. 

Alternatively, Chiari I may be silent. Many people with this version never know unless they have an MRI or CT scan for another reason. And yet another category of patients has little or no indication of Chiari I until well into adulthood when an event like a trauma or illness elicits new signs and symptoms that can only be resolved with Chiari I treatment.

Signs and Symptoms of Chiari Malformation Type I

When Chiari I symptoms develop, they are typically related to three factors: compression of the medulla oblongata (part of the brain stem) and spinal cord, compression of the cerebellum, and disruption in the flow of CSF. One common finding in this population is that the severity of the symptoms does not always correspond to the size of the cerebellar herniation.

Symptoms include severe headaches, even among very young children. The headaches focus on the back of the head and neck, which helps to distinguish them from tension-type and migraine headaches. They do not typically respond to ibuprofen or other over-the-counter medications. Headache pain is often aggravated when the person strains, as in sneezing, coughing, laughing, or passing a bowel movement. 

Sleep apnea is another symptom, if the part of the brain stem that manages respiratory reflexes is dysfunctional. Postural orthostatic tachycardia syndrome (POTS) and difficulty with temperature regulation reflect the dysautonomia that frequently affects people with Chiari I. Some people may have problems with swallowing, eye movements, muscle weakness, or lack of balance. If syringomyelia is present, the person may experience numbness and tingling in the arms, pain, weakness, and paralysis.

Chiari Malformation, Hypermobility EDS, and POTS

Chiari malformation often occurs alongside another condition, hypermobility Ehlers–Danlos syndrome (hEDS). I addressed EDS in the November/December 2019 issue of Massage & Bodywork (page 38). Chiari malformation and hEDS share some symptoms, including POTS—which is the subject of another Massage & Bodywork column from the January/February 2020 issue (page 36). Possibly because Chiari malformation and hEDS are often seen together, practitioners might be concerned about causing a central nervous system injury by working with the necks of clients with these challenges. However, no data suggests this is a significant risk, and many neurologists encourage their patients with Chiari I to engage in active exercise and physical activities—so long as they avoid contact sports. With this recommendation, it seems reasonable to suggest that massage therapy is a safe option for people with Chiari malformation.

Conventional Treatments for Chiari Malformation

People with mild or subclinical Chiari I are often not treated at all; if they are, they’re recommended to use over-the-counter pain relievers for occasional headaches. 

If the symptoms of Chiari I substantially interfere with quality of life, the typical treatment is surgery to decompress the cerebellum, brain stem, and spinal cord and to restore the flow of CSF. Decompression surgery can happen in a variety of ways, depending on the age of the patient, the presence of syringomyelia, and other factors. The risks of complications with this surgery are significant but manageable. The only other option is the permanent implantation of a shunt, which is considered to carry an even higher risk of complications than decompression surgery.

What About Massage?

Does Chiari I carry any special significance for our work? Can massage or other touch-based therapies offer any improvement in common symptoms? As always, the answer is, “It depends.” But here is a short list of what those determining factors are: how severe the condition is, how it affects the client’s quality of life (if at all), the client’s current treatment and side effects, and—above all—it depends on what the client hopes to accomplish with massage therapy. This will be different for every client. Here’s an example of how this decision-making process might play out.

Let’s suppose we have a 30-year-old client who has never received massage. They are otherwise healthy, and they used to enjoy playing softball and rock climbing. But after a recent illness, they developed frequent and severe headaches that make these activities impossible. They think the headaches might be connected to their Chiari I that was discovered at age 23, when they underwent testing for a mild concussion. Their doctor is noncommittal; these new headaches might be related to the Chiari I, but it’s not clear. Surgery is not recommended at this point. This client would like to see if massage therapy might help with their headaches so they can have less pain and avoid using narcotic painkillers in order to function.

Critical Thinking Steps and Possible Answers

If we put this scenario through a critical thinking process, here is where we
might land.

What Is the Key Question?

The client with Chiari I has frequent, severe headaches and would like to see if massage therapy might help reduce their pain.

What Are the Variables That Must Inform Clinical Decisions? 

A standard intake form may not capture all the important details about this situation. Some additional questions, along with rationales, include the following: 

  • Describe your headaches. Where do they hurt? How often and how severe are they? How long do they last?

    Rationale: To get a sense of how significant a problem they are and to set some baseline measures for frequency, severity, and duration. 

  • How long have they been a problem?

    Rationale: To establish if the headaches are a new pattern or one that is well established.

  • What activities make them worse? (Especially coughing, sneezing, or straining—do these impact headaches? What about certain postures or positions?)

    Rationale: To determine if the headaches are related to compression at the foramen magnum and/or tension on the dura mater.
     
  • What activities help ease the headache? (Especially putting the head or neck in a particular position)

    Rationale: To see if certain activities or positions impact the headaches, so we can accommodate during the massage. 

  • What other ways does Chiari malformation affect you?

    Rationale: Although headaches are the main issue, this client may have other consequences that contribute to their stress and quality of life. If these can be addressed by massage therapy, the headaches may also lessen.
  • Have you been diagnosed with syringomyelia and/or hydrocephalus?

    Rationale: If yes, it is important to gather more information on any numbness, tingling, weakness, or other signs of central nervous system damage. Consider, with the client’s permission, consulting with their health-care team to determine any other cautions.

What Have We Missed? What Assumptions Are We Making?

Is it a given that the client’s headaches are a result of Chiari I? Does this client also have signs of hEDS, which might contribute to muscle tension and therefore headaches? Or, is it possible that their headaches are related to something entirely different—like stress, eyestrain, TMJ disorder, or other factors?

What Have Others Done in Similar Situations?

Almost no information about Chiari I and manual therapies has been published in academic sources. This points to the need to gather good information on the interface between massage therapy and Chiari I. In the absence of more specific information, it is necessary to proceed with some caution.

Possible Treatment Plan and Predictable Accommodations

This must depend on you and your skills, the answers your client provides on those extra questions, and how you decide together to move toward the client’s goals. Some accommodations might include positioning their head in a neutral position (neither flexed nor extended) to promote the easiest possible flow of CSF. Craniosacral therapy could be a good choice if you have advanced education in that approach. Focusing on the neck and head muscles may also be helpful, since hypertonicity here may be a cause and/or effect of headaches. Observations about the client’s movement, posture, or other factors may also inform a treatment plan. 

Evaluate Results

This example allows for a straightforward way to track whether the strategy for massage therapy was helpful. We can get baseline information, then ask the client to track their headaches in terms of frequency, severity, and duration. Other ways to gauge effectiveness include getting feedback on what the client enjoyed most and least from the session and if they noticed anything new over the days following their session. This is all offered with the caveat that a client’s main goals may change over time, so the way we set goals and evaluate effectiveness must also change.

More Data Will Yield More Answers

Massage therapy is probably safe for this fictional client with Chiari I, but accommodations for positioning and technique may be needed. This client could be an excellent subject for a case report, if the practitioner captures some baseline information about headache frequency, duration, and severity. That data can be tracked over time with the additional intervention of whatever type of massage therapy the practitioner feels would be most beneficial. The symptoms and goals of your clients with Chiari I may be different from the example provided here. But with this information and some careful clinical decision-making, you can safely explore the possibilities that massage therapy may make a powerful positive difference in their lives. So little information is available on any kind of manual therapy for people with Chiari I that any published project would provide important guidance for other massage therapists. 

Note

1. American Association of Neurological Surgeons, “Chiari Malformation,” last modified April 29, 2024, www.aans.org/patients/conditions-treatments/chiari-malformation.  

Resources

Hidalgo, J. A., C. A. Tork, and M. Varacallo.
Arnold-Chiari Malformation. Treasure Island, Florida: StatPearls Publishing, 2023.
www.ncbi.nlm.nih.gov/books/NBK431076.

Knight, J., and O. De Jesus. Tonsillar Herniation. Treasure Island, Florida: StatPearls Publishing, 2024. www.ncbi.nlm.nih.gov/books/NBK562170.

National Institute of Neurological Disorders and Stroke. “Chiari Malformations.” Accessed May 21, 2024. www.ninds.nih.gov/health-information/disorders/chiari-malformations.

Pakzaban, P. “Chiari Malformation.” MedScape. Updated September 27, 2018. https://emedicine.medscape.com/article/1483583-overview?form=fpf.

Tay, V. S., A. Kornberg, and M. Cook. “Cerebellar Tonsil.” In Essentials of Neuroanesthesia and Neurointensive Care, eds. Anthony H. V. Schapira et al. (Saunders, 2007). www.sciencedirect.com/topics/veterinary-science-and-veterinary-medicine/cerebellar-tonsil.

Zheng, N., et al. “The Myodural Bridge Complex Defined as a New Functional Structure,” Surgical and Radiologic Anatomy 42 (2020): 143–53. https://doi.org/10.1007/s00276-019-02340-6.

 

Ruth Werner is a former massage therapist, a writer, and an NCBTMB-approved continuing education provider. She wrote A Massage Therapist’s Guide to Pathology (available at booksofdiscovery.com), now in its seventh edition, which is used in massage schools worldwide. Werner is available at ruthwerner.com.