An Overview of Neurosurgery and Spina 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: ¶ Winston KR. Hair and neurosurgery. Neurosurgery 1992;31:

St Christopher’s Removal of CSF Shunts: Endoscopic 3 rd 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: ¶ 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: ¶ Fukuhara T. Risk factors for failure of endoscopic third ventriculostomy for obstructive hydrocephalus. Neurosurgery 2000;46:

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: ¶ 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