Summary
In Chiari malformation, brain tissue extends into the spinal canal. This happens when a part of the skull is misshapen or smaller than usual, pushing the brain downward. Despite Chiari malformation being uncommon, imaging tests have led to diagnosis more frequently.
There are three types of Chiari malformation, based on the anatomy of the brain tissue that is displaced into the spinal canal and whether developmental problems of the brain or spine are present. Due to the growth of the skull and brain, the signs and symptoms of Chiari malformation type 1 may not appear until late childhood or adulthood. In pediatrics, Chiari malformation types 2 and 3 occur in birth (congenital).
Regular monitoring, medications, and surgery are the treatment options for Chiari malformation, depending on its form, severity, and associated symptoms. Treatment may not be necessary in some cases.
Symptoms
Many people with Chiari malformation have no symptoms and do not need treatment. Their condition is usually discovered only when tests are performed for unrelated conditions. Depending on the type and severity, Chiari malformation, can cause a variety of problems.
Chiari malformation is most commonly classified as Type 1 and Type 2. The signs and symptoms of these types still can disrupt a person's life, even though they are less severe than type 3.
Chiari malformation type 1
Children who develop Chiari malformation type 1 usually have the condition appear sometime in their late childhood or early adulthood.
People with Chiari malformation are often afflicted with extreme headaches, most commonly during coughing, sneezing, or other forms of strain. Individuals with Chiari malformation type 1 may also experience:
Headaches
Neck stiffness
Difficulty with fine coordination.
Hand and foot tingling
Feeling a little off balance.
Issues swallowing
Dry-heaving, easy gag-reflex, or vomiting.
Hoarseness
Speech problems
Ringing or buzzing in their ears, a.k.a. tinnitus.
Slow heartbeat
One of the possible effects of the curvature of the spine (scoliosis) is related to an impairment in the spinal cord.
Common breathing problems such as central sleep apnea, (which happens when a person ceases to breathe during sleep).
Chiari malformation type 2
A person with Chiari malformation type 2 will have a greater amount of tissue pushing downward from the skull than one with Chiari malformation type 1. Some of the symptoms may include those related to a form of spina bifida called myelomeningocele which accompanies Chiari malformation type 2. In myelomeningocele, the backbone and the spinal canal don't close before birth.
Signs and symptoms can be as follows:
Change in pattern of breathing.
Difficulty swallowing and bad gag reflexes.
Quick, downward, horizontal eye movements.
Weakness of the arms
Pregnant women may be able to discover via ultrasound if she has a fetus with Chiari malformation type 2. Sometimes, it's not discovered until the infant is born or in the first few months of their life.
Chiari malformation type 3
In the most severe type of the condition, Chiari malformation type 3, a portion of the lower back part of the brain (cerebellum) or the brainstem extends through an opening in the back of the skull. This form of Chiari malformation is diagnosed at birth or with an ultrasound during pregnancy. This type of Chiari malformation has a higher mortality rate and may also cause neurological problems.
When to see a doctor
When the brain does not develop properly during pregnancy, the skull may be too small to accommodate the brain, which results in pressure on the brain and spinal cord. This can result in muscle weakness, difficulty swallowing, and loss of bladder control, among other symptoms. In some cases, surgery is required to relieve pressure on the spinal cord, with varying levels of success depending on the severity of the malformation. This guide will explain what chiari malformation is, how it affects the body, and how it can be treated.
Consult your doctor if you or your child show any of the symptoms associated with Chiari malformation. Several Chiari malformation symptoms can also be associated with other disorders, making a thorough medical examination vital.
Causes
The cerebellum (lower part of the cerebellum) protrudes into the upper spinal canal because a section of the skull containing the cerebellum is too small or deformed. Almost always, Chiari malformation type 2 is associated with myelomeningocele, a type of spina bifida.
The cerebellum can interfere with the flow of cerebrospinal fluid, which protects the brain and spinal cord, when pushed into the upper spinal canal. When cerebrospinal fluid circulation is impaired, signals from the brain to the body are blocked, or spinal fluid builds up in the brain and spinal cord. Alternatively, the cerebellum can exert pressure on the spinal cord or lower brain stem, causing neurological symptoms.
Risk factors
There's evidence that Chiari malformation is hereditary. However, research into a possible hereditary component is still in its early phase.
Complications
There are some cases in which Chiari malformation can become progressive, leading to serious complications. In other cases, there may not be any symptoms, and no intervention is required. Complications associated with this condition include:
Hydrocephalus - As a result of excessive cerebrospinal fluid accumulation in the brain (hydrocephalus), a flexible tube (shunt) can be inserted to redirect and drain the fluid.
Spina bifida - During Chiari malformation, the spinal cord or its covering may not fully develop, resulting in spina bifida. Chiari malformation type 2 is characterized by an exposed part of the spinal cord, causing serious conditions such as paralysis. In most cases, people with Chiari malformation type 2 have a type of spina bifida called myelomeningocele.
Syringomyelia. - Some people with Chiari malformation also develop a condition called syringomyelia, in which a cavity within the spinal column forms a cyst (syrinx).
Tethered cord syndrome - In which the spinal cord is attached to the spine and stretches. A serious injury to the lower body's nerves and muscles can result from this.
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