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Purpose: Lumbar kyphosis occurs in approximately 8-20% of patients with myelomeningocele (MMC). The purpose of this article is to analyze the risks and benefits of vertebrectomy and spinal stabilization in MMC children with severe lumbar kyphosis and to establish treatment guidelines., Methods: This is an IRB-approved retrospective analysis of 59 patients with MMC who underwent kyphectomy and posterior instrumentation in three centers. Average age at surgery was 7.9 years (2 weeks-17 years). Sitting trunk position, skin status, kyphosis angle, and thoracic lordosis were analyzed preoperatively, postoperatively, and at an average follow-up of 8.2 years (range 2.5-16). The correction was maintained by applying a short posterior instrumentation in 6 patients, and extending to the pelvis in 53 cases. Pelvic fixation was achieved using the Warner and Fackler technique in 24 patients, the Dunn-McCarthy in 8, Luque-Galveston in 8, sacral screws in 2, and ilio-sacral screws in 11., Results: Sitting position improved postoperatively in 47 of the 53 patients who underwent pelvic fixation and only in one patient with short instrumentation. All 6 patients with long instrumentation and poor postoperative sitting balance were in the Dunn-McCarthy fixation group. Skin sores at the apex of the deformity disappeared postoperatively in all patients but recurred in two patients with short instrumentations. Kyphosis angle improved from 109° (45°-170°) preoperatively to 10° (0°-45°) postoperatively and 21° (0°-55°) at last follow-up. The best results were seen in cases where a cross-k-wire fixation of the kyphectomy site was used, augmented with a long thoraco-pelvic instrumentation consisting of Luque sublaminar wires in the thoracic region and a Warner-Fackler type of pelvic fixation. Good results were also found with the bipolar technique and ilio-sacral screw fixation. Six over 24 patients with the Warner and Fackler technique showed gradual dislodgment or hardware failure, with subsequent nonunion of the kyphectomy site in four. Infection, with or without wound dehiscence and/or hardware exposure, occurred in 17 cases, necessitating hardware removal in 9 patients., Conclusion: Lumbar kyphosis in MMC children is best managed by resection of enough vertebrae from the apex to produce a flat lumbar spine, with perfect bone-to-bone contact and long thoraco-pelvic instrumentation using the Warner and Fackler technique through the S1 foramina or the bipolar technique with ilio-sacral screw fixation. Additional local fixation of the osteotomy site using cross-wires with or without cerclage increases the stability of the construct. The majority of complications occurred in patients with short instrumentations or where residual kyphosis persisted postoperatively regardless of the type of pelvic fixation or hardware density. The Dunn-McCarthy technique for pelvic fixation following kyphectomy in MMC was less successful in producing stable pelvic fixation and should not be considered in this patient category., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Back pain occurs commonly in adults and is multifactorial in nature. This study aimed to assess the prevalence and intensity of back pain during young adulthood in subjects with adolescent idiopathic scoliosis (AIS), as well as factors that may be associated with its prognosis., Methods: Subjects with AIS aged 20-39 treated conservatively were included in this study. Patient-reported outcome measures in adulthood involved episodes of back pain, and scales of self-image, depression, anxiety, and stress. Additionally, pain, self-image, and mental health scores were retrieved at the first clinic consultation. Occurrence of back pain was defined as a numeric pain rating scale ≥ 6., Results: 101 participants were enrolled. The prevalence of back pain in the lifetime, past 12 months, past 6 months, past 1 month, past 7 days, and past 24 h were 37%, 35%, 31%, 27%, 23%, and 20%, respectively. Male, self-image, and depression were significant associated factors for the development of back pain at all time points. Furthermore, the analyses of the initial presentation of participants have shown that participants with back pain in adulthood were characterised by poor self-image and mental health during their adolescence., Conclusion: The present study addressed the natural history of back pain in young adults with conservatively treated AIS. Psychological makeup has been shown to constitute the development of back pain and is strongly hinted as an early sign of having back pain in adulthood among subjects with AIS., (© 2023. The Author(s).)

Purpose: It has been suggested that the cause of the balance disorder seen in adolescent idiopathic scoliosis (AIS) originates from the central nervous system. However, the extent of the balance problem and the dysfunction of which part of the central nervous system has not been investigated in detail. This study aimed to correlate the values obtained by balance analysis and cerebellum volume measurement in female individuals with AIS with healthy individuals., Methods: Cerebellum volume was calculated via the cloud-based software " https://volbrain.upv.es " using brain magnetic resonance images of 27 healthy and 26 individuals with AIS. The duration of stay in the test positions, the movement strategy used during this time and the amount of postural sway were analyzed by using a computer-assisted force platform and compared statistically., Results: Significant differences were found between the AIS and control groups in cerebellum total volume, vermis cerebelli volume (cm 3 ), and trunk oscillation velocity (mm/s) parameters (p < 0.05). Cerebellum and vermis cerebelli volumes were found to be lower and trunk oscillation velocity was found to be greater in patients with AIS., Conclusion: Balance problems in patients with AIS are correlated with decreased cerebellum volume and increased trunk oscillation velocity., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: There is still no consensus on the optimum pedicle screw density required for the desired thoracic kyphosis restoration in adolescent idiopathic surgery (AIS). The aim of this study to evaluate the effect of pedicle screw density on thoracic kyphosis restoration in AIS surgery., Methods: The data of 106 patients from two centers that operated for Lenke type 1 and 2 AIS were retrospectively reviewed. Two groups were constituted according to the pedicle screw density: intermittent pedicle screw constructs (IPSC) (n = 52 patients) and consecutive pedicle screw construct (CPSC) (n = 54 patients) groups. The preoperative and at least 24-month follow-up radiographs and SRS-22 scores were evaluated. The Cobb angle of the main and concomitant curves in the coronal plane and the sagittal plane were measured and compared., Results: The mean follow-up period for the IPSC and CPSC groups was 72.3 ± 37.2 and 62.9 ± 28.8 months, respectively. In the SRS-22 questionnaire, there was no significant difference between the two groups in terms of self-image/appearance domain scores (p = 0.466), but better results were obtained in the IPSC group in terms of treatment satisfaction domain scores (p = 0.010) and better thoracic kyphosis restoration was achieved in IPSC group radiologically for Lenke type 1 curves with - 81.4 ± 81.4% in the IPSC group and 6.8 ± 83.8% in the CPSC group (p < 0.001)., Conclusion: It was considered that better thoracic kyphosis restoration could be achieved with the less lordotic effect of IPSC in Lenke type 1 curves. Although the current situation had a significant impact on radiological outcomes, its effect on SRS-22 scores was limited., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Adolescent idiopathic scoliosis (AIS) is believed to be caused by genetic, neurological, osseous growth anomalies, histological variables including muscle fiber percentage and core structure changes, metabolic and hormonal dysfunction, vestibular dysfunction, and platelet microarchitecture. The objective of this study was to contribute to the determination of the cause of AIS by analyzing the changes in pineal gland volume in AIS cases., Methods: Study (AIS) and control group were each comprised of 26 patients who met the inclusion requirements. Scoliosis radiograph and MRI of the pineal glands were used for radiological examinations. The distribution of age, gender, Risser grading for skeletal radiological development, and sexual maturation according to Tanner categorization were uniform and statistically insignificant between groups., Results: When the pineal gland volumes of the cases were evaluated according to age, the AIS group was found to have significantly reduced pineal gland volumes in all age groups. The pineal gland volume was found to be 38.1% lower in the AIS group compared to the control group (p˂0.001). In the AIS group, patients aged 13 years had the lowest pineal gland volume (77.2 ± 13.86 mm 3 ), while patients aged 15 years had the highest volume (97.9 ± 16.47 mm 3 )., Conclusion: Changes in pineal gland volume support the role of the pineal gland in the etiopathogenesis of AIS., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To describe the process and outcome of vertebral column resection (VCR) at the subapical vertebra for correction of angular kyphosis associated with neurofibromatosis type 1(NF1)., Methods: A review and summary of the medical history, radiographs, operative procedure, and complications of a 16-year-old male presenting with severe angular kyphosis associated with NF1 with dyspnea., Results: A 16-year-old male presented with severe angular kyphosis associated with NF1 with dyspnea. Preoperative radiographs demonstrated multiple vertebrae were rotated in the vicinity of the apical vertebra, with a wedge-shaped deformity, dysplasia, T10-T12 kyphotic angle of 160°, and T2-L2 kyphotic angle of 95°. VCR at the L1 vertebra (distal end of the apical vertebra) with bone grafting and internal fixation was performed. Postoperative imaging revealed that the T2-L2 Cobb angle was 20°, denoting a correction rate of 79%. The patient's height increased from 130 to 150 cm. The position of internal fixation was not displaced, and the correction angle was maintained at 2-year follow-up., Conclusions: The novel strategy of performing VCR at the subapical vertebra, with posterior displacement of the distal end, and remodeling of the spinal canal is potentially a safe and efficacious option to correct sharp angular kyphosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To present a case of delayed aortic perforation due to a nondisplaced fracture of the 9th rib after vertebral osteotomy for degenerative kyphoscoliosis in patients with osteoporosis., Methods: A 78-year-old female patient with osteoporosis had undergone T9-iliac correction surgery for degenerative kyphoscoliosis. After 2 years, the patient underwent T10 pedicle subtraction osteotomy for a T10 vertebral fracture and progression of kyphosis. Postoperatively, the patient had been doing well for 3 weeks; however, just before the day of discharge, she died following a cardiopulmonary arrest. An autopsy was performed with the consent of her family., Results: Autopsy revealed a large amount of blood and a clot in the left thoracic cavity. Aortic perforation was found just in front of a nondisplaced fracture of the left 9th rib., Conclusion: This report describes a new critical complication after spinal correction surgery. Even without pedicle screw malposition, aortic injuries can happen to patients with osteoporosis after corrective osteotomy for degenerative kyphoscoliosis due to positional change of aorta and fragility of the ribs. The spine surgeon should be aware of this type of complication, and rib fractures around the aorta after vertebral osteotomy should not be neglected even when there is no displacement., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Surgical site infection (SSI) is a major complication after adolescent idiopathic scoliosis (AIS) surgery, with an incidence ranging from 0.5 to 7%. Intraoperative wound decontamination with povidone-iodine (PVP-I) irrigation and/or vancomycin powder in adult spinal surgery has gained attention in the literature with controversial results. The aim of this study was to investigate the impact of using intrawound PVP-I irrigation and local vancomycin powder (LVP) on the incidence of early SSI in AIS surgery., Methods: All AIS patients who underwent posterior spinal fusion between October 2016 and December 2019 were retrospectively reviewed. The incidence of early SSI was reported and compared between 2 groups defined by the treating spinal surgeons' preferences: group 1-intrawound irrigation with 2L of PVP-I and application of 3 g LVP before closure and control group 2-patients that did not receive either of these measures., Results: Nine early cases of SSI (2.9%) were reported among the 307 AIS posterior spinal fusion patients. Incidence of SSI in group 1 (2/178 = 1.1%) was significantly lower than in group 2 (7/129 = 5.4%; p = 0.04). There were no adverse reactions to the use of PVP-I and LVP in our study. At latest follow-up, rate of surgical revision for mechanical failure with pseudarthrosis was significantly lower in group 1 (2/178 = 1.1%) than in group 2 (9/129 = 7.0%; p = 0.01)., Conclusion: Intraoperative use of intrawound PVP-I irrigation and vancomycin powder is associated with a significant reduction of early SSI after AIS spine surgery., Level of Evidence Iv: Retrospective study., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To report on the phenomenon of body drift in neurofibromatosis scoliosis and discuss its implication on surgical safety., Materials and Methods: Ten dystrophic neurofibromatosis scoliosis (NF) and ten adolescent idiopathic scoliosis (AIS) were studied by radiographs, CT, and MRI. The curve characteristics and a detailed analysis of the morphology of the apical and three adjacent vertebral segments above and below were done. The coronal alignment and the presence of a drift of the vertebral body in relationship to the lamina were carefully studied in both groups and compared., Results: The mean cobb angle in the NF group was 77.6°, and 63.7° in the AIS group. All the studied vertebra in the NF group had extensive pedicle changes, which were more severe at the apical and periapical regions. Body drift was noted in 29 vertebral segments, with 9/10 of apical segments showing a significant drift. The body drift was associated with significant pedicle dystrophic changes and was independent of the curve magnitude. In comparison, in AIS, no body drift was noted despite a larger deformity and more severe vertebral rotation., Conclusion: The 'body drift' phenomenon was unique to neurofibromatosis scoliosis and was secondary to severe pedicle morphology changes. This was present even in curves less than 60° and could result in cord injury while instrumenting the concave pedicle. Therefore, a thorough preoperative assessment and planning by a 3D CT are mandatory., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The objective of this retrospective study was to provide the radiographic outcomes and complications for pedicle subtraction osteotomy (PSO) performed at the low lumbar spine, i.e., L4 or L5 for ASD patients with fixed sagittal malalignment., Methods: ASD patients who underwent L4 or L5 PSO with a minimum 2-year follow-up were included. Preoperative and postoperative radiographs, and complications were collected. Radiographic analysis included lumbar lordosis (LL), pelvic incidence (PI), pelvic tilt (PT), thoracic kyphosis (TK), sagittal vertical axis (SVA), spinal lordosis (SL) ratio and global tilt (GT) on standing long-cassette radiographs., Results: A total of 102 patients from 2 spinal centers were analyzed. 66 patients underwent PSO at L4 and 36 patients at L5. From preoperatively to the final follow-up, significant improvements occurred in LL (from - 31° to - 52°), SVA (from 13 to 5 cm), and GT (from 44° to 27°) (all, p < 0.05). 12 patients had transient neurological deficits, and 8 patients had persistent neurological deficit. 23 patients underwent revision for PJK (2), pseudarthrosis (10), neurological deficit (2), epidural hematoma (1), or deep surgical site infection (8). No PJK was observed in any of the patients with L5 PSO., Conclusions: PSO at the level of L4 or L5 remains a challenging technique but with an acceptable rate of complications and revisions. It enables correction of fixed sagittal malalignment in ASD patients with a globally satisfactory outcome. In comparison with L4 PSO, L5 PSO patients did not show PJK as a mechanical complication. Distal lumbar PSO at the level of L5 may represent one of the factors that may help preventing the proximal junctional kyphosis complication., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To clarify the relationship between sarcopenia and spinopelvic parameters., Methods: Among outpatients of spine surgery department, 126 patients (mean age 77.2 years. M/F = 71/55) were included. We diagnosed patients with sarcopenia using the diagnostic algorithm of the Asian Working Group for Sarcopenia. Spinopelvic parameters and the prevalence of spinopelvic mismatch (pelvic incidence minus lumbar lordosis ≥ 10°) were investigated and compared between patients with and without sarcopenia. Furthermore, we compared the spinopelvic parameters between the Sarcopenia and No Sarcopenia groups under each condition of spinopelvic match and mismatch., Results: The prevalence of sarcopenia in this study was 21.4%. Overall, the spinopelvic parameters except thoracic kyphosis (TK) (Sarcopenia: 34.7°, No Sarcopenia: 24.3°, p < 0.01) were not significantly different between the Sarcopenia and No Sarcopenia groups. Prevalence of patients with spinopelvic mismatch was also not significantly different between the Sarcopenia and No Sarcopenia groups (37.0% vs. 42.4%, p = 0.66). Among patients without spinopelvic mismatch, there was no spinopelvic parameter with a significant difference between the 2 groups. However, among patients with spinopelvic mismatch, sagittal vertebral axis (SVA) (115.7 mm vs. 58.7 mm, p < 0.01) and TK (36.6° vs. 21.3°, p < 0.01) of the Sarcopenia group were significantly larger than those of the No Sarcopenia group. Moreover, sarcopenia was independently related to a significant increase in SVA (β = 50.7, p < 0.01) and TK (β = 14.0, p < 0.01) in patients with spinopelvic mismatch, after adjustment for age., Conclusions: Sarcopenia is related to spinal sagittal imbalance because of insufficient compensation by flattening thoracic kyphosis in patients with spinopelvic mismatch. These slides can be retrieved under Electronic Supplementary Material.

Background: Several reports in the literature have suggested a causative association between oral occlusion and spinal deformity such as scoliosis and Scheuermann's disease or kyphosis. Based on these findings, a growing number of adolescents with spinal deformity receive orthodontic treatment, supposing a beneficial effect on the spine., Objective: The aim of this study was to verify the association between spinal deformity and malocclusion in the orthopedic population and potential effect of orthodontic treatment on the spinal deformity., Method: The databases: MEDLINE, CINAHL, EMBASE, Cochrane Register, OTseeker and ScienceDirect were searched up to August 2017 for studies reporting on associations between spinal and occlusal conditions. Case series, cohort, case-control studies and randomized clinical trials were considered for analysis. Two reviewers independently selected studies, conducted quality assessment and extracted results. Methodological quality was assessed using MINORS score., Results: Nine publications reporting on 1424 patients were included. Studies were two case series, five case-control studies, one cohort study and one randomized clinical trial. The methodological quality was poor in 8/9 studies., Conclusion: Evidence from three low-quality studies suggests an increased prevalence of occlusal dysfunction in patients with known spinal deformity, but the conclusions have a high risk of bias. No evidence of beneficial effects of orthodontic treatment on spinal deformity was found. These slides can be retrieved under Electronic Supplementary Material.

Introduction: Proximal junctional kyphosis (PJK) is a frequent complication, up to 46%, in adolescent idiopathic scoliosis surgical treatment (AIS). Several risk factors have been evoked but remain controversial. The purpose of this study was to analyze the incidence of PJK in a multicenter cohort of AIS patient and to determine risk factor for PJK., Materials and Methods: Lenke I and II AIS patients operated between 2011 and 2015 (minimum of 2-years follow-up) were included. On fullspine X-rays, coronal and sagittal radiographic parameters were measured preoperatively, postoperatively and at final follow-up. Occurrence of radiological PJK corresponding to a 10° increase in the sagittal Cobb angle, measured between the upper instrumented vertebra (UIV) and UIV + 2, between postoperative and 2-years follow-up X-rays, was reported., Results: Among the 365 patients included, 15.6% (n = 57) developed a PJK and only 10 patients required a revision surgery. Preoperatively, PJK patients had significantly larger pelvic incidence (57° ± 13° vs. 51° ± 12°), larger lumbar lordosis (LL) (63° ± 12° vs. 57° ± 11°) and bigger C7 slope. Postoperatively (3 months), in the non-PJK group, thoracic kyphosis (TK) was increased and LL was not significantly different. However, postoperatively, in the PJK group, no significant change was observed in TK, whereas C7 slope decreased and LL significantly increased. There was also a postoperative change in inflection point which was located at a more proximal level in the PJK group. Between postoperative time and final follow-up, TK and LL significantly increased in the PJK group., Conclusion: PJK is a frequent complication in thoracic AIS, occurring 16%, but remains often asymptomatic (less than 3% of revisions in the entire cohort). An interesting finding is that patients with high pelvic incidence and consequently large LL and TK were more at risk of PJK. As demonstrated in ASD, one of the causes of PJK might be postoperative posterior imbalance that can be due to increased LL, insufficient TK or inflection point shift during surgery. These slides can be retrieved under Electronic Supplementary Material.

Purpose: Reciprocal mechanisms for standing alignment have been described in thoraco-lumbar deformity but have not been studied in patients with primary cervical deformity (CD). The purpose of this study is to report upper- and infra-cervical sagittal compensatory mechanisms in patients with CD and evaluate their changes post-operatively., Methods: Global spinal alignment was studied in a prospective database of operative CD patients. Inclusion criteria were any of the following: cervical kyphosis (CK) > 10°, cervical scoliosis > 10°, cSVA (C2-C7 Sagittal vertical axis) > 4 cm or CBVA (Chin Brow Vertical Angle) > 25°. For this study, patients who had previous fusion outside C2 to T4 segments were excluded. Patients were sub-classified by increasing severity of cervical kyphosis [CL (cervical lordosis): < 0°, CK-low 0°-10°, CK-high > 10°] and cSVA (cSVA-low 0-4 cm, cSVA-mid 4-6 cm, cSVA-high > 6 cm) and were compared for pre- and 3-month post-operative regional and global sagittal alignment to determine compensatory recruitment., Results: 75 CD patients (mean age 61.3 years, 56% women) were included. Patients with progressively larger CK had a progressive increase in C0-C2 (CL = 34°, CK-low = 37°, CK-high = 44°, p = 0.004), C2Slope and T1Slope-CL (p < 0.05). As the cSVA increased, there was progressive increase in C2Slope, T1Slope and TS-CL (p < 0.05) and patients compensated through increasing C0-C2 (cSVA-low = 33°, cSVA-mid = 40°, cSVA-high = 43°, p = 0.007) and pelvic tilt (cSVA-low = 14.9°, cSVA-mid = 24.1°, cSVA-high = 24.9°, p = 0.02). At 3 months post-op, with significant improvement in cervical alignment, there was relaxation of C0-C2 (39°-35°, p = 0.01) which positively correlated with magnitude of deformity correction., Conclusions: Patients with cervical malalignment compensate with upper cervical hyper-lordosis, presumably for the maintenance of horizontal gaze. As cSVA increases, patients also tend to exhibit increased pelvic retroversion. Following surgical treatment, there was relaxation of upper cervical compensation.

Purpose: In using posterior vertebral column resection (PVCR) to treat severe kyphoscoliosis, it is unavoidable to ligate and cut off several segmental arteries (SAs) of the spinal cord for exposure and hemostasis, but which would raise the neurological risks. The aim of this study is to explore the changes of intraoperative spinal cord monitoring (IOM) following ligating different numbers of SAs in PVCR., Methods: Twenty-one consecutive patients with severe kyphoscoliosis were included and treated by PVCR correction. In operation, according to ligate different numbers of SAs, the IOM changes were recorded, respectively. Examinations of the covariance between different numbers of SAs ligations and IOM changes were performed to reveal the effect to the spinal cord by SAs ligations., Results: In all the 21 cases, averaging 1.9 pairs of SAs were ligated. With the increased numbers of ligations, SSEP amplitudes and latencies were changed more obviously: from 1 to 3 pairs ligations, the mean decreased percentages of amplitudes were from 53.20 to 78.15%, the mean increased percentages of latency were from 1.23 to 1.40%, and the mean durations of decreased SSEP amplitudes were from 3.23 to 5.2 min; but without abnormal MEP changes. None occurred postoperative or delayed neurological deficit. Correlation analysis identified significant correlations between the number of SAs ligation and decreased percentage of SSEP amplitude (r = 0.945, P < 0.0001), and between the number of SAs being ligated and the duration of SSEP change (r = 0.945, P = 0.0002)., Conclusions: Following the increased number of SAs ligation, the amplitude of SSEP is decreased more obviously with a much longer duration of recovery and the risk to spinal cord will be increased greatly. In the PVCR correction on the basis of spinal shortening, the numbers of SAs ligations should be as less as possible for neurological safety.

Purpose: To evaluate the association between spinopelvic sagittal parameters and leg pain in patients with adult spinal deformity (ASD) after adjusting for demographic and surgical variables., Methods: A multicenter prospective ASD database (European Spine Study Group) was retrospectively reviewed. The characteristics (age, sex, body mass index, comorbidity, history of spine surgery, and radiographical coronal and sagittal parameters) of patients with preoperative and 6-month postoperative leg pain (PostLP; numerical rating scale score ≥5) were analyzed using univariate and multivariate analyses., Results: In this study, 204 patients (164 women and 40 men; mean age 53.1 years) were included. Fifty-three percent of the patients had preoperative leg pain (PreLP). The patients with PreLP had significantly worse sagittal parameters and less coronal Cobb angle than those with less leg pain; however, this association disappeared after adjustment for covariates. The PreLP of the ASD patients was successfully treated surgically in most cases; however, 24 % of the patients still had unexpected residual leg pain. Postoperative sagittal malalignment (sagittal vertical axis ≥40 mm, T1 sagittal tilt ≥0°, pelvic tilt ≥30°) was a significant risk factor of PostLP even after adjusting for covariates., Conclusions: Leg pain in patients with ASD was significantly associated with sagittal malalignment especially after surgical treatments. As these patients lose flexibility in the fused spinal segment, they can only depend on the remaining compensatory mechanisms below the pelvis (e.g., the hips and knees) to maintain a balanced posture. This may lead to a predisposition of these patients to postoperative leg symptoms related to spinal sagittal malalignment.

Purpose: It has been reported that the incidence of post-operative segmental nerve palsy, such as C5 palsy, is higher in posterior reconstruction surgery than in conventional laminoplasty. Correction of kyphosis may be related to such a complication. The aim of this study was to elucidate the risk factors of the incidence of post-operative C5 palsy, and the critical range of sagittal realignment in posterior instrumentation surgery., Methods: Eighty-eight patients (mean age 64.0 years) were involved. The types of the disease were; 33 spondylosis with kyphosis, 27 rheumatoid arthritis, 17 athetoid cerebral palsy and 11 others. The patients were divided into two groups; Group P: patients with post-operative C5 palsy, and Group NP: patients without C5 palsy. The correction angle of kyphosis, and pre-operative diameter of C4/5 foramen on CT were evaluated between the two groups. Multivariate logistic regression analysis was used to determine the critical range of realignment and the risk factors affecting the incidence of post-operative C5 palsy., Results: Seventeen (19.3 %) of the 88 patients developed C5 palsy. The correction angle of kyphosis in Group P (15.7°) was significantly larger than that in Group NP (4.5°). In Group P, pre-operative diameters of intervertebral foramen at C4/5 (3.2 mm) were significantly smaller than those in Group NP (4.1 mm). The multivariate analysis demonstrated that the risk factors were the correction angle and pre-operative diameter of the C4/5 intervertebral foramen. The logistic regression model showed a correction angle exceeding 20° was critical for developing the palsy when C4/5 foraminal diameter reaches 4.1 mm, and there is a higher risk when the C4/5 foraminal diameter is less than 2.7 mm regardless of any correction., Conclusions: This study has indicated the risk factors of post-operative C5 palsy and the critical range of realignment of the cervical spine after posterior instrumented surgery.

Objective: Compressive myelopathy in severe angular kyphosis is rare and challenging for surgical treatment. The goal of this retrospective study was to report a series of ten patients with compressive myelopathy in severe angular kyphosis and the results of surgical decompression and correction of kyphosis., Methods: Between 2010 and 2014, 10 patients were surgically treated for severe angular kyphosis with a progressive onset or a sudden onset of paraplegia in investigator group. In these ten patients (seven males and three females), the etiologic diagnosis included eight cases of congenital kyphosis and two of neurofibromatosis; the distribution of spine level was from C5 to T11; the duration from onset until surgery ranged from 1 to 120 months; follow-up ranged from 12 to 26 months (mean 18.5 months); the kyphosis angle of the patients ranged from 50° to 180°. Magnetic resonance imaging demonstrated the spinal cord thinning and compression at apex in most of patients. All patients underwent decompressive surgery by single-stage posterior vertebral column resection or both anterior corpectomy fusion and posterior fixation. Neurological status was evaluated using the ASIA impairment classification and the motor score., Results: Postoperatively, all patients had different kyphosis correction rate from 24 to 100 %. Nine patients showed neurological improvement; one patient showed no improvement. Among them, one sudden onset ASIA A adolescent paraplegic patient improved to ASIA E within 1 year of follow-up. One ASIA C adolescent paraplegic patients deteriorated neurologically to ASIA A after surgery and improved to ASIA D with 12-month follow-up., Conclusions: Compressive myelopathy in severe angular congenital kyphosis is usually occurred high incidence rate at apex of upper thoracic spine (T1-T4). The duration from onset of paraplegia until surgery and the severity of paraplegia before surgery are two key factors for neurological prognosis after surgery.

Background: It is known that sagittal compensating mechanisms are created for counteracting sagittal imbalance problems; however, they can sometimes be associated with incidents which affect the plan of management., Purpose: The purpose of this study was to report a case of the occurrence of common iliac vessel displacement into the intervertebral disc space following one of the spinal compensatory mechanisms., Material and Methods: The authors demonstrated this case by showing the patient history, physical examination, imaging studies, and treatment strategy as well as by reviewing some related literature., Results: An 81-year-old woman presented with a long history of low back pain with claudication. An upright plain radiograph and flexion-extension study demonstrated a progressive local thoracolumbar kyphosis and losing of lumbar lordosis with significant widening of the intervertebral disc space of L4-L5. An MRI scan and 3D volume rendering spiral computed tomography (3D-CT) revealed an abnormal content which was depicted as common iliac vessels inside the disc space of L4-L5. Consequently, a rare case of the occurrence of common iliac vessel displacement into the intervertebral disc space following one of the spinal compensatory mechanisms was reported., Conclusion: The occurrence of vascular displacement into the intervertebral disc space related to lumbar hyperextension, as a compensating mechanism, is a rare incident but can occur. Consequently, when this mechanism presents with abnormal widening of the intervertebral disc space, especially at the low lumbar level, it should raise surgeon's concern about the probability of vascular injury when performing a disc procedure. Thorough investigation with imaging studies and selecting the optimum surgical treatment are warranted.

Purpose: To investigate the causes of late-onset, progressive neurological deficits in patients with severe angular kyphosis caused by spondylitis secondary to tuberculosis (TB spondylitis)., Methods: From 2000 to 2011, 36 patients with severe angular kyphosis secondary to TB spondylitis (TB kyphosis) were enrolled in the study. All patients had late-onset, progressive neurological deficits. The causes of these deficits were classified with respect to the level of the causative lesion. Group A (n = 25, 69.4%) comprised patients whose neurological deficits resulted from the kyphosis itself. Patients in group B (n = 11, 30.6%) had developed neurological symptoms related to a lesion cephalad or caudal from the kyphosis. Surgical intervention was performed in 23 patients; 13 patients were treated conservatively. Clinical outcomes were evaluated using the American Spinal Injury Association (ASIA) impairment scale., Results: The late onset of neurological deficits was attributed to cord compression, pure cord distraction, stenosis, and instability above or below the level of the angular kyphosis. An improvement of the neurological symptoms at the cord level after surgical intervention, as indicated by a change from a non-ambulatory (ASIA impairment scale A/C) to an ambulatory (ASIA D/E) status, occurred in four of nine (44.4%) surgically treated patients. However, only 2 of 10 (20.0%) patients treated conservatively showed cord level improvement, as assessed using the ASIA impairment scale., Conclusions: In their evaluation of paraplegic patients, spine surgeons should consider the many potential causes of late-onset neurological deficits in TB spondylitis to avoid performing unnecessary surgery. A simpler procedure may yield equivalent results.

Purpose: To evaluate the outcome and complications of anterior release, posterior internal distraction and subsequent posterior spinal fusion for the treatment of severe kyphoscoliosis., Methods: The medical records and spinal imaging of 17 consecutive severe kyphoscoliosis patients who underwent anterior release, posterior internal distraction and subsequent posterior spinal fusion were reviewed., Results: Patients underwent spinal deformity correction at the mean age of 22.6 ± 8.4 years (range 15-44 years) with mean follow-up of 37.4 ± 12.0 months (range 24-60 months). The preoperative thoracic kyphosis of 83.5° ± 16.3° (range 60°-115°) was corrected to 37.5° ± 7.9° (range 20°-49°) at the final follow-up. The preoperative major curve of 110.1° ± 16.8° (range 92°-145°) was corrected to 33.5° ± 13.9° (range 12°-63°) at the final follow-up, with a scoliosis correction of 70.3 %. At the most recent follow-up, the correction rate of proximal thoracic curve and thoracolumbar or lumbar curve was 57.1 and 74.8 %, respectively. Transient dyspnoea occurred in one patient after the initial surgery and subsequently resolved. No ventilator support was needed. Three patients complained the pain of concave soft tissue after the first distraction. There were no neurological complications or any deep wound infections. No complication of instrumentation was found at final follow-up., Conclusions: In the treatment of severe kyphoscoliosis, anterior release, posterior internal distraction and subsequent posterior spinal fusion, which obviates three-column osteotomy, is a good alternative correction method.

Purpose: To evaluate the outcome and complications of a novel technique for the treatment of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis (MPS)., Methods: The medical records and spinal imaging of four consecutive paediatric patients who underwent a single stage anteroposterior spinal fusion with segmental pedicle screw instrumentation were reviewed., Results: Patients underwent spinal deformity correction at the mean age of 3 years (2.4-3.7) with mean clinical follow-up of 3.2 years (2.1-4.5) and mean postoperative radiographic follow-up was 2.4 years (0.8-3). Preoperative kyphosis was corrected from a mean angle of 65º (63º-70º) to 6.5º (-12º-13º). Vertebral subluxation at the apex of the deformity was corrected from an average 64% (56-83%) to 12% (0-24%). Spinal cord monitoring with somatosensory evoked potentials (SSEP) was successfully obtained and stable throughout surgery. No instrumentation failure, loss of correction or junctional problems occurred at final follow-up., Conclusions: Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation is a safety and reliable technique for the treatment of severe thoracolumbar kyphosis in children with MPS. This technique achieves excellent correction of the deformity with adequate decompression of the spinal canal. The fusion is limited to the thoracolumbar junction and interferes minimally with the longitudinal growth of the thorax. No neurological complications or intraoperative spinal cord monitoring events occurred. No loss of correction or junctional kyphosis was observed.

Purpose: Proximal junctional kyphosis (PJK) is a common radiographic finding following long spinal fusions. Whether PJK leads to negative clinical outcome is currently debatable. A systematic review was performed to assess the prevalence, risk factors, and treatments of PJK., Methods: Literature search was conducted on PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials using the terms 'proximal junctional kyphosis' and 'proximal junctional failure'. Excluding reviews, commentaries, and case reports, we analyzed 33 studies that reported the prevalence rate, risk factors, and discussions on PJK following spinal deformity surgery., Results: The prevalence rates varied widely from 6 to 61.7%. Numerous studies reported that clinical outcomes for patients with PJK were not significantly different from those without, except in one recent study in which adult patients with PJK experienced more pain. Risk factors for PJK included age at operation, low bone mineral density, shorter fusion constructs, upper instrumented vertebrae below L2, and inadequate restoration of global sagittal balance., Conclusions: Prevalence of PJK following long spinal fusion for adult spinal deformity was high but not clinically significant. Careful and detailed preoperative planning and surgical execution may reduce PJK in adult spinal deformity patients.

Purpose: To analyze the role of spine alignment in post-traumatic paraplegic patient as a potential cause of late Charcot spine disease (CSD)., Method: A retrospective review of three cases in which the disease appeared more than 15 years after a spinal cord injury treated by posterior fusion. A review of the literature concerning spine balance in sitting position, especially referred to paraplegic patients, is done to validate this hypothesis., Results: Lumbar kyphosis in paraplegic patients during the sitting position may increase the mechanical load on disks and ligament below the previously fused area. This phenomenon, in combination with lack of protective mechanism because of poor muscular support and lack of sensitivity can speed up and amplify the normal degenerative changes in the disk and ligaments., Conclusions: More investigations are required to fully understand all the mechanisms underlying CSD pathogenesis to prevent it. Until then, a systematic long-term clinical and radiological follow-up in all post-trauma paraplegic patients is suggested. Combined anterior and posterior fusion, when feasible, can restore the sagittal balance providing a better quality of life in these patients.

Introduction: Even if diastematomyelia is a rare condition, it always has to be identified in case of diagnosing and treating a case of congenital scoliosis. The consequence of missing the diagnosis of such a malformation may be devastating to the involved patient. This paper wants once again to make aware the physicians of the eventual presence of a spinal dysraphic malformation when dealing with a congenital spinal malformation., Methods: The presence of diastematomyelia was noticed in two of our cases, one with congenital scoliosis and another with congenital kyphoscoliosis. First of all in these cases, we performed the resection of the bony septum, followed by spinal fusion in a single-stage surgical procedure. We noticed no complications during and after surgery related to the resection of the bony septum., Results: In both patients, we obtained partial correction and stabilization of the congenital spinal malformation after a safe excision of the bony septum., Conclusions: Diastematomyelia is a rare condition. It has to be taken into consideration when dealing with a congenital scoliosis. The first step in the surgical procedure has to be the resection of the diastematomyelic septum. In case of a scoliosis ranging up to 30° and not presenting a progressive potential, the expectative-evaluation attitude is a correct one.

Purpose: Regardless of the etiology, severe cervical deformities can be extremely debilitating and are a challenge to correct. Often a multi-modality team approach is required to safely and effectively reduce the deformity, provide adequate decompression, and ensure solid fixation and fusion. In cases of iatrogenic cervical deformity necessitating five-level corpectomy and fixation, the feasibility, safety, and durability of this procedure remains unknown., Results: We describe a patient who presented with debilitating pain and inability to eat due to an iatrogenic chin-on-chest cervical kyphotic deformity. The patient underwent a back-front-back staged procedure requiring five-level cervical vertebrectomy, C3-T1 anterior fixation, and occipital to T5 posterior fusion, resulting in successful reduction of cervical kyphosis from 75 to 0 degrees. At 6 months post-operatively, the patient demonstrated marked improvement in neurologic function and reported substantial improvements in neck pain-specific disability (NDI) and quality of life (SF-12 and EQ-5D)., Conclusion: The feasibility and safety of five-level vertebrectomy and reconstruction for chin-on-chest deformity remains poorly described. The current case suggests that thoughtful planning that involves maximizing the patient's health status, judicious use of traction under direct neurological examination, staged circumferential release, and design of a construct that provides anterior and posterior column support with several points of fixation beyond the axis of rotation will attenuate the risk of peri-operative morbidity and potentiate the durability of deformity correction.

Purpose: Sagittal spine and pelvic alignment of adolescent idiopathic scoliosis (AIS) is poorly described in the literature. It generally reports the sagittal alignment with regard to the type of curve and never correlated to the thoracic kyphosis. The objective of this study is to investigate the relationship between thoracic kyphosis, lumbar lordosis and sagittal pelvic parameters in thoracic AIS., Methods: Spinal and pelvic sagittal parameters were evaluated on lateral radiographs of 86 patients with thoracic AIS; patients were separated into hypokyphosis group (n = 42) and normokyphosis group (n = 44). Results were statistically analyzed. The lumbar lordosis was lower in the hypokyphosis group, due to the low proximal lordosis. The thoracic kyphosis was not correlated with any pelvic parameters but with the proximal lordosis. The pelvic incidence was correlated with sacral slope, pelvic tilt, lumbar lordosis and highly correlated with distal lumbar lordosis in the two groups. There was a significant linear regression between thoracic kyphosis and proximal lordosis and between pelvic incidence and distal lordosis., Conclusions: We can consider that the proximal part of the lordosis depends on the thoracic kyphosis and the distal part depends on the pelvic incidence. The hypokyphosis in AIS is independent of the pelvic parameters and could be described as a structural parameter, characteristic of the scoliotic deformity.

Objective: The objectives of this study was to clarify the relationship between kyphosis and gastroesophageal reflux disease (GERD) by evaluation of spinal alignment, obesity, osteoporosis, back muscle strength, intake of oral drugs, and smoking and alcohol history in screening of a community population to determine the factors related to GERD symptoms. GERD increases with age and is estimated to occur in about 30% of people. Risk factors for GERD include aging, male gender, obesity, oral medicines, smoking, and alcohol intake. It has also been suggested that kyphosis may influence the frequency of GERD, but the relationship between kyphosis and GERD is unclear., Subjects and Methods: We examined 245 subjects (100 males and 145 females; average age 66.7 years old) in a health checkup that included evaluation of sagittal balance and spinal mobility with SpinalMouse, GERD symptoms using the Frequency Scale for Symptoms of GERD (FSSG) questionnaire, body mass index, osteoporosis, back muscle strength, number of oral drugs taken per day, intake of nonsteroidal anti-inflammatory drugs (NSAIDs), intake of bisphosphonates, and smoking and alcohol intake., Results: Multivariate logistic regression analysis including all the variables showed that lumbar lordosis angle, sagittal balance, number of oral drugs taken per day, and back muscle strength had significant effects on the presence of GERD (OR, 1.10, 1.11, 1.09 and 1.03; 95% CI, 1.03-1.17, 1.02-1.20, 1.01-1.18 and 1.01-1.04; p = 0.003, 0.015, 0.031 and 0.038, respectively). The other factors showed no association with GERD., Conclusion: This study is the first to show that lumbar kyphosis, poor sagittal balance; increased number of oral drugs taken per day, and decreased back muscle strength are important risk factors for the development of GERD symptoms. Thus, orthopedic surgeons and physicians should pay attention to GERD in elderly patients with spinal deformity.

Objective: To report the first case of surgical treatment for severe kyphoscoliosis associated with respiratory disorder in a patient with congenital neuromuscular disease with uniform type 1 fibers (CNMDU1), including management of the possible onset of malignant hyperthermia (MH) in general anesthesia. CNMDU1 is rare among congenital neuromuscular diseases, and surgery for spinal deformity in CNMDU1 has not been described. Onset of MH in general anesthesia is a concern in this disease., Methods: A 13-year-old female with motor retardation, suspected myopathy, and severe spinal deformity was followed at another pediatric hospital before referral to Meijo Hospital. Symptoms at the initial consultation were mild general muscular weakness and muscular atrophy. The rib hump was 60° and trunk balance was poor. The tendon reflex showed hyporeflexia, and blood tests were normal. Vital capacity was 0.69 L and forced expiratory volume percentage in 1 s was 75.5%, showing a restrictive and obstructive ventilatory defect. A plain radiograph showed severe kyphoscoliosis with thoracic scoliosis of 130° (T5-L1) and thoracic kyphosis of 110° (T2-T12) with almost no flexibility in bending or traction film., Results: After preoperative halo traction for 2 months, one-stage anterior and posterior correction and fusion from T2 to L3 was conducted. MH did not occur, but recovery of respiratory function required 8 days by intubation after surgery. Postoperatively, thoracic kyphosis improved to 25° and thoracic scoliosis was 66° (correction rate: 49%). Pathological results of an intraoperative muscle biopsy from the paraspinal muscles confirmed the diagnosis of CNMDU1. At 6 years after surgery, the patient has no problems in daily life and no respiratory difficulty., Conclusion: Spinal deformity in CNMDU1 has a risk of severe progression, which makes early diagnosis by biopsy important. The surgery may be recommended before severe progression of spinal deformity and respiratory disorder. Perioperative MH is a concern, but can be managed by appropriate procedures.

Introduction: The treatment of rigid and severe scoliosis and kyphoscoliosis is a surgical challenge. Presurgical halo-gravity traction (HGT) achieves an increase in curve flexibility, a reduction in neurologic risks through gradual traction on a chronically tethered cord and an improvement in preoperative pulmonary function. However, little is known with respect to the ideal indications for HGT, its appropriate duration, or its efficacy in the treatment of rigid deformities., Materials and Methods: To investigate the use of HGT in severe deformities, we performed a retrospective review of 45 patients who had severe and rigid scoliosis or kyphoscoliosis. The analysis focused on the impact of HGT on curve flexibility, pulmonary function tests (PFTs), complications and surgical outcomes in a single spine centre., Results: PFTs were used to assess the predicted forced vital capacity (FVC%). The mean age of the sample was 24±14 years. 39 patients had rigid kyphoscoliosis, and 6 had scoliosis. The mean apical rotation was 3.6°±1.4°, according to the Nash and Moe grading system. The curve apices were mainly in the thoracic spine. HGT was used preoperatively in all the patients. The mean preoperative scoliosis was 106.1°±34.5°, and the mean kyphosis was 90.7°±29.7°. The instrumentation used included hybrids and pedicle screw-based constructs. In 18 patients (40%), a posterior concave thoracoplasty was performed. Preoperative PFT data were obtained for all the patients, and 24 patients had ≥3 assessments during the HGT. The difference between the first and the final PFTs during the HGT averaged 7.0±8.2% (p<.001). Concerning the evolution of pulmonary function, 30 patients had complete data sets, with the final PFT performed, on average, 24 months after the index surgery. The mean preoperative FVC% in these patients was 47.2±18%, and the FVC% at follow-up was 44.5±17% (a difference that did not reach statistical significance). The preoperative FVC% was highly predictive of the follow-up FVC% and the response during HGT. The mean flexibility of the scoliosis curve during HGT was only 14.8±11.4%, which was not significantly different from the flexibility measures achieved on bending radiographs or Cotrel traction radiographs. In rigid curves, the Cobb angle difference between the first and final radiographs during HGT was only 8°±9° for scoliosis and 7°±12° for kyphosis. Concerning surgical outcomes, 13 patients (28.9%) experienced minor and 15 (33.3%) experienced major complications. No permanent neurologic deficits or deaths occurred. Additional surgery was indicated in 12 patients (26.7%), including 7 rib-hump resections. At the final evaluation, 69% of the patients had improved coronal balance, and at a mean follow-up of 33±23.3 months, 39 patients (86.7%) were either satisfied or very satisfied with the overall outcome., Conclusion: The improvement of pulmonary function and the restoration of sagittal and coronal balance are the main goals in the treatment of severe and rigid scoliosis and kyphoscoliosis. A review of the literature showed that HGT is a useful tool for selected patients. Preoperative HGT is indicated in severe curves with moderate to severe pulmonary compromise. HGT should not be expected to significantly improve severe curves without a prior anterior and/or posterior release. The data presented in this study can be used in future studies to compare the surgical and pulmonary outcomes of severe and rigid deformities.

We retrospectively studied the cases with tuberculous spondylitis of thoracolumbar region with two or more levels of involvement that underwent posterior instrumentation and fusion and anterior fusion with titanium mesh following anterior decompression using simultaneous successive posterior-anterior-posterior surgery. Among all patients with tuberculous spondylitis accompanied by medium or severe kyphosis, 20 patients who underwent simultaneous successive decompression, fusion and instrumentation with posterior-anterior-posterior surgery between 1999 and 2004 were included in the study. Patients were evaluated for fusion formation and neurological and functional status. Kyphosis angles were measured at early and long-term follow-up. Antituberculosis chemotherapy was initiated in all patients and continued for 9 months; initially as quadruple therapy for 3 months, and then as triple therapy. Average follow-up period was 52.7 months (range 37-94). Solid fusion was achieved in all patients. All patients returned to their previous occupation; 75% (15 subjects) with mild pain or no pain and 15% (3 subjects) with major limitations. There were 11 patients with neurological deficit, 9 of these achieved complete neurological recoveries. Regarding kyphosis angle, an average 35.1° correction (84.8%) was obtained in postoperative period (p < 0.001) and there was no significant correction loss during the follow-up period (p < 0.05). There were no grafts or instrumentation-related stabilization problems. In subjects with tuberculous spondylitis with involvements at two or more levels accompanied by medium and severe kyphosis, decompression, fusion and instrumentation by simultaneous successive posterior-anterior-posterior surgery is an effective and safe management method for effective kyphosis correction with high fusion rates.

Degenerative changes of the cervical spine are commonly accompanied by a reduction or loss of the segmental or global lordosis, and are often considered to be a cause of neck pain. Nonetheless, such changes may also remain clinically silent. The aim of this study was to examine the correlation between the presence of neck pain and alterations of the normal cervical lordosis in people aged over 45 years. One hundred and seven volunteers, who were otherwise undergoing treatment for lower extremity problems in our hospital, took part. Sagittal radiographs of the cervical spine were taken and a questionnaire was completed, enquiring about neck pain and disability in the last 12 months. Based on the latter, subjects were divided into a group with neck pain (N = 54) and a group without neck pain (N = 53). The global curvature of the cervical spine (C2-C7) and each segmental angle were measured from the radiographs, using the posterior tangent method, and examined in relation to neck complaints. No significant difference between the two groups could be found in relation to the global curvature, the segmental angles, or the incidence of straight-spine or kyphotic deformity (P > 0.05). Twenty-three per cent of the people with neck pain and 17% of those without neck pain showed a segmental kyphosis deformity of more than 4 degrees in at least one segment--most frequently at C4/5, closely followed by C5/6 and C3/4. The average segmental angle at the kyphotic level was 6.5 degrees in the pain group and 6.3 degrees in the group without pain, with a range of 5-10 degrees in each group. In the group with neck pain, there was no association between any of the clinical characteristics (duration, frequency, intensity of pain; radiating pain; sensory/motor disturbances; disability; healthcare utilisation) and either global cervical curvature or segmental angles. The presence of such structural abnormalities in the patient with neck pain must be considered coincidental, i.e. not necessarily indicative of the cause of pain. This should be given due consideration in the differential diagnosis of patients with neck pain.

Indications for surgery in Scheuermann disease are not well codified and remain rare, as the natural history of the disease is in most cases benign. In the immature adolescent, conservative treatment, such as bracing or casting, can be tried for moderate curves. For larger curves, or in the adult, conservative treatment is usually not effective, and surgery can be considered. Such indications are mostly cosmetic for large curves above 75 degrees. Pain over the deformity or in the low back may represent another surgical indication, especially in the adult group. The question of anterior release or straight posterior fusion has become more of an actuality with the advent of powerful, third-generation stiff segmental instrumentation. However, the long-term results of a modern, posterior-only instrumentation fusion are not known. Concern about loss of correction, late pseudarthrosis or the need to remove instrumentation for infected hardware or due to late pain at the operative site must make us careful about choosing this method. Very rigid and large curves still require an anterior release, either done in a conventional or mini-open fashion, or through video-assisted thoracoscopic surgery. The extent of the posterior instrumentation has now been better defined. One must fuse the whole Cobb angle without hypercorrection and stop distally, above the first lordotic disc, to avoid sagittal decompensation. New approaches such as short anterior fusion with bone-on-bone techniques and pedicle substraction osteotomies have not yet been reported in the literature as having been used for treating Scheuermann's kyphosis. These should be considered experimental.

Rigid congenital kyphosis in myelomeningocele is associated with an important morbidity with skin breakdown, recurrent infection, and decreased function. Kyphectomy is the classic treatment to restore spinal alignment; however, surgery is associated with an important morbidity and long-term correction is uncertain. The authors retrospectively reviewed 9 patients with a mean age of 8.8 years who underwent a two stage surgical procedure: first a posterior kyphectomy with a modified Dunn-McCarthy fixation consisting of lumbar pedicle screws and long S-shape rods buttressing the anterior sacrum. Then a second stage done several weeks later consisting of a thoraco-abdominal approach to the spine with an inlay strut graft classically from T10-S1. The mean follow-up was 34 months (range 1-5 years). The kyphosis was corrected from a mean of 110 degrees of Cobb angle (range 70-130 degrees) to 15 degrees after surgery (45-0 degrees). There was no instrumentation failure, no loss of correction and no pseudarthrosis. Complications consisted of one intra-operative cardiac arrest fortunately reversible, a wound necrosis, one deep venous thrombosis and one late aseptic bursitis on the posterior hardware. Congenital kyphosis in myelomeningocele can be treated successfully with an initial posterior approach correction and instrumentation followed by an anterior approach allowing for anterior inlay impacted structural graft. The authors believe that this technique improves biomechanical and biological fusion mass anteriorly and will prevent late instrumentation failure and loss of correction.

Background: In previous studies, typical radiological findings in the cervical spine of patients with diastrophic dysplasia (DD) have been kyphosis, displacement of the vertebrae, spina bifida occulta (SBO), anterior hypoplasia of vertebrae C3-5, and hyperplasia and dysmorphism of the odontoid process., Objectives: To make a radiological analysis of the cervical spine in patients with DD., Materials and Methods: The study comprised 122 patients (50 males, 72 females), with an average age of 19 years (range newborn-63 years). Follow-up was available on 62 patients (51%), for an average duration of 11 years. Cervical spine alignment was measured according to Cobb's method. The height (H) and depth (D) of the vertebral body and sagittal diameter (S) of the spinal canal were measured. H/D and S/D ratios were then calculated from the measurements. The shape of the vertebrae was assessed. Displacement and movement of cervical vertebrae in neutral and bending radiographs were measured. RESULTS. The average lordosis in the last radiograph was 17(degrees) (range 4 degrees -55(degrees)). Five (4%) patients had a cervical kyphosis with an average of 92(degrees) (range 10-165(degrees)) on their last radiograph. The H/D ratio increased slowly during growth and showed significant correlation with age. There was no growth spurt at puberty. The S/D ratio was fairly stable until 7-8 years of age, when it started to decline slowly. The percentage of vertebrae with a flat vertebral body and narrow spinal canal value tended to increase with age. Vertebral hypoplasia and displacement between vertebrae were most common in the mid-cervical region and resolved spontaneously with age. Degenerative changes seemed to increase with age and were already visible during the second decade of life. SBO was noted in 79% of patients., Conclusions: The most common alignment in the cervical spine is lordosis in adulthood. The vertebral bodies are flattened and the spinal canal is narrowed. Vertebral body hypoplasia and displacement usually resolve spontaneously during growth. Degenerative changes in the cervical spine are common, but vertebral anomalies are rare. Prevalence of SBO is high.

We report a 29-month-old girl with osteopathia striata with cranial sclerosis (OS-CS), who showed several unusual manifestations, including short stature, muscular hypotonia, short lingual frenulum, an accessory ear and a granuloma at the oral edge. The most unusual distressing finding was severe cervical kyphosis with a dysplastic cervical spine. MRI revealed impingement of the upper spinal cord. To clarify the mode of inheritance of this disease in this patient, we performed a methylation-specific PCR analysis. It eventually showed random inactivation of X-chromosomes, suggesting an autosomal dominant trait.

Congenital pedicle abnormalities are rare. Unilateral aplastic and hypoplastic lumbar pedicles have been reported, but these were usually discovered incidentally and did not need surgical treatment. We present a case of absence of both pedicles and the neural arch of L2, with associated kyphoscoliosis with neurological involvement, that needed a two-stage corrective surgery. An L1-L4 fusion was achieved with relief of the symptoms.

Neurological deficit is a serious though not well-known complication associated with spinal deformity. Sharp-angle kyphosis may be congenital, traumatic, degenerative, infectious, or iatrogenic in origin. Many kyphotic deformities are underestimated, thus leading to severe neurological deficit. In order to determine exactly what procedures of angulation the patients should undergo to stabilize the spine, which are major operations, the authors analyzed in an experimental model the effects of progressive sharp angulation on the anatomy of spinal canal and cord. We found that sharp anterior angulation of 50 degrees causes anterior-posterior stenosis and the dura will touch the spinal cord. At 90 degrees of angulation, the spinal cord will be squeezed and the pressure in the canal will be double what it was initially, probably leading to ischemia. The experimental confirmation (determination) of these angulations allows the physician in charge to define early in the treatment program when a surgical stabilization procedure should be included, before the angulation causes any neurological damage.

A case of spondylo-epimetaphyseal dysplasia with joint laxity (SEMDJL) in an Italian girl is reported. This condition is mainly observed in the Afrikaans population of South Africa with an ancestral founder believed to be localized in West Germany. This case might support a link with the European origin of SEMDJL.

Mid-cervical kyphosis in diastrophic dysplasia (DD) with cord compression and weakness has been recognized for the last three decades. A strikingly similar situation exists in Larsen's syndrome (LS), an otherwise unrelated problem of weakness with lay joints and dislocations with typical facies. Forty patients with DD were studied of whom seven had cervical spine kyphosis: one patient had spontaneous correction over the ensuing two decades. Seven patients with LS were studied of whom four had cervical spine changes with cord compression. There was one sudden death among the patients with LS and no cases of spontaneous correction. Muscle weakness in DD and LS should lead to evaluation of cervical spine kyphosis with cord compression. Plain films, CT and recently MRI are useful.

During a brief period of 21 months (November 1988 to August 1990) the neurospinal unit of our institution registered three cases of Scheuermann's kyphosis with herniation of the thoracic discs. Local and intercostal pain, progressive spasticity with gait disturbance and urinary hesitancy were the most common presenting symptoms. All of the patients were surgically managed by posterolateral decompression, interbody fusion (posterolateral approach) and pedicular screw plate fixation. The average length of follow-up was 25 months. Results were excellent in two patients and good in one. All patients had improvement in neurological status, were pain-free and demonstrated sound fusion within 5 months. A new approach to management of this rare clinical entity is documented.

Homotransplantation of preserved sclera was successfully performed for post-traumatic scleral staphyloma in a 38-year-old woman with Ehlers-Danlos syndrome type VI. The transplanted tissue was preserved by glycerol dehydration. For 1 year postoperatively, no sign of graft failure or recurrence of scleral staphyloma has been found. To our knowledge, this is the first report of the use of a preserved scleral homograft to treat this ocular complication in a patient with Ehlers-Danlos syndrome.