Chiari Malormation

Research shows the first recorded cases of Chiari malformation dates back to 1883. This disorder, named after Hans Chiari, an Austrian pathologist who classified Chiari malformations into types I through III in 1891. Chiari's colleague, Julius Arnold, made additional contributions to the definition of Chiari II malformation. The first known malformation was a 17-year-old female in 1883. CM is a congenital anomaly in which parts of the brain protrude through the opening in the base of the skull into the spinal column. Chiari used to be considered a rare disorder, although, due to advances in modern technology, it is said that there is more people diagnosis. One in one thousand children, approximately, are born with this disorder every year.
Chiari Malformation is classified into four different types. Type I: by far the most commonly observed type in children and adults. In this type, the lower part of the cerebellum, but not the brain stem extends into an opening at the base of the skull. The opening is called the foramen magnum. Normally, only the spinal cord passes through this opening. Type II: “this condition has skull, Dural, brain, spinal, and spinal cord manifestations, including downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal, as well as elongation of the puns and fourth ventricle, probably due to a relatively small posterior fossa.” (Medscape). This is usually only seen in children born with Spina Bifida; which is the incomplete development of the spinal cord and/or its protective covering. Type III: This is the most serious form of Chiari malformation. It involves the protrusion or herniation of the cerebellum and brain stem through the foramen magnum and into the spinal cord. This usually causes severe neurological defects. Type III is a rare type. Type IV: This involves an incomplete or undeveloped cerebellum. It sometimes is associated with exposed parts of the skull and spinal cord. Type IV is an extremely rare type of Chiari Malformation. Physicians say that every case of CM is never the same. Cases of CM, all have different symptoms and each have different related disorders. This is the reason why it can be hard to diagnosis a patient with this disorder.
Chiari never comes alone; it is usually accompanied by other disorders.
Such as: Syringromyelia, Hydrocephalus, Myelomeningocele, Neuropathic pain syndrome, intracranial hypertension, POTS, Scoliosis, Spina Bifida, and EDS. Since there are so many related disorders the list of symptoms can be endless. “Chiari has a wide ranging, diverse set of symptoms and affects everyone differently. Research shows that 95% of patients experience at least 5 symptoms.” (Conquerchiari.org) The symptoms that are related to CM are; neck pain, headache, unsteady gait, dizziness, poor hand coordination, weakness, central sleep apnea, vision problems, tinnitus, speech problems, swallowing problems, and the list goes on. There is currently no cure for Chiari Malformation. There are only treatments that can be done to control symptoms. If the CM is severe enough the neurosurgeon might suggest a decompression of the brain to minimize these symptoms. The cause of this disorder is unknown. There are some theories, but there is no actual proof what causes CM. There is a theory that the child develops CM in the womb due to the mother’s lack of vitamins. Another is that simply the skull is just to small or deformed to fit the brain properly. Chiari Malformation types I-III is not considered fatal, but complications due to either surgery or symptoms can
be.
Chiari Malformation type IV is a “term some authors gave to describe a form of extreme cerebella hypoplasia.
This can be associated with hypoplasia of pons as well as a small funnel shaped posterior fossa. It is now considered to be an obsolete term.” (Radiopaedia). This type is considered to be the most dangerous form. There is a lack of research of this type, so it is not fully understood. “. Type 4 actually involves a lack of development of a portion of the base of the brain. The prognosis is very poor with death often occurring during infancy.” (Healthgrades Right Diagnosis). The symptoms in this type are completely different from types I through III. The symptoms of type IV include: Hypotonia, ataxia, developmental delays, seizures, mental retardation, involuntary eye movements, Nystagmus, headache, extreme breathing problems, vertigo, and hearing impairment. Encephalocele that is sometimes known by the Latin name cranium bifidum, a neural tube defect characterized by sac-like protrusions of the brain. It is almost always accompanied by type IV. It is often diagnosed while the baby is still in the womb by an ultrasound. An unborn child that is diagnosis with type IV is often a stillbirth. Others pass away within a few
months.
Since, prognosis is poor and many patients pass away shortly after birth, there is currently no treatment for type IV. A doctor can treat the symptoms and related disorders, but there is no cure. Children that are born with this disorder spend their life in a hospital, so they can be closely monitored and made comfortable. This disorder is a death sentence before the child if even born.
Patients that are diagnosed with Chiari Malformation are often overlooked and not taken seriously. Some might not have any symptoms, but the most of the patients that have any type of CM are highly systemic and live with constant pain. It is hard to get diagnosis because it is a rare disorder that many doctors don’t know about or they misdiagnosis because of all the related disorders and symptoms that come with Chiari Malformation.