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

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