What is Chiari?

Before MJ’s MRI discovery in January Dan and I had never heard of Chiari Malformation – let alone have any idea what it was. So to understand our journey I thought it best to share some information with you that we had a crash course in when our son’s condition was discovered and surgery was scheduled within 4 weeks.

MJ has been diagnosed with 2 new neurological conditions (Autism being his first neurological diagnosis in September 2012). The first one he was diagnosed with Chiari Malformation after an MRI on January 11, 2013. His brain surgery was performed to alleviate some of the symptoms and possible complications due to this diagnosis.

What is Chiari Malformation exactly?

Chiari Malformation (kee-AR-ee) (CM) - is a serious neurological disorder where the bottom of the brain (cerebellum) is crowded in the skull cavity, and forces the lower tips of the cerebellar hemi-spheres (tonsils) into the hole in the bottom of the skull (foramen magnum). This causes blockage of cerebrospinal fluid (CSF) flow from the cranial cavity into the spinal canal.

• MJ has CM1 (Chiari Malformation Type 1) which is congenital (he was born with it)
• MJ has a Small Posterior Fossa - Back of the skull is too small for the normal sized brain.



Photo from Chiari Institute

What are the symptoms?

• Severe headache in the back of the head brought on by straining, coughing, etc.
• Sleep apnea
• Trouble Swallowing (MJ has this)
• Balance and Coordination issues (MJ has/had this)

Symptoms are due to compression of brain and spinal tissue, disruption of the natural flow cerebrospinal fluid, and increased pressure in the brain. It is not known why, but symptoms can develop at any age and some people with malformations are asymptomatic, meaning they have no symptoms.

 Treatment

• Pain/Symptom Relief - Depending on the severity of symptoms sometimes treatment can be symptom relief such as therapies or pain medications while watching to ensure the condition does not worsen.
• Surgery - In MJ’s case his condition required surgery due to the complete blockage of flow of spinal fluid causing an additional neurological condition called Syringomyelia.

 MJ’s brain surgery performed on February 28, 2013 consisted of the following:

• Craniectomy- A piece of the skull is removed
• Laminectomy- Part of one or more vertebrae are removed (Mj had this on the C1 vertebrae
• Duraplasty- The covering of the brain is opened and a patch is sewn in to make it bigger
• Tissue removal- Cerebellar tonsils are cauterized (part of the brain tissue that is “dead” due to compression)

 


Diagrams from Mayfield Chiari Center

 Possible complications

• Growth spurts can cause new crowding of his brain depending on the rate of his skull growth vs. brain growth.
• It is not uncommon for the skull bone that was surgically removed to grow back (often during a growth spurt).
• Scar tissue can build up around the surgical areas and cause new/additional blockage of CSF (cerebral spinal fluid).

Prognosis

Surgery does not take away MJ’s Chiari – It simply alleviates some of the brain compression and slows the progression of permanent neurological damage.

Chiari presents itself and can be a risk throughout childhood growth spurts. MJ’s surgeon advises we will need to be aware of MJ’s condition and any changes at growth spurts from 5-8 yrs, 10-12 yrs, and 15-18 yrs.

MJ will have increase risks of brain and spinal cord injuries and possible symptoms that may make daily life more difficult.

 

 

**Information used from the following sources:

Chiari Institute, Mayfield Chiari Center, www.conquerchiari.org, and www.csfinfo.org