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Prevention
Chiari malformation is a congenital anomaly, and no method of prevention is known.
Parental concerns
Chiari malformation can have a significant impact on both the child and the family. The full extent of problems associated with Chiari malformation may not be evident at birth, especially for children with Type I malformation. Children with Type I malformation may experience months of subtle but progressive symptoms before a diagnosis is made. Parents must play an active role in securing appropriate health care and an accurate diagnosis for their child. The symptoms of Chiari Type I malformation when observed individually may not seem significant. However, these symptoms form the basis of the initial diagnosis. Parents should document all unusual events their child experiences such as dizziness, headaches, slurred speech, fainting spells, and numbness or pain in the arms and legs. The first step for diagnosing Chiari malformation Type I is an accurate and detailed history and physical examination. Following this, an MRI is performed. The MRI usually provides a definitive diagnosis and helps determine if the child is a candidate for decompression surgery.
Medical treatment is a multidisciplinary team effort often involving the pediatrician, a neurosurgeon, a rhematologist, and a neurologist. Once diagnosed, the child and family face the prospect of surgery and recuperation. A child will typically be hospitalized for four to seven days after surgery. The prognosis for children with Chiari malformation Type I is very good. Over 70 percent of those who have surgery to correct Type I malformation experience a significant reduction in symptoms; however, there are some symptoms that may not resolve. There may be continued discomfort in the neck and lower head, and some muscle weakness may be permanent.
KEY TERMS
Brain stem—The part of the brain that is continuous with the spinal cord and controls most basic life functions. It is the last part of the brain that is destroyed by Alzheimer's disease.
Catheter—A thin, hollow tube inserted into the body at specific points in order to inject or withdraw fluids from the body.
Cerebellum—The part of the brain involved in the coordination of movement, walking, and balance.
Cerebrospinal fluid—The clear, normally colorless fluid that fills the brain cavities (ventricles), the subarachnoid space around the brain, and the spinal cord and acts as a shock absorber.
Cerebrum—The largest section of the brain, which is responsible for such higher functions as speech, thought, vision, and memory.
Computed tomography (CT)—An imaging technique in which cross-sectional x rays of the body are compiled to create a three-dimensional image of the body's internal structures; also called computed axial tomography.
Congenital—Present at birth.
Dura mater—The strongest and outermost of three membranes that protect the brain, spinal cord, and nerves of the cauda equina.
Embryonic—Early stages of life in the uterus.
Foramen magnum—The opening at the base of the skull, through which the spinal cord and the brain-stem pass.
Herniation—Bulging of tissue through opening in a membrane, muscle, or bone.
Hydrocephalus—An abnormal accumulation of cerebrospinal fluid within the brain. This accumulation can be harmful by pressing on brain structures, and damaging them.
Hypoplasia—An underdeveloped or incomplete tissue or organ usually due to a decrease in the number of cells.
Magnetic resonance imaging (MRI)—An imaging technique that uses a large circular magnet and radio waves to generate signals from atoms in the body. These signals are used to construct detailed images of internal body structures and organs, including the brain.
Neurologist—A doctor who specializes in disorders of the nervous system, including the brain, spinal cord, and nerves.
Neurosurgeon—Physician who performs surgery on the nervous system.
Rhematologist—A physician who specializes in the treatment of disorders of the connective tissue structures, such as the joints and related structures.
Scoliosis—An abnormal, side-to-side curvature of the spine.
Syringomyelia—Excessive fluid in the spinal cord.
Syrinx—A tubular fluid-filled cavity within the spine.
Ultrasonography—A medical test in which sound waves are directed against internal structures in the body. As sound waves bounce off the internal structure, they create an image on a video screen. Ultrasonography is often used to diagnose fetal abnormalities, gallstones, heart defects, and tumors. Also called ultrasound imaging.
Ventricles—Four cavities within the brain that produce and maintain the cerebrospinal fluid that cushions and protects the brain and spinal cord.
When discussing Chiari malformation and surgery with their child, parents should use words the child can understand. How illness and surgery are discussed depends on the age of the child. A younger child needs short simple answers, a school-aged child may understand more complicated explanations, and an adolescent will probably be present at all meetings with doctors and should be encouraged to talk to healthcare providers himor herself. Regardless of the age of the child, mental health experts agree that children should be told about medical procedures and surgeries before they occur. Depending on the age of the child, parents may choose to talk to him a day before or weeks ahead. The child may have questions. Parents can answer them simply and honestly. While a younger child may ask many questions, an adolescent may be reluctant to appear ignorant and may
Children with Chiari II malformation have a much poorer prognosis than those with Type I malformation and will usually be quite ill. Multiple surgeries are required, and many of these children die at an early age. All of these factors have a significant impact on the family. These children require multiple services, including surgery, physical and occupational therapy, and special education. Many have developmental delays and impaired intellectual functioning in addition to the physical limitations often caused by the accompanying spina bifida. Parents may need support services in addition to the team of healthcare providers. Many parents find it helpful to participate in a support group of other families of children with special needs. Most states and children's hospitals offer these services and know of organizations in the area that may help these families. Parents can work closely with the hospital's social work department to learn more about available resources.
See also Hydrocephalus; Spina bifida.
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Author Info: Deborah L. Nurmi MS, Thomson Gale, Gale, Detroit, Gale Encyclopedia of Children's Health, 2006 |
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