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Craniocervical instability is more common in people with a connective tissue disease, including Ehlers-Danlos syndromes, [1] osteogenesis imperfecta, and rheumatoid arthritis. [2] It is frequently co-morbid with atlanto-axial joint instability, Chiari malformation , [ 3 ] or tethered spinal cord syndrome .
Cervicocranial syndrome or (craniocervical junction syndrome, CCJ syndrome) is a combination of symptoms that are caused by an abnormality in the cervical vertebrae leading to improper function of cervical spinal nerves. Cervicocranial syndrome is either congenital [1] or acquired. [2]
Klippel–Feil syndrome (KFS), also known as cervical vertebral fusion syndrome, is a rare congenital condition characterized by the abnormal fusion of any two of the seven bones in the neck (cervical vertebrae).
Patients who exhibit extreme joint hypermobility and connective tissue weakness as a result of Ehlers–Danlos syndrome or Marfan syndrome are susceptible to instabilities of the craniocervical junction; thus they are at risk for acquiring a Chiari malformation. Headache, neck pain, unsteady gait usually during childhood [4] 1.5
Craniocervical instability is caused by trauma(s) to the head and neck areas such as concussion and whiplash. Ligaments in the neck are unable to heal properly, so the neck structure cannot support the skull, which can then sink into the brain stem, blocking the flow of cerebrospinal fluid, which in turn causes autonomic dysfunction.
The distances between the dens and surrounding structures are also key features that can suggest the diagnosis, with the normal distance between the dens and basion (i.e., dens–basion interval; BDI) measuring less than 9 mm on CT, and the distance between the atlas and dens (i.e., atlas–dens interval; ADI) measuring less than 3 mm on CT, although this can be increased in cases of ...
X-ray of the cervical spine with a Hangman's fracture. Left without, right with annotation. It can be seen clearly that C2 (red outline) is moved forward with respect to C3 (blue outline).
A Jefferson fracture is a bone fracture of the anterior and posterior arches of the C1 vertebra, [1] though it may also appear as a three- or two-part fracture. The fracture may result from an axial load on the back of the head or hyperextension of the neck (e.g. caused by diving), causing a posterior break, and may be accompanied by a break in other parts of the cervical spine.