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The Chiari malformation can be divided in 3 types, althouh some doctors include a fourth type within the classification
The posterior fossa is uaually small, and some part of the brain (cerebellum or brain stem) protrudes into the neck. This can cause a "blockage" reduction in cerebrospinal fluid pathways and hence symptoms.
Classification
- Chiari 1 Cerebellar tonsils protrude through the formaen magnum
- Chiari 2 Cerebellum, brain stem and part of the IV ventricle protrude
- Chiari 3 The above accociated with an encephlocoele
- Chiari 4 Incompatible with life
Chiari 1
- Often asymptomatic
- May cause headaches with activities which increase the pressure in the brain
- Headache aggtivated with coughing or sneezing
- 2-10% associated with hydrocephalus
- May lead to a syrinx
- Treatment only if symptomatic or causing a syrinx
Treatment of a Chiari 1
- Always treat the hyrocephalus first (shunt)
- Ideally a bony foramen magnum decompression
- Cerebellar tonsillectomy if the bony decompression fails
- Treatment of the syrinx (decompression or shunt)
- Only if the syrinx fails to settle with treatment of the chiari
Chairi 2
- Always associated with an open myelomeningocoele (see spina bifida)
- May present at birth with respiratory and feeding problems-urgent decompression
- In early childhood can cause bulbar problems (feeding, drooling)
Treatment of Chiari 2
- Need to treat and outcome is controversial
- The surgey is a cervical laminectomy
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