Chiari Malformation

Chiari Malformation md top10mdChiari Malformation is a birth defect that affects the central nervous system organs. The disorder affects both the brain and the spinal cord. The brain and spinal cord are separate structures that are connected by the brain stem. In Chiari Malformation, the brain tissue extends beyond the skull into the spinal canal. It is not a condition that many people are aware of, but it affects over 1 in 1,000 babies. Chiari Malformation is primarily a defect of structure rather than function. Chiari Malformation does not cause any brain damage, but you will have other physical symptoms. Other types of Chiari Malformation will affect a child mentally and physically. It is rare for Chiari Malformation to occur after birth, but it is possible. There are different types of Chiari Malformation which may all be treated by surgery.

Cause

The brain and spinal cord are important parts of the central nervous system. They are, in fact, the only organs in the central nervous system. The brain is made of tissue, but the spinal cord is just a bundle of nerves. There is no tissue at all. The skull covers the brain and protects it. Once you reach the spine, the skull ends, and the vertebrae begin. The vertebrae form around the nerves and makes up the backbone. The vertebrae are spaced out which gives the back flexibility. The brain has three parts: the cerebellum, cerebrum, and brain stem. The brain stem connects the brain to the spinal cord. When the brain is not structured properly, the tissue will fall into the spinal cord causing problems.

Types of Chiari Malformations

There are several types of Chiari Malformations. The types of Chiari Malformations vary in severity. Type 1 Chiari Malformation is the extension of only the cerebellum into the spinal column. This type of Chiari Malformation can be acquired. It is the only type that does not have to be a birth defect. It can be acquired through injury or infection. Type II Chiari Malformation is the extension of both the cerebellum and brain stem into the spinal column. This condition may also be called Arnold-Chiari malformation. This is strictly seen in children with spina bifida. Type III Chiari Malformation is extremely rare and extremely serious. It will leave a child mentally challenged. Instead of just extending into the spinal column, both the cerebellum and brain stem completely protrudes into that area. Type IV is the last type. This is a result of a cerebellum that has not completely formed. There may be partial exposure of the skull and spinal cord.

Chiari Malformation md top10md Symptoms

When talking about symptoms, you have to differentiate which type of Chiari Malformation is being talked about. Each type does not have the same symptoms.

In children, Chiari Malformation causes developmental problems.  Any time a child has spina bifida, they will automatically have developmental challenges. Chiari Malformation only adds to those challenges. As a child gets older, they will have a lot of pain in their head when doing small things like sneezing and coughing. There may be trouble with breathing and swallowing. Type I Chiari Malformation is not always diagnosed, because symptoms are not always present. It may start with pain in the back or neck. When pressure increases with sneezing and coughing, your pain intensifies. Sleep is abnormal, and dizziness is a common factor.

Diagnosis & Treatment 

For Type II-IV Chiari Malformation a diagnosis is typically made at birth. It can be confirmed through diagnostic imaging tests. Type I Chiari Malformation is acquired, so many people may not even know they have it. Sometimes while looking for something else, a doctor realizes an abnormal head structure. This abnormality will lead them to believe you have Chiari Malformation. When you are not showing symptoms, treatment is not necessary. In more serious cases, treatment is the only thing that will save your life.

If you are only experiencing pain and discomfort, you can take medication. There is no need for surgery especially when it is not severe. When there are serious defects, surgery is necessary. The main goal of surgery is to relieve the pressure on the spinal cord and brain. By relieving this pressure, you lower your risk of nervous system damage. The nerves are not being smashed or pinched. Surgery will also cause normal fluid circulation. There is fluid in the brain, and spinal cord that has to be balanced, or else brain damage can occur. Having the right amount of fluid ensures the proper functioning of the brain. Surgery may involve the use of electrical currents or the removal of certain parts of the skull.

Chiari Malformation is not something we hear about every day, but it is affecting a family almost every day. Although it is is more common in newborns, injuries and infections can make you susceptible to the disease. If you are not experiencing any symptoms, there is nothing for you to worry about. If you are suffering from symptoms, you should see a doctor. Medication can ease your pain, but if that is not enough, surgery is available. It is good to do your best to protect yourself from these conditions. There is no way to protect your child against it, but the good news is that it is rare and if it were to happen, treatment is available.

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