An Overview of Neurosurgery and Spina Bifida

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An Overview of NeurosurgeryandSpina Bifida

• Joseph H Piatt, Jr, MD, FAAP
• Section
• of
• Neurosurgery

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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

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CSF shunts

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

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Better CSF Shunts

• the Shunt Design Trial
• Bioglide
• programmable valves

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differential pressure
Delta
Orbis-Sigma
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Better CSF Shunt Surgery

• the Endoscopic Shunt Insertion Trial
• ultrasound
• image guidance
• shaveless CSF shunt surgery

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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 199726180-184.
• Winston KR. Hair and neurosurgery. Neurosurgery
  199231320-329.

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Removal of CSF ShuntsEndoscopic 3rd
Ventriculostomy

• If it aint broke, dont fix it.
• E3V for CSF shunt failure
• urgent
• elective for slit ventricle
• E3V for CSF shunt infection

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Management of Shunt Failure with E3V

• Cinalli G. The role of endoscopic third
  ventriculostomy in the management of shunt
  malfunction. Neurosurgery 1998431323-7.
• Teo C. Management of hydrocephalus by endoscopic
  third ventriculostomy in patients with
  myelomeningocele. Pediatr Neurosurg
  19962557-63.

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Management of Shunt Infection with E3V

• Jones R. Third ventriculostomy for shunt
  infections in children. Neurosurgery
  199332855-9.
• Fukuhara T. Risk factors for failure of
  endoscopic third ventriculostomy for obstructive
  hydrocephalus. Neurosurgery 2000461100-9.

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Management of Slit Ventricle Syndrome with E3V

• Baskin J. Ventricular shunt removal the ultimate
  treatment of the slit ventricle syndrome. J
  Neurosurg 199888478-84.
• Reddy, K. Slit ventricle syndrome with aqueduct
  stenosis third ventriculostomy as definitive
  treatment. Neurosurgery 198823756-9.

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E3V
shunts
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Avoidance of CSF ShuntsAntenatal MM Repair

• Rationale
• Expected benefit lower extremities
• Observations Chiari
• Putative benefit hydrocephalus
• Risks and ethical issues

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Avoidance of CSF Shuntsthe MRCT

• Vanderbilt, CHoP, UCSF
• NO repairs outside trial!
• Endpoint CSF shunt insertion
• adjudication
• Secondary endpoints
• Freezing of technology

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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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pf
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Spinal Complications

• syringomyelia / syringobulbia
• spinal arachnoid cyst
• diastematomyelia
• dermoid cyst
• tethering

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Spinal Cord Tethering

• progressive neurological deficits
• pain
• bladder dysfunction
• a diagnosis of exclusion
• surgical risks and benefits

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