FACTS
Chiari | What Herniates | Brainstem/4V position | Define | Comments |
0 | nothing | normal | < 5mm tonsillar herniation but with crowded posterior fossa and large syringes that resolve with decompression | |
1 | tonsils > 5mm | normal | Tonsils below foramen magnum > 5mm brainstem normal position +/- syrinx | #1 most common Etiology: congenitally small p fossa or acquired high pressure from above cerebellum or low pressure below cerebellum |
1.5 | tonsils > 5mm (like CM1) | herniated (like CM2) | caudal tonsillar displacement ~ CM1 but brainstem/4th ventricle are low (~CM2) | not associated with NTDs |
2 | vermis + brainstem | herniated | vermis + myelo/encepalocele | #2 most commonUsually accompanied by open myelo |
3 | foramen magnum encephalocele | foramen magnum encephalocele | - hydro common - very severe neurodev deficits | |
4 | none | normal | Hypoplasia/aplasia of cerebellum | - not really applicable |
HPI
Symptom complexes
1. Occipitovervical headaches: pain at neck/occiput worse w/ Valsalva
- irritability/grabbing
2. Anatomical
2a. Bulbar Symptoms
2b. Brainstem
- 3/6: extraocular paresis
- 9-11: downbeat nystagmus (medullary), gagging, sleep apnea, dysphagia, poor feeding, FTT, asp pna, stridor/hoarseness (rare)
NOTE: Chiari 2 patients especially p/w life-threatening apnea (breath-holding spells), inspiratory stridor, dysphagia, bradycardia
- 12: tongue atrophy
dysfunction
2c. Cerebellar syndromes (rare)
2d. Myelopathy (syrinx)
3. Scoliosis
A/P
- complete pan spine MRI to eval syrinx
- SLP consult for swallow exam
- Outpatient apnea testing
- Optho eval as indicated for hydro
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