Chiari Malformation

By: Elizabeth Feczko

https://www.nhs.uk/conditions/chiari-malformation/
 What is a Chiari Malformation?

• Also known as CM
• Opening in back of skull, called foramen magnum
• Structural defects cause the cerebellum and brain stem to also pass through
• 5 different types
 Causes of Chiari Malformations
• Primary or congenital
• During fetal development
• More common
• Acquired or secondary
• Traumatic injury, disease, or infection
• Causes CSF to drain excessively
 Type 1

• Cerebellar tonsils extends into foramen magnum

• Most common form
• May not cause symptoms initially
 Type 2

• Both cerebellum and brain stem extend into the foramen magnum
• Surrounding nerve tissue may be missing or only partially formed
• Sometimes accompanied by a myelomeningocele
• Also known as Arnold-Chiari malformations
 Type 3

• Most rare and serious form

• Abnormal opening in the back of the skull
• Brain stem and cerebellum herniate through the hole
• Symptoms appear in infancy and are usually severe
 Type 4

• Cerebellum is incomplete or underdeveloped

• Also known as cerebellar hypoplasia
• Cerebellum is located in its normal position, but parts of it are missing
 Symptoms

• Neck pain
• Ringing or buzzing in ears
• Hearing or balance problems • Scoliosis
• Muscle weakness or numbness • Insomnia
• Dizziness
• Difficulty swallowing or speaking
• Depression
• Vomiting • Hand-eye coordination problems
• Seizures • Mental and physical delays
• Paralysis
 Symptoms in Infants

• Difficulty swallowing
• Gagging
• Irritability while being fed
• Vomiting
• Excessive drooling
• Arm weakness
• Weak crying
• Stiff neck
• Developmental delays
• Breathing problems
• Inability to gain weight

http://www.the-
registry.org/Credentials/InfantToddler.aspx
 Associated Conditions

• Hydrocephalus
• Spina Bifida
• Syringomyelia
• Tethered Cord Syndrome
• Spinal Curvature
http://www.scoliosissos.com/news/post/levoconvex-scoliosis
 Diagnosis

• Ultrasound
• MRI
• X-rays
• CT scan
• Memory, cognition, balance, touch, reflexes, sensation, and motor skills.
 Treatment

• Can become fatal over time

• No cure currently exists
• Chiari malfunctions show no symptoms
• Regular MRI monitoring
• Medication
• More severe symptoms
• Posterior fossa
https://www.mayoclinic.org/diseases-conditions/chiari-
malformation/symptoms-causes/syc-20354010

• Third ventriculostomy
 Research

• Genetics
• Brain mechanisms
• Treatments
http://www.humanbrainfacts.org/interesting-brain-facts-for-
kids.php
 Statistics and Facts

• Every 1 in 1000 children are born with a Chiari malformation

• About 50% of people become symptom free after surgery, 20-30% improve
significantly, 20% experience no relief or become worse
• Surgery also fails 20% of the time for an unknown reason
• The size of the malformation is not related to severity of symptoms
• Many doctors aren’t familiar with the disease, therefore people often go
years without a proper diagnosis
 Bibliography
• “Chiari Malformation Awareness Sheet.” Chiari Malformation Awareness Sheet,
www.conquerchiari.org/awareness/documents/Chiari%20Awareness%20Sheet.pdf.
• “Chiari Malformation.” Edited by Joseph H. Piatt Jr., KidsHealth, The Nemours Foundation, Jan. 2017,
kidshealth.org/en/parents/chiari.html.
• “Chiari Malformation Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department
of Health and Human Services, www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-
Sheets/Chiari-Malformation-Fact-Sheet#3.
• “Chiari Malformation.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 7 Mar. 2018,
www.mayoclinic.org/diseases-conditions/chiari-malformation/symptoms-causes/syc-20354010.
• “Chiari Malformation: Signs, Diagnosis & Treatment Options.” Chiari: The Most Important Facts You Need
to Know | CSF, csfinfo.org/education/patient-information/chiari-malformation/.