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The Journal of Bone & Joint Surgery British Volume
Vol. 77-B, Issue 5 | Pages 768 - 770
1 Sep 1995
Loder R Urquhart A Steen H Graziano G Hensinger R Schlesinger A Schork M Shyr Y

The variability in measurement of angles in congenital scoliosis is not known, but it is postulated that it is larger than that in adolescent idiopathic scoliosis due to skeletal immaturity, incomplete ossification, and anomalous development of the end-vertebrae. To determine this variability, we selected 54 radiographs of adequate quality showing 67 scoliotic curves from children with congenital scoliosis. The end-vertebrae were preselected. Each curve was measured by the Cobb method on two separate occasions by six different observers, using the same goniometer and marker. The intraobserver variability was +/- 9.6 degrees and the interobserver variability +/- 11.8 degrees. If 'significant progression' is to be used as a criterion for surgical fusion in congenital scoliosis, there should be at least a 23 degrees increase, the entire range of the interobserver variability, in the curvature to ensure that the perceived increase is not due to variability in measurement


The Journal of Bone & Joint Surgery British Volume
Vol. 68-B, Issue 2 | Pages 194 - 196
1 Mar 1986
Pool R

The term "congenital scoliosis" contributes little to our understanding of aetiology, for "congenital" simply means "to be born with" and is applicable to deformities present at birth whether these are genetically determined or acquired in utero. The presentation of monozygotic twins, one of whom has congenital scoliosis (vertebral anomalies) while the other is normal, provides a rare opportunity to study the cause of this deformity. Three pairs of monozygotic twins, previously unreported, are presented with a review of the previous literature. These cases add weight to the argument that congenital scoliosis may be acquired in utero rather than being genetically determined


Bone & Joint 360
Vol. 12, Issue 4 | Pages 38 - 41
1 Aug 2023

The August 2023 Children’s orthopaedics Roundup. 360. looks at: DDH: What can patients expect after open reduction?; Femoral head deformity associated with hip displacement in non-ambulatory cerebral palsy; Bony hip reconstruction for displaced hips in patients with cerebral palsy: is postoperative immobilization indicated?; Opioid re-prescriptions after ACL reconstruction in adolescents are associated with subsequent opioid use disorder; Normative femoral and tibial lengths in a modern population of USA children; Retrospective analysis of associated anomalies in 636 patients with operatively treated congenital scoliosis; Radiological hip shape and patient-reported outcome measures in healed Perthes’ disease; Significantly displaced adolescent posterior sternoclavicular joint injuries


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_III | Pages 495 - 495
1 Sep 2009
Garrido E Tome F Tucker S Noordeen H Morley T
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Study Design: Retrospective study with clinical and radiological evaluation of 29 patients with congenital scoliosis who underwent 31 short segment anterior instrumented fusions of lateral hemivertebrae. Objective: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital scoliosis in children below the age of 6. Summary of background data: A variety of treatments have been described in the literature for the treatment of HV. We report the results of a novel technique. Materials and Methods: Between 1996 and 2005, 29 consecutive patients with 31 lateral HV and progressive scoliosis underwent short segment anterior instrumentation and fusion with preservation of the HV. Mean age at surgery was 2.9 years. Mean follow-up period was 6.3 years. Results: Preoperative segmental Cobb angle averaging 39°, was corrected to 150 after surgery, being 15º at the last follow up (60% of improvement). Compensatory cranial and caudal curves corrected by approximately 50% and did not change significantly on follow up. The angle of segmental kyphosis averaged 13º before surgery, 12º after surgery, and 12° at follow up. There was 2 wound infection requiring surgical debridment, 1 intraoperative fracture of the vertebral body and 1 case lost correction due to implant failure. All went on to stable bony union. There were no neurologic complications. Conclusions: Early diagnosis and early and aggressive surgical treatment are mandatory for a successful treatment of congenital scoliosis and to prevent the development of secondary compensatory deformities. Anterior instrumentation is a safe and effective technique capable of transmitting a high amount of convex compression allowing short segment fusion which is of great importance in the growing spine


The Journal of Bone & Joint Surgery British Volume
Vol. 84-B, Issue 2 | Pages 305 - 306
1 Mar 2002
FREEMAN BJ OULLET JA WEBB JK


The Journal of Bone & Joint Surgery British Volume
Vol. 83-B, Issue 4 | Pages 496 - 500
1 May 2001
Deviren V Berven S Smith JA Emami A Hu SS Bradford DS

We present a study of ten consecutive patients who underwent excision of thoracic or thoracolumbar hemivertebrae for either angular deformity in the coronal plane, or both coronal and sagittal deformity. Vertebral excision was carried out anteriorly alone in two patients. Seven patients had undergone previous posterior spinal fusion. Their mean age at surgery was 13.4 years (6 to 19). The mean follow-up was 78.5 months (20 to 180). The results were evaluated by radiological review of the preoperative, postoperative and most recent follow-up films.

The mean preoperative coronal curve was 78.2° (30 to 115) and was corrected to 33.9° (7 to 58) postoperatively, a mean correction of 59%. Preoperative coronal decompensation of 35 mm was improved to 11 mm postoperatively. Seven patients had significant coronal decompensation preoperatively, which was corrected to a physiological range postoperatively. There were no major complications and no neurological damage.

We have shown that resection of thoracic and thoracolumbar hemivertebrae can be performed safely, without undue risk of neurological compromise, in experienced hands.


Orthopaedic Proceedings
Vol. 106-B, Issue SUPP_18 | Pages 35 - 35
14 Nov 2024
Bulut H Abasova F Basaran T Balaban P
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Introduction. Congenital scoliosis is a prevalent congenital spinal deformity, more frequently encountered than congenital lordosis or kyphosis. The prevailing belief is that most instances of congenital scoliosis are not hereditary but rather stem from issues in fetal spine development occurring between the 5th and 8th weeks of pregnancy. However, it has been linked to several genes in current literature. Our goal was to explore potential pathways through an exhaustive bioinformatics analysis of genes related to congenital scoliosis. Method. The literature from the 1970s to February 2024 was surveyed for genes associated with CS, and 63 genes were found to be associated with AIS out of 1743 results. These genes were analyzed using DAVID Bioinformatics. Result. Our pathway analysis has unveiled several significant associations with congenital scoliosis. Notably, “Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate” (P-Value:8.8E-3, Fold Enrichment: 20.6), “Central carbon metabolism in cancer” (P-Value:1.3E-3, Fold Enrichment: 10.3), and “Lysine degradation” (P-Value: 9.0E-3, Fold Enrichment: 9.1) emerge as statistically significant pathways. Additionally, “Endocrine resistance” (P-Value:4.4E-3, Fold Enrichment:7.4) and”EGFR tyrosine kinase inhibitor resistance” (P-Value: 1.7E-2, Fold Enrichment:7.3) pathways are noteworthy. These findings suggest a potential involvement of these pathways in the biological processes underlying congenital scoliosis. Furthermore, “Signaling pathways regulating pluripotency of stem cells” (P-Value:4.0E-4, Fold Enrichment:7.1), “Notch signaling pathway” (P-Value:6.7E-2, Fold Enrichment: 7.0), and “TGF-beta signaling pathway” (P-Value:6.2E-3, Fold Enrichment: 6.7) exhibit a less pronounced yet intriguing association that may warrant further investigation. Conclusion. In conclusion, our comprehensive analysis of the genetic etiology of congenital scoliosis has revealed significant associations with various pathways, shedding light on potential underlying biological mechanisms. While further research is needed to fully understand these associations and their implications, our findings provide a valuable starting point for future investigations into the management and treatment of congenital scoliosis


The Bone & Joint Journal
Vol. 103-B, Issue 3 | Pages 547 - 552
1 Mar 2021
Magampa RS Dunn R

Aims. Spinal deformity surgery carries the risk of neurological injury. Neurophysiological monitoring allows early identification of intraoperative cord injury which enables early intervention resulting in a better prognosis. Although multimodal monitoring is the ideal, resource constraints make surgeon-directed intraoperative transcranial motor evoked potential (TcMEP) monitoring a useful compromise. Our experience using surgeon-directed TcMEP is presented in terms of viability, safety, and efficacy. Methods. We carried out a retrospective review of a single surgeon’s prospectively maintained database of cases in which TcMEP monitoring had been used between 2010 and 2017. The upper limbs were used as the control. A true alert was recorded when there was a 50% or more loss of amplitude from the lower limbs with maintained upper limb signals. Patients with true alerts were identified and their case history analyzed. Results. Of the 299 cases reviewed, 279 (93.3%) had acceptable traces throughout and awoke with normal clinical neurological function. No patient with normal traces had a postoperative clinical neurological deficit. True alerts occurred in 20 cases (6.7%). The diagnoses of the alert group included nine cases of adolescent idiopathic scoliosis (AIS) (45%) and six of congenital scoliosis (30%). The incidence of deterioration based on diagnosis was 9/153 (6%) for AIS, 6/30 (20%) for congenital scoliosis, and 2/16 (12.5%) for spinal tuberculosis. Deterioration was much more common in congenital scoliosis than in AIS (p = 0.020). Overall, 65% of alerts occurred during rod instrumentation: 15% occurred during decompression of the internal apex in vertebral column resection surgery. Four alert cases (20%) awoke with clinically detectable neurological compromise. Conclusion. Surgeon-directed TcMEP monitoring has a 100% negative predictive value and allows early identification of physiological cord distress, thereby enabling immediate intervention. In resource constrained environments, surgeon-directed TcMEP is a viable and effective method of intraoperative spinal cord monitoring. Level of evidence: III. Cite this article: Bone Joint J 2021;103-B(3):547–552


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_11 | Pages 26 - 26
1 Sep 2021
Palliyil N Estefan M Gessara A Shafafy M
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SUMMARY. A retrospective cohort study of 19 patients of EOS who underwent MCGR rod instrumentation with subsequent serial distractions, measured using ultrasonography. The degree of distraction achieved during each session were analyzed and subgroup analysis done. HYPOTHESIS. The degree of distraction/ distractibility of the MCGR rod is closely linked to the etiology of EOS. The degree of distraction achieved at each session progressively diminishes with successive distractions. STUDY DESIGN. Retrospective cohort study. INTRODUCTION. MCGR rods have gained popularity as an alternative to traditional growing rods for the treatment of Early-onset scoliosis (EOS), serially distracting with an aim to mimic the normal spinal growth, which can be achieved in an outpatient setting using external remote controller. The use of ultrasound as a tool to measure the degree of distraction achieved has been validated previously. However the association between the etiological diagnosis of EOS and the degree of distraction achieved has not been studied previously. METHODS. We performed a retrospective cohort study of 19 patients with EOS of varying etiologies who underwent MCGR rod instrumentation at our institution since 2016. Their hospital records were analyzed to assess the demographics, distraction interval and the degree of distraction achieved at each session. RESULTS. 19 patients (12 females and 7 males) were included in the study. The average age of the study population was 7 years (4–13 years). The average follow up was 2.1 years (1–4). The following were the etiological diagnoses: Idiopathic EOS(8), Neuromuscular scoliosis(5), Syndromic(5), Congenital(1). The average interval between each distraction was 109.8days (88.2–140.6). The overall mean distraction was 1.66mm (0.6–3.427). The mean distraction achieved for the concave rod was 1.69mm (0.6–3.03) as against 1.59mm (0.2–3.427) for the convex rod. The degree of distraction achieved per session was the greatest in patients with neuromuscular scoliosis 2.79mm (0.94–4.62), while it was the least in Congenital scoliosis 0.99mm (0.8–2.93). The degree of distraction in Syndromic and Idiopathic scoliosis groups were 2.19mm (0.1–4.2) and 1.50mm (0.2–4.45) respectively. The mean distraction achieved during the first session after MCGR instrumentation was 2.82mm (0.4–9.8) as against 1.98mm (0.1–4.2) and 1.18mm (0.2–2.3), achieved during the 5. th. and 10. th. distractions respectively. CONCLUSIONS. The average distraction of the MCGR rod achieved per session depends upon the etiological diagnosis of EOS. Neuromuscular curves are the most amenable to MCGR distractions, while the congenital curves are the least. The degree of distraction achieved progressively diminishes with each successive distractions. The distraction achieved slumps to 70% of the initial distraction by the 5. th. session and further declines to 41% of the initial distraction by the 10. th. session. TAKE HOME MESSAGE. The distractibility of the MCGR rod is closely linked to the etiology of EOS. MCGR rods are most effective in cases of neuromuscular scoliosis, while least effective in congenital scoliosis. The law of diminishing returns holds true even with MCGR rods


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 198 - 198
1 Mar 2003
Elsebaie H Basu P Noordeen M
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Background: The incidence of intra-spinal abnormality in congenital scoliosis is high. McMaster et al found an 18% incidence of myelographic abnormality in a series of 251 patients. Our objective was to report the MRI findings in a large series of patients with congenital scoliosis. Method: The notes, X-rays and MRI of 126 congenital scoliosis patients were reviewed to note the vertebral abnormality, curve progression, MRI findings and the presence of non-spinal congenital abnormality. These findings were then correlated to detect any association between them. Result: Forty-six patients (37%) had intra-spinal abnormalities detected on MRI. Sixty-six patients had failure of formation, 10 had failure of segmentation, 34 had mixed vertebral anomaly and 16 had congenital kyphosis or dislocation. MRI abnormality was significantly higher among patients with mixed anomaly (41%), congenital kyphosis (57%) and segmentation anomaly (40%) than those with failure of formation (29%). Presence of MRI abnormality did not correlate with curve progression or the presence of congenital abnormality affecting other organs. Conclusion: Intra-spinal abnormality in congenital scoliosis occurred in 37% cases. The incidence of such abnormality is higher in patients with congenital kyphosis, failure of segmentation and mixed vertebral anomalies


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 4 | Pages 474 - 479
1 Apr 2008
Tsirikos AI Howitt SP McMaster MJ

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 231 - 231
1 May 2006
Tsirikos A McMaster M
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Background: Congenital anomalies of the chest wall and Sprengel’s shoulder are often associated with congenital deformities of the spine. It has been suggested that extensive rib fusions on the concavity of a congenital scoliosis may adversely affect progression of the spine deformity, thoracic function and growth of the lungs, which can lead to a thoracic insufficiency syndrome. Methods: This is a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital spine deformities. The rib anomalies were classified into simple and complex and the presence of a Sprengel’s shoulder was recorded. Subjects: There were 497 patients (80%) with scoliosis, 88 patients (14%) with kyphoscoliosis, and 35 patients with kyphosis (6%). The mean rate of scoliosis deterioration without treatment, age and curve size at surgery was compared for the different types of vertebral abnormalities in patients with and without rib anomalies. Results: Rib anomalies occurred in 124 patients. The rib anomalies were simple in 97 patients (78%) and complex in 27 (22%). The most common simple rib anomaly (70 patients; 72%) was a fusion of two or three ribs. The most common complex rib anomaly (20 patients; 74%) was a fusion of multiple ribs associated with a large chest wall defect. Rib anomalies were most commonly associated with a congenital scoliosis (116 patients; 93.6%), and much less frequently with a congenital kyphoscoliosis or kyphosis (8 patients). In those patients with a scoliosis, the rib anomalies were simple in 91 patients (78.5%) and complex in 25 patients (21.5%). Eighty-eight of the 124 patients (76%) with rib abnormalities had a scoliosis due to a unilateral unsegmented bar with or without contra-lateral hemivertebrae at the same level, and 8 patients had mixed unclassifiable vertebral anomalies. In contrast, only 17 patients with a scoliosis and rib anomalies had hemivertebrae alone. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (107 patients; 92.2%) and occurred on the concavity in 81 patients (70%), convexity in 27 patients (23%), and were bilateral in 8 patients. Sprengel’s deformity occurred in 45 patients; 43 patients with congenital scoliosis (8.6%), and 2 with kyphoscoliosis or kyphosis. A Sprengel’s shoulder occurred most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (28 patients). The elevated shoulder was on the concavity of scoliosis in 26 patients and on the convexity in 16 patients. We compared the mean rate of curve progression without treatment, the age and curve size at spine surgery for the different types of spine deformities in patients with and without rib anomalies either simple or complex and found no significant difference (p> 0.05); the only statistical difference was that the mean age at surgery was higher for patients with a unilateral unsegmented bar without rib anomalies (p=0.005). Conclusion: Congenital rib anomalies and Sprengel’s shoulder occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis due to a unilateral failure of vertebral segmentation. However, these anomalies do not have an adverse effect on curve size or rate of progression


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 198 - 198
1 Mar 2003
Elsebaie H Basu P Noordeen M
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Background: Surgical treatments described for congenital spinal deformity are i) convex growth arrest, ii) posterior or combined anterior and posterior fusion and iii) hemivertebrectomy. Posterior instrumentation is used as an adjunct to fusion, whenever possible. Anterior instrumentation for correction of congenital scoliosis has not been described. A preliminary report of the use of anterior instrumentation following hemi-vertebrectomy for correction of congenital spinal deformity is reported. Method: 15 patients with congenital scoliosis and 5 patients with congenital kyphosis underwent hemiverte-brectomy and anterior instrumentation with fusion for single-stage correction of deformity . The average age of the patients at the time of surgery was 31 months and at last follow-up 59 months. All patients had pre-operative MRI. Twelve patients had normal and 8 had abnormal MRI. The average operating time was 135 minutes and average blood loss was 462 ml. Implants used were downsize Synergy, Orion Colorado and AO Cervifix. Average sagittal Cobb angle for the scoliosis patients was 45.5° pre-operatively and 16.8° post-operatively. Average coronal Cobb angle in patients with congenital kyphosis was 61° pre-operatively and 21° postoperatively. At an average follow-up of 17 months, the correction is well maintained in all except one. This patient developed pseudarthrosis at 19 months post-operatively. This was treated with posterior instrumented fusion. There were no cases of neurologic compromise or deep wound infection. Conclusion: Because of the young age at which hemiver-tebrectomy is performed in congenital scoliosis patients, instrumentation is difficult. Posterior instrumentation has been well described in literature. Our early experience with anterior instrumentation after hemivertebrectomy shows promising results with very good correction of the deformity and no increase in complication rate


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_III | Pages 450 - 450
1 Aug 2008
Kiely P Steele N Schueler A Breakwell L Medhian S Grevitt M Webb J Freeman B
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Study design: A retrospective review of patient records with recent clinical and radiographic assessment. Objective: Long-term evaluation of the Luque trolley for posterior instrumentation in congenital scoliosis. Summary of background data: From a group of 51 cases treated with the Luque trolley, we review 10 patients with progressive congenital scoliosis (5male, 5female) for a mean follow-up period of 14.8 years, to mean age of 19 years. The mean Cobb angle of the primary curve before surgery was 69.5 degrees. The mean Cobb angle of the secondary cervico-thoracic curve before surgery was 37.1 degrees and of the caudal secondary curve was 26.4 degrees. The mean age at surgery was 5.0 years. 8 patients had a selective epiphysiodesis procedure, 2 with hemi-vertebrectomy, and all underwent single- stage (7 patients) or dual-staged (3 patients) posterior instrumentation with a Luque trolley growing construct. Method: Clinical evaluation and sequential measurements of Cobb angle were done, with recording of further surgical procedures, associated complications, and final coronal balance. The thoracolumbar longitudinal spinal growth (T1-S1) and growth in the instrumented segmented were also calculated. Results: The mean preoperative primary curve Cobb angle of 69.5degrees, corrected to a mean postoperative angle of 30.6 degrees, with progression from here to curve magnitude of 38.8 degrees on latest follow up (approximate rate of progression of 0.55 degrees per year). The mean pre-operative cephalic (cervico-thoracic) Cobb angle of 37.1degrees, corrected to 22 degrees, with progression to 26.6 degrees. The mean pre-operative caudal (lumbar) Cobb angle of 26.4degrees, corrected to16.2 degrees, this later progressed to 20.6 degrees. Coronal plane translation measured 1.68 cm at latest follow up [range 0.5–5.1cm]. The thoracolumbar longitudinal growth measured a mean of 8.81cm (approx0.8 cm/year) with a recorded lengthening of 2.54 cm (approx 0.23cm/year) in the instrumented segmented. Half the patients did not require further surgery. Conclusion: Selective fusion does not always prevent further deformity in congenital scoliosis. The addition of posterior growing construct instrumentation did demonstrate capacity for good correction of primary and secondary curvatures and a limited capacity for further longitudinal growth


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 7 | Pages 950 - 955
1 Jul 2012
Helenius I Serlo J Pajulo O

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful procedure


The Journal of Bone & Joint Surgery British Volume
Vol. 57-B, Issue 4 | Pages 422 - 429
1 Nov 1975
James JIP

This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_III | Pages 481 - 481
1 Aug 2008
Suzuki N Iida T
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Cervico-thoracic congenital scoliosis is a difficult deformity to obtain good correction due to its anatomical characteristics and lack of proper instrumentation. Surgical treatments often end up with poor correction by convex epiphysiodesis alone, making hideous residual head tilt. This is a report of 2 cases with cervico-thoracic congenital scoliosis, which underwent total excision of hemivertebra, instrumentation and fusion through posterior approach alone. Case 1. 8y2m old Girl who had T1, T3, T7 hemivertebrae with a left convex curve from C7 to T11. At age 5. she had tilted head and left convex 33 degrees scoliosis. Only regular observation was done. At age 8y2m, the scoliosis had progressed to 49degrees. Total excision of T1 hemivertebra was performed. At age 10y8m, total excision of T7 hemivertebra, extension of instrumentation and fusion to T10 was performed. These procedures brought almost normal alignment on both sagittal and coronal plane. However, lower compensatory curve progressed later on, fusion was extended to L2 at age 13 resulting in excellent balance. Case 2: Girl. 2y7m. Multi-level hemivertebrae. C6-L1 L100 degrees. Total excision of T12 hemi, short fusion and instrumentation reduced the scoliosis to 50 degrees. Five months later, total excision of T9 hemi was done. Four months later, concave side instrumentation from T2 to L2 without fusion was done. At age 4y2m, total excision of T1 hemi was done using cervical pedicle screw. The scoliosis is being controlled at 35 degrees with one extension of the rod later on


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_6 | Pages 114 - 114
1 Jul 2020
Thompson G Hardesty C Son-Hing J Ina J Tripi P Poe-Kochert C
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Intrathecal morphine (IM) is a common adjunct in paediatric spinal deformity surgery. We previously demonstrated with idiopathic scoliosis it provides safe and effective analgesia in the immediate postoperative period. This study represents our 25 year experience with IM in all diagnostic groups. Our prospective Pediatric Orthopaedic Spine Database (1993–2018) was reviewed to identify all patients undergoing spinal deformity surgery who received IM and who did not. Patients 21 years of age or less who had a posterior spinal fusion (PSF) with segmental spinal instrumentation (SSI), and received 9–19 mcg/kg (up to 1 mg) of IM were included. Early onset scoliosis surgical patients were excluded. We assessed demographics, pain scores, time to first dose of opioids, diagnoses, surgical time, paediatric intensive care unit (PICU) admission and IM complications (respiratory depression, pruritus, nausea/ vomiting). There were 986 patients who met inclusion criteria. This included 760 patients who received IM and 226 who did not. IM was not used for short procedures (< 3 hrs), respiratory concerns, unsuccessful access of intrathecal space, paraplegia, and anesthesiologist decision. Both groups followed the same strict perioperative care path. The patients were divided into 5 diagnostic groups (IM / non IM patients): idiopathic (578/28), neuromuscular (100/151), syndromic (36/17), and congenital scoliosis (32/21) and kyphosis (14/9). Females predominated over males (697/289). The first dose of opioids after surgery was delayed for a mean of 10.6 hrs in IM group compared to 2.3 hrs in the non-IM group (p=0.001). The postoperative pain scores were significantly lower in the IM groups in the Post Anesthesia Care Unite (p=0.001). Only 17 IM patients (2%) were admitted to the PICU for observation secondary to respiratory depression, none required re-intubation. None of the IM group were re-intubated. Forty-nine patients (6%) experienced pruritus in the IM group compared to 4 of 226 patients (2%) in the non IM group. There were 169 patients (22%) of the IM patients and 21 patients (9%) of the non IM had nausea and vomiting postoperatively. Three patients (0.39%) had a dural leak from the administration of IM but did not require surgical repair. There were no other perioperative complications related to the use of IM. There were no significant group differences. Pre-incision IM is a safe and effective adjunct for pain management in all diagnostic groups undergoing spinal surgery. The IM patients had lower pain scores and a longer time to first administration of post-operative opioids. Although there is an increased frequency of respiratory depression, pruritus, and nausea/vomiting in the IM group, there were no serious complications


Orthopaedic Proceedings
Vol. 87-B, Issue SUPP_II | Pages 109 - 109
1 Apr 2005
Dohin B Filipeti P Vernet P
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Purpose: The risk of injuring the radial nerves during spine instrumentation to correct spinal deformity is well known and accounts for about 50% of the neurological complications associated with this type of surgery. We describe a technique for monitoring the nerve roots during spinal surgery. Radicular monitoring was described by Hormes in 1993. Material and methods: We report a retrospective analysis of 73 procedures for spinal deformity during which the nerve roots were monitored. The series included 27 men and 46 women, mean age 23.9 years (range 4.5–74.9). Forty patients were less than 18 years old. Procedures included posterior arthrodesis (n=65) and anterior arthrodesis (n=8). Indications were: idiopathic scoliosis (n=32), neurological scoliosis (n=21), congenital scoliosis (n=4), spondylolisthesis (n=2) and kyphosis (n=3). The study group included 68 patients (168 roots) with recordings obtained under the required conditions. The routine procedure involved permanent electrophysiological monitoring of muscle activity with a multi-channel electromyograph. We used microwires implanted within the muscle itself for electrodes. Target muscles depended on the position of the planned implants and the topography of the roots likely to be endangered during the surgical procedure or instrumentation. Explored roots were: T12 (n=9), L1 (n=24, L2 (n=40), L3 (n=24), L4 (n=23), L5 (n=11), S1 (n=22). Monitoring prohibited use of curare during anaesthesia. Results: Prior to radicular monitoring, we had had two root injuries (T12 and L3) which resolved spontaneous (n=139). During the study, changes in the radicular signal were observed in seven patients. All signal anomalies triggered a modification of the surgical procedure and no postoperative deficit was observed. Incidents observed concerned congenital scoliosis (n=2), neurological scoliosis (n=2), and idiopathic scoliosis (n=3). Roots involved were L1 (n=1), L2 (n=2), L3 (n=2), L4 (n= 4), i.e. 11/163. Discussion: Continuous intraoperative monitoring of the spinal roots exposed to surgery for spinal deformity enabled us to identify eleven cases of root suffering among 163 recordings. This permanent monitoring system enabled us to immediately modify the surgical procedure and to control and conflict between the instrumentation and the roots or possible stretching during the correction. This technique requires permanent monitoring during the spinal procedure to avoid false negatives. Curare cannot be used. Conclusion: Intraoperative radicular monitoring is an effective way to avoid radicular complications of this type of surgery. The technique is sensitive and allows immediate adaptation of the surgical procedure. It requires close collaboration between the neurophysiologist, the orthopaedic surgeon and the anaesthesiologist


Orthopaedic Proceedings
Vol. 87-B, Issue SUPP_II | Pages 185 - 185
1 Apr 2005
La Rosa G Crostelli M
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There can be no doubt that bone morphogenetic proteins play a hierarchic role in the osteogenetic cascade. Pre-clinical and clinical trials have confirmed their decisive role in achieving anterior lumbar fusion, as they direct mesenchymal stem cells toward osteoblastic lineages.The present study is concerned with initial experience in the application of autologous mesen-chymal stem cells and various growth factors (BMP-7,VEGF,TGFbeta) in the treatment of paediatric spinal pathologies. Eleven patients affected by serious forms of congenital infantile scoliosis, idiopathic scoliosis and grade I spondylolisthesis received surgical treatment. In three patients with congenital infantile scoliosis, ages ranging from 3 to 12 years, the surgical procedure was an anterior and posterior fusion at the level of the hemiver-tebra, extending it to a level above and below it by means of in situ decortication of the vertebral plates and laminae on the convex side and delivery of stem cells taken from the iliac bone and applied in situ by means of bovine collagen sponge (Healos system). No fixation device was added. Plaster and brace were used during the postoperative course for 9 months. In two cases of intertransverse in situ fusion for grade I spondylolisthesis the age of the patients was 13 and 16 years, respectively, and the surgical procedure consisted in the standard technique to which was added delivery of a mixture composed of small bone chips obtained from decortication, 3.5 mg of eptotermin alpha (Op-1, BMP7), and autologous stem cells taken from iliac bone. A special form of informed consent was obtained for these two patients because of their incomplete bone maturity. TLSO was used in the postoperative course for 2 months. In the six patients with idiopathic scoliosis, ages ranged from 13 to 15 years and the treatment consisted in posterior instrumentation and fusion by means of rods, transpedicular screws and hooks; standard fusion techniques were supported by local bone chips obtained from decortication, placed on collagen sponges and combined with autologous stem cells taken from iliac bone with the addition of platelet gel derived from the autologous preoperative blood collection. No bone chips were taken from iliac wing. The results were evaluated by X-rays and CT at intervals of 1, 2, 4, 6 and 12 months. In the cases of congenital scoliosis a solid fusion area was obtained only for posterior hemiephysiodesis, without a parallel bone signal of fusion at the anterior level. In the cases of intertransverse in situ fusions for spondy-lolisthesis there was a beginning fusion already visible on the first X-ray control 1 month postopoeratively, confirmed at successive check-ups and maintained in the follow-up. The cases of idiopathic scoliosis showed an initial ossification of the grafts and signals of fusion at the 6-month check-up. The isolated use of stem cells, although promising from a theoretical point of view, did not prove encouraging in the cases of anterior fusion. It is most probable that the absence of instrumentation induced a defect in the stable fixation of the fused segments, the latter being a crucial factor.The cases of in situ fusion for spondylolisthesis confirm the hierarchic role of the bone morphogenetic protein 7 in inducing the mesenchymal stem cells, released in situ from decortication, toward osteoblastic lineage. To our knowledge these represent the first two cases of use of OP-1 in patients younger than 18 years. In the fusion areas which are more extensive in length (idiopathic scoliosis) the added use of autologous stem cells mixed with platelet gel seems to improve the physiological processes of fusion. It will be necessary to monitor the long-term results of these procedures with special regard to loss of correction and weakness of the fusion area causing torsional or flexion-extension stress. These possible costs have to be compared with the verified benefits of a better use of blood in its capacity to save on corresponding haemotransfusion, combined with the absence of comorbidity related to the donor site of iliac bone chips