Treatment options vary depending on the severity of symptoms, the extent of tonsillar herniation, and the presence of other conditions such as syringomyelia, hydrocephalus, and disorders of the skull and spine.
Observation (watch and wait)
If a person has mild or no symptoms, monitoring by regular check-ups and periodic MRI scans may be recommended. Some people experience headache relief with anti-inflammatory or pain-relieving drugs.
Follow these self-care tips to minimize neck strain in daily activities:
• Ice packs for 20 minutes can help relieve neck and shoulder pain.
• Get at least 8 hours of sleep and use a good pillow.
• Have a sleep study and evaluation for sleep apnea. A CPAP (Continuous Positive Airway Pressure) machine can greatly improve your sleep quality and reduce fatigue.
• If you are overweight, shed extra pounds to reduce the strain on your arms and legs and help with numbness/tingling sensations.
• Eat a healthy diet, include fiber, and drink plenty of water.
• Stay active with low-impact activities, such as walking, cycling, or water aerobics.
• Play cards, crossword or Sudoku puzzles to sharpen your thinking.
• Tai Chi or Yoga can help stretch and tone muscles, improve balance, and reduce stress. Avoid poses that aggravate your symptoms.
Avoid these activities if you have a Chiari, with or without syringomyelia:
• High-velocity chiropractic manipulation that can make the herniation worse and aggravate the spinal cord.
• Cervical traction.
• Trampolines, roller coasters, scuba diving, and other activities that apply G forces to the neck.
• Contact sports to avoid include football, soccer (heading the ball), diving, running, weight lifting, etc.
• Constipation and straining during bowel movements. Straining can cause formation or worsening of a syrinx. A high fiber diet, plenty of water, and stool softeners can help, especially if you take narcotic pain relievers (Vicodin, Percocet).
• Lumbar punctures (spinal taps) and epidurals can be dangerous for a person with Chiari. Ask your doctor to review this important literature on these procedures with respect to increasing herniation. Or discuss with your neurosurgeon.
Childbirth (bearing down and pushing) can also increase cerebellar herniation and formation of a syrinx. Make sure your OB/GYN is aware of your Chiari and tell your neurosurgeon if you become pregnant.
It is important for patients to closely monitor their symptoms. Some patients find it helpful to keep a symptom diary (download a PDF). By keeping track daily of how you feel and what you do, you may be able to find patterns, identify triggers, and notice subtle changes over time. Bring the symptom diary to each appointment to help you communicate more clearly with your doctor. Knowing what symptoms you experience most, and to what degree, can help shape your diagnosis and treatment. If your symptoms worsen or if any new ones develop, call your neurosurgeon's office.
Figure 13. Posterior fossa decompression surgery removes bone and creates more space for the brainstem and cerebellum.
In patients with severe symptoms or with a syrinx, surgery is advisable. The goals of surgery are to stop or control the progression of symptoms caused by herniation of the cerebellar tonsils, and relieve compression of the brainstem.
In patients with a syrinx, the goal of surgery is to prevent or control progression. Symptoms related to the obstruction of CSF should decrease as flow normalizes.
• Posterior fossa decompression is a surgical procedure that removes bone at the back of the skull and spine to widen the space for the tonsils and brainstem (Fig. 13). The surgeon opens the dura overlying the tonsils and sews a patch to expand the CSF space, similar to letting out the waistband on a pair of pants.
Many patients ask about minimally invasive or endoscopic surgery. Minimally invasive can mean different things: shorter skin and muscle incision, no dura opening, no shrinkage of the tonsils, or use of ultrasound and endoscopes. Despite what the words “minimally invasive” suggest, the amount of bone removal needed to effectively restore normal CSF flow depends on the individual patient’s anatomy and size of Chiari. The amount of bone removal should be the same in any procedure, endoscopic or standard “open” technique. For other common questions, see the Q&A.
• Spinal fusion may be performed in addition to posterior fossa decompression surgery in certain patients with spine instability. The neck area of the spine may be unstable due to scoliosis, Ehler-Danlos syndrome, or another bone abnormality. Rods and screws are inserted to structurally reinforce the skull and neck vertebrae.
• Shunting is used to reroute CSF. The shunt includes a flexible tube with a 1-way valve that directs the fluid out in the desired direction. For a syrinx in the spinal cord, one end of the tubing is placed in the syrinx. The other end is placed outside the spinal cord. For hydrocephalus, one end of the tubing is place in the ventricle of the brain. The other end is placed in the abdomen (called a ventriculoperitoneal shunt). A shunt remains inside the body after surgery. However, shunts pose risks and often become clogged or dislodge. Repeated surgeries may be necessary.
• Transoral decompression is a surgical procedure to treat basilar invagination. The surgery is performed through the mouth, to the back of the throat, to remove an abnormal odontoid bone (C2 vertebra).
reviewed by: Andrew Ringer, MD, John M. Tew, MD, and Nancy McMahon, RN
University of Cincinnati Department of Neurosurgery