An Overview of
Neurosurgery
and
Spina Bifida
Joseph H Piatt, Jr, MD, FAAP
Section
of
Neurosurgery
Spina Bifida and Neurosurgery
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better CSF shunts
better CSF shunt surgery
removal of CSF shunts
avoidance of CSF shunts
Chiari malformation type 2
spinal cord complications
St Christopher’s
CSF shunts
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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
St Christopher’s
Better CSF Shunts
¶ the
Shunt Design Trial
¶ Bioglide
¶ programmable valves
St Christopher’s
differential
pressure
Delta
Orbis-Sigma
Better CSF Shunt Surgery
the Endoscopic Shunt Insertion Trial
¶ ultrasound
¶ image guidance
¶ shaveless CSF shunt surgery
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St Christopher’s
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Shaveless CSF Shunt Surgery
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Horgan MA, Piatt JH Jr.
Shaving of the scalp may
increase the rate of
infection in CSF shunt
surgery. Pediatr
Neurosurg 1997;26:180184.
Winston KR. Hair and
neurosurgery.
Neurosurgery
1992;31:320-329.
St Christopher’s
Removal of CSF Shunts:
Endoscopic 3rd Ventriculostomy
If it ain’t broke, don’t fix it.
¶ E3V for CSF shunt failure
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• urgent
• “elective” for slit ventricle
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E3V for CSF shunt infection
St Christopher’s
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.
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St Christopher’s
Management of Shunt Infection
with E3V
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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.
St Christopher’s
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.
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St Christopher’s
E3V
shunts
Avoidance of CSF Shunts:
Antenatal MM Repair
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Rationale
Expected benefit – lower extremities
Observations – Chiari
Putative benefit – hydrocephalus
Risks and ethical issues
St Christopher’s
Avoidance of CSF Shunts:
the MRCT
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Vanderbilt, CHoP, UCSF
NO repairs outside trial!
Endpoint – CSF shunt insertion
• adjudication
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Secondary endpoints
“Freezing” of technology
St Christopher’s
Chiari malformation type 2
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posterior fossa too small
hindbrain developmentally abnormal
medullary dysfunction – infancy
• Compression?
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medullary dysfunction – later
• CSF shunt failue?
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syringomyelia
St Christopher’s
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Spinal Complications
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syringomyelia / syringobulbia
spinal arachnoid cyst
diastematomyelia
dermoid cyst
tethering
St Christopher’s
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Spinal Cord Tethering
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progressive neurological deficits
pain
bladder dysfunction
a diagnosis of exclusion
surgical risks and benefits
St Christopher’s