Chiari malformation

Diagnosis

To diagnose your condition, your doctor will review your medical history and symptoms and conduct a physical examination.

Your doctor will also order imaging tests to diagnose your condition and determine its cause. Tests may include:

• Magnetic resonance imaging (MRI). An MRI is often used to diagnose Chiari malformation. An MRI uses powerful radio waves and magnets to create a detailed view of the body.

  This safe, painless test produces detailed 3D images of structural differences in the brain that may be contributing to symptoms. It can also provide images of the cerebellum and determine whether it extends into the spinal canal.

  An MRI can be repeated over time, and it can be used to monitor the progression of the disorder.

• Computerized tomography (CT) scan. Your doctor may recommend other imaging tests such as a CT scan.

  A CT scan uses X-rays to obtain cross-sectional images of the body. This can help to reveal brain tumors, brain damage, bone and blood vessel problems, and other conditions.

Treatment

Treatment for Chiari malformation depends on the severity and the characteristics of your condition.

If you have no symptoms, your doctor likely will recommend no treatment other than monitoring with regular examinations and MRIs.

When headaches or other types of pain are the primary symptom, your doctor may recommend pain medication.

Reducing pressure with surgery

Doctors usually treat symptomatic Chiari malformation with surgery. The goal is to stop the progression of changes in the anatomy of the brain and spinal canal, as well as ease or stabilize symptoms.

When successful, surgery can reduce pressure on the cerebellum and spinal cord and restore the normal flow of spinal fluid.

In the most common surgery for Chiari malformation, called posterior fossa decompression, the surgeon removes a small section of bone in the back of the skull, relieving pressure by giving the brain more room.

In many cases, the covering of the brain, called the dura mater, may be opened. Also, a patch may be sewn in place to enlarge the covering and provide more room for the brain. This patch may be an artificial material, or it could be tissue harvested from another part of the body.

Your doctor may also remove a small portion of the spinal column to relieve pressure on the spinal cord and allow more space for the spinal cord.

The surgical technique may vary, depending on whether a fluid-filled cavity (syrinx) is present, or if you have fluid in your brain (hydrocephalus). If you have a syrinx or hydrocephalus, you may need a tube (shunt) to drain the excess fluid.

Surgical risks and follow-up

Surgery involves risks, including the possibility of infection, fluid in the brain, cerebrospinal fluid leakage or problems with wound healing. Discuss the pros and cons with your doctor when deciding whether surgery is the most appropriate treatment for you.

The surgery reduces symptoms in most people, but if nerve injury in the spinal canal has already occurred, this procedure won't reverse the damage.

After the surgery, you'll need regular follow-up examinations with your doctor, including periodic imaging tests to assess the outcome of surgery and the flow of cerebrospinal fluid.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

Preparing for your appointment

You're likely to start by seeing your family doctor. However, when you call to set up an appointment, you may be referred to a doctor trained in brain and nervous system conditions (neurologist).

Because appointments can be brief, and because there's often a lot to talk about, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready for your appointment and know what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment. For example, even though your primary complaint may be headaches, your doctor will want to know about any changes you may have noticed in your vision, speech or coordination.
• Write down key personal information, including any major stresses and recent life changes.
• Make a list of your key medical information, including other conditions you're being treated for and the names of the medications that you're taking.
• Take a family member or friend along, if possible. Sometimes it can be difficult to recall all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Prepare a list of questions so that you can make the most of your limited time with your doctor. List your questions from most important to least important in case time runs out. For Chiari malformation, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Other than the most likely cause, what are possible causes for my symptoms or condition?
• What kinds of tests do I need?
• Do I need treatment?
• If you don't think I need to be treated now, how will you monitor me for changes in my condition?
• If you recommend surgery, what should I expect from my recovery?
• What is the risk of complications from surgery?
• What is my long-term prognosis after surgery?
• I have other health conditions. How can I best manage them together?
• Are there any restrictions that I need to follow?
• Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
• Are there any brochures or other printed material that I can take home with me? What websites do you recommend visiting?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous or occasional?
• If you experience head and neck pain, is it made worse by sneezing, coughing or straining?
• How severe is your head and neck pain?
• Have you noticed any change in your coordination, including problems with balance or with hand coordination?
• Do your hands and feet feel numb or do they tingle?
• Have you developed any difficulty swallowing?
• Do you experience episodes of dizziness or faintness? Have you ever passed out?
• Have you developed any problems with your eyes and ears, such as blurred vision or a ringing or buzzing in your ears?
• Have you had problems with bladder control?
• Has anyone ever noticed that you stop breathing during sleep?
• Have you been taking pain relievers or using other approaches to relieve your discomfort? Does anything seem to work?
• Do you have any additional symptoms, such as hearing loss, fatigue, or changes in your bowel habits or appetite?
• Have you been diagnosed with any other health conditions?
• Has anyone in your family been diagnosed with Chiari malformation?