• Steinbok, Paul

    Titles
    Investigator, BC Children's Hospital
    Professor, Division of Neurosurgery, Department of Surgery, University of British Columbia
    Degrees / Designations
    B.Sc., MBBS, FRCSC
    Primary Area of Research
    The M.I.N.D
    Secondary Area(s) of Research
    Phone
    604-875-2094
    Fax
    604-875-3109
    Lab Phone
    Assistant
    Helen McGregor
    Assistant Phone
    604-875-2094
    Mailing Address
    BC Children's Hospital
    Room K3-159, 4480 Oak Street
    Vancouver, BC V6H 3V4
    Affiliate Websites
    Research Areas
  • Outcomes of interventions in treatment of children with spastic cerebral palsy
  • Assessment of infants with occipital plagiocephaly
  • Occult tethered cord syndrome in children
  • Multiple areas of pediatric neurosurgery - including craniosynostosis treatment, epilepsy surgery and brain tumours.
Summary
Cerebral palsy is a brain disorder that occurs around the time of birth. Many children with cerebral palsy have increased stiffness (spasticity) in their limbs that may contribute to poor motor function. The past 20 years new treatments to decrease spasticity have become available, including selective dorsal rhizotomy, intrathecal baclofen and botox injections. In selective dorsal rhizotomy, parts of the nerves that bring sensation from the lower limbs to the brain are cut using intraoperative guidance with electrical stimulation of the nerves. Intrathecal baclofen involves continuous administration of a drug (baclofen) into the spinal fluid via an implanted pump. My research has centered on evaluating outcomes in children with spastic cerebral palsy after both dorsal rhizotomy and intrathecal baclofen, and involves multiple assessments by physiotherapists, occupational therapists, orthopaedic surgeons and neurosurgeons. This research has led to a better understanding of which children with cerebral palsy could benefit most from these procedures and expected results of the interventions.
Current Projects

Review of the long-term changes in the hips and spine after selective dorsal rhizotomies
The dorsal rhizotomy procedure has the potential to affect the curvature of the spine and stability of the hips in both positive and negative ways. The goal of this study is to examine the results of X-rays of the hips and spine done many years after dorsal rhizotomies, and to compare these findings with those noted prior to surgery.

Survey of selective dorsal rhizotomies and intrathecal baclofen usage internationally
The goal of this study is to determine access to selective dorsal rhizotomy and intrathecal baclofen around the world. In addition, we wish to determine if there are differences in the way the procedures are performed in various parts of the world. This is being done as a web-based survey in association with the International Society for Pediatric Neurosurgery. The results of this survey will allow us to direct resources and educational activities toward the areas of the world most in need.

Intraobserver variability in anthropometric measurements in occipital plagiocephaly
Occipital plagiocephaly refers to an abnormal head shape, with flattening of one side of the back of the head, causing an abnormal appearance. Our research is focussed on measurement of the asymmetry of the skull using callipers, the determination of how variable these measurements are when done by more than one observer, and how the measurements relate to the cosmetic appearance. The study results will allow us to better evaluate the outcome of different types of treatment for this condition.

Occult tethered cord syndrome in children
The entity of a tethered cord syndrome secondary to a tight filum terminale is recognized as a clinically important problem in children and adults. The pathogenesis of the clinical syndrome is thought to be traction on the lower end of the spinal cord by a thickened filum terminale.  This traction on the conus leads to decreased blood flow and decreased oxidative metabolism, which may result in the clinical symptoms and signs of the tethered cord syndrome.  The clinical features of the syndrome include neurological, musculoskeletal and urological abnormalities, which are often reversed or improved by section of the filum.  The tethered cord syndrome is typically associated with a low lying conus medullaris, with the tip of the conus located below the L2 vertebral level.  The thickening of the filum and subsequent tethering of the spinal cord are thought to result from abnormal development of the filum during the retrogressive differentiation phase of secondary neurulation.  As such, other evidence of spinal dysraphism, such as midline lumbar or sacral cutaneous lesions and bony spina bifida are commonly identified in this population of patients.

More recently, the concept of a tethered cord syndrome in which the conus was not low has been proposed (Occult tethered cord syndrome or OTCS).  Children with preumed OTCS present with urinary incontinence secondary to a neurogenic bladder, similar to what may be seen in patients with the usual tethered cord syndrome, but on MRI scans the spinal cord ends in a normal position. In a number of uncontrolled case series, section of the filum terminale resulted in improvement in the clinical findings. At BCCH we compared patients with OTCS who had been operated on with similar patients who were observed and found a better outcome in the surgical group.

At the present time, the concept of the “occult tethered cord”, in which there is no evidence anatomically of a low lying conus is controversial, and the indications for section of the filum in this context are equally controversial. 

We are currently leading a multicentered a randomized controlled study to compare the regimen of symptomatic medical treatment only with a regimen of surgical section of the filum terminale plus symptomatic medical treatment in children with neurogenic urinary incontinence, as part of an “occult tethered cord syndrome."

Selected Publications

Schrader DV, Steinbok P, Connolly M.: Urgent, resective surgery for medically refractory, convulsive status epilepticus. Eur J Paediatr Neurol. 2009 Jan;13(1):10-7.

Lam FC, Irwin BJ, Poskitt KJ, Steinbok P.: Cervical spine instability following cervical laminectomies for Chiari II malformation: a retrospective cohort study. Childs Nerv Syst. 2009 Jan;25(1):71-6.

Vitali AM, Steinbok P.: Depressed skull fracture and epidural hematoma from head fixation with pins for craniotomy in children. Childs Nerv Syst. 2008 Aug;24(8):917-23; discussion 925.

Hentschel S, Steinbok P, Cochrane DD, Kestle J. Reduction of Transfusion Rates in the Surgical Correction of Sagittal Synostosis. J Neurosurgery 2002; 97:503-509.

Hader WJ, Steinbok P, Hukin J, Fryer C: Intratumoral chemotherapy with bleomycin for cystic craniopharyngioma. Pediatric Neurosurgery 2000; 33: 211-218.

Steinbok P, O'Donnell, M: Baclofen infusion for spastic cerebral palsy. Clinical Neurosurgery 1999;47:440-457.

Gul S, Steinbok P, MacLeod K: Long-term outcome after selective posterior rhizotomy in children with spastic cerebral palsy. Pediatric Neurosurgery 1999; 31; 84-95.

Loewen P, Steinbok P, Holsti L, MacKay M: Upper extremity performance and self care skill changes in children with spastic cerebral palsy following selective posterior rhizotomy. Pediatric Neurosurgery 1998; 29: 191-198.

Steinbok P, Schrag C: Complications after selective posterior rhizotomy for spasticity in children with cerebral palsy. Pediatric Neurosurgery 1998; 28:300-313.

Steinbok P, Reiner A, Beauchamp R, Armstrong RW, Cochrane DD: A randomized clinical trial to compare selective posterior rhizotomy plus physiotherapy with physiotherapy alone in children with spastic diplegic cerebral palsy. Dev Med Child Neurol 1997; 39: 178-184.

Grants
Honours & Awards
Barbados Ball Award, Barbados Ball Canada Aid - 2014
PHSA Patient & Family Centred Care Award, Honorable Mention - 2013

Richmond Cerebral Palsy Award - American Academy for Cerebral Palsy and Developmental Medicine - for best paper presented at their 1996 Annual Meeting - 1997
Outstanding Achievement by an Investigator award - BC Research Institute for Child and Family Health - 1996

Research Group Members

Cerebral palsy group
Richard Beauchamp - Orthopedic surgeon
Maureen O'Donnell - Developmental pediatrician
Diane Wickenheiser - Physiotherapist
Patricia Mortenson - Occupational therapist
Tufan Hicdonmez- Fellow
Bonnie Sawatzky - Director of Pediatric Orthopedic Research

Plagiocephaly group
Patricia Mortenson - Occupational therapist