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Craniocervical Instability & Ehlers Danlos Syndrome: Know the Facts

Gymnastics and yoga were easy as you were super flexible.  With continued training your shoulders and knees kept popping out of place.  A series of small but significant traumas left you sidelined both from athletics, academics and socially.  Brain fog and fatigue were common themes.  You have seen countless physicians with conflicting information.  Your chiropractor thinks you may have loose neck ligaments. What is Ehlers Danlos Syndrome?  What are the different types of Ehlers Danlos Syndrome? What is Craniocervical Instability? What is Craniocervical Instability Ehlers Danlos Syndrome? What are the eight most common symptoms of Craniocervical Instability? What are common causes of Craniocervial Instability in patients with EDS? How is Craniocervical Instability Ehlers Danlos Syndrome diagnosed? What are the treatment options for Craniocervical Instability Ehlers Danlos Syndrome? What is the PICL procedure?  Let’s dig in.

Understanding Craniocervical Instability and Ehlers Danlos Syndrome

Ehlers Danlos Syndrome is a connective tissue disorder that can affect ligaments.  Craniocervical Instability is a medical condition where the ligaments that hold your head onto your neck are weakened or loose.  Patients with Ehlers Danlos Syndrome because of their connective tissue disorders are predisposed to develop Craniocervical Instability.

What Is Ehlers Danlos Syndrome (EDS)?

Ehlers Danlos Syndrome (EDS) is a group of inherited disorders that affect and weaken the connective tissues such as tendons and ligaments (1). It is a hereditary disorder which means you are born with it.  EDS has many different signs and symptoms which can vary significantly from patient to patient.  It most commonly affects the skin, joints, and blood vessels.  The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000 (2).  Joints are typically hypermobile with excessive joint range of motion as a result of a defect in collagen formation.

Are There Different Types of EDS? (Hypermobile, Classic, Vascular)

Yes.

The three most common types of EDS are:

Hypermobile

Hypermobile EDS ( hEDS) is the most common form of EDS.

Classic

Classic is the second most common type of EDS.  Previously is was also called EDS Type I & II.

Vascular

Vascular EDS is quite rare and is the most severe type of EDS.  Vascular EDS is much different from Hypermobile and Classic EDS. In addition to loose joints, and translucent skin these patients are a risk for life-threatening rupture of the intestine, uterus, and arteries.

What Is Craniocervical Instability (CCI)?

Cranial Cervical Instability (CCI) is a medical condition where the strong ligaments that hold your head to your upper neck are loose or lax (3). The major ligaments involved are the Alar, Transverse and Accessory ligaments.   To learn more about CCI please click on the video below.

What Is Craniocervical Instability Ehlers Danlos Syndrome?

Ehlers Danlos Syndrome (EDS) is a group of inherited connective tissue disorders that affect and weaken tendons and ligaments.  As a result of weakened or loose ligaments, patients with EDS are predisposed to Craniocervical Instability (4). Craniocervical Instability Ehlers Danlos Syndrome describes the medical condition of CCI that affects patients with EDS as a result of their compromised ligaments.

What Are the Eight Most Common Symptoms of Craniocervical Instability?

Excessive movement between your head and neck can cause movement and damage of the upper cervical facet joints, discs, nerves, and blood vessels.  Symptoms vary depending upon the amount of instability.  Most common symptoms include:

1. Painful, Heavy Head

A constant to near-constant head pain can be described as feeling like the head is too heavy for the neck to support.  Many describe feeling like they have a “bobblehead”. Neck weakness along with ligament instability results in head forward posture in many patients.  This has significant consequences as the weight of your head dramatically increases as it moves forward from neutral alignment.  This is illustrated below.  On the left, in neutral spinal alignment, your head weighs 12 lbs. With just 2 inches forward posture the weight of the head increases to 32lbs.  Add another 1 inch forward and the weight increases to 42lbs.  This is an enormous amount of weight that can not be sustained or supported.  Pain, limitation, and compromised posture ensures and curve of the thoracic spine starts (kyphosis).

2. Headache

This is not your normal headache caused by your in-laws or excessive consumption of alcohol.  This is a debilitating headache that is constant in duration and miserable. Headache is often caused by the upper neck joints, muscles, and tendons getting injured due to ongoing instability.

3. Rapid Heart Rate

Elevated heart rate may occur as the vagus nerve gets irritated by the extra motion in the upper neck.  Another cause of elevated heart rate is Postural Orthostatic Tachycardia Syndrome (POTS).  POTS is a medical condition that causes malfunction of the autonomic nervous system. It is estimated to impact between 1,000,000 and 3,000,000 Americans.  The autonomic nervous system controls involuntary body functions such as heart rate and blood pressure.  Malfunction of the autonomic nervous system causes a number of symptoms which include rapid heart rate.

4. Brain Fog

Patients with CCI  can struggle with memory, concentration, and ability to complete tasks. Every day can feel like your brain is stuffed full of cotton.

5. Neck Pain

Pain is typically localized at the base of the skull and aggravated with flexion and rotation.  The pain can shoot up into the base of the skull, top of the head, frontal area or behind the eyes.

6. Visual Problems

Visual disturbances can vary from mild to severe involving a number of symptoms.  Visual disturbances can happen because the upper neck supplies information to the brain to guide eye position and vice versa (5).

7. Dizziness dizziness associated with craniocervical instability ehlers danlos syndrome

Balance problems can be a significant issue (6).  Dizziness or imbalance is a feature related to the fact that the upper neck is a major contributor to balance (4).  The upper neck provides position sense that has to be coordinated with balance information from the eyes and inner ear.

8. Chronic Fatigue

Excessive movement between the head and neck can cause irritation and dysfunction of the nervous system.  The autonomic nervous system is the most affected.  A common symptom of autonomic dysfunction is chronic fatigue.

Common Causes of Craniocervical Instability in EDS Patients

EDS is a group of inherited disorders that affect and weaken the connective tissues such as tendons and ligaments.  This predisposes EDS patients to CCI.  Other causes of Craniocervical Instablity in patients with EDS include:

Trauma

Whether it is one major event or a series of repetitive traumas over time, trauma to the cervical spine can cause cervical instability.  For example during a motor vehicle accident ligaments in the neck can be overextended, disrupted and injured leading to chronic cervical instability. Other examples include sports injuries or forceful chiropractic adjustments

Inflammation

Chronic inflammatory diseases can be debilitating.  In such cases, a patient’s own immune system attacks itself resulting in inflammation, injury, and pain.  Common examples include Rheumatoid Arthritis, Scleroderma, and Lupus.  Patients with Rheumatoid Arthritis are at risk for CCI.

How Is Craniocervical Instability Diagnosed in EDS Patients?

Treatment of Craniocervical Instability Ehlers Danlos Syndrome can be challenging. Not having an accurate diagnosis can make it even more challenging.  A lack of diagnosis can send patients on a many-year journey looking for assistance.  Some will be dismissed as their complaints are considered not valid or genuine whereas others will be labeled “hormonal”, anxious or psychiatric and referred elsewhere or medicated with anti-depressants, opioids, or anti-anxiety medications.

In a previous blog I outlined the major criteria for diagnosing Craniocervical Instability.  They include:

Mechanism of Injury:  AKA how did it occur.  Was it traumatic in nature or due to underlying condition such as EDS?

Symptoms

Findings on Physical Examination

Radiographic Studies: Unfortunately, traditional MRI and CT studies do not evaluate the upper cervical spine or Alar and Transverse ligaments. Special radiographic studies are required which include Upper Craniocervical Junction MRI, Upright Cervical MRI with flexion/extension, Rotatory CT scan or DMX.

Response to Conservative Care

Response to Diagnostic Injections

Level of Disability

Treatment Options for Craniocervical Instability Ehlers Danlos Syndrome

There are three major treatment options for patients with Craniocervical Instability Ehlers Danlos Syndrome:  conservative care, posterior injections and surgery. The specific type of treatment will depend upon the amount of instability and a patient’s clinical presentation.

Conservative Treatment Options

Patients with small amounts of instability can oftentimes be managed with conservative care.  Treatment options include:

  • Upper cervical chiropractic care.  Look for members of NUCCA (National Upper Cervical Chiropractic Association)
  • Physical Therapy
  • Bracing with either a soft or hard cervical collar
  • Curve restoration. Look for chiropractors that specialize:  Chiropractic BioPhysics or CBP is a protocol utilized by a select group of chiropractors who look to spinal rehabilitation and postural correction as a means to address pain, disease, and dysfunction.

Posterior Injections Craniocervical Instability Ehlers Danlos Syndrome

Precise x-ray or ultrasound guided injections are a treatment option for those patients that have failed to receive significant benefit from conservative care.  Posterior injections refers to injections performed at the backside of the head and neck.  PRP or stem cells are treatment options.  Steroids should be AVOIDED as they can weaken the affected ligaments or damage the cartiage in the facet joints.  Examples of posterior injections include:

  • C0/1 and C1/2 facet injections.  A facet is a paired joint on the backside of the spine that provides support and limits rotation of the spine.  It can also be irritated or injured causing significant neck and head pain (7).   Few clinics perform this injection due to the skill required.
  • Occipitals Nerve block.  The occipital nerves arise from the upper cervical spine and travel up into the back of the head.  There is one nerve on each side of the head.  When irritated, injured, or compressed they can cause significant pain in the back and top of the head (8).
  • Ligament injections to stabilize injured or lax ligaments.  Ligaments are thick pieces of connective tissue that connect bone to bone.  Loose or damaged ligaments can cause significant instability, pain, and dysfunction.

cerviccal fusion of patient with Craniocervical Instability Ehlers Danlos Syndrome Surgical Treatment Options

Craniocervical Instability Surgery always involves a fusion.  Fusion is a surgical procedure where one or more of the bones in the spine are joined together by screws, bolts, and rods.  The hardware provides mechanical stability.  In most cases the upper portion of the neck (C1 and C2 vertebral bodies)  is fused to the skull.  The skull is also called the occiput.  The surgery can also be called an Occipitocervical Fusion.  This is major surgery and rehabilitation.

What Are the Risks Associated with Craniocervical Instability Surgery?

Fusion of the head to the neck is a major surgery that is associated with signficant risk and complications.  The 10 most common complications include:

Screw loosening:  The implanted screw can back out of the bone.

Screw failure:  The screw that is inserted into the occiput or C1 or C2 can fracture, bend or break.  In most cases surgical revision is necessary.

Infection: The infection can be localized to the skin or may penetrate deeper into the muscles or bone.

Vertebral Artery injury: The vertebral artery provides critical blood flow to the spinal cord and brain.  The artery can be compressed, irritated, or injured during the surgery.

Spinal Cord Injury

Cervical Facet Injury: The screw can be advanced into the facet joint thereby injuring the joint cartilage leading to arthritis, pain, and restriction in range of motion.

Dural Leak: The dura is a thin layer of connective tissue that covers the brain and spinal cord.  It can be injured during the surgery resulting in leakage of spinal fluid 

Hematoma:  A Hematoma is a collection of blood that can compress or injure nerves, arteries, and veins.

Limited Neck range of motion:  Fusion of the skull base to the upper neck can severely restrict the range of motion in the upper neck.

Adjacent Segment Disease (ASD): Fusion of the spine significantly alters the biomechanics of the spine.  The fused segment is no longer able to absorb the forces of daily living.  As such these forces are then transferred above and below the fusion.  This additional force can overload the discs, facet joints, muscle, and ligaments above and below the fusion which start to break down.  To learn more about adjacent segment disease please click on the video below.

 

Revolutionary Way of Treating Craniocervical Instability for EDS Patients

In 2015 a nonsurgical treatment option for patients with Craniocervical Instability (CCI) was developed at the Centeno-Schultz Clinic. It involved the injection of a patient’s own Bone Marrow Concentrate into the damaged Alar and Transverse ligaments.  The b

Bone Marrow Concentrate is rich in stem cells.  The procedure is called a PICL:  Percutaneous Implantation of Cervical Ligaments.  It is a very demanding procedure and is only performed at the Centeno-Schultz Clinic in Broomfield Colorado. Clinical success stories have been discussed in previous blogs. To learn more about this groundbreaking procedure please click on the video below.

In Conclusion

Ehlers Danlos Syndrome (EDS) is a group of inherited disorders that affect and weaken the connective tissues such as tendons and ligaments.

Cranial Cervical Instability (CCI) is a medical condition where the strong ligaments that hold your head to your upper neck are loose or lax.

Patients with EDS due to their ligament laxity are predisposed to develop Craniocervical Instability.

Craniocervical Instability Ehlers Danlos Syndrome describes the medical condition of CCI that affects patients with EDS because of their compromised ligaments.

The most common symptoms of CCI are: brain fog, neck pain, painful heavy head, headache, rapid heart rate, visual problems, dizziness and fatigue.

Treatment options for Craniocervical instability Ehlers Danlos Syndrome depend upon the severity of the instability and a patient’s clinical presentation.

When appropriate first line treatment should be conservative care including chiropractic care and physical therapy.

Craniocervical Instability surgery involves a fusion of the head to the spine.  It is associated with significant risks and complications.

The PICL is a nonsurgical treatment option where Bone Marrow Concentrate is injected into the damaged ligaments.

If you or a loved one has Ehlers Danlos Syndrome and has ongoing headaches, neck pain, fatigue that has not been responsive to conservative care, please schedule a telephone Candidacy discussion with a board-certified, fellowship-trained physician.  From the comfort of your home or office, learn what CCI treatment options are available for you. At the Centeno-Schultz Clinic we are experts in the nonsurgical treatment of Craniocervical Instability.  Call today and stop the pain, misery, and suffering.


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4.Spiessberger A, Dietz N, Gruter B, Virojanapa J. Ehlers-Danlos syndrome-associated craniocervical instability with cervicomedullary syndrome: Comparing outcome of craniocervical fusion with occipital bone versus occipital condyle fixation. J Craniovertebr Junction Spine. 2020;11(4):287-292. doi:10.4103/jcvjs.JCVJS_166_20

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