Chiari Malformation Awareness Month
Photo courtesy of MesserWoland/Wikimedia Commons
Chiari Malformation Awareness is commonly associated with a purple ribbon and it is also a symbol of strength for those suffering.
September is Chiari Malformation Awareness Month. Chiari is a lesser-known condition in which the brain extends downwards into the spinal canal due to a lack of space in the skull. While this disorder is not common, there has been an increase in diagnoses due to more advanced medical technology.
Many patients with Chiari experience little to no symptoms—it was simply an incidental finding during other testing. A scarcer population of these patients experience debilitating symptoms due to the malformation. There are several forms in which Chiari malformation may present; these are classified as Chiari malformation type I, II, III, and IV.
Chiari malformation type I is the most common of the four types, which means that the cerebellum has been forced downwards through the opening of the skull, merging into the spinal canal. Usually, the condition develops at birth, but is not diagnosed until childhood or early adulthood. There are no solid medical explanations as to why type I develops. Some signs that a child may have type I would be the presence of a syrinx—a cyst putting pressure on the spinal cord—or a diagnosis or misdiagnosis of scoliosis.
The signs of Chiari malformation type II almost always include myelomeningocele–a child's spine or spinal canal did not properly close before birth–and the compression of the cerebellar vermis, brainstem, and fourth ventricle. If not treated properly, severe mobility issues or even death may develop. Patients with a manageable version of type II will usually experience difficulty swallowing, facial weakness, aspiration, syringomyelia, and many other mobility issues. Surgery is often required in type II cases in order to address the most serious problems, like myelomeningocele, and to fix the cerebrospinal fluid (CSF) flow.
Chiari malformation type III is a truly horrific condition, as the patient either dies or is left with severe disabilities as a result of the condition. This type is normally diagnosed during pregnancy through an ultrasound or at birth. Patients are born with parts of their brain being forced downwards out through the skull opening, and in some cases, pushed out from the neck or back of the head.
The rarest type is type IV, in which the cerebellum may be underdeveloped or missing integral pieces. Most infants will not survive Chiari malformation type IV.
Chiari malformation, in all of its forms, is a horrific and under-researched medical condition. The malformation will present itself with a multitude of symptoms, including difficulty with swallowing or breathing, tinnitus, speech difficulty, weakness in extremities, lack of motor coordination, neck pain, and constant pain throughout the body.
MRI and CT scans are the proper forms of testing to determine if a patient may be suffering from Chiari, although a patient could have a Chiari Malformation (usually a type I) and be asymptomatic. The malformation only affects a small group of people who have it, which makes it even more difficult for doctors to understand the condition and provide resources for those suffering.
Furthermore, there is no known cure for Chiari at this time. While decompression surgery is recommended for some patients, it is not a cure or even effective in some cases for treating symptoms. The surgery is required in patients who have a syrinx or whose CSF flow is being blocked due to a lack of space in the skull.
With September being Chiari Malformation Awareness Month, it is an opportunity to learn more about the condition. In their September newsletter, the Bobby Jones Chiari & Syringomyelia Foundation states, “Too often, Chiari is misunderstood or invisible—but by speaking up and standing together, we shine a light on what it truly means to live with this condition. This month, we come together not only to spread awareness, but also to celebrate resilience, support one another, and hold onto hope for better answers and brighter days ahead.”