An Overview of Neurosurgery and Spina Bifida

1. An Overview ofNeurosurgeryandSpina Bifida Joseph H Piatt, Jr, MD, FAAP Section of Neurosurgery

2. Spina Bifida and Neurosurgery • better CSF shunts • better CSF shunt surgery • removal of CSF shunts • avoidance of CSF shunts • Chiari malformation type 2 • spinal cord complications

3. CSF shunts • 85 – 90% prevalence among MM pts • shunt failure: acute or insidious • > 1 / 3 fail in first year • 5 – 10% infection rate per operation • 1 – 2% mortality (!) per failure • exacerbation of CM2 / syringomyelia

5. Better CSF Shunts • the Shunt Design Trial • Bioglide • programmable valves

6. differential pressure Delta Orbis-Sigma

10. Better CSF Shunt Surgery • the Endoscopic Shunt Insertion Trial • ultrasound • image guidance • shaveless CSF shunt surgery

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17. Shaveless CSF Shunt Surgery • Horgan MA, Piatt JH Jr. Shaving of the scalp may increase the rate of infection in CSF shunt surgery. Pediatr Neurosurg 1997;26:180-184. • Winston KR. Hair and neurosurgery. Neurosurgery 1992;31:320-329.

18. Removal of CSF Shunts:Endoscopic 3rd Ventriculostomy • If it ain’t broke, don’t fix it. • E3V for CSF shunt failure • urgent • “elective” for slit ventricle • E3V for CSF shunt infection

19. Management of Shunt Failure with E3V • Cinalli G. The role of endoscopic third ventriculostomy in the management of shunt malfunction. Neurosurgery 1998;43:1323-7. • Teo C. Management of hydrocephalus by endoscopic third ventriculostomy in patients with myelomeningocele. Pediatr Neurosurg 1996;25:57-63.

20. Management of Shunt Infection with E3V • Jones R. Third ventriculostomy for shunt infections in children. Neurosurgery 1993;32:855-9. • Fukuhara T. Risk factors for failure of endoscopic third ventriculostomy for obstructive hydrocephalus. Neurosurgery 2000;46:1100-9.

21. Management of “Slit Ventricle Syndrome” with E3V • Baskin J. Ventricular shunt removal: the ultimate treatment of the slit ventricle syndrome. J Neurosurg 1998;88:478-84. • Reddy, K. Slit ventricle syndrome with aqueduct stenosis: third ventriculostomy as definitive treatment. Neurosurgery 1988;23:756-9.

28. E3V shunts

29. Avoidance of CSF Shunts:Antenatal MM Repair • Rationale • Expected benefit – lower extremities • Observations – Chiari • Putative benefit – hydrocephalus • Risks and ethical issues

30. Avoidance of CSF Shunts:the MRCT • Vanderbilt, CHoP, UCSF • NO repairs outside trial! • Endpoint – CSF shunt insertion • adjudication • Secondary endpoints • “Freezing” of technology

31. Chiari malformation type 2 • posterior fossa too small • hindbrain developmentally abnormal • medullary dysfunction – infancy • Compression? • medullary dysfunction – later • CSF shunt failue? • syringomyelia

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34. Spinal Complications • syringomyelia / syringobulbia • spinal arachnoid cyst • diastematomyelia • dermoid cyst • tethering

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37. Spinal Cord Tethering • progressive neurological deficits • pain • bladder dysfunction • a diagnosis of exclusion • surgical risks and benefits