Cranio-vertebral Junction Anomalies

The cranio-vertebral junction (CVJ) is present between the base of the skull and the upper region of the cervical spine. Any deviations or deformities in this region are known as cranio-vertebral junction anomalies. They can occur as congenital anomalies, developmental or secondary to other disease processes. Cranio-vertebral anomalies are classified into different types based on their causative factors.

Causes and risk factors of Cranio-vertebral Junction Anomalies

There are various factors associated with CVJ anomalies. Some of them include:

  • Skeletal structure abnormalities
  • Congenital systemic disorders such as Achondroplasia, Down syndrome, etc.
  • Infections
  • Metastatic Tumours effecting bones
  • Slow growing tumours in the CVJ
  • Rheumatoid Arthritis
  • Traumatic injuries by an accident, a fall etc.

Signs and symptoms of Cranio-vertebral Junction Anomalies

The signs and symptoms of CVJ anomalies vary based on the cause, severity and specific structures affected. However, some common signs and symptoms may include:

  • Neck pain
  • Headache in the back region of the head
  • Worsening head and neck pain with movement.
  • Decreased range of motion in the CVJ
  • Increased pain intensity while coughing or bending

Symptoms associated with spinal cord compression in the CVJ may include:

  • Weakness in arms, legs or both.
  • Numbness and tingling sensation on bending the neck.
  • Decreased sensations of heat, cold and pain in the legs/hands/feet.
  • Confusion and dizziness

Symptoms associated with brain stem dysfunction may include:

  • Sleep apnea
  • Impaired balance and coordination (Ataxia)
  • Involuntary eye movements (Nystagmus)
  • Facial paralysis

Symptoms associated with lower cranial nerve dysfunction may include:

  • Difficulty in swallowing
  • Muscle weakness
  • Impaired hearing
  • Paralysis of soft palate and tongue atrophy

Diagnosis of Cranio-vertebral Junction Anomalies

CVJ anomalies are diagnosed based on medical history, physical examination and diagnostic imaging scans. Lateral view X-rays, CT scan and MRI are used to visualise the structural abnormalities in the CVJ. CT scan is the most common diagnostic tool used for CVJ anomalies. However, if further investigations are required, MRI and CT myelography are considered.

Treatment of Cranio-vertebral Junction Anomalies

Surgery is the treatment of choice for CVJ anomalies. The type of surgery and approach is decided based on some factors which include: traumatic causes, non-traumatic causes, reducible or irreducible lesions. Considering these factors, the basic surgical procedures involved are reduction, immobilization, decompression and fixation. Treatment may include any one of the procedures or a combination which is determined based on the nature, severity and risk factors of the disease.


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