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 4 th 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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