Chiari Malformation Education

Patients can be born with CM or develop it later in life. Before birth, CM can be caused by a structural defect in the brain and spinal cord. The defect causes part of the brain to push into the spinal canal. Research suggests that CM can be inherited in some families.

In children, teens and adults, CM can develop if cerebrospinal fluid is drained excessively from the spine due to injury, exposure to harmful substances or infection.

If CM is suspected in a newborn or infant, a neurologist will perform a physical exam and an MRI to look for structural issues in the brain. Infants with CM may have one or more of these symptoms:

 

• Arm weakness
• Breathing problems
• Developmental delays and/or inability to gain weight
• Difficulty swallowing
• Excessive drooling
• Gagging or vomiting
• Irritability when being fed
• Stiff neck
• Weak cry

 In older patients, CM may be discovered at symptom onset or during treatment of another condition. In these cases, we will conduct a thorough review of your medical history, a physical exam and an MRI. Our neurologists may also order a specialized cerebrospinal fluid flow test to rule out other conditions.

Adults with CM may have one or more of these symptoms, which can range from barely noticeable to excruciating:

 

• Balance problems
• Burning or tingling in the fingers, toes or lips
• Chronic fatigue
• Depression
• Difficulty swallowing (dysphagia)
• Dizziness
• Headache and neck pain
• Insomnia 
• Muscle weakness or numbness or other abnormal feelings in the arms or legs (known as paresthesia)
• Ringing or buzzing in the ears (also called tinnitus) or hearing loss
• Vision problems
• Vomiting

Treatment of CM depends on its form, severity and associated symptoms. If symptoms do not interfere with normal daily activities, treatment may not be needed. However, patients should continue to work with a CM specialist to reduce the risk of severe complications. 

Medication can effectively manage mild to moderate symptoms in many patients. Some may benefit from posterior fossa decompression surgery to reestablish normal cerebral fluid flow. In this procedure, a neurosurgeon will remove a small portion of bone from the back of the skull to make room for the brainstem. Once normal flow is established, the skull is repaired. Most patients can go home within 24-48 hours of surgery. Some patients require a second procedure to achieve optimal results.