Kraniozervikale Instabilität (CCI): Ursachen, Symptome und umfassendes Management

The Pathophysiology Behind CCI

The craniocervical junction serves as a pivotal axis for both neural and vascular structures. Instability, dynamically, in this region may lead to:

• Compression of the brainstem and upper spinal cord
• Tension or kinking of the vertebral arteries, affecting cerebral perfusion
• Disruption of cerebrospinal fluid flow

This can result in complex neurological symptoms and exacerbate conditions such as Chiari malformation or tethered cord syndrome.

Diagnosis: Precision Is Key

CCI is diagnosed through Upright Magnetic Resonance Imaging (uMRI) or upright cone-beam CT scanning (uCBCT), which evaluates the junction in neutral, flexion, extension, and rotational positions. Standard supine MRI often misses dynamic instabilities.

Key radiological measurements include:

• CXA (Clivo-axial angle)
• Grabb-Oakes Line
• BDI (Basion-Dens Interval)
• BAI (Basion-Axial Interval)
• ADI (Atlanto-Dental Interval)

Complementary tests may include lateral cervical x-rays, flexion-extension views, 3D CT scans, and cervical cineradiology, which records joint movement throughout the occipitocervical, atlantoaxial, and subaxial regions.

A clinical-radiological correlation is crucial for accurate diagnosis.

How We Approach CCI at DrGilete.com

Our center in Barcelona is one of the few in Europe to integrate genetic, immunological, and biomechanical assessments to determine the root cause of each patient’s instability. We combine:

• Structural assessment through upright and dynamic imaging
• Neurological and neurophysiological evaluations
• Genetic and immunological profiling
• Personalized treatment planning: both surgical and/or conservative

„We have pioneered an innovative imaging protocol for evaluating CCI and Tethered Cord that is second to none.“

Treatment Options for CCI

Conservative Treatment

• Cervical collars and orthotics
• Physical therapy tailored to hypermobility
• Neuromodulation and regenerative therapies
• Anti-inflammatory and immunomodulatory treatments 

Surgical Treatment

When instability is severe and unresponsive to conservative treatments, surgical intervention may be necessary. Procedures include:

• Occipitocervical fusion (C0-C2)
• Atlantoaxial fusion (C1-C2)
• Occipitothoracic fusion (C0-T2)

Occipitocervical Fusion

Surgical treatment of Craniocervical Instability usually consists of the posterior arthrodesis and fusion of Occipital, Atlas (C1), and Axis (C2). In the Axis, pedicle screws are usually placed, though depending on the patient’s anatomy, screws can be placed in the isthmus. The Atlas screws are generally placed in the lateral masses.

In regard to the fixation of the cranium to the cervical vertebrae, it can be done in two main ways:

1. Placement of screws in the Occipital bone (most frequent and widespread)
2. Placement of screws in the Occipital condyle

In both methods, screws are joined by lateral bars which give rigidity to the fusion system. Bone grafts are typically applied—autologous (from the iliac crest or rib) or allografts (from a bone bank). We typically use structural bone graft from bone banks. Additionally, DBM (demineralized bine matrix) is added to enhance bone fusion.

Surgical Planning and Execution

Preoperative and individualized evaluation of each case is essential to identify anatomical differences, particularly the trajectory of the Vertebral Artery, pedicle dimensions, and lateral mass configurations.

Diagnosis can be supported with Upright MRI or CT scan with 3D reconstruction. Flexion-extension and cervical rotation imaging is crucial.

In the Operating Room

Surgery is performed under general anesthesia, using:

• Neurophysiological monitoring (SSEP – somatosensory evoked potentials)
• Neuronavigational guidance
• Intraoperative CT scan

These measures ensure real-time monitoring of spinal cord and nerves to reduce the risk of complications.

Postoperative Care

After surgery, patients stay:

• 1–2 days in ICU
• 5–7 days in the Neurosurgical Ward

Patients usually begin sitting up on the second-third day and walking shortly after. Weekly outpatient checks are done post-discharge, and we recommend staying in Barcelona for 10–15 days to ensure optimal follow-up.

„Preoperative and individualized evaluation of each case is extremely important since anomalies in the anatomy and trajectory of the Vertebral Artery should be ruled out.“

Prognosis and Quality of Life

With proper diagnosis and management, patients can experience substantial improvement. Post-treatment outcomes often include reduced pain, better cognitive function, and restored mobility.

„From the moment I became their patient they were very caring and respectful… I’m now slowly getting my life back from being mostly bed bound for several years.“

Our Commitment to Complex Cases

„In my many years of practicing medicine and operating on complex cases, I have been introduced to a group of patients in Europe who suffer from what is known as connective tissue disorders.“

„Knowing so many connective tissue patients have been refused help from their own countries and come to us with such hope, encourages us to continue in our expertise and compassionate approach, with the goal of providing patients with a diagnosis that justifies their suffering and surgical options that can offer freedom and independence to their lives.“

Frequently Asked Questions (FAQs)

What is the main cause of CCI?

The most common causes include Ehlers-Danlos Syndrome, trauma, and congenital malformations affecting the upper cervical spine.

How do I know if I have CCI?

Symptoms such as neck pain, dizziness, headaches, and brain fog—especially when triggered by movement—may be suggestive of CCI. An upright MRI or CBCT is necessary for accurate diagnosis.

Can CCI be treated without surgery?

Yes, in mild to moderate cases, conservative treatments like physical therapy and bracing can be effective.

What happens if CCI is left untreated?

Depending on the case, some patients develop progressive neurological deterioration, worsening symptoms, and reduced quality of life are possible if the condition is not addressed, though some keep stable in a “plateu” phase.

Does CCI always occur with EDS?

No, but patients with EDS have an inherent higher risk due to ligamentous laxity. Each case is evaluated individually.

Final Thoughts: Our Commitment to Your Recovery

Craniocervical Instability is a serious yet often misunderstood condition. With proper evaluation and expert care, recovery is possible. At DrGilete.com, we lead this field through evidence-based practice, compassionate treatment, and continuous innovation.

Learn more about our evaluation process, or request a consultation to begin your journey to recovery.