Syringobulbia

1. Syringobulbia Mark R. Lee, MD, PhD Pediatric Neurosurgery Dell Children’s Medical Center

2. Syringobulbia • Very rare entity • Slit-like fluid cavity in brainstem • Associated with • Chiari malformation • Tumors • Tethered cord • Idiopathic

3. Syringobulbia and Chiari Malformation • Strongly associated with Chiari malformation with syringomyelia • But very rare… • 3-5% of patients with Chiari malformation

4. Symptoms • Head and neck pain • Snoring • Diplopia • Sensory disturbances • Unsteady gait • Dysphagia • Paresthesias and numbness

5. Clinical Findings • Cranial nerve deficits • All patients • Unilateral not uncommon • Nystagmus • Weakness • Hyperreflexia • Scoliosis

6. MRI Findings

7. Treatment • Restore CSF pathways between cranial and spinal compartments • Decompression of craniovertebral junction • Posterior fossa decompression • Suboccipital craniectomy • C1 laminectomy • Duraplasty (or dural splitting)

8. Syringobulbia in a pediatric population. Neurosurgery, 2005Greenlee, Menezes, et. al

9. Resolution of Syringobulbia

10. Timing of Syringobulbia Resolution?

11. 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

12. “Clefts” in the Brainstem

13. Syringomyelia and Chiari Malformation

14. Extension of Syringomyelia into Brainstem

15. Summary • Syringobulbia is rare • Associated with syringomyelia • ? Extension of syringomyelia • Has cranial nerve dysfunction • Treated with craniovertebral decompression • Outcomes are usually good

16. Thank You for Your Attention!