Chiari Malformation Treatment

Chiari Malformation

Chiari malformation is a condition in which brain tissue protrudes into the spinal canal. Obstructing the normal cerebrospinal fluid (CSF) pathway, this tissue can lead to a buildup of fluid on the brain (hydrocephalus) or in the spine (syringomyelia).

Types of Chiari Malformation

Chiari malformation is categorized by four types, based on anatomy of brain tissue and occurrence of developmental brain/spine abnormalities.

•  This condition develops while the brain and skull are still growing, and patients with type 1 typically don’t have symptoms until late childhood or adulthood. Patients with other types of Chiari malformation are often diagnosed in utero, during birth or during early infancy.
• Chiari malformation type II: Involves both the brainstem and cerebellum below the foramen magnum. This condition is usually associated with spina bifida and myelomeningocele, which is a sac-like opening in the back where the spinal cord is exposed to the air. This is found in children and is also sometimes known as Arnold Chiari malformation.
• Chiari malformation type III: Type III is the most serious and is relatively uncommon. The cerebellum and the brainstem produce a pouch-like structure containing brain matter that protrudes from the back of the head and neck. Chiari III causes severe neurological deficits.
• Chiari malformation type IV: Also very uncommon, this type of Chiari malformation involves an incomplete or underdeveloped cerebellum.

Symptoms of Chiari Malformation

The most common symptom of Chiari malformation is an occipital headache, often described as intense pressure that begins at the back of the head and radiates forward. These headaches may be brought on by neck extension or excessive straining during coughing, sneezing, bending over or lifting heavy objects.

Additional Chiari malformation symptoms may also include:

• Pain in the neck and shoulders
• Trouble swallowing
• Occasional trouble speaking
• Sleep apnea & respiratory problems
• Loss of bladder or bowel control
• Loss of fine motor control
• Numbness in the hands or feet
• Weakness or stiffness in arms or legs
• Balance & coordination problems
• Electric shock-like sensations down the spine

Other reported symptoms of Chiari malformation type I include:

• Tinnitus (ringing in the ears)
• Nausea
• Facial pain
• Visual symptoms

Untreated Chiari malformation type I may result in the development of fluid-filled cavities in the brain (hydrocephalus) or spinal cord (syrinx), which may eventually lead to syringomyelia. This condition can lead to irreversible brain or spinal cord damage.

Causes of Chiari Malformation

“Congenital” or primary Chiari malformation is most common. This CM is caused by structural defects in the central nervous system that develop in utero—possibly due to genetic mutation or a lack of sufficient nutrients during pregnancy. Secondary or “acquired” CM arises later in life with excessive loss of cerebrospinal fluid caused by infection, disease or traumatic injury.

Chiari malformation symptoms are caused by one or more of the following:

• Cranial nerve compression
• Brainstem compression
• Cerebellum compression
• Disruption to normal cerebrospinal fluid (CSF) flow
• Increase in CSF pressure
• Nerve damage

Concurrent conditions like syringomyelia, intracranial hypertension or other genetic disorders may complicate Chiari symptoms.

Diagnosis of Chiari Malformation

The development and availability of magnetic resonance imaging (MRI) has shown that Chiari malformation is not as rare as once thought. This complex and serious neurological disorder presents with a wide range of symptoms. Most patients exhibit multiple symptoms, but some have none at all. Each case is different, so diagnosis requires an experienced neurosurgeon.

Patients with Chiari malformation type I are often non-symptomatic, so the condition may be found during an exam for another health concern. Diagnosis is made through a combination of patient history, neurological examination and magnetic resonance imaging (MRI). Three-dimensional CT imaging or special MRI scans called CINE studies may also be used to confirm a diagnosis.

In Chiari type II, infants may present with difficulty swallowing, irritability, drooling, gagging or vomiting, arm weakness, neck stiffness or developmental delays.

Once a Chiari malformation is diagnosed, it should be evaluated by an experienced neurosurgeon specializing in the disorder. Early diagnosis and treatment are important, and surgical results for Chiari malformation type I offer the best outcomes when the condition is treated promptly. Many patients continue to experience significant symptom improvement after surgery and are able to return to daily life without activity restrictions.

Dr. Mark McLaughlin and the doctors at Princeton Brain, Spine & Sports Medicine offer state-of-the-art diagnosis and treatment of this serious condition.

Treatment & Management of Chiari Malformation

There is no cure for Chiari malformation, but treatment helps to relieve symptoms and restore quality of life. Chiari type I treatment is based on a number of factors, including symptom severity and whether or not a syrinx exists. When a patient has no symptoms or neurological findings, your neurosurgeon will often manage the condition using watchful surveillance. When symptoms are mild but manageable, pain-relieving medications may be prescribed.

Surgery may become necessary if a patient notices symptom progression, reports neurological deficits or experiences an enlarging syrinx. When surgery is required, it typically begins with an incision that starts in the middle of the back of the skull and extends midway down the back of the neck. Muscles are gently separated from the base of the skull, as well as the back of the first cervical vertebra.

Once the muscles are separated, a craniectomy is performed to enlarge the opening of the base of the skull and allow additional CSF flow space next to the brain. To do this, a specialized surgical instrument is used to carefully shave a small piece of bone about the size of a silver dollar. The first cervical vertebra is then carefully removed during a laminectomy procedure. In some cases, removal of additional cervical lower lamina may be necessary.

Request a First or Second Opinion for Chiari Malformation

PBSSM’s Dr. Mark McLaughlin, board-certified neurosurgeon specializing in treatment for Chiari malformation and neuralgia, provides primary treatment for all types of CM. To request an appointment, verify insurance or speak with a member of our medical team, call 609.921.9001 in NJ or 215.741.3141 in PA. Providing skilled, compassionate care in seven area facilities, PBSSM neurosurgeons also hold privileges at many reputable area hospitals.

Additional Resources

To learn more about Chiari malformation, view these educational videos provided by Dr. McLaughlin:

• What Is Chiari Malformation?
• Surgical Treatment of Chiari Malformation
• Chiari Malformation Treatment Options
• Chiari Malformation Symptoms