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Chiari malformation is a congenital anomaly (a condition that is present at birth), in which parts of the brain protrude through the opening in the base of the skull into the spinal column.

Description

In order to explain the various components of Chiari malformation, it is helpful to describe a few parts of the brain and their functions. There are four major regions of the brain affected in Chiari malformation: the cerebellum, the brain stem, the ventricles, and the cerebrum. The cerebellum is located at the base of the skull and is divided into two parts or hemispheres with a third section that connects the hemispheres. Its main purpose is to coordinate body movements. The brain stem is located in front of the cerebellum and is composed of two parts. It regulates involuntary actions the body must conduct to survive, such as breathing, swallowing, and blinking the eyes. There are four ventricles in the brain. They are located above and in front of the cerebellum, and their function is to produce and circulate cerebrospinal fluid (CSF), the protective fluid that circulates through the brain and the spinal cord. The cerebrum is the largest part of the brain and is divided into two halves or hemispheres as well. It is located above the cerebellum and is responsible for the higher functions of the brain, such as thought. In Chiari malformation, one or more of these parts of the brain function improperly or are malformed. In addition to brain anomalies, Chiari malformation can also involve defects in the base of the skull and in the bony part of the spine.

There are four types of Chiari malformation. In Type I malformation, the lower portions of the cerebellum, known as the cerebellar tonsils, protrude through the opening in the skull known as the foramen magnum and into the spinal cord canal. It is often accompanied by a condition known as syringomyelia in which pockets of CSF form in the spinal cord. This type is usually diagnosed in adolescence or early adulthood when symptoms most commonly appear; however, with the availability of magnetic resonance imaging (MRI), many children are diagnosed at a much younger age.

Type II malformation, sometimes called Arnold Chiari malformation, is more severe than Type I and involves herniation of a more significant part of the cerebellum, part of the fourth ventricle, and parts of the brain stem. The brain tissues protrude farther into the spinal column than in Type I. These malformations are part of a larger syndrome seen in children with spina bifida, a condition in which the spine and spinal cord have not formed properly. Approximately 80-90 percent of children with Chiari malformation Type II also have hydrocephalus, a condition in which one or more of the ventricles becomes enlarged due to an accumulation of CSF. In these children, hydrocephalus is caused by obstruction of the fourth ventricle due to its herniation into the spinal column. Type II Chiari malformation may be diagnosed prenatally by ultrasound or shortly after birth during medical evaluation of the accompanying spina bifida.

In Type III malformation, parts of the cerebellum and the brain stem protrude into a spina bifida defect located at the base of the skull, on the neck. Type III malformation occurs rarely. Some neurologists do not consider it a Chiari malformation but rather a specific type of spina bifida called an encephalocervical meningocele.

Type IV malformation consists of an underdevelopment of the cerebrum and involves no herniation of brain tissue into the spinal area. As with Type III malformation, many neurologists do not consider this a Chiari malformation but rather cerebellar hypoplasia (underdevelopment). Both Type III and IV Chiari malformations are extremely rare, and this term is not often used in diagnosis of these conditions. The remainder of this entry only discusses Chiari malformation Types I and II.