Syringobulbia
Mark R. Lee, MD, PhD
Pediatric Neurosurgery
Dell Children’s Medical Center
Syringobulbia
• Very rare entity
• Slit-like fluid cavity in
brainstem
• Associated with
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–
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–
Chiari malformation
Tumors
Tethered cord
Idiopathic
Syringobulbia and Chiari
Malformation
• Strongly associated
with Chiari
malformation with
syringomyelia
• But very rare…
– 3-5% of patients with
Chiari malformation
Symptoms
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•
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•
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•
•
Head and neck pain
Snoring
Diplopia
Sensory disturbances
Unsteady gait
Dysphagia
Paresthesias and numbness
Clinical Findings
• Cranial nerve deficits
– All patients
– Unilateral not uncommon
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•
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Nystagmus
Weakness
Hyperreflexia
Scoliosis
MRI Findings
Treatment
• Restore CSF pathways between cranial
and spinal compartments
• Decompression of craniovertebral junction
• Posterior fossa decompression
– Suboccipital craniectomy
– C1 laminectomy
– Duraplasty (or dural splitting)
Syringobulbia in a pediatric population.
Neurosurgery, 2005
Greenlee, Menezes, et. al
Resolution of Syringobulbia
Timing of Syringobulbia
Resolution?
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
“Clefts” in the Brainstem
Syringomyelia and Chiari
Malformation
Extension of Syringomyelia into
Brainstem
Summary
• Syringobulbia is rare
• Associated with syringomyelia
– ? Extension of syringomyelia
• Has cranial nerve dysfunction
• Treated with craniovertebral
decompression
• Outcomes are usually good
Thank You for Your Attention!