Syringobulbia Mark R. Lee, MD, PhD Pediatric Neurosurgery Dell Children’s Medical Center.
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Transcript of Syringobulbia Mark R. Lee, MD, PhD Pediatric Neurosurgery Dell Children’s Medical Center.
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
– 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
• Head and neck pain
• Snoring
• Diplopia
• Sensory disturbances
• Unsteady gait
• Dysphagia
• Paresthesias and numbness
Clinical Findings
• Cranial nerve deficits– All patients– Unilateral not uncommon
• 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!