1	Feeling Lost? A quick overview of Chiari & Syringomyelia
2	Arnold -WHO? Chiari malformation (ACM, CM) is a condition where part of the brain, the cerebellar tonsils, descend out of the skull and crowd the spinal cord Hans Chiari first identified the disorder in the 1890’s Type I is shown above, Type II is more serious and is associated with Spina Bifida, Type III and IV are very rare Cause unknown, believed to be primarily a congential condition Affects people of all ages, however usually diagnosed in children and adults in late 20’s – early 30’s Classic definition involves the length of the herniation (>3-5mm) Research has shown little correlation between length of herniation and symptoms; focus now on flow of cerebrospinal fluid (CSF); malformation blocks normal flow Leads to a secondary condition, syringomyelia, in some people
3	Leads To What? Syringomyelia (SM) is a neurological condition where a fluid filled cyst, or syrinx, forms in the spinal cord The syrinx expands the spinal cord, sometimes to more than twice its normal diameter, and stretches the nerve tissue paper thin Why a syrinx forms is unknown Several theories, none proven or without problems SM affects children and adults of all races and ethnicities Can cause permanent nerve damage and paralysis
4	CM & SM: Two Sets Of Problems Chiari Malformation (CM) is the leading cause of syringomyelia (SM), so some people must deal with two sets of problems The direct link between CM and SM is poorly understood Spinal trauma and tumors are other leading causes of SM
5	Symptoms
6	Diagnosis Everyone is different Some people are diagnosed soon after onset of symptoms Some people go years before an accurate diagnosis Some asymptomatic (or slightly symptomatic) people are diagnosed incidental to another medical event/condition In the end most people see a Neurosurgeon Neurological exam Check for loss of sensation in hands, balance problems, reflexes, walking, strength, etc. MRI (Magnetic Resonance Imaging) Lay perfectly still inside a giant magnet that makes strange noises, but also makes great pictures of your brain and spinal cord CINE MRI MRI shows static anatomy, CINE MRI shows the flow of CSF (cerebrospinal fluid) and whether it is obstructed Surgery is the only real treatment Based on MRI and symptoms, the patient and Neurosurgeon will decide whether surgery is warranted Sometimes the Neurosurgeon will recommend monitoring the situation if the symptoms aren’t that bad and there is no syrinx
7	Surgery Is A Treatment, Not A Cure Many neurosurgeons consider decompression surgery to be “technically” straightforward There are many variations on the surgical technique; none are conclusively proven better than others Unfortunately, surgery doesn’t always work; repeat surgeries are sometimes necessary Surgery is fairly traumatic and can not reverse nerve damage that has already occurred Complications include infection and spinal fluid leakage (from dura patch)
8	Surgery: What To Expect
9	Surgical Outcomes For Chiari Only – Data is not conclusive, but… Up to 80% may have significant improvement Repeat surgeries are sometimes necessary Factors that influence outcome may include complex anatomy, duration of symptoms, co-existing conditions Lasting problems may include exertional headaches, balance problems, trouble bending over Long-term recurrence of symptoms is a problem For Chiari with syringomyelia – Even less conclusive data, but… Fewer people recover fully Lingering symptoms are more common Chronic pain is a common lasting effect Other lasting problems may include bad shoulders (from nerve damage), trouble walking and intolerance to heat Syrinx can take up to a year to shrink
10	Things To Keep In Mind Surgeons tend to use a radiological definition of success Syrinx has shrunk, restored CSF flow Discuss specific outcomes with doctor – what is the chance of being symptom free? Will my main symptoms go away? Not clear why surgery fails (up to 20% of the time) Each person is unique Anatomy, symptoms, other conditions Over time symptoms will come and go, there is always a chance of recurrence Traumas, such as car accidents, can flare up symptoms Most people will modify their lifestyle to fit their situation Some go on disability Some seek pain treatments Some modify their activities
11	How Many People Are Affected?
12	State of Research
13	However, There Are Many Areas Of Active Research Surgical technique The best surgical approach is a highly debated topic with many publications How much bone to remove? Should the dura be opened? Should some of the cerebellar tonsils be removed? When should a shunt be put in? Radiological The advent of MRI, and now CINE MRI, revolutionized the diagnosis of ACM and SM Radiologists are developing new protocols to obtain even more information CSF characterization Bioengineers and others are working to mathematically quantify the spinal system in order to gain a fundamental understanding of the conditions Genetic There are enough family clusters of Chiari to indicate a possible genetic factor in the disease. Geneticists are working to find a “Chiari” gene Syrinx formation There are several active theories of how syrinxes form Pain Management Many types of doctors are researching novel treatments for pain
14	US Government Has Not Stepped Up To The Plate
15	Economic Impact Estimated Annual Costs of SM/CM in US: Doctors Visits $ 32,000,000 MRI Scans $ 200,000,000 Surgeries $ 750,000,000 Rehab/Home Care $ 240,000,000 Disability Payments $ 1,475,000,000 Total $ 2,697,000,000 Lack of knowledge about SM/CM translates to ~2.7 Billion in annual costs; this does not include lost wages, productivity, etc.
16	C&S Patient Education Foundation
17	Thank You Return To Main Page