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Syringobulbia Mark R. Lee, MD, PhD Pediatric NeurosurgeryDell Children’s Medical Center
Syringobulbia Very rare entity Slit-like fluid cavity in brainstemAssociated with Chiari malformation Tumors Tethered cord Idiopathic
Syringobulbia and Chiari MalformationStrongly associated with Chiari malformation with syringomyelia But very rare… 3-5% of patients with Chiari malformation
Symptoms Head and neck pain Snoring Diplopia Sensory disturbancesUnsteady gait Dysphagia Paresthesias and numbness
Clinical Findings Cranial nerve deficits Nystagmus WeaknessAll patients Unilateral not uncommon Nystagmus Weakness Hyperreflexia Scoliosis
Treatment Restore CSF pathways between cranial and spinal compartmentsDecompression of craniovertebral junction Posterior fossa decompression Suboccipital craniectomy C1 laminectomy Duraplasty (or dural splitting)
Syringobulbia in a pediatric populationSyringobulbia 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
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