An Overview of Neurosurgery

andSpina Bifida

Joseph H Piatt, Jr, MD, FAAP

Section

of

Neurosurgery

St Christopher’s

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

St Christopher’s

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

St Christopher’s

Better CSF Shunts

¶ the Shunt Design Trial¶ Bioglide¶ programmable valves

Orbis-Sigma

differential pressure Delta

St Christopher’s

Better CSF Shunt Surgery

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

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St Christopher’s

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.

St Christopher’s

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

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.

St Christopher’s

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.

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.

shunts

E3V

St Christopher’s

Avoidance of CSF Shunts:Antenatal MM Repair

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

St Christopher’s

Avoidance of CSF Shunts:the MRCT

¶ Vanderbilt, CHoP, UCSF¶ NO repairs outside trial!¶ Endpoint – CSF shunt insertion

• adjudication¶ Secondary endpoints¶ “Freezing” of technology

St Christopher’s

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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St Christopher’s

Spinal Complications

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

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St Christopher’s

Spinal Cord Tethering

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