Publications by authors named "Reinhard Zeller"

32 Publications

The Use of Halo Gravity Traction in Severe, Stiff Scoliosis.

J Pediatr Orthop 2021 Apr 8. Epub 2021 Apr 8.

Department of Orthopaedic Surgery, Hospital for Sick Children, Toronto, ON, Canada Department of Trauma and Orthopaedic Surgery, Mater Misericordiae University Hospital and Children's University Hospital, Rotunda, Dublin, Ireland.

Purpose: The correction of severe, stiff scoliosis in children is challenging. One method used to reduce the risk is preoperative halo gravity traction (HGT). In this study, the authors sought to define the efficiency and safety of HGT and characterize the chronology of the correction seen.

Method: A consecutive group of pediatric patients with severe spinal deformities was treated with HGT before definitive correction. A standard protocol with the daily addition of weight to 50% of body weight at 3 weeks was used. Traction remained in place until signs of impending neurological complication or 6 weeks, whichever was sooner.

Results: Twenty-four patients were included with a mean age of 11.8 years. The mean coronal deformity was 123 degrees, with a T1-L5 height of 234 mm. The mean duration of traction was 42 days with a mean improvement in height of 72 mm with 82% occurring over the first 3 weeks. Hundred percent of the angular and 98% of T1-L5 height correction was reached by 6 weeks.One patient showed early signs of a cranial nerve palsy prompting early surgery and 8 patients showed pin loosening, 1 of which required revision of their halo. One patient underwent a slower progression of traction because of transitory urinary disturbance. Following fusion, angular correction of the major curve was 49%.

Conclusion: HGT is a safe treatment for severe, stiff scoliosis because it can respond to early signs of impending neurological impairment. The first 3 weeks of treatment, reaching 50% of body weight as a traction force accounts for 80% of correction, with the remaining 20% in the following 2 weeks. At least 4 weeks of traction is recommended when following this protocol.
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April 2021

The use of three rods in correcting severe scoliosis.

Spine Deform 2021 Feb 10. Epub 2021 Feb 10.

Department of Orthopaedic Surgery, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1XB, Canada.

Purpose: The three-rod technique, utilising a short apical concavity rod is an option to achieve controlled correction in severe scoliosis. We describe this technique, the complications encountered, and the long-term outcomes.

Method: All paediatric patients who had at least 2 years follow-up after undergoing corrective surgery for scoliosis ≥ 100° using 3 parallel rods were included. Radiographs were assessed to evaluate the correction and clinical records examined for any loss of correction, complications, revision procedures or neuromonitoring events.

Results: Twenty-five patients met the inclusion criteria. Four underwent prior anterior fusion to prevent crankshaft phenomenon. The mean angle of the deformity was 112.0° (range 100.3-137.1). Mean maximal kyphosis was 48.8° (range 11.4-78.8°) and mean curve flexibility 4.4% (range 0-37.0%). Intraoperative traction achieved an average of 70.4% (95% CI 56.6-84.1%). Nine patients (39%) showed a reduction in MEPs during definitive surgery. All returned to within 75% of baseline by the end of surgery. All patients had normal postoperative neurology. One patient underwent removal of hardware for late infection. The mean overall Cobb correction was 55.7° (95% CI 50.2-61.2°), equating to 50.2% (95% CI 44.9-55.4%) of the mean initial deformity. Thoracic kyphosis reduced by a mean of 18.2° (95% CI 12.8-23.6°).

Conclusion: Our series suggests that three-rod constructs are able to safely and effectively achieve 50% correction of severe scoliosis.
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February 2021

Congenital Kyphosis: Progressive Correction With an Instrumented Posterior Epiphysiodesis: A Preliminary Report.

J Pediatr Orthop 2021 Mar;41(3):133-137

Department of Orthopaedic Surgery, Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada.

Purpose: Congenital kyphosis is a rare condition. In this case series we sought to identify the outcomes and complications of posterior instrumented fusion and the resultant epiphysiodesis effect in uniplanar congenital kyphosis in pediatric patients.

Method: Pediatric patients were included if treated for a uniplanar congenital kyphotic deformity treated with posterior instrumented spinal fusion between October 2006 and August 2017, with a minimum of 2 years of follow-up. Patients were excluded if a coronal deformity >10 degrees was present.

Results: Six patients met the inclusion criteria. Mean age at surgery was 3.6 years. The mean kyphotic deformity before surgery was 49.7 degrees. All patients underwent posterior instrumented fusion with autogenous iliac crest graft and a cast or brace postoperatively. One patient showed a loss of motor evoked potential on prone positioning which returned to normal on supine positioning. No patient showed any postoperative neurological deficits. One patient was diagnosed with a wound infection which was successfully treated with oral antibiotics.By a follow-up of 5.4 years (range, 2.2 to 10.9 y) there was no failure of instrumentation. An epiphysiodesis effect (a difference of ≥5 degrees in the kyphotic deformity measured between the immediate postoperative and final follow-up lateral whole spine XR) of 16.2 degrees (range, 7.2 to 30.9 degrees) was seen in 5 patients. The mean annual epiphysiodesis effect was 2.7 degrees (95% confidence interval, 1.4-4.1 degrees). No kyphosis proximal to the instrumentation was observed for the duration of follow-up.

Conclusion: Posterior instrumented fusion and epiphysiodesis is safe and effective. The epiphysiodesis effect occurs in 5/6 of cases, and our data suggests that the procedure is associated with an acceptable blood loss and a low incidence of neurological complications.
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March 2021

What is the Optimal Surgical Method for Achieving Correction and Avoiding Neurological Complications in Pediatric High-grade Spondylolisthesis?

J Pediatr Orthop 2021 Mar;41(3):e217-e225

Department of Orthopaedic Surgery, Hospital for Sick Children, Toronto, ON, Canada.

Background: Controversy persists in the treatment of high-grade spondylolisthesis (HGS). Surgery is recommended in patients with intrusive symptoms and evidence debates the competing strategies. This study compares the radiologic outcomes and postoperative complications at a minimum of 2 years follow-up for patients with HGS treated with instrumented fusion with partial reduction (IFIS) with those treated with reduction, decompression, and instrumented fusion (RIF). We hypothesize that IFIS leads to a lower rate of complication and revision surgery than RIF.

Methods: A retrospective comparative methodology was used to analyze consecutive HGS treated surgically between 2006 and 2017. Patients diagnosed with ≥grade 3 spondylolisthesis treated with arthrodesis before the age of 18 years with a minimum of 2 years follow-up were included. Patients were excluded if surgery did not aim to achieve arthrodesis or was a revision procedure. Cases were identified through departmental and neurophysiological records.

Results: Thirty patients met the inclusion criteria. Mean follow-up was 4 years. Ten patients underwent IFIS and the remaining 20 underwent RIF. The 2 groups showed no difference in demographics, grade of slip, deformity or presenting symptoms. Of 10 treated with IFIS, the SA reduced by a mean of 10 degrees and C7 sagittal vertical line changed by 31 mm. In the RIF cohort, SA reduced by 16 degrees and C7 sagittal vertical line reduced by 26 mm. PT was unchanged in both groups. In IFIS cohort, 2 patients showed postoperative weakness, resolved by 2 years. None required revision surgery. In the RIF group, 4 sustained dural tears and 1 a laminar fracture, 7 showed postoperative weakness or dysaesthesia, 3 of which had not resolved by 2 years. Eight patients underwent unplanned further surgery, 3 for pseudarthrosis.

Conclusions: RIF and IFIS show similar radiologic outcomes. RIF shows a higher rate of unplanned return to surgery, pseudarthrosis and persisting neurological changes.

Level Of Evidence: Level III-retrospective comparative study.
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March 2021

Impact of Various Weights in the Intraoperative Skull-Skeletal Traction on Correction of Adolescent Idiopathic Scoliosis.

Global Spine J 2020 Dec 21;10(8):1015-1021. Epub 2019 Oct 21.

7979The Hospital for Sick Children, Toronto, Ontario, Canada.

Study Design: A retrospective study.

Objectives: Intraoperative skull-skeletal traction (ISST) facilitates the surgical scoliosis correction, but it is also associated with neurological risk. The objective of the present study was to investigate the impact of various traction weights on neurophysiological change and curve correction in surgery for adolescent idiopathic scoliosis (AIS).

Methods: A retrospective review of a consecutive series of posterior spinal fusions for AIS patients undergoing corrections with the use of ISST by 2 surgeons in one institution was performed. Intraoperative prone, post-traction radiographs were performed on all cases. The cases were divided into 2 groups, high and low traction weights, based on whether the weight used was ≥35% or <35% of body weight. The frequency of neurophysiological changes and the curve correction were compared between the 2 groups.

Results: The intraoperative correction magnitudes by ISST were significantly larger in the high ISST group than in the low ISST group (35° vs 26°, < .001). Changes in motor-evoked potential (MEP) were more frequently observed in the high ISST group (47% vs 26%, = .049). A multivariate analysis showed that high ISST was associated with 3 times higher risk of MEP change (95% confidence interval = 1.1-8.0, = .03) and higher final postoperative correction rates (68% vs 60%, = .001).

Conclusions: The high ISST for AIS was associated with increased intraoperative and ultimate curve corrections, and potentially facilitated better final correction. However, the high weight group was associated with an increased frequency of intraoperative MEP changes.
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December 2020

Adolescent idiopathic scoliosis detection and referral trends: impact treatment options.

Spine Deform 2021 Jan 11;9(1):75-84. Epub 2020 Aug 11.

Division of Rehabilitation Services, Hospital for Sick Children, Toronto, ON, Canada.

Study Design: Retrospective cross-sectional study.

Objective: To analyze the patient demographic referred for scoliosis to the Hospital for Sick Children to determine the proportion of patients suitable for brace treatment, as per the Scoliosis Research Society guidelines. There is level 1 evidence that bracing in adolescent idiopathic scoliosis (AIS) decreases the risk of curve progression and need for surgery, but optimal brace treatment requires early curve detection.

Methods: We performed a retrospective review of 618 consecutive patients who underwent initial assessment in our Spine Clinic between Jan. 1 and Dec. 31, 2014. We included children 10-18 years, with scoliosis greater than 10°, excluding those diagnosed with non-idiopathic curves. Primary outcomes were Cobb angle, menarchal status, and Risser score. We analyzed the effect of specific referral variables (family history, the person who first noticed the curve, and geographic location of residence) on presenting curve magnitude.

Results: During the study period, 335 children met the inclusion criteria, with an average age of 14.1 ± 1.8 years and a mean Cobb angle of 36.8 ± 14.5°. Brace treatment was indicated in 17% of patients; 18% had curves beyond optimal curve range for bracing (> 40°), and 55% were skeletally mature, therefore not brace candidates. The majority of curves (54%) were first detected by the patient or family member and averaged 7° more than curves first detected by a physician. A family history of scoliosis made no difference to curve magnitude, nor did geographic location of residence.

Conclusion: The majority of AIS patients present too late for effective management with bracing.

Level Of Evidence: III.
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January 2021

Spinal Casting for the Treatment of Severe Early Onset Scoliosis: Utilization of a Nonsurgical Health Care Provider for Cast Application.

J Pediatr Orthop 2020 Oct;40(9):e805-e810

Hospital for Sick Children.

Background: Spinal casting with the aim of delaying surgical intervention is a widely accepted but resource intensive conservative management strategy used by spine surgeons to treat severe early onset scoliosis. Opting to use a nonsurgical health care provider for cast application may be an effective use of human resources if the quality of care is not compromised. This study aimed to evaluate the outcomes of spinal cast treatment utilizing a nonsurgical health care provider for cast application.

Methods: This is a chart and radiographic review of all 30 patients that initiated spinal cast treatment between 2007 and 2018 and have at least 2-year follow-up. A spine surgeon applied all casts before July 14; a physical therapist (PT) applied all subsequent casts. Comparative analyses were performed for baseline data and the amount of correction achieved in initial cast. Complications and delay time to surgery were recorded.

Results: The PT cast 16 patients (12 female, 10 idiopathic, 64 casts total), average age 3.8±1.4 years and mean major curve of 63±18 degrees. Similarly the spine surgeon cast 14 patients (11 female, 8 idiopathic, 53 casts total), average age 4.4±1.7 years (P=0.30) and mean major curve of 63±11 degrees (P=0.93). In the initial cast the PT obtained 55%±13% (28±12 degrees) correction and the surgeon 44%±12% (36±12 degrees, P=0.09). The average number of casts was similar (4.0 vs. 3.8, P=0.7). The PT had 1 patient develop superior mesenteric artery syndrome, which resolved with cast removal, and the surgeon had 2 patients require extra care related to skin breakdown. With average follow-up of 4.15 years (range, 2.0 to 5.8 y) 11/14 PT patients demonstrate curve improvement compared with their initial presentation and 2 patients have undergone surgical intervention (3.2 and 4.3 y after first cast). The surgeon's average delay time to surgery was 4.3 years (range, 1.8 to 8.7 y after first cast, n=10).

Conclusions: The results of this study support our current model of care utilizing a nonsurgical health care practitioner for spinal cast application.

Level Of Evidence: Level III-retrospective comparative.
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October 2020

Postoperative Distal Coronal Decompensation After Fusion to L3 for Adolescent Idiopathic Scoliosis Is Affected by Sagittal Pelvic Parameters.

Spine (Phila Pa 1976) 2020 Nov;45(21):E1416-E1420

Division of Orthopaedics, Hospital for Sick Children, Toronto, Ontario, Canada.

Study Design: Retrospective study.

Objective: To identify on early postoperative radiographs the risk factors for late distal decompensation in adolescent idiopathic scoliosis (AIS) patients undergoing posterior fusion surgery to L3.

Summary Of Background Data: Sparing distal fusion levels in AIS surgery is considered beneficial for postoperative mobility and outcomes; nonetheless, late distal decompensation is of concern. L3 is often advocated as lower instrumented vertebra in posterior fusion, but progressive angulation of the L3/4 disc is commonly observed.

Methods: A retrospective analysis was conducted on 78 AIS patients who underwent posterior fusion to L3 from 2007 to 2014. Patients' demographic data, early and 2-year postoperative standing radiographs by biplanar imaging system were investigated. Late decompensation was defined as progressive increase of L3-4 disc wedging angle at 2-year follow-up. Coronal, sagittal, and rotational radiographic parameters were compared between those with and without decompensation. SRS-30 scores were reviewed.

Results: Mean age was 14.5-year, and fusion levels averaged 12.0 (range: 6-15); 43 out of 78 patients (55%) experienced progressive L3-4 disc wedging, with 6 showing wedging >5°. L3 translation from the central sacral vertical line (13.9 vs. 11.1 mm, P = 0.13) and increased pelvic tilt (13.3° vs. 8.6°, P = 0.06) on the early postoperative radiograph were associated with increased L3-4 disc wedging. Multivariate analysis revealed that larger pelvic tilt was a significant risk factor for decompensation (odds ratio = 1.1 per 1°, 95% confidence interval: 1.0-1.1, P = 0.04). SRS-30 scores did not differ significantly between the two groups (4.0 vs. 4.1, P = 0.44).

Conclusions: Pelvic retroversion and increased translation of L3 from the central sacral line on the early postoperative radiograph were associated with late L3-4 disc wedging in AIS fusions to L3. Careful surgical planning and correction of sagittal alignment are imperative to ensure the long-term outcomes.

Level Of Evidence: 4.
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November 2020

Klippel Feil Syndrome: Clinical Phenotypes Associated With Surgical Treatment.

Spine (Phila Pa 1976) 2020 Jun;45(11):718-726

Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto, Ontario, Canada.

Study Design: Retrospective study.

Objective: To define distinct Klippel-Feil syndrome (KFS) patient phenotypes that are associated with the need for surgical intervention.

Summary Of Background Data: KFS is characterized by the congenital fusion of cervical vertebrae; however, patients often present with a variety of other spinal and extraspinal anomalies suggesting this syndrome encompasses a heterogeneous patient population. Moreover, it remains unclear how the abnormalities seen in KFS correlate to neurological outcomes and the need for surgical intervention.

Methods: Principal component (PC) analysis was performed on 132 KFS patients treated at a large pediatric hospital between 1981 and 2018. Thirty-five variables pertaining to patient/disease-related factors were examined. Significant PCs were included as independent variables in multivariable logistic regression models designed to test associations with three primary outcomes: cervical spine surgery, thoracolumbar/sacral spine surgery, and cranial surgery.

Results: Fourteen significant PCs accounting for 70% of the variance were identified. Five components, representing four distinct phenotypes, were significantly associated with surgical intervention. The first group consisted of predominantly subaxial cervical spine fusions, thoracic spine abnormalities and was associated with thoracolumbar/sacral spine surgery. The second group was largely represented by axial cervical spine anomalies and had high association with cervical subluxation and cervical spine surgery. A third group, heavily represented by Chiari malformation, was associated with cranial surgery. Lastly, a fourth group was defined by thoracic vertebral anomalies and associations with sacral agenesis and scoliosis. This phenotype was associated with thoracolumbar/sacral spine surgery.

Conclusion: This is the first data-driven analysis designed to relate KFS patient phenotypes to surgical intervention and provides important insight that may inform targeted follow-up regimens and surgical decision-making.

Level Of Evidence: 3.
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June 2020

Responding to Intraoperative Neuromonitoring Changes During Pediatric Coronal Spinal Deformity Surgery.

Global Spine J 2019 May 8;9(1 Suppl):15S-21S. Epub 2019 May 8.

AOSpine Knowledge Forum Deformity, Davos, Switzerland.

Study Design: Retrospective case study on prospectively collected data.

Objectives: The purpose of this explorative study was: 1) to determine if patterns of spinal cord injury could be detected through intra-operative neuromonitoring (IONM) changes in pediatric patients undergoing spinal deformity corrections, 2) to identify if perfusion based or direct trauma causes of IONM changes could be distinguished, 3) to observe the effects of the interventions performed in response to these events, and 4) to attempt to identify different treatment algorithms for the different causes of IONM alerts.

Methods: Prospectively collected neuromonitoring data in pre-established forms on consecutive pediatric patients undergoing coronal spinal deformity surgery at a single center was reviewed. Real-time data was collected on IONM alerts with >50% loss in signal. Patients with alerts were divided into 2 groups: unilateral changes (direct cord trauma), and bilateral MEP changes (cord perfusion deficits).

Results: A total of 97 pediatric patients involving 71 females and 26 males with a mean age of 14.9 (11-18) years were included in this study. There were 39 alerts in 27 patients (27.8% overall incidence). All bilateral changes responded to a combination of transfusion, increasing blood pressure, and rod removal. Unilateral changes as a result of direct trauma, mainly during laminotomies for osteotomies, improved with removal of the causative agent. Following corrective actions in response to the alerts, all cases were completed as planned. Signal returned to near baseline in 20/27 patients at closure, with no new neurological deficits in this series.

Conclusion: A high incidence of alerts occurred in this series of cases. Dividing IONM changes into perfusion-based vs direct trauma directed treatment to the offending cause, allowing for safe corrections of the deformities. Patients did not need to recover IONM signal to baseline to have a normal neurological examination.
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May 2019

Staged insertion of growing rods in severe scoliosis.

Eur Spine J 2018 09 23;27(9):2203-2212. Epub 2018 Mar 23.

Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.

Purpose: The objective of this retrospective chart and radiographic review was to present the clinical outcomes and complication rate of a staged approach to modern dual growing-rod (GR) surgery when treating children with severe early onset scoliosis.

Methods: Fifteen patients received a 6-mm dual GR system. During Stage 1, pairs of end vertebra were exposed in a subperiosteal fashion, instrumented, grafted, and fused. Stage 2 was performed, on average, 5 months later (range 8-35 weeks) and the fused foundations were connected with two growing rods under skull-femoral traction. Clinical and operative notes were reviewed and all complications were recorded. Radiographic measurements were assessed at pre-index, with intraoperative traction during Stage 1, post-Stage 2 and at most recent follow-up. Statistical analyses were performed to evaluate change in scoliosis and kyphosis.

Results: At initial surgery, the average age was 8.17 ± 1.5 years. The mean Cobb angle was 88.1° ± 14.0°, corrected to 60.3° ± 8.7° (p < 0.001) with intraoperative traction in Stage 1, preserved after Stage 2 instrumentation (59.5° ± 9.6°, p = 0.69), and maintained with subsequent lengthenings (60.6° ± 12.8°, p = 0.73). Hyperkyphosis (11/15 patients) improved from 70.8° ± 15.7° to 46.6° ± 9.7° (p < 0.001). At minimum 2-year follow-up (range 24-80 months, mean 49.5), the complication rate was 14 (0.93 complications/patient), including 6 rod breakages, 6 superficial infections, and 2 deep infections. No anchor migration or pull-out was noted. Seven patients have undergone definitive posterior spinal fusion.

Conclusions: Staged insertion of dual GR systems permits strong distraction, with acceptable correction of severe deformities and minimal complications. These slides can be retrieved under Electronic Supplementary Material.
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September 2018

Three-dimensional EOS Analysis of Apical Vertebral Rotation in Adolescent Idiopathic Scoliosis.

J Pediatr Orthop 2017 Dec;37(8):e543-e547

Division of Orthopaedic Surgery, The Hospital for Sick Children, Toronto, ON, Canada.

Background: Apical vertebral rotation (AVR) is increasingly recognized as one of the important radiographic parameters in adolescent idiopathic scoliosis (AIS). EOS enables us to precisely measure AVR by 3-dimensional reconstruction. The objective of the present study was to describe the postoperative correction and the long-term follow-up of AVR in posterior spinal fusion with direct vertebral rotation and elucidate the factors that affected the correction.

Methods: We retrospectively reviewed 153 consecutive posterior spinal fusion surgeries for AIS performed between 2009 and 2012. Among them, 55 patients who fulfilled the study inclusion criteria with complete preoperative, immediate postoperative, and last follow-up (>1 y) EOS images were included in the present study. EOS 3-dimentional reconstructions were undertaken for each patient. Postoperative AVR correction and the loss of correction were calculated.

Results: Preoperative AVR of the major curve averaged 19 degrees (SD=7 degrees), and AVR on immediate postoperative images averaged 9 degrees (SD=6 degrees, P<0.001). AVR at final follow-up averaged 11 degrees (SD=6 degrees, P=0.06). Postoperative correction was larger in all-screw construct than in hybrid construct (55% vs. 36%, P=0.03).

Conclusions: The present study is the first study to measure AVR in a large population of AIS patients using EOS 3-dimensional reconstruction. We report the correction magnitude was significantly affected by the construct.

Level Of Evidence: Level IV-therapeutic study (case series).
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December 2017

The "slide technique": an improvement on the "funnel technique" for safe pedicle screw placement in the thoracic spine.

Eur Spine J 2014 Jul 14;23 Suppl 4:S452-6. Epub 2014 May 14.

Department of Pediatric Orthopaedics, Université Pierre et Marie Curie Paris 6, Armand Trousseau Hospital, 26 avenue du Dr Arnold Netter, 75571, Paris Cedex 12, France,

Study Design: Technical note.

Objectives: To report and describe a new free-hand technique for pedicle screw placement in the thoracic spine especially in severe deformities. Because of distortion of anatomic landmarks scoliosis, this free-hand placement technique based on pedicle access through the decancelled transverse process is a safe procedure.

Methods: Transverse process is widely exposed and its posterior cortex is decorticated. The cancellous bone content of the transverse process is completely removed using a small curette. Bone wax is applied to avoid local bleeding and then the decancelled transverse process is inspected. The entry of the pedicle is then easily identified by the presence of remaining cancellous bone. A pedicular probe is then inserted and gently advanced. During pedicle probe insertion, the cortex of the anterior aspect of transverse process and the lateral margin of the pedicle act as a "slide" to permit safe insertion of the instrument.

Results: In our experience, no patient required additional procedures for screw revision, and no neurologic deficit occurred stemming from malpositioning of pedicle screws. The key point of the "slide technique" is to use the cortex of the anterior aspect of transverse process and the lateral margin of the pedicle as a "slide" to permit correct probe positioning during pedicle probe insertion.

Conclusions: This technique is very close to the "funnel technique". The "funnel" and then the "slide" technique are mostly useful in complex spinal deformities as in neuromuscular patients. The "slide technique" is a safe, effective and cost-effective technique for pedicle screw placement in the thoracic spine especially in severe deformities.

Level Of Evidence: IV.
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July 2014

Three dimensional analysis of brace biomechanical efficacy for patients with AIS.

Eur Spine J 2013 Nov 20;22(11):2445-8. Epub 2013 Jul 20.

Pediatric Orthopedic Surgery Division, Hospital for Sick Children, 555 University Av, Toronto, ON, M5G 1X8, Canada,

Purpose: Corrective three dimensional (3D) effect of different braces is debatable. We evaluated differences in in-brace radiographic correction comparing a custom thoracic-lumbo-sacral-orthosis (TLSO) (T) brace to a Chêneau type TLSO (C) brace using 3D EOS reconstruction technology. Our primary research question was the 3D effect of brace on the spine and in particularly the apical vertebra rotation (AVR).

Methods: This was a retrospective comparative analysis of patients with adolescent idiopathic scoliosis who had orthogonal AP and lateral X-rays with and without brace. A 3D image of the spine was reconstructed. Coronal, sagittal and axial spine parameters were measured before bracing and then on the first post-brace X-ray. Brace efficacy in controlling coronal, sagittal and axial parameters was evaluated.

Results: Eighteen patients treated with the C brace and ten patients treated with the T brace were included. No difference was found regarding patients' age, gender, magnitude of Cobb angle, sagittal parameters or AVR at inclusion. Following bracing, AVR was significantly reduced by the C brace compared to the T brace [average correction of 8.2° vs. 4.9° (P = 0.02)]. Coronal and sagittal correction did not differ significantly between the two groups.

Conclusions: By utilizing a novel 3D reconstruction technology, we were able to demonstrate that braces differ in their immediate effects on the spine. Although clinical relevance should be evaluated in a future trial we feel that the ability to measure treatment effects in 3D, and especially the transverse plane, is an important tool when evaluating different treatments.
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November 2013

Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery.

J Pediatr Orthop 2013 Jul-Aug;33(5):471-8

Department of Orthopaedic Surgery, Columbia University, New York, NY 10032, USA.

Background: Perioperative surgical site infection (SSI) after pediatric spine fusion is a recognized complication with rates between 0.5% and 1.6% in adolescent idiopathic scoliosis and up to 22% in "high risk" patients. Significant variation in the approach to infection prophylaxis has been well documented. The purpose of this initiative is to develop a consensus-based "Best Practice" Guideline (BPG), informed by both the available evidence in the literature and expert opinion, for high-risk pediatric patients undergoing spine fusion. For the purpose of this effort, high risk was defined as anything other than a primary fusion in a patient with idiopathic scoliosis without significant comorbidities. The ultimate goal of this initiative is to decrease the wide variability in SSI prevention strategies in this area, ultimately leading to improved patient outcomes and reduced health care costs.

Methods: An expert panel composed of 20 pediatric spine surgeons and 3 infectious disease specialists from North America, selected for their extensive experience in the field of pediatric spine surgery, was developed. Using the Delphi process and iterative rounds using a nominal group technique, participants in this panel were as follows: (1) surveyed for current practices; (2) presented with a detailed systematic review of the relevant literature; (3) given the opportunity to voice opinion collectively; and (4) asked to vote regarding preferences privately. Round 1 was conducted using an electronic survey. Initial results were compiled and discussed face-to-face. Round 2 was conducted using the Audience Response System, allowing participants to vote for (strongly support or support) or against inclusion of each intervention. Agreement >80% was considered consensus. Interventions without consensus were discussed and revised, if feasible. Repeat voting for consensus was performed.

Results: Consensus was reached to support 14 SSI prevention strategies and all participants agreed to implement the BPG in their practices. All agreed to participate in further studies assessing implementation and effectiveness of the BPG. The final consensus driven BPG for high-risk pediatric spine surgery patients includes: (1) patients should have a chlorhexidine skin wash the night before surgery; (2) patients should have preoperative urine cultures obtained; (3) patients should receive a preoperative Patient Education Sheet; (4) patients should have a preoperative nutritional assessment; (5) if removing hair, clipping is preferred to shaving; (6) patients should receive perioperative intravenous cefazolin; (7) patients should receive perioperative intravenous prophylaxis for gram-negative bacilli; (8) adherence to perioperative antimicrobial regimens should be monitored; (9) operating room access should be limited during scoliosis surgery (whenever practical); (10) UV lights need NOT be used in the operating room; (11) patients should have intraoperative wound irrigation; (12) vancomycin powder should be used in the bone graft and/or the surgical site; (13) impervious dressings are preferred postoperatively; (14) postoperative dressing changes should be minimized before discharge to the extent possible.

Conclusions: In conclusion, we present a consensus-based BPG consisting of 14 recommendations for the prevention of SSIs after spine surgery in high-risk pediatric patients. This can serve as a tool to reduce the variability in practice in this area and help guide research priorities in the future. Pending such data, it is the unsubstantiated opinion of the authors of the current paper that adherence to recommendations in the BPG will not only decrease variability in practice but also result in fewer SSI in high-risk children undergoing spinal fusion.

Level Of Evidence: Not applicable.
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February 2014

Three-dimensional imaging of the spine using the EOS system: is it reliable? A comparative study using computed tomography imaging.

J Pediatr Orthop B 2013 Sep;22(5):409-12

Department of Orthopedics, Odense University Hospital, Odense C, Denmark.

The aim of this study was to evaluate the precision of three-dimensional geometry compared with computed tomography (CT) images. This retrospective study included patients who had undergone both imaging of the spine using the EOS imaging system and CT scanning of the spine. The apical vertebral orientation was also measured using the EOS imaging system and by CT. Other measures such as the Cobb angle and apical vertebral rotation and translation were used as the control variables to evaluate the potential discrepancy between the standing position in EOS imaging and the supine position in CT scanning. The apical vertebral orientations were 8.7° for the first measurement and 8.4° for the second measurement made by the first author, and 10.3° for the measurement made by the second author. The average of these measurements was 9.3° compared with 6.6° (P=0.65) obtained on CT scanning. The precision of EOS-based measurements of vertebral rotation has never been tested in clinical practice. Although it has limitations, this study suggests that the results obtained using EOS are comparable to those obtained on CT.
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September 2013

Medical and psychosocial considerations in rehabilitation care of childhood neuromuscular diseases.

Handb Clin Neurol 2013 ;113:1491-5

Neurology Service, CHU Sainte-Justine and Department of Pediatrics, Université de Montréal, Montréal, Canada. Electronic address:

Most neuromuscular diseases (NMD) are rare inherited conditions presenting a relentlessly progressive course. Some NMD are associated with intellectual impairment and many are complicated by musculoskeletal, cardiac, or respiratory problems. Rehabilitation care of NMD is thus best provided by a team of medical specialists and health professionals working closely together in an interdisciplinary fashion. Rehabilitation for patients with NMD should include physical evaluation but also assessment of their ability to manage their daily activities or work and their need for assistive devices. With appropriate physical, psychological, and social support, those patients can maintain a good quality of life and an adequate level of well-being. Surgical management of musculoskeletal problems in children related to neuromuscular disorders remains a highly controversial subject. This review is based mainly on the author's personal experience with conservative and periopeporative rehabilitation care for neuromuscular diseases. A multidisciplinary approach is advocated. The importance of proactive respiratory care is emphasized. Rehabilitation after surgical treatment of spinal deformities is analyzed outlining the particular aspects in spinal muscular atrophy, Duchenne's muscular dystrophy, congenital muscular dystrophy, and congenital myopathies. A disease- specific approach is recommended as the wide variety of different neuromuscular pathologies requires careful planning of treatment strategies best managed in a team approach.
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March 2014

Towards the development of a haptic simulator of surgical gestures in orthopaedic spine surgery.

Stud Health Technol Inform 2013 ;184:254-60

Institute of Biomaterials and Biomedical Engineering, University of Toronto, Canada.

The objective of the present work is to develop a haptic system that will permit the demonstration of a proof of concept for the simulation of haptic effects typical of pediatric spine surgery. The first phase of this project focused on the development of a custom haptic system, and was followed by evaluation with experienced orthopedic surgeons. Initial findings demonstrate promise of this approach for further development into an advanced haptic system.
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July 2013

Progressive anterior vertebral bars: a study of 16 cases.

Spine (Phila Pa 1976) 2011 Mar;36(6):E423-8

Department of Pediatric Orthopaedic Surgery, Timone Children Hospital, University of Marseille, Marseille, France.

Study Design: In a retrospective study, we report on 16 patients with congenital kyphosis due to progressively ossifying anterior unsegmented bars.

Objective: To specify the therapeutic strategy in such malformation.

Summary Of Background Data: Congenital kyphosis due to progressively ossifying anterior unsegmented bars is a particular and uncommon entity of congenital kyphosis. Progressive anterior vertebral bars result in slowly progressive kyphosis, which rarely lead to neurological compromise. Sagittal equilibrium of the spine is maintained by compensatory curves adjacent to the primary curve.

Methods: All patients had clinical assessment and successive measurements of segmental angle made on a standing lateral radiograph of the spine. Magnetic resonance imaging was obtained in four patients. Six patients were just observed. A brace was used in three. And seven were treated surgically.

Results: The compensatory capacity of the spine is reduced in lumbar area where there are few discs spaces below the bar. Presentation therefore tends to be at an earlier age and pain is more common.Magnetic resonance imaging enables definition of the structures immediately posterior to the bar and provides information regarding the integrity of the intervertebral disc.

Conclusion: There is usually no need for surgery in thoracic and thoracolumbar localization. In lumbar localization, if the diagnosis is done early in childhood and if magnetic resonance imaging shows beyond the anterior ossification a normal T2-weighted magnetic resonance imaging disc space signal, desepiphysiodesis bar resection and cement interposition is mandatory. If the disc magnetic resonance imaging signal is abnormal or if the disc space is totally ossified, we propose a surgical correction of the kyphosis. For such cases a posterior wedge osteotomy seems to be the more suitable procedure.
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March 2011

Short-term morbidity and mortality associated with correction of thoracolumbar fixed sagittal plane deformity: a report from the Scoliosis Research Society Morbidity and Mortality Committee.

Spine (Phila Pa 1976) 2011 May;36(12):958-64

Department of Neurosurgery, University of Virginia Medical Center, Charlottesville, VA, USA.

Study Design: Retrospective review.

Objective: Our objective was to assess the short-term complication rate in patients undergoing treatment of thoracolumbar fixed sagittal plane deformity (FSPD).

Summary Of Background Data: The reported morbidity and mortality for the surgical treatment of thoracolumbar FSPD is varied and based on studies with small sample sizes. Further studies are needed to better assess FSPD complication rate, and the factors that influence it.

Methods: The Scoliosis Research Society (SRS) Morbidity and Mortality Database was queried to identify cases of thoracolumbar FSPD from 2004 to 2007. Complications were analyzed based on correction technique, surgical approach, surgeon experience (SRS membership status used as a surrogate), patient age, and history of prior surgery.

Results: Five hundred and seventy-eight cases of FSPD were identified. Osteotomies were performed in 402 cases (70%), including 215 pedicle subtraction osteotomies (PSO), 135 Smith-Petersen osteotomies (SPO), 19 anterior discectomy with corpectomy procedures (ADC), 18 vertebral column resections (VCR), and 15 unspecified osteotomies. There were 170 complications (29.4%) in 132 patients. There were three deaths (0.5%). The most common complications were durotomy (5.9%), wound infection (3.8%), new neurologic deficit (3.8%), implant failure (1.7%), wound hematoma (1.6%), epidural hematoma (1.4%), and pulmonary embolism (1.0%). Procedures including an osteotomy had a higher complication rate (34.8%) than cases not including an osteotomy (17.0%, P < 0.001), and this remained significant after adjusting for the effects of patient age, surgeon experience, and history of prior surgery (P = 0.003, odds ratio = 2.070, 95% CI = 1.291-3.321). Not significantly associated with complication rate were patient age (P = 0.68), surgeon experience (P = 0.18), and history of prior surgery (P = 0.10). Complication rates were progressively higher from no osteotomy (17.0%), to SPO (28.1%), to PSO (39.1%), to VCR (61.1%).

Conclusion: The short-term complication rate for treatment of FSPD is 29.4%. The complication rate was significantly higher in patients undergoing osteotomies, and more aggressive osteotomies were associated with progressively higher complication rates.
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May 2011

Incidence of unintended durotomy in spine surgery based on 108,478 cases.

Neurosurgery 2011 Jan;68(1):117-23; discussion 123-4

University of Virginia Medical Center, Charlottesville, Virginia 22908, USA.

Background: Unintended durotomy is a common complication of spinal surgery. However, the incidences reported in the literature vary widely and are based primarily on relatively small case numbers from a single surgeon or institution.

Objective: To provide spine surgeons with a reliable incidence of unintended durotomy in spinal surgery and to assess various factors that may influence the risk of durotomy.

Methods: We assessed 108,478 surgical cases prospectively submitted by members of the Scoliosis Research Society to a deidentified database from 2004 to 2007.

Results: Unintended durotomy occurred in 1.6% (1745 of 108 478) of all cases. The incidence of unintended durotomy ranged from 1.1% to 1.9% on the basis of preoperative diagnosis, with the highest incidence among patients treated for kyphosis (1.9%) or spondylolisthesis (1.9%) and the lowest incidence among patients treated for scoliosis (1.1%). The most common indication for spine surgery was degenerative spinal disorder, and among these patients, there was a lower incidence of durotomy for cervical (1.0%) vs thoracic (2.2%; P = .01) or lumbar (2.1%, P < .001) cases. Scoliosis procedures were further characterized by etiology, with the highest incidence of durotomy in the degenerative subgroup (2.2% vs 1.1%; P < .001). Durotomy was more common in revision compared with primary surgery (2.2% vs 1.5%; P < .001) and was significantly more common among elderly (> 80 years of age) patients (2.2% vs 1.6%; P = .006). There was a significant association between unintended durotomy and development of a new neurological deficit (P < .001).

Conclusion: Unintended durotomy occurred in at least 1.6% of spinal surgeries, even among experienced surgeons. Our data provide general benchmarks of durotomy rates and serve as a basis for ongoing efforts to improve safety of care.
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January 2011

Consensus statement on standard of care for congenital muscular dystrophies.

J Child Neurol 2010 Dec 15;25(12):1559-81. Epub 2010 Nov 15.

Stanford University School of Medicine, Stanford, California, USA.

Congenital muscular dystrophies are a group of rare neuromuscular disorders with a wide spectrum of clinical phenotypes. Recent advances in understanding the molecular pathogenesis of congenital muscular dystrophy have enabled better diagnosis. However, medical care for patients with congenital muscular dystrophy remains very diverse. Advances in many areas of medical technology have not been adopted in clinical practice. The International Standard of Care Committee for Congenital Muscular Dystrophy was established to identify current care issues, review literature for evidence-based practice, and achieve consensus on care recommendations in 7 areas: diagnosis, neurology, pulmonology, orthopedics/rehabilitation, gastroenterology/ nutrition/speech/oral care, cardiology, and palliative care. To achieve consensus on the care recommendations, 2 separate online surveys were conducted to poll opinions from experts in the field and from congenital muscular dystrophy families. The final consensus was achieved in a 3-day workshop conducted in Brussels, Belgium, in November 2009. This consensus statement describes the care recommendations from this committee.
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December 2010

Morbidity and mortality in the surgical treatment of 10,242 adults with spondylolisthesis.

J Neurosurg Spine 2010 Nov;13(5):589-93

Department of Neurosurgery, University of Maryland Medical Center, Baltimore, Maryland, USA.

Object: This is a retrospective review of 10,242 adults with degenerative spondylolisthesis (DS) and isthmic spondylolisthesis (IS) from the morbidity and mortality (M&M) index of the Scoliosis Research Society (SRS). This database was reviewed to assess complication incidence, and to identify factors that were associated with increased complication rates.

Methods: The SRS M&M database was queried to identify cases of DS and IS treated between 2004 and 2007. Complications were identified and analyzed based on age, surgical approach, spondylolisthesis type/grade, and history of previous surgery. Age was stratified into 2 categories: > 65 years and ≤ 65 years. Surgical approach was stratified into the following categories: decompression without fusion, anterior, anterior/posterior, posterior without instrumentation, posterior with instrumentation, and interbody fusion. Spondylolisthesis grades were divided into low-grade (Meyerding I and II) versus high-grade (Meyerding III, IV, and V) groups. Both univariate and multivariate analyses were performed.

Results: In the 10,242 cases of DS and IS reported, there were 945 complications (9.2%) in 813 patients (7.9%). The most common complications were dural tears, wound infections, implant complications, and neurological complications (range 0.7%-2.1%). The mortality rate was 0.1%. Diagnosis of DS had a significantly higher complication rate (8.5%) when compared with IS (6.6%; p = 0.002). High-grade spondylolisthesis correlated strongly with a higher complication rate (22.9% vs 8.3%, p < 0.0001). Age > 65 years was associated with a significantly higher complication rate (p = 0.02). History of previous surgery and surgical approach were not significantly associated with higher complication rates. On multivariate analysis, only the grade of spondylolisthesis (low vs high) was in the final best-fit model of factors associated with the occurrence of complications (p < 0.0001).

Conclusions: The rate of total complications for treatment of DS and IS in this series was 9.2%. The total percentage of patients with complications was 7.9%. On univariate analysis, the complication rate was significantly higher in patients with high-grade spondylolisthesis, a diagnosis of DS, and in older patients. Surgical approach and history of previous surgery were not significantly correlated with increased complication rates. On multivariate analysis, only the grade of spondylolisthesis was significantly associated with the occurrence of complications.
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November 2010

Morbidity and mortality in the surgical treatment of 10,329 adults with degenerative lumbar stenosis.

J Neurosurg Spine 2010 May;12(5):443-6

Department of Neurosurgery, University of Virginia Health System, Charlottesville, Virginia 22908, USA.

Object: The purpose of this study was to evaluate the prospectively collected Scoliosis Research Society (SRS) database to assess the incidences of morbidity and mortality (M&M) in the operative treatment of degenerative lumbar stenosis, one of the most common procedures performed by spine surgeons.

Methods: All patients who underwent surgical treatment for degenerative lumbar stenosis between 2004 and 2007 were identified from the SRS M&M database. Inclusion criteria for analysis included an age >or= 21 years and no history of lumbar surgery. Patients were treated with either decompression alone or decompression with concomitant fusion. Statistical comparisons were performed using a 2-sided Fisher exact test.

Results: Of the 10,329 patients who met the inclusion criteria, 6609 (64%) were treated with decompression alone, and 3720 (36%) were treated with decompression and fusion. Among those who underwent fusion, instrumentation was placed in 3377 (91%). The overall mean patient age was 63 +/- 13 years (range 21-96 years). Seven hundred nineteen complications (7.0%), including 13 deaths (0.1%), were identified. New neurological deficits were reported in 0.6% of patients. Deaths were related to cardiac (4 cases), respiratory (5 cases), pulmonary embolus (2 cases), and sepsis (1 case) etiologies, and a perforated gastric ulcer (1 case). Complication rates did not differ based on patient age or whether fusion was performed. Minimally invasive procedures were associated with fewer complications and fewer new neurological deficits (p = 0.01 and 0.03, respectively).

Conclusions: The results from this analysis of the SRS M&M database provide surgeons with useful information for preoperative counseling of patients contemplating surgical intervention for symptomatic degenerative lumbar stenosis.
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May 2010

Intraoperative neurophysiological monitoring during complex spinal deformity cases in pediatric patients: methodology, utility, prognostication, and outcome.

Childs Nerv Syst 2010 Apr 7;26(4):523-44. Epub 2010 Mar 7.

Division of Neurosurgery, Hospital for Sick Children, University of Toronto, 555 University Ave, Toronto, ON, Canada.

Introduction: Complex spinal deformity (CSD) problems in pediatric patients result from a wide variety of congenital, acquired, neoplastic, or traumatic abnormalities that result in a combination of spinal deformity and spinal cord impingement. While these problems are rare, decompression, correction, instrumentation, and fusion are quite hazardous. Intraoperative neurophysiological monitoring (IONM) seems particularly beneficial in these patients.

Methodology: Somatosensory evoked potentials, transcranial electrical motor evoked potentials (MEPs), direct waves, and electromyography were used in a variety of CSD cases over a period when IONM was routine for most spinal cases. Examples of cases in which IONM provided important intraoperative information and significantly affected the course of the operation are illustrated.

Results: IONM is a useful tool particularly in CSD cases in pediatric patients but requires special expertise and anesthetic considerations. Loss of MEP appears to have particularly important adverse prognostic information. Conversely, maintenance of IONM provides significant reassurance that the spinal cord function is being maintained. Preserved but persistently diminished MEPs usually predict a neurological injury that will significantly improve and possibly completely recover. Issues concerning training, certification, oversight, standardization of equipment, and technique are partially but incompletely resolved.

Discussion: IONM is an extremely valuable tool for management of CSD pediatric patients. The utility of IONM is such and the detection of unexpected or unanticipated neurological injury frequent enough that a strong argument that it be used in every spinal surgery case can be made.
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April 2010

Do baclofen pumps influence the development of scoliosis in children?

J Neurosurg Pediatr 2010 Feb;5(2):195-9

Division of Neurosurgery, The Hospital for Sick Children, Toronto, Ontario, Canada.

Object: Intrathecal baclofen is an effective treatment for spasticity in patients with cerebral palsy. There has been increasing concern, however, that intrathecal baclofen may accelerate the development of scoliosis in this population. To this end, the authors reviewed their population of pediatric patients with baclofen pumps to assess the incidence of scoliosis.

Methods: This was a retrospective chart and radiology review of all pediatric patients with baclofen pumps. Cobb angles were measured preoperatively and on follow-up images.

Results: Of 38 patients identified, 32 had adequate data available for inclusion in the study (16 with cerebral palsy, 7 with dystonic cerebral palsy, 4 with head injury, and 5 with other diagnoses). The mean age at pump insertion was 10.6 years and the mean follow-up period was 31 months (range 1-118 months). The mean annual Cobb angle progression was 19 degrees (range 0-68 degrees, median 12 degrees).

Conclusions: In the authors' group of patients there was notable development and progression of scoliosis at a greater than previously reported rate for the same patient population, and also greater than previously reported patients with intrathecal baclofen pumps. The largest possible confounding factor in this study was the insertion of the pump before skeletal maturity and therefore coinciding with the time when scoliosis may be developing naturally. A prospective study is recommended to gather further data on the development of scoliosis in this particular population with intrathecal baclofen pumps.
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February 2010

Technique for drilling instrument monitoring electrical conductivity in pediatric cervical spine screw insertion: a preliminary report.

J Pediatr Orthop 2009 Oct-Nov;29(7):760-4

Department of Orthopedic Surgery , The Hospital for Sick Children, Toronto, ON, Canada.

Background: To detail a technique of assisted screw placement in pediatric patients with cervical spine disorders. The use of a recently produced wireless electronic freehand drilling instrument is documented.

Methods: We performed fixation of the cervical spine using different screws in 5 consecutive patients with various cervical spine disorders (posttraumatic, neoplastic or metabolic). Clinical and radiologic features of all cases are reported. The surgical technique is described.

Results: Twenty-six cervical screws (lateral mass, pars, and/or laminar) were placed with the use of the same pedicle screw pilot hole preparation device, and by the same surgical team. The average age of this patient group was 13.4 years (range: 6 to 16 y). Average follow-up was 10 months (range: 5 to 14 mo). All screws were inserted after the correct trajectory was identified. No breaches were detected. No screw failure was encountered. However, 8 of 26 (30.8%) screws were, on average, 1.3 mm longer that expected (range: 0.5 to 2.4 mm). None of the patients developed neurologic or vascular complications as a result of screw placement.

Conclusions: Cervical screws placement, although safe, is not free of complications: neurovascular injuries, dural tears, and paraplegia have been described. The use of a wireless electronic handheld pedicle screw pilot hole preparation instrument is a useful tool in the armamentarium of the spinal surgeon dealing with patients with complex spinal deformities or difficult anatomy. Our experience outlines the applicability of this technique at different cervical levels. However, this device is not satisfactory in predicting the length of the screws. The device should be modified or a preoperative computed tomography scan should be used to estimate safe the length of the screws.

Level Of Evidence: Level IV (case series).
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May 2010

The treatment of vertebral and chest wall deformities with expandable thoracoplasty and a prosthetic expandable implant.

J Pediatr Orthop 2010 Jan-Feb;30(1):90-8

NYU Hospital for Joint Diseases, LLC, New York 10016, USA.

Background: Expansion thoracoplasty is a temporary solution to allow complete pulomonary development in the setting of vertebral and rib cage deformities. After thoracoplasty, an expandable implant is needed to maintain the correction. The Growing Spine Profiler (GSP) is a new type of readjustable rib distracter. The purpose of this study was to evaluate the experiences of surgeons in Europe with the implantation of the GSP.

Methods: We retrospectively reviewed 44 patients that underwent placement of the GSP at 15 sites throughout Europe. The mean age at the time of surgery was 6.24 years. Basic demographic data and postoperative management were reviewed. All patients received an opening-wedge thoracostomy with device implantation and expansion thoracoplasty. Retrospectively, the original indications for implantation were determined. Digital images of the radiographs were used to measure coronal and sagittal parameters.

Results: The most common diagnoses for implantation were hemivertebrae (9), hemivertebrae with chest wall deformity (10), and unilateral bar (8). Outcomes were available for 43 patients with a mean follow-up period of 3.07 years. There were 23 patients that had an adverse event. Rib fractures were the most common adverse event, with 8 incidents in 7 patients. Radiographically, coronal curve magnitudes were maintained, whereas sagittal kyphosis gradually increased.

Conclusions: The GSP was effective in the maintenance of deformity, while providing a moderate complication rate. Further studies need to be performed to determine the safety and efficacy of this implant.
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March 2010

Management of osteoblastoma and osteoid osteoma of the spine in childhood.

J Neurosurg Pediatr 2009 Nov;4(5):434-8

Division of Neurosurgery, The Hospital for Sick Children, Toronto, Ontario, Canada.

Object: Osteoid osteomas and osteoblastoma of the spine are rare lesions in childhood, and management strategies have changed. The authors reviewed their recent experience with these 2 types of lesions to elucidate current treatment modalities and outcomes.

Methods: Case records and radiographic images from all cases of osteoid osteoma and osteoblastoma diagnosed between 1993 and 2008 were retrospectively reviewed, including those managed nonsurgically.

Results: Thirty cases were identified; 22 were treated surgically and 8 were managed nonsurgically. The patients' mean age at presentation was 13 years (range 3-17 years). Of 30 patients, 29 (97%) presented with pain; 7 (23%) had scoliosis at presentation; 12 (40%) experienced relief with nonsteroidal antiinflammatory medication. Osteoid osteoma was diagnosed in 7 (32%) of the 22 patients who underwent surgery, and osteoblastoma in 15 (68%). Nine (41%) of the 22 surgically treated patients underwent fusion procedures (bone onlay or instrumentation) at the time of surgery. Pain freedom without medication had been achieved in 16 (73%) of the 22 surgically treated patients at a mean follow-up of 28 months (range 2-75 months) and was confirmed in 3 (38%) of the 8 nonsurgically treated patients at a mean follow-up of 33 months (range 24-51 months).

Conclusions: Osteoid osteomas and osteoblastomas can present challenging management problems in pediatric patients. In the majority of cases in which conservative therapy fails or pathological diagnosis is required, surgery using modern intraoperative imaging and spinal instrumentation can provide symptom relief and tumor control.
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November 2009

Clinical heterogeneity of duchenne muscular dystrophy (DMD): definition of sub-phenotypes and predictive criteria by long-term follow-up.

PLoS One 2009 5;4(2):e4347. Epub 2009 Feb 5.

Department of Neuropediatrics, Neuromuscular Disease Reference Center Garches-Necker-Mondor-Hendaye, Necker-Enfants Malades Hospital, Paris, France.

Background: To explore clinical heterogeneity of Duchenne muscular dystrophy (DMD), viewed as a major obstacle to the interpretation of therapeutic trials

Methodology/principal Findings: A retrospective single institution long-term follow-up study was carried out in DMD patients with both complete lack of muscle dystrophin and genotyping. An exploratory series (series 1) was used to assess phenotypic heterogeneity and to identify early criteria predicting future outcome; it included 75 consecutive steroid-free patients, longitudinally evaluated for motor, respiratory, cardiac and cognitive functions (median follow-up: 10.5 yrs). A validation series (series 2) was used to test robustness of the selected predictive criteria; it included 34 more routinely evaluated patients (age>12 yrs). Multivariate analysis of series 1 classified 70/75 patients into 4 clusters with distinctive intellectual and motor outcomes: A (early infantile DMD, 20%): severe intellectual and motor outcomes; B (classical DMD, 28%): intermediate intellectual and poor motor outcome; C (moderate pure motor DMD, 22%): normal intelligence and delayed motor impairment; and D (severe pure motor DMD, 30%): normal intelligence and poor motor outcome. Group A patients had the most severe respiratory and cardiac involvement. Frequency of mutations upstream to exon 30 increased from group A to D, but genotype/phenotype correlations were restricted to cognition (IQ>71: OR 7.7, 95%CI 1.6-20.4, p<0.003). Diagnostic accuracy tests showed that combination of "clinical onset <2 yrs" with "mental retardation" reliably assigned patients to group A (sensitivity 0.93, specificity 0.98). Combination of "lower limb MMT score>6 at 8 yrs" with "normal or borderline mental status" reliably assigned patients to group C (sensitivity: 1, specificity: 0.94). These criteria were also predictive of "early infantile DMD" and "moderate pure motor DMD" in series 2.

Conclusions/significance: DMD can be divided into 4 sub-phenotypes differing by severity of muscle and brain dysfunction. Simple early criteria can be used to include patients with similar outcomes in future therapeutic trials.
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April 2009