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What is a Chiari Malformation?

/20 Mar 2018
/By admin

A Chiari malformation is a congenital (present at birth) defect in the area of the back of the head where the brain and spinal cord connect. The condition is also called Arnold Chiari malformation. There are four types of Chiari malformations:


Type I – this is the most common type of Chiari malformation. Commonly goes unnoticed until problems arise in the adolescent or adult years of life. In this condition, the base of the skull and the upper spinal area are not formed properly.

Type II – In this condition, part of the back of the brain shifts downward through the bottom of the skull area.

  • Type II Chiari malformations are typically seen in infants who are born with spina bifida, a neurological condition that causes a portion of the spinal cord and the surrounding structures to develop outside, instead of inside, the body.
  • Type II Chiari malformations can also be associated with a condition known as hydrocephalus. Hydrocephalus is a condition in which there is an overproduction or lack of absorption of the cerebral spinal fluid (CSF) that is found inside of the brain. The increased fluid causes the pressure inside of the head to increase and the skull bones to expand to a larger-than-normal appearance.

Type III – The back of the brain protrudes out of an opening in the back of the skull area.

Type IV – The back of the brain fails to develop normally.


According to The Spine Hospital at the Neurological Institute in New York City, the following are the most common symptoms of a Chiari malformation:

  • Headaches
  • Stiffness or pain in the neck or back of the head area
  • Poor feeding and swallowing
  • Decreased strength in the arms
  • Decreased sensation in the arms and legs
  • Rapid, back and forth, eye movement
  • Developmental delays
  • Weak cry
  • Breathing problems


Causes and Risk Factors

The exact cause of a Chiari malformation is unknown.  It is believed that a problem during fetal development may cause the abnormal brain formation. Chiari malformation may be caused by exposure to harmful substances during fetal development or associated with genetic problems or syndromes that may have a tendency to run in families.


Theories suggest that the following may predispose the fetus to problems that affect the normal development of the head during pregnancy:

  • Exposure to hazardous chemicals/substances
  • Lack of proper vitamins and nutrients in the diet
  • Infection
  • Prescription or illegal drug and alcohol consumption


There are many ways to treat Chiari malformations, but all require surgery. The basic operation is one of creating space in the area at the base of the cerebellum where it is pushing against the brainstem and spinal cord. This is done by removing a small portion of bone at the base of the skull deep to the neck muscles as well as often removing a part of the back of the first and occasionally additional spinal column segments.


If you think you or your loved one has suffered from Chiari malformations due to someone else’s negligence, you may have a case. Contact an attorney, like a medical malpractice lawyer New York, NY trusts, today.

Thanks to our friends and contributors from Okun Oddo & Babat, P.C. for their insight into medical malpractice.