| Introduction |
 Definitions
- syringomyelia
- a syrinx (fluid filled cavity) within the spinal cord that progressively expands and leads to neurologic deficits
- syringobulbia
- a syrinx within the brain stem that leads to neurologic symptoms
- Predisposing factors
- usually result from lesions that partially obstruct CSF flow including
- craniocervical junction abnormalities
- more than 50% related to Chiari malformations
- spinal cord trauma
- found in 4-10% of patients with spinal cord injury
- may become apparent years after the injury
- spinal cord tumors
- 30% of patients with a spinal cord tumor develop a syrinx
- Associated conditions
- developmental scoliosis
- scoliosis associated with syringomyelia in 25% to 80% of cases
- Klippel-Feil deformity
- Charcot's joints (occurs in < 5% with syringomyelia, often involves shoulder joint)
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| Presentation |
- Syringomyelia
- symptoms
- symptoms usually begin insidiously between adolescence and age 45
- syringomyelia often presents with a central cord syndrome
- physical exam
- flaccid weaknes, atrophy, and hyporeflexia of upper extremities
- deficits in pain and temperature sensation in a capelike distribution over the back of the neck
- light touch and position and vibration sensation are usually not affected
- Syringobulbia
- symptoms related to cranial nerve involvment
- tounge weakness and atrophy (CN XII)
- sternocleidomastoid & trapezius weakness (CN XI)
- dysphagia and dysarthia (CN IX, CN X)
- facial palsy (CN VII)
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| Imaging |
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- MRI
- diagnosis made my MRI
- obtain MRI with gadolinium enhancement to rule out associated tumor
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| Treatment |
- Nonoperative
- observation indicated if asymptomatic syrinx
- obtain MRI with gadolinium to rule out malignancy
- Operative
- surgical decompression of the foramen magnum and upper cervical cord
- usually does not reverse neurologic deterioration
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