In: Youmans and Winn Neurological Surgery. American Association of Neurological Surgeons. In: Adams & Victor's Principles of Neurology. Developmental diseases of the nervous system. Chiari malformations and syringohydromyelia. National Institute of Neurological Disorders and Stroke. This can cause serious nerve and muscle damage in the lower body. In this condition, the spinal cord attaches to the spine and causes the spinal cord to stretch. Some people with Chiari malformation also develop a condition called syringomyelia, in which a cavity or cyst (syrinx) forms within the spinal column. People with Chiari malformation type 2 usually have a form of spina bifida called myelomeningocele. Part of the spinal cord is exposed, which can cause serious conditions such as paralysis. Spina bifida, a condition in which the spinal cord or its covering isn't fully developed, may occur in Chiari malformation. An accumulation of excess fluid within the brain (hydrocephalus) may require placement of a flexible tube (shunt) to divert and drain the cerebrospinal fluid to another area of the body. The complications associated with this condition include: In others, there may be no associated symptoms, and no intervention is necessary. In some people, Chiari malformation can become a progressive disorder and lead to serious complications. However, research into a possible hereditary component is still in its early phase. There's evidence that Chiari malformation runs in some families. This impaired circulation of cerebrospinal fluid can lead to the blockage of signals transmitted from the brain to the body, or to a buildup of spinal fluid in the brain or spinal cord.Р”Р„lternatively, the pressure from the cerebellum on the spinal cord or lower brainstem can cause neurological signs or symptoms. When the cerebellum is pushed into the upper spinal canal, it can interfere with the normal flow of cerebrospinal fluid that protects the brain and spinal cord. The lower part of the cerebellum (tonsils) is displaced into the upper spinal canal.Р”В¬hiari malformation type 2 is nearly always associated with a form of spina bifida called myelomeningocele. Request an Appointment at Mayo Clinic CausesР”В¬hiari malformation type 1 occurs when the section of the skull containing a part of the brain (cerebellum) is too small or is deformed, thus putting pressure on and crowding the brain. If you or your child has any of the signs and symptoms that may be associated with Chiari malformation, see your doctor for an evaluation.Р”В«ecause many symptoms of Chiari malformation can also be associated with other disorders, a thorough medical evaluation is important. This type of Chiari malformation has a higher mortality rate and may also cause neurological problems. This form of Chiari malformation is diagnosed at birth or with an ultrasound during pregnancy. 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. It may also be diagnosed after birth or in early infancy. In myelomeningocele, the backbone and the spinal canal don't close properly before birth.Р”В¬hiari malformation type 2 is usually noted with ultrasound during pregnancy. The signs and symptoms can include those related to a form of spina bifida called myelomeningocele that nearly always accompanies Chiari malformation type 2. In Chiari malformation type 2, a greater amount of tissue extends into the spinal canal compared with that in Chiari malformation type 1. Breathing problems, such as central sleep apnea, which is when a person stops breathing during sleep.Curvature of the spine (scoliosis) related to spinal cord impairment.Ringing or buzzing in the ears (tinnitus).Less often, people with Chiari malformation may experience: Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting.Numbness and tingling of the hands and feet.Poor hand coordination (fine motor skills).People with Chiari malformation type 1 can also experience: They generally occur after sudden coughing, sneezing or straining. Headaches, often severe, are the classic symptom of Chiari malformation. In Chiari malformation type 1, signs and symptoms usually appear during late childhood or adulthood. The more common types of Chiari malformation are:Р”Р„lthough these types are less serious than the rarer pediatric form, type 3, signs and symptoms still can be life disrupting. However, depending on the type and severity, Chiari malformation can cause a number of problems. Their condition is detected only when tests are performed for unrelated disorders. Many people with Chiari malformation have no signs or symptoms and don't need treatment.