Side bending radiographs and lowest instrumented vertebra in adolescent idiopathic scoliosis: A French quality-of-care study.

BACKGROUND: Determining which spinal levels to instrument during surgical treatment of Lenke Type 1 adolescent idiopathic scoliosis (AIS) depends on the reducibility of the primary and secondary curve patterns. This reducibility can be evaluated in several ways, with the most popular being radiographs in bending for moderate thoracic and lumbar curvatures. Hypothesis Side-bending radiographs will alter the choice of the lowest instrumented vertebra (LIV) for the surgical treatment of AIS. METHODS: Thirteen experienced French spine surgeons were invited to perform surgical planning on 23 patients based on stereoradiographs with and without (standing) side-bending views. The surgical planning was repeated a second time to assess the intra- and inter-rater reliability. Variations in the choice of LIV were analyzed for each evaluation. RESULTS: The intra-rater reliability was moderate to substantial. The inter-rater reliability was low to moderate. The study compared 879 surgical plans. Selective fusion was chosen in 0.3% of the plans. The median LIV was L2. The availability of side bending views changed the plan in 39% of cases. However, 36% of the plans were changed in the control (test-retest) condition. No significant difference was found between the variations with side-bending radiographs and "control" variations (p>0.05). CONCLUSION: The use of radiographs in bending has no significant effect on the LIV choice in this study. This result is derived from statistically robust analysis made possible by one of the largest datasets available on this topic. Large inter-rater variability was observed and will be explored further in a future study. LEVEL OF EVIDENCE: II; non-randomized controlled comparative study.

Epidemiology of spinal fractures in children: Cross-sectional study.

INTRODUCTION: Epidemiological studies of fractures of the spine in children are all old, mostly single-centre, with series spanning periods of 5 to 20 years. HYPOTHESIS: As lifestyle is constantly changing, notably with an increase in sports activities and improvements in the prevention of road and household accidents, epidemiology has likely changed. OBJECTIVE: To update the description of spinal trauma in children and adolescents compared to the existing literature. MATERIAL AND METHOD: A multicentre cross-sectional study of spinal fracture, dislocation and spinal cord injury without radiological abnormality (SCIWORA) in children was carried out in 15 French university hospital centres, for a period of one year (2016). RESULTS: One hundred and sixty-five children were identified: 85 girls, 80 boys; mean age 11 years (range, 10 months-17 years); median, 12 years 6 months. One hundred and fifty-two children (92%) had fracture, 8 (5%) dislocation (including 7 C1-C2 rotary dislocations), and 5 (3%) SCIWORA. Fractures were multiple in 80 cases (49%), contiguous in 73 cases (91%) and non-contiguous in 7 (9%). Locations were cervical in 25 cases (15%), thoracic in 85 (52%), lumbar in 75 and sacral in 4 (2%). Fracture types comprised 234 vertebral compactions (78%), 25 burst fractures (8%), 5 chance fractures (2%), 2 odontoid fractures, and 33 other lesions. Causes comprised fall in 77 cases (47%), sports accidents in 56 (34%), road accidents in 29 (18%), and others in 3. In 52 cases (32%), there was ≥1 associated lesion: appendicular in 35 cases (67%), thoracic or abdominal in 31 (60%), and head in 16 (31%). Twenty-one cases had multiple lesions (40%). Eighteen cases showed neurological involvement (11%) including 5 SCIWORAs. Neurological complications were more frequent before 9 years of age. CONCLUSION: The epidemiology of spine fractures in children has slightly changed. There are now fewer cervical lesions. Causes are less often road accidents and more often sports accidents. Multi-level lesions remain frequent and the rate of neurological complications is around 10%. Compaction fracture is the most common type.

Enhanced recovery after surgery in pediatric orthopedics (ERAS-PO).

"Enhanced recovery after surgery" is now the official term: ERAS. Patients come to a specialized center for surgery, and early recovery is ensured by minimizing the impact of surgical stress, controlling pain and stimulating autonomy. Patient information and education concerning the process and care organization enable short hospital stay with early discharge. The expected benefits are fewer postoperative complications and shorter hospital stay. There is nothing to prevent this kind of program being implanted for children, so long as age and the parent-child relationship are taken into account. Lessons should be drawn from existing pediatric therapeutic education programs, to adapt information and training to the child's cognitive, motor and psycho-affective development. Setting up an ERAS program is the result firstly of medical and surgical reflection. All healthcare actors need to be actively involved, to set up a management program for the parent-child duo. Implementation, monitoring and assessment are the responsibilities of the physicians who initiate the program. Fewer postoperative complications, with earlier discharge and rehabilitation, should reduce costs and improve patient management in hospital. Such is, indeed, usually the case, but unfortunately drastic health expenditure curbs greatly attenuate the expected benefit in terms of care organization and cost savings.

Treatment of thoracolumbar kyphosis in patients with mucopolysaccharidosis type I: results of an international consensus procedure.

BACKGROUND: In all patients with mucopolysaccharidosis type I (MPS I), skeletal disease (dysostosis multiplex) is a prominent, debilitating, condition related complication that may impact strongly on activities of daily living. Unfortunately, it is not alleviated by treatment with hematopoietic cell transplantation (HCT) or enzyme replacement therapy (ERT). Although early kyphosis is one of the key features of dysostosis multiplex, there is no international consensus on the optimal management. Therefore, an international consensus procedure was organized with the aim to develop the first clinical practice guideline for the management of thoracolumbar kyphosis in MPS I patients. METHODS: A literature review was conducted to identify all available information about kyphosis and related surgery in MPS I patients. Subsequently, a modified Delphi procedure was used to develop consensus statements. The expert panel included 10 spinal orthopedic surgeons, 6 pediatricians and 3 physiotherapists, all experienced in MPS I. The procedure consisted of 2 written rounds, a face-to-face meeting and a final written round. The first 2 rounds contained case histories, general questions and draft statements. During the face-to-face meeting consensus statements were developed. In the final round, the panel had the opportunity to anonymously express their opinion about the proposed statements. RESULTS: Eighteen case series and case reports were retrieved from literature reporting on different surgical approaches and timing of thoracolumbar kyphosis surgery in MPS I. During the face-to-face meeting 16 statements were discussed and revised. Consensus was reached on all statements. CONCLUSION: This international consensus procedure resulted in the first clinical practice guideline for the management of thoracolumbar kyphosis in MPS I patients, focusing on the goals and timing of surgery, as well as the optimal surgical approach, the utility of bracing and required additional assessments (e.g. radiographs). Most importantly, it was concluded that the decision for surgery depends not only on the kyphotic angle, but also on additional factors such as the progression of the deformity and its flexibility, the presence of symptoms, growth potential and comorbidities. The eventual goal of treatment is the maintenance or improvement of quality of life. Further international collaborative research related to long-term outcome of kyphosis surgery in MPS I is essential as prognostic information is lacking.

Carpal tunnel syndrome in mucopolysaccharidosis I: a registry-based cohort study.

AIM: To characterize carpal tunnel syndrome (CTS) in patients with mucopolysaccharidosis I (MPS I). METHOD: Data were included for patients with MPS I who had either nerve conduction examination that included a diagnosis of CTS or who had CTS release surgery. Although this represented a subset of patients with CTS in the MPS I Registry, the criteria were considered the most objective for data analysis. RESULTS: As of March 2016, 994 patients were categorized with either severe (Hurler syndrome) or attenuated (Hurler-Scheie or Scheie syndromes) MPS I. Among these, 291 had a CTS diagnosis based on abnormal nerve conduction (n=54) or release surgery (n=237). Median ages (minimum, maximum) at first CTS diagnosis were 5 years 2 months (10mo, 16y 2mo) and 9y 11mo (1y 8mo, 44y 1mo) for patients with severe and attenuated MPS I respectively. Most patients had their first CTS diagnosis after MPS I diagnosis (94%) and treatment (hematopoietic stem cell transplant and/or enzyme replacement therapy) (74%). For 11% of patients with attenuated disease, CTS diagnosis preceded MPS I diagnosis by a mean of 7 years 6 months. INTERPRETATION: CTS is a rare complication in pediatric patients and should alert medical care providers to the potential diagnosis of MPS I. Significant delays exist between diagnosis of CTS and MPS I for patients with attenuated disease. WHAT THIS PAPER ADDS: There are significant delays in diagnosing carpal tunnel syndrome (CTS) in patients with mucopolysaccharidosis I (MPS I). Enzyme replacement therapy or hematopoietic stem cell transplant do not prevent the development of CTS. Testing for CTS in patients with MPS I is recommended to prevent irreparable damage. CTS in pediatric patients should alert physicians to potential diagnosis of MPS I.

Natural hydroxyapatite as a bone graft extender for posterolateral spine arthrodesis.

PURPOSE: The aim of this retrospective analysis of spinal fusion, was to document the clinical, functional, and radiological outcomes with a local bone graft plus the highly osteoconductive hydroxyapatite, bio-derived Orthoss®, with or without bone marrow aspirate. METHODS: Forty seven patients submitted to spinal posterolateral fusion were operated for four major indications: scoliosis in young patients (11), degenerative spine (18), lumbosacral transitional anomalies in young adults (14), and spine trauma (four). Sixteen patients had more than four levels fused. In addition to spinal decompression and instrumented fusion, autologous bone grafts from the excised lamina were augmented with Orthoss® granules in a 1:1 ratio. In addition iliac crest bone marrow aspirate was used in 70 % of the patients. The results were assessed clinically in terms of pain, and return to school or professional activities were checked at three, six, and 12 months following surgery with a mean follow-up of 20 months. In scoliotic patients, correction of the major angle was evaluated from one to four years after surgery. RESULTS: Pain persistence was reported only in four cases, after three months after surgery. A functional recovery was noted in almost all patients groups within these three months. Progressive bone formation with evidence of bone fusion masses were already observed at six months. No fusion failure was observed. CONCLUSIONS: Local bone enhanced by an osteoconductive long-term stable scaffold, used with and without bone marrow aspirate, led to successful fusion in all patients by six months while functional recovery was reported already within three to six months.

The change on vertebral axial rotation after posterior instrumentation of idiopathic scoliosis.

PURPOSE: Agreement between the correction objectives and the instrumentation strategies remains controversial in idiopathic scoliosis. Most studies have focus on the frontal and sagittal plane. The goal of this study was to evaluate the change on vertebral axial rotation after posterior instrumentation in fused and unfused segments. METHODS: Fourteen patients operated on for idiopathic scoliosis were prospectively included. Fusion and instrumentation were done by posterior approach. All patients had a pre-operative and a 10-day post-operative radiological evaluation with the EOS system. Axial orientation of the vertebrae with special interest to the apical, junctional, and unfused areas was obtained thanks to the reconstruction software. RESULTS: Mean apical vertebra axial rotation statistically decreased from 21° pre-operatively to 13° post-operatively. But, there were no statistically significant differences between pre-operative and post-operative mean axial intervertebral rotations in the main curve and axial rotation of the non-instrumented lower counter curve. CONCLUSIONS: 3D analysis of the spine in standing position is a great advancement for post-operative analysis of adolescent idiopathic scoliosis (AIS) corrections. This study confirmed that actual instrumentations are able to achieve "en bloc" 3D correction of the spine but not intervertebral axial rotation correction.

Tridimensional trunk surface acquisition for brace manufacturing in idiopathic scoliosis.

INTRODUCTION: Brace manufacturing for idiopathic scoliosis requires trunk surface acquisition. Two methods are currently available to design the trunk shape: craft made technique based on a plaster mold and computer-aided design with 3-D reconstruction of the trunk by optical scanning. The objective of the present review was to compare these two methods. METHODOLOGY: We describe the different steps to design and manufacture braces used for spinal deformities. RESULTS: Our prospective evaluation showed good results in terms of the correction achieved and regarding patients' in-brace comfort. DISCUSSION: Optical scanning for computer-aided design and manufacturing (CAD-CAM) of trunk orthoses have proven their effectiveness. These technologies may help in monitoring conservative treatment and may enhance the brace interaction with the spinal deformity when orthopedic treatment is indicated.

Predicting intraoperative feasibility of combined TES-mMEP and cSSEP monitoring during scoliosis surgery based on preoperative neurophysiological assessment.

BACKGROUND CONTEXT: Combined monitoring of muscle motor evoked potentials elicited by transcranial electric stimulation (TES-mMEP) and cortical somatosensory evoked potentials (cSSEPs) is safe and effective for spinal cord monitoring during scoliosis surgery. However, TES-mMEP/cSSEP is not always feasible. Predictors of feasibility would help to plan the monitoring strategy. PURPOSE: To identify predictors of the feasibility of TES-mMEP/cSSEP during scoliosis surgery. STUDY DESIGN/SETTING: Prospective cohort study in a clinical neurophysiology unit and pediatric orthopedic department of a French university hospital. PATIENT SAMPLE: A total of 103 children aged 2 to 19 years scheduled for scoliosis surgery. OUTCOME MEASURES: Feasibility rate of intraoperative TES-mMEP/cSSEP monitoring. METHODS: All patients underwent a preoperative neurological evaluation and preoperative mMEP and cSSEP recordings at both legs. For each factor associated with feasibility, we computed sensitivity, specificity, positive predictive value (PPV), and negative predictive value. A decision tree was designed. RESULTS: Presence of any of the following factors was associated with 100% feasibility, 100% specificity, and 100% PPV: idiopathic scoliosis, normal preoperative neurological findings, and normal preoperative mMEP and cSSEP recordings. Feasibility was 0% in the eight patients with no recordable mMEPs or cSSEPs during preoperative testing. A decision tree involving three screening steps can be used to identify patients in whom intraoperative TES-mMEP/cSSEP is feasible. CONCLUSIONS: Preoperative neurological and neurophysiological assessments are helpful for identifying patients who can be successfully monitored by TES-mMEP/cSSEP during scoliosis surgery.

Surgical management of thoracolumbar kyphosis in mucopolysaccharidosis type 1 in a reference center.

PURPOSE: Mucopolysaccharidosis (MPS) I is a rare autosomal recessive lysosomal storage disease. Thoracolumbar kyphosis is an early characteristic feature of the disease. Ossification failure in the anterosuperior quadrant of the vertebral body results in anterior dislocation. This study describes the surgical management of thoracolumbar kyphosis in MPS IH (Hurler syndrome) in a national reference center. METHODS: Among 72 MPS I patients followed in our institution, we treated surgically 14 MPS IH patients with severe thoracolumbar kyphosis. The decision was made after documented deformity progression. Mean age at surgery was 8 (3.5-15) years. Sagittal imbalance of the trunk was constant. One patient underwent extended fusion for associated scoliosis. We retrospectively reviewed 13 patients who underwent selective circumferential fusion at the thoracolumbar level. RESULTS: Average preoperative kyphosis was +57.5°(+30°; +90°). Surgical correction of the kyphosis was about 66 % and maintained at final follow-up. Fusion was obtained in all patients. Two patients required revision surgery consecutively to a previous posterior-only fusion, as a significant loss of correction occurred. One patient presented delayed neurologic deficit secondarily to cardiac embolism. One patient died postoperatively from cardiorespiratory failure. CONCLUSION: Surgery is necessary when kyphosis is progressive despite orthopedic management, aggravating the multifactorial trunk imbalance. Regarding our experience, circumferential arthrodesis should be recommended to achieve stable correction. Surgical management requires a multidisciplinary approach due to multisystemic failure and neurological risks specific to metabolic disorders.

Orthoses for mild scoliosis: a prospective study comparing traditional plaster mold manufacturing with fast, noncontact, 3-dimensional acquisition.

STUDY DESIGN: A prospective comparison of 2 different methods to make orthoses in mild scoliosis. OBJECTIVE: To evaluate the therapeutic efficiency of orthoses made by a computer-aided design procedure. SUMMARY OF BACKGROUND DATA: It is now possible to make orthoses by a computer-aided design procedure. In order to evaluate this new method, we carried out a comparative study between the traditional and computer-aided design-manufactured orthoses. METHODS: In this prospective study, we compared the 2 methods by studying the cases of 30 adolescents with mild scoliosis. For each patient requiring orthopaedic treatment, we made 2 ortheses: 1 using the traditional method and 1 using the computer-aided design method. There were 26 girls and 4 boys whose average age was 13 years and 3 months. Each body jacket was successively used in random order following the same protocol. Neither the prescriber nor the patient knew the origin of the orthosis used. The final choice of the orthosis was made using 3 criteria: first, improvement of the scoliotic curves on the frontal and lateral radiologic planes, and second, the patient's impression of comfort. RESULTS: For the 30 cases, 13 traditional and 16 computer-aided design body jackets were chosen. In 1 case, no significant difference allowed us to chose one body jacket rather than the other. For the frontal radiologic correction, the better results were obtained 3 times with the computer-aided design body jacket, 5 times with the traditional one, and in 22 cases, the results were equivalent. For the lateral radiologic correction, the better results were obtained 11 times with the computer-aided design body jacket, 3 times with the traditional one, and in 16 cases, the results were equivalent. For the comfort of the 2 body jackets for each patient, the better results were obtained 12 times with the computer-aided design body jacket, 8 times with the traditional one, and in 10 cases, the results were equivalent. CONCLUSION: Based on our results, we believe that the computer-aided design procedure is equally efficient to traditional method for mild scoliotic curves.