Arnold Chiari Development Disorder

Anomaly Arnold Chiari


Doctors subdivide the Arnold-Chiari syndrome (anomaly) into 3 types, depending on the structure of brain tissue that moves to the spinal canal, and on the presence of a pathology of the development of the brain or spinal cord.

Many patients with Chiari syndrome have no signs or symptoms of the disease, they do not require treatment. The disease is detected only during the diagnosis in connection with other diseases. However, depending on the type and severity, Arnold-Chiari syndrome can cause a number of problems in patients.

  • Headache triggered by coughing, sneezing, or physical exertion
  • Numbness and tingling in hands
  • Violation of temperature sensitivity in the hands, frequent burns
  • Pain in the neck and neck (going down to the shoulders for periods)
  • Uneven gait (balance problems)
  • Poor hand coordination (fine motor skills)

When you need to see a doctor

If you or your child have any sign or symptom that may be associated with Arnold-Chiari syndrome, see your doctor for an examination.

Since many of the symptoms of Chiari syndrome may also be associated with other diseases, it is important to undergo a thorough physical examination. A headache, for example, can be caused by a migraine, a disease of the paranasal sinuses, or a tumor of the brain or spinal cord. Other signs and symptoms are associated with other diseases, such as multiple sclerosis, carpal syndrome, cervical osteochondrosis, and hernias in the cervical spine.

Arnold-Chiari syndrome is an abnormal development of the brain in which the section of the skull that contains the cerebellum is too small or deformed, resulting in compression of the brain. The lower part of the cerebellum or tonsils are displaced to the upper part of the spinal canal. The pediatric form — Arnold Chiari Type III syndrome — is always associated with myelomeningocele (hernia of the spinal cord and meninges). The adult form, Chiari Type I Syndrome, develops due to an insufficiently large posterior part of the skull.

Arnold Chiari Development Disorder

When the cerebellum is pressed into the upper part of the spinal canal, it can interfere with the normal outflow of CSF, which protects the brain and spinal cord. Disturbed circulation of cerebrospinal fluid can lead to blockade of signals transmitted from the brain to the underlying organs or to the accumulation of cerebrospinal fluid in the brain and spinal cord. The pressure of the cerebellum on the spinal cord or lower part of the brainstem can cause syringomyelia.

Risk factors

There is evidence that Arnold-Chiari syndrome is a hereditary disease. However, studies examining the possible hereditary component of this disease are at an early stage.

Examination and diagnosis

The process of diagnosis begins with a survey of the doctor and with a full physical examination. The doctor will find out if you have symptoms such as headache and neck pain, ask you to describe this pain.

Arnold Chiari Development Disorder

If you suffer from a headache, but the cause of this symptom is not clear to the doctor, you will be asked to undergo magnetic resonance imaging (MRI), which will help you determine if you have a Chiari anomaly.

Sometimes it is necessary to introduce a contrast agent before the examination, through which, when it enters the brain, the contrast and clarity of the image are increased. MRI is a safe and painless method. Later, using repeated MRI, it will be possible to monitor the progression of this disease.

Arnold Chiari Development Disorder


In some patients, Arnold Chiari syndrome can become a progressive disease and lead to serious complications. In others, the symptoms associated with this disease may not occur and no intervention will be required.

Complications associated with this disease include:

  • Hydrocephalus. This accumulation of excess fluid in the brain may require placement of a flexible tube (shunt) for drainage and drainage of cerebrospinal fluid to another part of the body.
  • Paralysis. Paralysis can develop due to compression of the spinal cord. Paralysis may persist even after surgery.
  • Syringomyelia. Some patients suffering from Arnold Chiari syndrome develop a disease called syringomyelia, in which a cavity or cyst forms in the spine (a pathological cavity in the brain or spinal cord). Although the mechanism of connection between Chiari syndrome and syringomyelia is not clear, it may be associated with injury or displacement of nerve fibers in the spinal cord. When a cavity forms, it fills with fluid and can disrupt the functioning of the spinal cord.

Treatment for the Arnold-Chiari anomaly depends on the severity and condition of the patient. In the absence of symptoms, the doctor may prescribe only a review with regular check-ups as a treatment for you.

If the primary symptoms are headache or other types of pain, the doctor may recommend pain relievers. For some patients relief comes from taking anti-inflammatory drugs. This may prevent or delay the operation.

Operational Pressure Reduction Method

Most often, doctors prefer surgical treatment of the Arnold-Chiari anomaly. The purpose of the operation is to stop the progression of changes in the structure of the brain and spine, as well as stabilize the symptoms. If the operation is successful, the pressure on the cerebellum and spinal cord decreases and the normal outflow of CSF is restored.

During the most common operation with the Arnold Chiari anomaly — called craniectomy of the posterior fossa or decompression of the posterior fossa — the surgeon removes a small piece of bone in the back of the skull, reducing pressure and providing more space for the brain. The technique of the operation may be different, it depends on the presence or absence of hydrocephalus. We use the technique of Henry Marsh (London). The operation takes 1-2 hours; recovery usually takes 5-7 days.

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