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 Nystagmus Weakness 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 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

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