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Chiari Malformation

Department of Chiari Malformation

Chiari malformations (CM) are caused by problems in the structure of the brain and skull. In Chiari malformations, the lower part of the brain presses on and through an opening in the base of the skull and cerebellum into the spinal canal. The cerebellum is the part of the brain that controls balance. Normally the cerebellum and parts of the brain stem sit above an opening in the skull (called the foramen magnum) where the spinal cord connects to the brain. When part of the cerebellum extends below this opening and into the upper spinal canal, it is called a Chiari malformation. 

CM may develop when part of the skull is smaller than normal or misshapen, which can put pressure on the cerebellum, brain stem, and spinal cord and block the flow of cerebrospinal fluid (CSF) — the clear liquid that surrounds and cushions the brain and spinal cord. 

Types

1.Type I: Cerebellar tonsils extend below the foramen magnum but the brainstem remains in its normal position.

2.Type II (Arnold-Chiari Malformation): Involves both cerebellar tonsillar herniation and displacement of the brainstem, often associated with a form of spina bifida.

3.Type III: More severe, with cerebellar and brainstem herniation, as well as a visible sac protruding from the back of the skull.

Diagnosis

1.Imaging Studies: MRI (Magnetic Resonance Imaging) is the primary diagnostic tool to visualize the brain structures and identify the displacement of the cerebellar tonsils.

2.Clinical Evaluation: Assessment of symptoms such as headaches, neck pain, and neurological deficits aids in diagnosis.

Treatment

1.Surgery: Decompressive surgery, such as posterior fossa decompression, is a common approach to relieve pressure and create more space for the cerebellum.

2.Syrinx Drainage: If a syrinx (fluid-filled cyst) is present, surgical intervention may be necessary to drain or shunt the syrinx.

Chiari Malformation Overview

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