What is chiari malformation

CM Is most often caused by structural defects in the brain and spinal cord that occur during fetal development. This is called primary or congenital Chiari malformation. CM can also be caused later in life if spinal fluid is drained excessively from the lumbar or thoracic areas of the spine either due to traumatic injury, disease, or infection. This is called acquired or secondary Chiari malformation. Primary Chiari malformation is much more common than secondary Chiari malformation.

Chiari malformations are classified by the severity of the disorder and the parts of the brain that protrude into the spinal canal. Currently, no test is available to determine if a baby will be born with a Chiari malformation. Since CMs are associated with certain birth defects like spina bifida, children born with those defects are often tested for malformations. However, some malformations can be seen on ultrasound images before birth.

Many people with Chiari malformations have no symptoms and their malformations are discovered only during the course of diagnosis or treatment for another disorder. The doctor will perform a physical exam and check the person's memory, cognition, balance functions controlled by the cerebellum , touch, reflexes, sensation, and motor skills functions controlled by the spinal cord. A doctor also may order imaging tests to diagnose a Chiari malformation or show hydrocephalus or bone abnormalities that might be associated with the disorder.

Medications may be prescribed to ease headache and pain. In many cases, surgery is the only treatment available to ease symptoms or halt the progression of damage to the central nervous system.

Surgery can improve or stabilize symptoms in most individuals. More than one surgery may be needed to treat the condition. The most common surgery to treat Chiari malformation is posterior fossa decompression , which creates more space for the cerebellum and relieves pressure on the spinal cord and should help restore the normal flow of CFS.

It involves making an incision at the back of the head and removing a small portion of the bone at the bottom of the skull craniectomy. In some cases, the arched, bony roof of the spinal canal, called the lamina, may also be removed spinal laminectomy.

In some instances the surgeon may use a procedure called electrocautery to remove the cerebellar tonsils, allowing for more free space. These tonsils do not have a recognized function and can be removed without causing any known neurological problems.

Curvature of the spine is another condition that commonly occurs with type 1. The formation of CSF-filled cysts in the center of the spinal cord, a condition known as syringomyelia sear-IN-go-my-EEL-ya , may also be associated with Chiari malformation type 1. Search Term. Menu Button. Recently Visited. View More Results Loading Our neurosurgeons have decades of experience treating Chiari malformations.

Our state-of-the-art surgical facilities equip us to offer the best available treatment. What is Chiari Malformation?

What is Chiari malformation type 1? Understanding Chiari malformation The thick bundle of nerves connecting the brain to the spinal cord passes through a funnel-like opening at the base of the skull called the foramen magnum.

Chiari malformation symptoms Although the cranial defect that causes Chiari malformation type 1 may be present before birth, symptoms typically take years to emerge. Frequency Chiari malformation type 1 is uncommon, diagnosed in fewer than 1 in 1, children. Other types of Chiari malformation. Chiari malformation type 1 and type 1. Association with other conditions Chiari malformations are frequently associated with other medical conditions. Different symptoms may emerge depending on the location and extent of the cyst and may include: Weakening of the arms and legs Incontinence Stiffness in the shoulders, back, arms or legs Chronic pain.

Find Doctors Locations Services Classes. Surgery is not usually needed. For children with severe symptoms, doctors often recommend a surgery called posterior fossa decompression.

This surgery makes more space for the cerebellum and lowers pressure on the spinal cord. Kids with related problems such as syringomyelia, hydrocephalus, sleep apnea, or scoliosis may need medical treatment for those conditions. Some doctors recommend that kids with Chiari I malformation avoid certain activities like gymnastics, wrestling, and contact sports. Many kids with Chiari I malformation do not have symptoms and never need treatment. But call your doctor right away if your child does show any symptoms, such as headache, balance or coordination problems, changes in pee or poop habits, or voice changes.

Reviewed by: Jeffrey W. Campbell, MD. Neurology at Nemours Children's Health. Larger text size Large text size Regular text size.