How are Chiari Malformations Diagnosed?

Diagnosis

Chiari malformations are sometimes diagnosed at birth. Other times, the diagnosis is made later after specific symptoms appear. If a child is suspected of having a Chiari malformation, they will be referred to a pediatric neurosurgeon for evaluation.

An MRI scan is used to confirm a diagnosis of a Chiari malformation, but your child’s doctor may recommend another imaging test, such as a CT scan.

  • MRI (Magnetic resonance imaging can show the part of the brain that is pushed down through the base of the skull (the foramen magnum), which is typical for Chiari malformation. In some instances, an MRI scan that is performed for another reason may discover Chiari malformations.
  • CT (Computerized tomography) scan uses X-rays to obtain cross-sectional images of the body. This image can help to reveal brain tumors, brain damage, and other conditions.

How are Chiari Malformations Treated?

Treatments

A pediatric neurosurgeon can recommend a course of treatment for a child diagnosed with a Chiari malformation. Depending on the symptoms and diagnosis, this may include ongoing monitoring or surgery.

Ongoing monitoring

Treatment may not be necessary for children diagnosed with Chiari malformation Type I without symptoms, but ongoing monitoring of the development of the head as the child grows is recommended.

Surgery

Surgery is typically necessary for children diagnosed with Chiari malformations Type II, III, and IV and some children with Chiari malformations Type I.  Surgery for Chiari malformation may be the only available treatment to improve symptoms and prevent further damage to the central nervous system. In some cases, more than one surgery may be needed to treat the condition.

  • Posterior fossa decompression surgery is the most common surgery to treat Chiari malformation. This surgical procedure is performed to make more room at the site of the compression. In this surgery, a piece of the skull and spinal column is removed and reshaped to restore the flow of brain fluid around the brain and the spinal cord. This surgery allows the brain more room at the base and relieves pressure on the spinal column.
  • Endoscopic third ventriculostomy (ETV) is a minimally invasive procedure that may be recommended to drain the ffluid in some patients who have hydrocephalus
  • Ventriculoperitoneal shunting is done to treat hydrocephalus by draining trapped fluid in the head through a small hole that is drilled in the skull
  • Untethering involves separating the spinal cord and releasing tension in the spine. It is done in children with Type I Chiari malformation with a tethered spinal cord, which is abnormally attached to the spine.
  • Spinal fixation – Patients with Type I Chiari malformation hypermobility syndrome may require surgery to stabilize their spine
Get Care

Trust NewYork-Presbyterian for Chiari Malformation Treatment

The NewYork-Presbyterian pediatric neurosurgery program offers therapies for children with congenital spine disorders who need specialized care. Our pediatric neurosurgery team has clinical expertise in pediatric brain and spine disorders, including congenital spine conditions such as Chiari malformation, spina bifida, scoliosis, and tethered spinal cord. 

Learn more about pediatric neurological conditions and types and symptoms of Chiari malformations, or contact us today to schedule an appointment.