What is Cerebellar Tonsillar Ectopia?

A tightening of the area around the brain stem, also called Chiari Malformation, is a birth defect of varying severity. Due to a genetic defect in a developing fetus, Chiari Malformation shrinks the space at the back of the skull, putting pressure on the growing brain and pushing the developing cerebellar tonsils downwards through the opening in the skull. The most prominant sign of Cerebellar Tonsillar Ectopedia consists of sudden headaches immediatly following coughing or sneezing. While Chiari Malformation can have serious effects, neurologists classify the disease as uncommon, with the most severe form appearing even less frequently.

What Are The Common Symptoms And Causes of Cerebellar Tonsillar Ectopia?

Most patients with Cerebellar Tonsillar Ectopia usually report headaches as a common symptom. These headaches usually cause pain near the base of the skull at the top of the neck. This headache sometimes radiates down into the neck and shoulders. Patients usually describe this as a brief, sharp, throbbing pain. These symptoms usually get worse after straining, sneezing, or coughing. Additional symptoms of Cerebellar Tonsillar Ectopia include:

  • Loss of coordination and balance
  • Difficulties swallowing or speaking
  • Fainting
  • Trouble Sleeping
  • Tingling or burning sensations in the fingers, lips, and toes.

The signs of Chiari malformation can differ from one person to another. Some individuals may not show any symptoms or remain asymptomatic while others may develop serious symptoms like neurological deficits. Chiari malformation can produce eye abnormalities in patients such as blurred vision, double vision, nystagmus, abnormal sensitivity to light and pain behind the eyes.

How Do Neurosurgeons Treat a Cerebellar Tonsillar Ectopia?

Treatment of conditions associated with Cerebellar Tonsillar Ectopia usually depends upon the symptoms exhibited by the patient. This requires a patient-specific treatment by a professional medical team from multiple specialties. This team may include neurosurgeons, pediatricians, and ophthalmologists. Patients with asymptomatic Chiari I Malformations do not need treatment. But physicians recommend monitoring this condition regularly to check the progress. Most patients with mild symptoms respond well to non-surgical and conservative treatment. Other treatment protocol includes:

  • Medications: This includes the administration of nonsteroidal anti-inflammatory drugs (NSAIDs) and muscle relaxants to relieve symptoms of muscle pain.
  • Physical therapy: This includes stretching and light exercises that can reduce pressure on your nerves and relieve minor pains.

Reducing the patient’s activities can also help to recover from mild symptoms. Neurosurgeons usually treat Chiari malformations with headaches with analgesia to control pain. When a patient with Chiari malformation does not respond to conservative treatment, the treating physician will then recommend surgery. Symptomatic Cerebellar Tonsillar Ectopia often requires surgery. The most common surgical procedure for treating Cerebellar Tonsillar Ectopia includes posterior fossa or Chiari decompression surgery. Almost 80% of patients with Cerebellar Tonsillar Ectopia experience a significant improvement in their headaches or neck pain after this surgery.

This procedure includes the removal of a piece of the skull (craniectomy) to relieve pressure and compression on the brain stem. Also, this involves the removal of part of the bony covering of the spinal canal, called a laminectomy, to provide more room for CSF circulation. Ultimately, this enlarges the foramen magnum and improves the patient’s condition.