The images will help your doctor look for abnormalities in the bone structure, brain tissues, organs, and nerves. Treatment depends on the type, severity, and symptoms. For cases where symptoms do interfere or there is damage to your nervous system, your doctor will recommend surgery.
The type of surgery and number of surgeries you need depends on your condition. For adults: Surgeons will create more space by removing a part of the skull. This relieves pressure on the spinal column. The surgeon may use electrical current to shrink the cerebellar tonsils. It may also be necessary to remove a small part of the spinal column to create more space.
For infants and children: Infants and children with spina bifida will need surgery to reposition their spinal cord and close the opening in their back. If they have hydrocephalus, the surgeon will install a tube to drain excess fluid to relieve pressure. In some cases, they may make a small hole to improve fluid flow. Surgery is effective for relieving symptoms in children. Surgery can help relieve symptoms, but the evidence from research is somewhat mixed on how effective treatment is.
Certain symptoms tend to be more likely to improve after surgery than others. In the long-term, people who have surgery to treat CM will need frequent monitoring and retesting to check for changes in symptoms and functioning. The outcome for each case is different. Type 1: Chiari type 1 is not considered fatal.
One study looked at 29 people with CM type 1 and found that 96 percent reported improvement six months after surgery. One person reported no change. All participants still felt residual symptoms after surgery. The most common symptoms felt after treatment included pain and loss of sensation. CM and syringomyleia: A review study followed up on cases of CM-related syringomyelia. It found a 90 percent chance of long-term improvement or stabilization.
Each outcome depends on the person. Talk to your doctor about your condition, operation risks, and other concerns. It may help to define success, which can vary from improving symptoms to eliminating symptoms. Pregnant women can avoid some of the possible causes of CM by getting proper nutrients, especially folic acid, and avoiding exposure to hazardous substances, illegal drugs, and alcohol.
The cause of CM is the subject of ongoing research. Currently, researchers are looking at genetic factors and risks for developing this disorder.
You can also read personal perspectives and stories about people with this condition at Conquer Chiari. Clumsiness can be more than poor coordination, especially if you have other symptoms. Learn about possible causes and treatments.
Hydromyelia is condition that causes damage to the nerves around the spinal cord. The vagus nerve is the longest of the 12 cranial nerves. This is known as acquired or secondary Chiari malformation type I. The exact cause of a congenital Chiari malformation type I is not known. A problem during fetal growth may cause the defect. It may be caused by contact with harmful substances during pregnancy. Or it may be linked with genetic problems that run in families. An acquired Chiari malformation type I happens to a person after birth.
It is caused by excess leaking of spinal fluid from the lower back lumbar or chest thoracic areas of the spine. This can happen because of an injury, contact with harmful substances, or an infection.
Alan Cohen , chief of pediatric neurosurgery, gives an overview of the different types of Chiari malformations and how they're treated. Cohen also explains why not all cases will need surgery. Chiari malformations are associated with the formation of a syrinx, a fluid-filled pocket, or cyst, in the spinal cord.
This condition also known as syringomyelia. As the cyst fills with cerebrospinal fluid, it expands, putting pressure on the spinal cord. In some patients with a Chiari malformation, increasing pressure from a syrinx can affect neuromuscular function, causing limb weakness or difficulties with walking or breathing.
Some children will show signs of a spine syrinx, but others will not. In these situations, an MRI scan may be needed for a definite diagnosis. In children younger than 16 whose spines are still growing, the presence of a syrinx can also be associated with the development of scoliosis, an abnormal, lateral side-to-side curvature of the spine.
Toddlers, children and teens with undiagnosed type 1 Chiari malformations may develop headaches, which are typically located at the back of the head and neck, and are often made worse by exertion. Type 2 Chiari malformations can also be associated with hydrocephalus, a condition in which there is an obstruction of the flow of cerebrospinal fluid that is found inside of the ventricles fluid-filled areas inside of the brain.
Sleep apnea is another problem that occurs in people with a Chiari malformation. This is a serious sleep disorder characterized by brief interruptions in breathing during sleep.
A sleep study can confirm the presence of sleep apnea so a doctor can prescribe treatment. If you have no symptoms, the problem may be found when you have imaging tests for other reasons. If you have symptoms, your healthcare provider will ask about your health history and give you a physical exam.
He or she may refer you to a specialist. Imaging tests are done to detect a Chiari malformation type I. Your child may have 1 or more of these tests:. This test is the one most often used to diagnose Chiari malformations. It uses large magnets and a computer to make detailed pictures of the inside of the body. CT scan. This test uses a series of X-rays and a computer to create detailed pictures of the inside of the body. A CT scan is more detailed than a regular X-ray.
You may be treated by a neurologist or neurosurgeon. These are chronic disorders involving the spinal cord, and may be expanding or extending over time. As the fluid cavity expands, it can displace or injure the nerve fibers inside the spinal cord. A wide variety of symptoms can occur, depending upon the size and location of the syrinx.
Loss of sensation in an area served by several nerve roots is one typical symptom, as is the development of scoliosis. Syringomyelia can arise from several causes. Chiari malformation is the leading cause of syringomyelia, although the direct link is not well understood.
It is thought to be related to the interference of normal CSF pulsations caused by the cerebellar tissue obstructing flow at the foramen magnum. This condition can also occur as a complication of trauma, meningitis, tumor, arachnoiditis or a tethered spinal cord. In these cases, the syrinx forms in the section of the spinal cord damaged by these conditions.
As more people are surviving spinal cord injuries, increased cases of post-traumatic syringomyelia are being diagnosed. Hydromyelia is usually defined as an abnormal widening of the central canal of the spinal cord. The central canal, a very thin cavity in the middle of the spinal cord, is a remnant of normal development. There are several tests that can help diagnose and determine the extent of Chiari malformation and syringomyelia, listed most common to least commonly ordered.
Magnetic resonance imaging MRI : A diagnostic test that produces three-dimensional images of body structures using magnetic fields and computer technology. It can provide an accurate view of the brain, cerebellum and the spinal cord, is very good at defining the extent of malformations, and distinguishing progression.
The MRI provides more information than the CT scan when analyzing the back of the brain and spinal cord, and is usually the preferred test. It can also evaluate the extent of fluid blockage and neural movement at the foramen magnum using CSF flow studies. Computed tomography scan CT or CAT scan : A diagnostic test that creates an image by computer reconstruction of x-rays; it is particularly good at defining the size of the cerebral ventricles and showing an obvious blockage.
It is most useful for evaluating bony anomalies at the base of the skull and the cervical canal. It is less effective for analysis of the posterior fossa contents or the spinal cord. Sleep study: Involves sleeping overnight in an room where they can monitor breathing, snoring, oxygenation and seizure activity to determine if there is any evidence of sleep apnea.
Swallowing study: Fluoroscopy XRays used to watch the internal swallowing process to determine if there is an abnormality suggestive of lower brainstem dysfunction. Brainstem auditory evoked potential BAER : An electrical test to examine the function of the hearing apparatus and brainstem connections. This is used to determine if the brainstem is working properly. Myleogram: An X-ray of the spinal canal following injection of a contrast material into the CSF space; can show pressure on the spinal cord or nerves due to malformations.
This test is performed less frequently now. Treatment of Chiari malformations and syringomyelia is very dependent on the exact type of malformation, as well as progression in anatomy changes or symptoms. Chiari I malformations that are asymptomatic should be left alone this involves the majority of Chiari malformations. There is no indication for "prophylactic" surgery on these. If the malformation is defined as symptomatic, or is causing a syrinx, treatment is usually recommended. Chiari II malformations are treated if the patient is symptomatic, and physicians have determined that there are no complications from hydrocephalus.
In some patients, consideration of a tethered cord is also explored. In many infants who become symptomatic from a Chiari II malformation, the symptom onset and progression are severe and rapid, and this requires an urgent or emergency approach. Surgical treatment of these malformations depends on the type of malformation. The goal of surgery is to relieve the symptoms or stop the progression of the syrinx or symptoms. Chiari I malformations may be treated surgically with only local decompression of the overlying bones, decompression of the bones and release of the dura a thick membrane covering the brain and spinal cord or decompression of the bone and dura and some degree of cerebellar tissue resection.
Occasionally some patients will also require cervical spinal fusion. Decompression is performed under general anesthesia.
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