Arnold-Chiari malformation is a serious disease where part of the brain is pushed into the spinal canal, often diagnosed at birth. Surgery can help relieve symptoms, but damage caused by spinal defects is usually permanent. Taking folic acid supplements before pregnancy can help prevent this condition.
Certain types of defects in the lower part of the brain or in the cerebellum and base of the skull can create a normal condition in which the brainstem and cerebellum shift downwards, inhibiting the movement of spinal fluid and causing a large number of symptoms, depending on the severity. This is called a Chiari malformation and there are four types that can occur, including a type I which can be relatively mild and may never be diagnosed. Type II is specifically known as Arnold-Chiari malformation and is a serious disease that can require multiple types of treatment.
In Arnold-Chiari malformation, which is often diagnosed at or before birth, part of the brain has been pushed into the spinal canal. By itself, this can create problems such as balance problems, headaches, coordination delays, feeding difficulties, breathing difficulties, and others. This is complicated by the fact that brain malformations are present with an open spine, or spina bifida. Symptoms of this condition combine with those of Arnold-Chiari malformation, and children born with the condition may have poor bladder control and fractional or total paralysis in the lower body.
The specific type of spina bifida associated with Arnold-Chiari malformation is myelomeningocele. This is often classified as a severe type and typically requires early surgery. One of the big problems is that fluid in the brain can build up quickly. Early surgery may be needed to create a shunt in the brain to help process this extra fluid and drain it, and further surgery may be needed to close the spine. Spinal shunt and repair do not necessarily address all problems associated with the condition
The increasing pressure on the brain, even with a shunt for fluid drainage, may require more extensive brain surgery. Neurosurgeons may decide to treat Arnold-Chiari malformation or other types of Chiari malformations with surgery that helps give the brain more room. Each surgery might be customized to the patient’s needs, but most can remove some bone in the skull, usually in the back, to create a less constricted space for the cerebellum.
Although surgery can improve some symptoms associated with Arnold-Chiari malformation, it doesn’t solve all problems. The damage caused by a spinal defect is usually permanent, even if the defect can be repaired. Early treatment, however, can make a huge difference.
For those interested in preventing Chiari type II and spina bifida, the most important step is to start taking folic acid supplements several months before pregnancy. This supplement has been shown to dramatically reduce the incidence of neural or spinal tube defects. While there is some suggestion of a weak genetic link with both of these disorders, folic acid use may help negate or reduce this risk.
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