A Chiari malformation is a condition in which a small part of the brain at the back of the skull sits lower than usual and extends into the upper spinal canal. This can crowd the area where the brain and spinal cord meet and may affect the normal flow of cerebrospinal fluid (CSF), which cushions and protects the brain and spinal cord.
Chiari malformation is not a defining feature of lymphatic malformations. In some individuals with complex lymphatic anomalies (CLAs), it may be identified during imaging performed as part of broader medical evaluation.
Possible Symptoms
Symptoms vary widely. Some people have no symptoms, while others may experience:
- Headaches, especially with coughing, sneezing, or straining
- Neck pain
- Dizziness or balance problems
- Weakness, numbness, or tingling in the arms or legs
- Swallowing difficulties or choking
Why It May Come Up in CLA Care
Because CLAs can affect multiple body systems, imaging of the brain or spine may be recommended. In some cases, this imaging may reveal Chiari malformation or related findings, such as a syrinx (a fluid-filled space within the spinal cord), which can contribute to symptoms.
In Gorham–Stout disease (GSD), bone loss involving the skull base, cervical spine, or upper vertebrae may alter normal anatomy in this region. In rare cases, these structural changes may prompt evaluation for Chiari malformation or Chiari-like findings.
Management
Management depends on symptoms, imaging findings, and whether there are signs of nerve or spinal cord involvement. Some individuals are monitored over time, while others may be referred to a specialist if symptoms are significant or worsening.
Important to Know
Not everyone with a CLA or GSD will have Chiari malformation. When it is identified, care is individualized and guided by symptoms and clinical findings.
