If your child has Chiari I but does not have a syrinx or symptoms, generally no treatment is necessary. The medical team will perform follow-up magnetic resonance imaging (MRI) scans to monitor your child’s condition. If the malformation is causing neck pain or headache, pain-relief medication may help. If your child is experiencing swallowing issues, speech therapy may be helpful.

However, if the pain is severe, or your child is experiencing other symptoms that affect his or her quality of life, the medical team may recommend decompression surgery to relieve symptoms. Without surgery, a child will likely continue to have symptoms, which may worsen over time.

Children with Chiari II typically do not undergo surgery, unless the malformation is causing breathing and swallowing difficulties. If those symptoms are present, the medical team may recommend decompression surgery.

Headaches and pain associated with Chiari malformations can often be managed by pain medications. Regular monitoring ensures symptoms do not get worse over time, at which point surgery may be needed. The family and neurosurgeon will decide if surgery is the best option.

Decompression surgery is an umbrella term used to describe a number of different procedures used to remove the bone compressing the cerebellar tonsils (the lower part of the cerebellum). When successful, this reduces pressure on the brain and spinal cord, and restores the normal flow of cerebrospinal fluid.

Decompression surgery is performed under general anesthesia.

• First, a small cut is made at the back of the head.
• The muscle is gently moved out of the way.
• A small piece of bone is removed from the bottom of the skull to create the extra space.

During surgery, the surgeon uses imaging technology to view the movement of the cerebellar tonsils and surrounding cerebrospinal
fluid to determine whether the patient will benefit from more extensive decompression. If the space is okay, the surgery is over and the incision is closed.

If more space is needed:

• The surgeon makes a cut in the covering of the brain, called the dura mater.
• The surgeon may need to sew a patch over the opening to keep cerebrospinal fluid from leaking.
• Sometimes, the surgeon may shrink the cerebellar tonsils to make more room for the cerebrospinal fluid to circulate. These tonsils do not have any known purpose.

The surgical technique may vary. If your child has a syrinx or hydrocephalus, he or she may need a tube (shunt) to drain the excess fluid.

Decompression surgery can take two to three hours. Every patient is different, but the recovery process typically includes one night in the intensive care unit and three to four days in the hospital. Your child will experience pain and swelling after surgery, but our team will prescribe medication to make him or her comfortable during the healing process.

After returning home, your child will need to avoid activities, such as heavy lifting and contact sports, for a couple of months. During this time it is important for your child to avoid activities that cause neck strain, such as carrying a heavy backpack. During follow-up appointments in the clinic, your child’s doctor will provide specific instructions, and let you know when your child can resume all of his or her usual activities.

Some children never require treatment for a Chiari malformation. Others are able to enjoy a good quality of life with the help of non-surgical therapies, such as pain-relief medicine or speech therapy.

When surgery is needed, results are often very positive. Many children are symptom free or have significantly fewer symptoms. Long-term monitoring includes performing MRIs at regular intervals to evaluate the results of the decompression, the response of any associated syrinx, and any scar tissue development.

In a small number of cases, a second decompression surgery is needed.

Neurosurgeries take place in the BrainSUITE at Cincinnati Children’s. There, surgeons use advanced technology to create precise, three-dimensional maps of the brain and tailor their approach to each patient, minimizing trauma to surrounding areas of the brain that control speech, movement and other vital functions. As a result, they are able to treat patients whose conditions previously would have been considered inoperable.

Cincinnati Children’s was the first pediatric hospital in the world to offer this technology and thanks to recent upgrades, our BrainSUITE now features the most sophisticated equipment available for pediatric neurosurgery.