Chiari malformation

What is it?

Chiari malformation is a congenital defect in the area of the back of the head where the brain and spinal cord connect. There are four types of Chiari malformations:

  • Type 1: This is the most common type of Chiari malformation, where the base of the skull and the upper spinal area are not formed properly. It commonly goes unnoticed until problems arise in the adolescent or adult years of life.
  • Type 2: In this condition, part of the back of the brain shifts downward through the bottom of the skull area.
  • Type 3: The back of the brain protrudes out of an opening in the back of the skull area.
  • Type 4: The back of the brain fails to develop normally.

Symptoms

Each child experience symptoms differently. In infants and older children born with this condition, symptoms may include breathing problems, headaches, stiffness or pain in the neck, poor feeding, developmental delays, decreased strength in the arms, and decreased sensation in the arms and legs.

Diagnosis

If a Chiari malformation occurs with other congenital defects, the diagnosis is made at birth. Other times, the diagnosis is made after the onset of specific symptoms, and after diagnostic testing. In most cases, Magnetic Resonance Imaging (MRI) is sufficient for the diagnosis.

Treatment

Surgical procedure is at the base of every treatment. Specific treatment for a Chiari malformation will be determined by the child’s physician based on the child’s age, overall health medical history, extent of the condition, type of condition, and tolerance for specific medications, procedures, or therapies. The goal of Chiari decompression surgery is to stop the progression of the changes in the brain and spinal canal, as well as ease or stabilize symptoms. With this technique, the surgeon removes a small section of bone in the back of the skull.