Chiari Malformation

Chiari Malformation

A Chiari (pronounced key-AR-ee) Malformation, also known as an Arnold-Chiari Malformation, is a condition in which the back part of the brain extends into the base of the spinal canal.  There are three categories of Chiari malformation; however, the treatment for each of these is similar.  Headaches are the classic symptom of a Chiari Malformation, however children may complain of neck pain, balance issues, upper extremity weakness, dizziness or difficulty swallowing or choking or they may be diagnosed with central sleep apnea.

What to expect from your visit: 

If you are coming to visit one of our Neurosurgeons because you have been told you have a Chiari malformation on recent imaging it is helpful to bring that report and a disc of the imaging to your visit.  During the visit our Neurosurgeons will discuss treatment options that are based on both symptoms and severity of the Chiari on the images.  An MRI is the best exam to view a Chiari malformation; if an MRI has not been completed we will set it up during your appointment.  It is possible we may want to repeat an MRI as some special tests we perform at Children’s Hospital of Michigan can be helpful to both monitor and diagnose a Chiari Malformation.  

Rarely, an area of fluid can develop within the spinal cord itself and this is known as a syrinx.  It is more likely to occur if a Chiari malformation is present.  If you have not had an MRI of the entire spine your Neurosurgeon may discuss obtaining one in the future.  

What to expect from Surgery: 

Not all Chiari malformations require surgery, however, if you and your surgeon decide that surgery is the best option this is what to expect.  Families will be given a date and time for surgery during their clinic appointment.  It is standard that children do not have anything to eat past midnight on the day of surgery.  We understand this is difficult for our very young patients and take special care to schedule those surgeries as early in the day as possible.  It is important you bathe your child and wash their hair either the night before or the morning of surgery to help prevent post operative infections.

In the morning your surgeon will review everything discussed in the office and be sure to answer any questions caregivers or children have.  The surgery generally requires 2-4 hours to complete and updates will be provided by our OR staff.  There is some additional time required at the beginning of your child’s surgery while the anesthesia team places an IV and helps your child fall asleep.  

After surgery your child will be sent to our wake up (PACU) area.  After discussing the surgery with your Neurosurgeon you are welcome to rejoin your child there.  They will be monitored carefully as the anesthesia begins to wear off.  From there they will be taken to the intensive care unit for monitoring following after surgery.  

There will be a small incision, about an inch long at the base of the neck.  This incision can be painful and is often associated with some neck stiffness and muscle pain.  We are very proactive with pain control after surgery, but it is not possible to be pain free.  Fortunately the pain often subsides by the second or third day.  It is important to encourage your child to gently turn their head from side to side in the days after surgery to prevent a stiff neck.

Following surgery the natural pathway of the fluid that flows around the brain has been restored.  Although this is the goal of surgery, it can feel very unnatural in the first few days following surgery and often causes significant nausea.  In addition, the vomiting center in the brain is very close to the area where surgery is completed, so it is not uncommon to have some nausea in the first few days following surgery.  This nausea resolves in time as the brain heals and the child acclimates to the normal flow of the fluid.  

Generally, children are hospitalized from 3-5 days following surgery.  During this time frame their pain becomes easy to manage on oral medications that can be given at home and they resume a normal diet.  

Follow up: 

To care for the incision wash daily with a mild soap and dry gently.  The incision will have small clear sutures that will dissolve on there own in approximately 10 days.  Patients should avoid submerging the incision completely underwater for 14 days following surgery, so this means taking modified baths and no swimming in pools or lakes.

Caregivers and patients should monitor for any redness or swelling that occurs in the days or weeks following surgery.  Rarely a superficial wound infection could occur and is easily treatable if detected early.  Please notify the Neurosurgery department at (313)833-4490 of any redness, swelling, or drainage and one of Advance Practice Providers will provide you immediate guidance, and a same day appointment if necessary.  

A scheduled “wound check” appointment will be made 10-14 days after surgery with one of our Advanced Practice Providers.  This is a good time to talk about returning to school, day care, or other activities.  This is another great opportunity to sit and answer any and all questions now that the stress of the hospital is behind you.   In addition to the wound check, a scheduled follow up with your surgeon will be scheduled for 4-6 weeks after surgery.