Craniocervical Junction Abnormalities

Craniocervical junction abnormalities are disorders of the bones that join the head and neck. They include the congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae.

Causes of Craniocervical Junction Abnormalities

Craniocervical junction disorders may be present at birth (congenital) or acquired later.
 Congenital disorders: They may be isolated (affect only the craniocervical junction) or general disorders (that also affect many other parts of the body)

• Isolated disorders like: 
  • Platybasia
  • Basilar invagination
  • Atlas assimilation
  • Atlantoaxial subluxation or dislocation
  • Atlas hypoplasia
• General disorders   
  • Achondroplasia
  • Down syndrome   

Acquired disorders: They can result from injuries or certain disorders later in the life.  

• Rheumatoid arthritis
• Paget disease of bone
• Tumors

Symptoms of Craniocervical junction abnormalities

• Neck pain
• Headache that starts at the back of the head
• Difficulty sensing pain, vibrations and temperature in the neck, upper arms, and parts of the back
• Short, webbed, or twisted neck
• Limited movement of the neck
• Muscle weakness
• Dizziness
• Impaired vision
• Paralysis (hands)
• Inability to move eyes in certain directions
• Involuntary movement of eyes
• Hoarse voice
• Difficulty swallowing
• Slurred speech
• Lost coordination
• Sleep apnea
• Repeatedly stopping of breathing

Diagnosis of Craniocervical junction abnormalities

Diagnosis includes thorough history, physical and neurological examination of the patient along with following diagnostic tests:  

• X-ray
• CT scan
• MRI   
• Myelography with CT
• Angiography

Treatment of Craniocervical junction abnormalities  

It is considered an emergency condition as in most of the cases symptoms may appear suddenly or get worse suddenly. Prompt diagnosis and treatment may prevent permanent disability.

Treatment is done to relieve pressure on brain, spinal cord or nerves and often involves:

• Realignment (reduction) of the structures by using traction with a device that encircles and is fixed to the head (called a halo crown or ring)
• Manipulation of the head into different positions
• Immobilization via surgery or an external device (halo crown)
• Surgery may be required to relieve the pressure, stabilize the structures, or both
• Radiation therapy in case of bone tumor
• Drug therapy by bisphosphonates or calcitonin in case of Paget disease

Authored by Dr. Shyam Sundar K