Syringobulbia

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Syringobulbia

• Mark R. Lee, MD, PhD
• Pediatric Neurosurgery
• Dell Childrens Medical Center

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Syringobulbia

• Very rare entity
• Slit-like fluid cavity in brainstem
• Associated with
• Chiari malformation
• Tumors
• Tethered cord
• Idiopathic

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Syringobulbia and Chiari Malformation

• Strongly associated with Chiari malformation with
  syringomyelia
• But very rare
• 3-5 of patients with Chiari malformation

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Symptoms

• Head and neck pain
• Snoring
• Diplopia
• Sensory disturbances
• Unsteady gait
• Dysphagia
• Paresthesias and numbness

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Clinical Findings

• Cranial nerve deficits
• All patients
• Unilateral not uncommon
• Nystagmus
• Weakness
• Hyperreflexia
• Scoliosis

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MRI Findings
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Treatment

• Restore CSF pathways between cranial and spinal
  compartments
• Decompression of craniovertebral junction
• Posterior fossa decompression
• Suboccipital craniectomy
• C1 laminectomy
• Duraplasty (or dural splitting)

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Syringobulbia in a pediatric population.
Neurosurgery, 2005Greenlee, Menezes, et. al
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Resolution of Syringobulbia
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Timing of Syringobulbia Resolution?
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What Causes Syringobulbia?

• Always associated with syringomyelia
• Propagation of syringomyelia into brainstem.
• rupture of cervical syrinx
• Extension of dilated central canal
• Direct entry of CSF into brainstem
• opening in floor of 4th ventricle

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Clefts in the Brainstem
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Syringomyelia and Chiari Malformation
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Extension of Syringomyelia into Brainstem
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Summary

• Syringobulbia is rare
• Associated with syringomyelia
• ? Extension of syringomyelia
• Has cranial nerve dysfunction
• Treated with craniovertebral decompression
• Outcomes are usually good

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Thank You for Your Attention!