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Craniocervical Junction Disorders


Michael Rubin

, MDCM, New York Presbyterian Hospital-Cornell Medical Center

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Craniocervical junction disorders may involve bones that are

  • Fused together

  • Abnormally formed or underdeveloped

  • Out of alignment (misaligned)


Craniocervical junction disorders can put pressure on the lower parts of the brain, the top part of the spinal cord, or nearby nerves. The resulting symptoms can be serious. They include paralysis, weakness, and loss of sensation.



Present at birth


Isolated disorders include the following:

  • Atlantoaxial subluxation or dislocation: The first and second spinal bones are misaligned, sometimes putting pressure on the spinal cord.


  • Atlas hypoplasia: The first spinal bone develops incompletely.

  • Basilar invagination: Part of the second spinal bone is pushed up and partly into the skull. As a result, the neck is shortened, and pressure is put on parts of the brain, nerves, and spinal cord.

  • Klippel-Feil malformation: The first two spinal bones are fused, or the first spinal bone is fused to the skull. As a result, movement of the neck is limited. Usually, this disorder causes no other problems, except sometimes spinal cord damage after a minor injury.

  • Os odontoideum: Part of the second spinal bone is detached from the rest of the second spinal bone.

  • Platybasia: The bone at the base of the skull (occipital bone) is flattened. It is often accompanied by Chiari malformation (protrusion of the cerebellum, which controls balance, through the opening in the occipital bone). The protruding cerebellum may put pressure on the brain stem or spinal cord. People with this disorder have a short neck.

General disorders may cause the same abnormalities as isolated disorders, but the abnormalities occur as part of a disorder that affects the body as a whole, as in the following:

  • Disorders that affect bone formation in children (such as achondroplasia): These disorders affect all newly developing bone, particularly the long bones in the arms and legs. The long bones may be abnormally short and malformed, causing dwarfism. These disorders sometimes also cause the opening in the occipital bone to narrow or the occipital bone to fuse with the first spinal bone (atlas assimilation), putting pressure on the brain stem or spinal cord.

  • Down syndrome, mucopolysaccharidosis (a rare hereditary disorder that impairs the body's processing of carbohydrates), or osteogenesis imperfecta (a rare hereditary disorder that results in fragile bones): These disorders cause various symptoms, which may include misalignment of the first two spinal bones.




The most common disorders that affect craniocervical structures are

Tumors can also affect craniocervical structures. If tumors spread to the bones of the neck (metastatic tumors to bone), the first two spinal bones may become misaligned. A rare, slow-growing bone tumor called a chordoma or a common, slow-growing noncancerous brain tumor called a meningioma can develop at the craniocervical junction and press on the brain or spinal cord.


Symptoms may start after a slight neck injury or for no apparent reason.

Typically, people with a craniocervical junction disorder have neck pain, often with a headache that starts at the back of the head. Moving the head usually makes neck pain and headache worse, and coughing or bending forward can trigger the pain. Neck pain may spread to the arms.





In many people with Chiari malformation, a cavity (called a syrinx) forms in the spinal cord. These people may lose their ability to feel pain and temperature in the neck, upper arms, and parts of the back. The muscles may feel weak or become paralyzed, particularly in the hands.


  • Imaging tests

If problems appear suddenly or suddenly worsen, people should see a doctor immediately. Immediate diagnosis and treatment of craniocervical junction disorders are essential and can sometimes reverse symptoms or prevent permanent disability.

Doctors suspect a craniocervical junction disorder if people have


  • Certain involuntary movement of the eyes (nystagmus)

The diagnosis can be confirmed by imaging tests, usually magnetic resonance imaging (MRI) or computed tomography (CT). Because problems that appear suddenly or suddenly worsen are an emergency, an imaging test is done immediately. CT shows bone better than MRI and may be done more easily in an emergency. If MRI and CT are unavailable, plain x-rays are taken.

If MRI and CT are inconclusive, myelography with CT may be done. For this procedure, x-rays are taken after a radiopaque contrast agent—one that can be seen on x-rays—is injected into the space around the spinal cord.

If MRI or CT suggests abnormalities that affect blood vessels, angiography, which provides detailed images of bloods vessels, is done. Doctors may use magnetic resonance angiography (which uses a strong magnetic field and very high frequency radio waves rather than x-rays), CT angiography (CT done after a radiopaque contrast agent is injected), or conventional angiography (which uses x-rays taken after a radiopaque contrast agent is injected).


  • Realignment, traction, and immobilization of affected structures

  • Possibly surgery

  • Other treatments depending on the cause

If the craniocervical junction structures are putting pressure on the brain, spinal cord, or nerves, doctors try to realign (reduce) the structures by using traction or by manipulating the head into different positions. These techniques may relieve the pressure. After the structures are realigned, a device is used to keep the head and neck from moving (to immobilize them).

Problems that appear suddenly or suddenly worsen require immediate realignment.

Usually, realignment requires traction. Traction typically involves a device that encircles and is fixed to the head (called a halo crown or ring). It may have to remain in place for 5 to 6 days. After the structures are realigned, the halo ring is attached to a brace around the person's torso. The brace, also called a halo vest, immobilizes the neck. It must remain in place for 8 to 12 weeks. After it is applied, x-rays are taken to make sure the structures are being held securely in the correct alignment.


If problems are due to a bone tumor that has spread (metastasized), radiation therapy and a rigid collar (neck brace) to keep the neck from moving often help.


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