Chiari 1 Malformation at North Austin Campus

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A Chiari (key-AR-ee) type 1 malformation occurs when a small part of the bottom part of the brain (cerebellum) is lower than should be and is at risk of being under pressure. Chiari malformations can occur in some craniofacial conditions such as Pfeiffer syndrome, Crouzon syndrome, Apert syndrome, or multiple suture craniosynostosis. In these conditions, the back of the skull is smaller or has an unusual shape.

What does a Chiari 1 Malformation look like on imaging?

Magnetic Resonance Image (MRI) of a Chiari 1 malformation. The tonsil of the cerebellum (yellow dotted line) falls below the level of the skull base (red dashed line).

How do you diagnose a Chiari malformation?

At Texas Children’s North Austin, our neurosurgery team specializes in identifying Chiari malformations in children with craniofacial conditions. For children diagnosed with a craniofacial syndrome at risk for a Chiari malformation, we screen using MRI imaging to evaluate the condition early.

What are the symptoms of a Chiari malformation?

While some individuals with a Chiari malformation have no symptoms, others may experience the following:

1. Headache:

Classically occipital or suboccipital
Exacerbated by Valsalva maneuvers (coughing, sneezing, straining).

2. Neurological Symptoms:

Cranial nerve dysfunction: Dysphagia, hoarseness, or nystagmus.
Sensory disturbances: Numbness or paresthesias.
Motor deficits: Weakness or ataxia.
Autonomic symptoms: Syncope or dizziness.

3. Spinal Symptoms:

Secondary to associated syringomyelia (syrinx): Pain, scoliosis, weakness, or bowel/bladder dysfunction.

How do you treat a Chiari malformation?

Most children with a Chiari malformation who do not display symptoms require no immediate treatment. However, regular follow-up is essential to monitor for changes over time.

We consider treatment if:

1. The malformation blocks the flow of cerebrospinal fluid, increasing pressure on the brain or spinal cord.

2. Symptoms such as headaches, balance problems, or other neurological issues appear or worsen.

Treatment Plan:

If treatment is necessary, it typically involves surgery to create more space at the back of the skull, relieving pressure on the brain and spinal cord. This procedure, known as posterior fossa decompression, aims to restore normal fluid flow and alleviate symptoms.
Post-surgical care includes close follow-up with our team to ensure recovery and long-term stability.