COL11A2 as a candidate gene for vertebral malformations and congenital scoliosis.

Human vertebral malformations (VMs) have an estimated incidence of 1/2000 and are associated with significant health problems including congenital scoliosis (CS) and recurrent organ system malformation syndromes such as VACTERL (vertebral anomalies; anal abnormalities; cardiac abnormalities; tracheo-esophageal fistula; renal anomalies; limb anomalies). The genetic cause for the vast majority of VMs are unknown. In a CS/VM patient cohort, three COL11A2 variants (R130W, R1407L and R1413H) were identified in two patients with cervical VM. A third patient with a T9 hemivertebra and the R130W variant was identified from a separate study. These substitutions are predicted to be damaging to protein function, and R130 and R1407 residues are conserved in zebrafish Col11a2. To determine the role for COL11A2 in vertebral development, CRISPR/Cas9 was used to create a nonsense mutation (col11a2L642*) as well as a full gene locus deletion (col11a2del) in zebrafish. Both col11a2L642*/L642* and col11a2del/del mutant zebrafish exhibit vertebral fusions in the caudal spine, which form due to mineralization across intervertebral segments. To determine the functional consequence of VM-associated variants, we assayed their ability to suppress col11a2del VM phenotypes following transgenic expression within the developing spine. While wildtype col11a2 expression suppresses fusions in col11a2del/+ and col11a2del/del backgrounds, patient missense variant-bearing col11a2 failed to rescue the loss-of-function phenotype in these animals. These results highlight an essential role for COL11A2 in vertebral development and support a pathogenic role for two missense variants in CS.

Incidence of Tether Breakage in Anterior Vertebral Body Tethering.

BACKGROUND: Thoracic anterior vertebral body tethering (TAVBT) is an emerging treatment for adolescent idiopathic scoliosis. Tether breakage is a known complication of TAVBT with incompletely known incidence. We aim to define the incidence of tether breakage in patients with adolescent idiopathic scoliosis who undergo TAVBT. The incidence of tether breakage in TAVBT is hypothesized to be high and increase with time postoperatively. METHODS: All patients with right-sided, thoracic curves who underwent TAVBT with at least 2 and up to 3 years of radiographic follow-up were included. Tether breakage between 2 vertebrae was defined a priori as any increase in adjacent screw angle >5 degrees from the minimum over the follow-up period. The presence and timing of tether breakage were noted for each patient. A Kaplan-Meier survival analysis was performed to calculate expected tether breakage up to 36 months. χ 2 analysis was performed to examine the relationship between tether breakage and reoperations. Independent t test was used to compare the average final Cobb angle between cohorts. RESULTS: In total, 208 patients from 10 centers were included in our review. Radiographically identified tether breakage occurred in 75 patients (36%). The initial break occurred at or beyond 24 months in 66 patients (88%). Kaplan-Meier survival analysis estimated the cumulative rate of expected tether breakage to be 19% at 24 months, increasing to 50% at 36 months. Twenty-one patients (28%) with a radiographically identified tether breakage went on to require reoperation, with 9 patients (12%) requiring conversion to posterior spinal fusion. Patients with a radiographically identified tether breakage went on to require conversion to posterior spinal fusion more often than those patients without identified tether breakage (12% vs. 2%; P =0.004). The average major coronal curve angle at final follow-up was significantly larger for patients with radiographically identified tether breakage than for those without tether breakage (31 deg±12 deg vs. 26 deg±12 deg; P =0.002). CONCLUSIONS: The incidence of tether breakage in TAVBT is high, and it is expected to occur in 50% of patients by 36 months postoperatively. LEVEL OF EVIDENCE: Level IV.

Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.

BACKGROUND: Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1. METHODS: Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs. RESULTS: Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs. CONCLUSION: We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

Do drains help reduce the risk of complications after thoracoplasties in posterior spinal fusions for adolescent idiopathic scoliosis?

PURPOSE: In adolescent idiopathic scoliosis (AIS), the rib prominence is a major cosmetic concern which can be improved using thoracoplasties. We sought to determine if the use of deep drains helps minimize the development of pleural effusions after thoracoplasties. METHODS: Retrospective study of 45 patients with AIS undergoing posterior spinal fusion (PSF) and thoracoplasties. RESULTS: Thirty six out of 45 patients (80.0%) required placement of a deep surgical drain, and 16 out of 45 (35.6%) developed pleural effusions after PSF with concomitant thoracoplasty. Of the 16 patients who developed pleural effusion, 12 of 36 (33.3%) required a placement of a deep drain (p > 0.05). Of the total 45 patients in this cohort, 3 patients (6.7%) required chest tubes, and 4 patients (8.9%) developed surgical site infections (SSIs). We found that deep drains were associated with a lower incidence of SSI (2.8% vs 33.3%; p = 0.021). Patients who had a pleural effusion had longer ICU stays (p = 0.037) and longer requirements of nasal oxygen (p = 0.025). DISCUSSION: The presence of a pleural effusion in patients with AIS undergoing PSF with thoracoplasty was associated with longer oxygen requirements and length of hospital ICU admission. Thirty six percent of patients with thoracoplasties developed pleural effusions, but deep subfascial drains did not significantly decrease the incidence of pleural effusions.

Risk factors for proximal junctional kyphosis after pediatric spinal deformity surgery with halo gravity traction.

PURPOSE: To determine risk factors for proximal junctional kyphosis (PJK) in pediatric patients with scoliosis undergoing halo gravity traction (HGT) prior to posterior spinal fusion (PSF). METHODS: Data from consecutive patients who underwent PSF after HGT with 2-year follow-up were retrospectively collected from a single center. Patients were divided into two groups: PJK vs. no PJK. RESULTS: Twenty-five patients (age 13.6 ± 3.1 years) underwent HGT for a mean of 42 ± 37 days. Eight patients (32%) developed radiographic PJK and 1 (4%) developed proximal junctional failure. PJK patients had greater loss of cervical lordosis (-17.4 ± 23.2 vs. 2.7 ± 16.2°, p < 0.05), greater increase in lumbar lordosis (9.3 ± 19.5 vs. -2.8 ± 12.8°; p = 0.034) during traction, and smaller overall major coronal curve angle correction (-16.8 ± 30.6 vs. -36.6 ± 16.4°; p = 0.026). From postoperative to last follow-up, PJK patients had a greater increase in upper end vertebrae (UEV) slope (3.3 ± 7.8 vs. -4.0 ± 7.7°; p = 0.004). Selection of UIV based on which vertebra was most level either pre-traction or in-traction was not associated with PJK development (p > 0.05). CONCLUSION: In-traction radiographic changes of decreased cervical lordosis, decreased major coronal curve, increased lumbar lordosis, and disruption of cervical sagittal balance at last follow-up may have implications for level selection and risk of PJK.

Crossing the cervicothoracic junction in complex pediatric deformity using anterior cervical discectomy and fusion: a case series.

PURPOSE: Proximal instrumentation failure is a challenge in posterior spinal fusions (PSFs) crossing the cervicothoracic junction. High rates of proximal junctional kyphosis (PJK) and loss of fixation have been reported. In this single-center retrospective cohort study, we evaluate the utility of anterior cervical discectomy and fusion (ACDF) in addition to traditional PSF crossing the cervicothoracic junction in order to mitigate implant-related complications. METHODS: All patients who underwent PSF across the cervicothoracic junction with ACDF with 2 years of follow-up data were reviewed. We analyzed clinical, surgical, and radiographic measures such as operative details, presence of PJK, complications, instrumentation migration, curve angles, and vertebral translation. Measurements were compared statistically using paired samples t-tests. RESULTS: Ten patients (6 girls, 4 boys) met inclusion criteria with a mean age at surgery of 12.8 ± 3.3 years and follow-up of 3.38 ± 0.9 years. All patients underwent ACDF (range 1-3 levels), and 8 (80%) underwent traction. The average number of levels fused posteriorly was 16.7 ± 4.7 and anteriorly was 2.4 ± 0.7. The major coronal curve averaged 48.8 ± 34.7° preoperatively and 23.3±13.3° postoperatively (p = 0.028). The average major sagittal curve was 83.5 ± 24.2° preoperatively, resolving to 53.9 ± 25.5° (p=0.001). One patient suffered rod breakage at T7, and another developed symptomatic PJK 19 months postoperatively. CONCLUSION: Our data suggest that ACDF procedures added to PSFs crossing the cervicothoracic junction offer promise for reducing risk for instrumentation-related complications. ACDF also significantly helps improve and maintain both coronal and sagittal correction over 2 years. LEVEL OF EVIDENCE: 4.

Use of postoperative neurophysiological testing to help guide management in a case of delayed neurological injury.

Bimodal intraoperative neuromonitoring (IONM), combining transcranial motor-evoked potentials (tcMEP) and somatosensory-evoked potentials (SSEP), enables real-time detection and prevention of spinal cord injury during pediatric spinal deformity correction. Although rare, false-positive and false-negative signal alerts have been reported. However, no previously published accounts have described the use of postoperative neurophysiological testing to both identify new-onset neurological injury and guide reintervention. Here, we describe the case of an 18-year-old young man with achondroplasia, thoracolumbar kyphosis, and L2 wedge vertebra who underwent T12-L4 posterior spinal fusion with L2 vertebral column resection. Despite two intraoperative decreases in tcMEP amplitude, corrective measures on both occasions produced a return of IONM signal. Curiously, despite movement of the bilateral lower extremities upon waking, continued observation demonstrated minimal movement of the left lower extremity. Postoperative neurophysiological testing then identified limited muscle group activation below the left quadriceps, prompting operative reintervention. After cage removal and laminectomy lengthening, the patient recovered bilateral lower extremity function. He later returned to surgery for repeat cage placement at L2 via a retroperitoneal exposure, with no noted IONM changes and subsequent neurological improvement.

Congenital Scoliosis of the Pediatric Cervical Spine: Characterization of a 17-Patient Operative Cohort.

BACKGROUND: Congenital cervical scoliosis is rare, and there is a paucity of literature describing surgical outcomes. We report surgical outcomes in a 17-patient cohort with surgical correction for congenital cervical scoliosis and identify risk factors associated with complications. METHODS: Data were retrospectively collected from a single-center cohort of 17 consecutive patients (9 boys, 8 girls) receiving surgical deformity correction for congenital cervical scoliosis. The mean age at surgery was 7.1±3.4 years with an average follow-up of 3.6±1.1 years. RESULTS: There were 24 operations performed on 17 patients, and 4 complications (17%) were reported in the series, including one each of pressure ulcer, asystole, vertebral artery injury, and pseudarthrosis. The mean preoperative major curve angle was 36±20 degrees, which improved to 24±14 degrees (P=0.02). The mean operative time was 8±2 hours with a mean estimated blood loss of 298±690 mL. Halo-gravity traction was used in 5 patients and 6 cases were staged with anterior/posterior procedures. CONCLUSIONS: Congenital scoliosis of the cervical spine is a complex process. The spinal deformity of this nature can be managed successfully with carefully planned and executed surgical correction. LEVEL OF EVIDENCE: Level IV-retrospective review.

Preoperative SRS pain score is the primary predictor of postoperative pain after surgery for adolescent idiopathic scoliosis: an observational retrospective study of pain outcomes from a registry of 1744 patients with a mean follow-up of 3.4 years.

BACKGROUND: Traditionally, adolescent idiopathic scoliosis (AIS) has not been associated with back pain, but the increasing literature has linked varying factors between pain and AIS and suggested that it is likely underreported. PURPOSE: Our objective was to investigate factors associated with post-op pain in AIS. METHODS: A prospectively collected multicenter registry was retrospectively queried. Pediatric patients with AIS having undergone a fusion with at least 2 years of follow-up were divided into two groups: (1) patients with a postoperative SRS pain score ≤ 3 or patients having a reported complication specifically of pain, and (2) patients with no pain. Patients with other complications associated with pain were excluded. RESULTS: Of 1744 patients, 215 (12%) experienced back pain after postoperative recovery. A total of 1529 patients (88%) had no complaints of pain, and 171 patients (10%) had pain as a complication, with 44 (2%) having an SRS pain score ≤ 3. The mean time from date of surgery to the first complaint of back pain was 25.6 ± 21.6 months. In multivariate analysis, curve type (16% of Lenke 1 and 2 curves vs. 10% of Lenke 5 and 6, p = 0.002) and a low preoperative SRS pain score (no pain 4.15 ± 0.67 vs. pain 3.75 ± 0.79, p < 0.001) were significant. When comparing T2-4 as the upper instrumented vertebrae in a subgroup of Lenke 1 and 2 curves, 9% of patients had pain when fused to T2, 13% when fused to T3, and 18% when fused to T4 (p = 0.002). CONCLUSION: 12% of all AIS patients who underwent fusion had back pain after postoperative recovery. The most consistent predictive factor of increased postoperative pain across all curve types was a low preoperative SRS pain score. These slides can be retrieved under Electronic Supplementary Material.

Improving Health-related Quality of Life for Patients With Nonambulatory Cerebral Palsy: Who Stands to Gain From Scoliosis Surgery?

INTRODUCTION: It is unclear what factors influence health-related quality of life (HRQOL) in neuromuscular scoliosis. The aim of this study was to evaluate which factors are associated with an improvement in an HRQOL after spinal fusion surgery for nonambulatory patients with cerebral palsy (CP). METHODS: A total of 157 patients with nonambulatory CP (Gross Motor Function Classification System IV and V) with a minimum of 2-year follow-up after PSF were identified from a prospective multicenter registry. Radiographs and quality of life were evaluated preoperatively and 2 years postoperatively. Quality of life was evaluated using the validated Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire. Patients who had an increase of 10 points or greater from baseline CPCHILD scores were considered to have meaningful improvement at 2 years postoperatively. 10 points was chosen as a threshold for meaningful improvement based on differences between Gross Motor Function Classification System IV and V patients reported during the development of the CPCHILD. Perioperative demographic, clinical, and radiographic variables were analyzed to determine predicators for meaningful improvement by univariate and multivariate regression analysis. RESULTS: A total of 36.3% (57/157) of the patients reported meaningful improvement in CPCHILD scores at 2 years postoperatively. Preoperative radiographic parameters, postoperative radiographic parameters, and deformity correction did not differ significantly between groups. Patients who experienced meaningful improvement from surgery had significantly lower preoperative total CHPILD scores (43.8 vs. 55.2, P<0.001). On backwards conditional binary logistic regression, only the preoperative comfort, emotions, and behavior domain of the CPCHILD was predictive of meaningful improvement after surgery (P≤0.001). CONCLUSION: Analysis of 157 CP patients revealed a meaningful improvement in an HRQOL in 36.3% of the patients. These patients tended to have lower preoperative HRQOL, suggesting more "room for improvement" from surgery. A lower score within the comfort, emotions, and behavior domain of the CPCHILD was predictive of meaningful improvement after surgery. Radiographic parameters of deformity or curve correction were not associated with meaningful improvement after surgery. LEVEL OF EVIDENCE: Level II-retrospective review of prospectively collected data.

Instrumented arthrodesis for non-traumatic craniocervical instability in very young children.

PURPOSE: Occipitocervical instrumentation is infrequently required for stabilization of the axial and subaxial cervical spine in very young children. However, when it is necessary, unique surgical considerations arise in children when compared with similar procedures in adults. METHODS: The authors reviewed literature describing fusion of the occipitocervical junction (OCJ) in toddlers and share their experience with eight cases of young children (age less than or equal to 4 years) receiving occiput to axial or subaxial spine instrumentation and fixation. Diagnoses and indications included severe or secondary Chiari malformation, skeletal dysplastic syndromes, Klippel-Feil syndrome, Pierre Robin syndrome, Gordon syndrome, hemivertebra and atlantal occipitalization, basilar impression, and iatrogenic causes. RESULTS: All patients underwent occipital bone to cervical spine instrumentation and fixation at different levels. Constructs extended from the occiput to C2 and T1 utilizing various permutations of titanium rods, autologous rib autografts, Mersilene sutures, and combinations of autografts with bone matrix materials. All patients were placed in rigid cervical bracing or halo fixation postoperatively. No postoperative neurological deficits or intraoperative vascular injuries occurred. CONCLUSION: Instrumented arthrodesis can be a treatment option in very young children to address the non-traumatic craniocervical instability while reducing the need for prolonged external halo vest immobilization. Factors affecting fusion are addressed with respect to preoperative, intraoperative, and postoperative decision-making that may be unique to the toddler population.

Risk Factors for Wound Infections after Deformity Correction Surgery in Neuromuscular Scoliosis.

OBJECTIVE: This study aims to elucidate surgical risk factors in neuromuscular scoliosis (NMS) with respect to wound site infection after spinal fusion. METHODS: A retrospective review was performed of all patients treated surgically for NMS between January 2008 and December 2016 (minimum 6 months' follow-up). A sub-cohort of 60 patients with minimum 2 years of follow-up data was also analyzed. RESULTS: In 102 patients (53 boys and 49 girls), the mean age at surgery was 14.0 years (SD ±2.7). Mean follow-up was 2.53 years (±1.66), and mean time to presentation of infection was 2.14 months (±4.95). The overall perioperative complication rate was 26.5%, with 14.7% of patients developing deep wound infection. Gram-negative bacteria were responsible for 60% of infections; 20% were Gram positive, and 20% involved both types. Pulmonary comorbidities (p = 0.007), pre- to postoperative increase in weight (p = 0.010), exaggerated lumbar lordosis at follow-up (p = 0.008), history of seizures (p = 0.046), previous myelomeningocele repair (p = 0.046), and previous operations (p = 0.013) were significant risk factors for infection. CONCLUSION: Our data suggest that in the pediatric population treated surgically for NMS, wound infection is strongly associated with postoperative increase in body weight, residual lumbar lordosis, pulmonary comorbidity, history of myelomeningocele repair, seizures, and previous operations.

A comprehensive review of the diagnosis and management of congenital scoliosis.

PURPOSE: To provide the reader with a comprehensive but concise understanding of congenital scoliosis METHODS: We have undertaken to summarize available literature on the pathophysiology, epidemiology, and management of congenital scoliosis. RESULTS: Congenital scoliosis represents 10% of pediatric spine deformity and is a developmental error in segmentation, formation, or a combination of both leading to curvature of the spine. Treatment options are complicated by balancing growth potential with curve severity. Often associated abnormalities of cardiac, genitourinary, or intraspinal systems are concurrent and should be evaluated as part of the diagnostic work-up. Management balances the risk of progression, growth potential, lung development/function, and associated risks. Surgical treatment options involve growth-permitting systems or fusions. CONCLUSION: Congenital scoliosis is a complex spinal problem associated with many other anomalous findings. Treatment options are diverse but enable optimization of management and care of these children.

Insertion of magnetically controlled growing rods in a patient with a diaphragmatic pacemaker: case report.

Phrenic stimulators offer an alternative to standard mechanical ventilation as well as the potential for ventilator independence in select patients with chronic respiratory failure. Young patients (< 10 years old) with high cervical spinal cord injuries often develop paralytic scoliosis due to loss of muscle tone caudal to their spinal cord lesion. Growing rod systems allow for stabilization of spinal deformity while permitting continued growth of the spine and thoracic cavity. Magnetically controlled growing rods (MCGRs) offer the advantage of noninvasive expansion, as opposed to the operative expansion required in traditional growing rod systems. To the authors' knowledge, this is the first reported case of MCGRs in a patient with a diaphragmatic pacemaker (DP). A 7-year-old boy with ventilator dependence after a high cervical spinal cord injury presented to the authors' institution with paralytic scoliosis that progressed to > 120°. The patient had previously undergone insertion of phrenic nerve stimulators for diaphragmatic pacing. The decision was made to insert MCGRs bilaterally to stabilize his deformity, because the planned lengthening surgeries that are necessary with traditional growing rods would be poorly tolerated in this patient. The patient's surgery and postoperative course were uneventful. The DP remained functional after insertion and lengthening of the MCGRs by using the external magnet. The DP had no effect on the expansion capability of the MCGRs. In conclusion, the MCGRs appear to be compatible with the DP. Further studies are needed to validate the long-term safety and compatibility of these 2 devices.

Intraoperative neuromonitoring alerts in a pediatric deformity center.

OBJECTIVE Intraoperative neuromonitoring (IONM) involves the use of somatosensory evoked potentials (SSEPs) and transcranial electric motor evoked potentials (TceMEPs). In this retrospective study the authors examined the sensitivity and specificity of both SSEPs and TceMEPs during pediatric spinal deformity surgeries. METHODS The authors performed a retrospective quantitative analysis of data obtained in 806 patients (197 males and 609 females) treated from December 2011 until October 2015. All patients were diagnosed with scoliosis that was classified as one of the following: adolescent idiopathic scoliosis (AIS) (38%), congenital scoliosis (22%), or syndromic scoliosis (40%). Also, 53 patients underwent vertebral column resection (VCR). All surgeries were monitored by high-level neuromonitoring specialists and were performed with total intravenous anesthesia. Alerts were described as a decrease in amplitude by 50% or greater (bilateral or unilateral) in SSEPs, TceMEPs, or both. RESULTS True-positive alerts for TceMEPs were observed in 60 of the 806 patients (7.4%). True-positive alerts for SSEPs were observed in 7 of the 806 patients (0.9%). In contrast, there were no false-positive or false-negative outcomes. Only 1 case (0.1%) was reported with a permanent postoperative deficit. No reported false negatives or false positives were observed, and thus sensitivity was 100% and specificity was 93%-100% for TceMEPs. The rate of sensitivity was 13.2% and the rate of specificity was 100% for SSEPs. The breakdown of total alert was as follows: 6.6% in AIS cases, 24.5% in congenital scoliosis cases, and 10.2% in syndromic scoliosis cases. Neurological injury rates were significantly lower than in previous studies, as there were 0% for AIS cases (p = 0.12), 0.6% for congenital scoliosis cases (p = 0.17), and 0% for syndromic scoliosis cases (p = 0.07). One injury in a patient with congenital scoliosis occurred during a VCR procedure, which brought the injury rate to 1.9% (p < 0.005). IONM alerts occurred during 34% of rod/correction cases, 25% of thoracic screw placements, 20% of the osteotomies, 17% of the resections, 3% of the cage insertions, and 2% of the sublaminar wiring procedures. CONCLUSIONS The authors hypothesize that the results of this study will support the necessity, as a standard of care, of multimodality neuromonitoring during high-risk pediatric spinal deformity surgery because of the decrease in postoperative deficits. Their data suggest that the TceMEPs are more sensitive than SSEPs, but when used in combination, they offer the patient a level of safety that would otherwise not exist. Last, these findings support the notion that better outcomes are achieved with high-level IONM professionals.

Neuromuscular scoliosis complication rates from 2004 to 2015: a report from the Scoliosis Research Society Morbidity and Mortality database.

OBJECTIVE Postoperative complications are one of the most significant concerns in surgeries of the spine, especially in higher-risk cases such as neuromuscular scoliosis. Neuromuscular scoliosis is a classification of multiple diseases affecting the neuromotor system or musculature of patients leading to severe degrees of spinal deformation, disability, and comorbidity, all likely contributing to higher rates of postoperative complications. The objective of this study was to evaluate deformity correction of patients with neuromuscular scoliosis over a 12-year period (2004-2015) by looking at changes in postsurgical complications and management. METHODS The authors queried the Scoliosis Research Society (SRS) Morbidity and Mortality (M&M) database for neuromuscular scoliosis cases from 2004 to 2015. The SRS M&M database is an international database with thousands of self-reported cases by fellowship-trained surgeons. The database has previously been validated, but reorganization in 2008 created less-robust data sets from 2008 to 2011. Consequently, the majority of analysis in this report was performed using cohorts that bookend the 12-year period (2004-2007 and 2012-2015). Of the 312 individual fields recorded per patient, demographic analysis was completed for age, sex, diagnosis, and preoperative curvature. Analysis of complications included infection, bleeding, mortality, respiratory, neurological deficit, and management practices. RESULTS From 2004 to 2015, a total of 29,019 cases of neuromuscular scoliosis were reported with 1385 complications, equating to a 6.3% complication rate when excluding the less-robust data from 2008 to 2011. This study shows a 3.5-fold decrease in overall complication rates from 2004 to 2015. A closer look at complications shows a significant decrease in wound infections (superficial and deep), respiratory complications, and implant-associated complications. The overall complication rate decreased by approximately 10% from 2004-2007 to 2012-2015. CONCLUSIONS This study demonstrates a substantial decrease in complication rates from 2004 to 2015 for patients with neuromuscular scoliosis undergoing spine surgery. Decreases in specific complications, such as surgical site infection, allow us to gauge our progress while observing how trends in management affect outcomes. Further study is needed to validate this report, but these results are encouraging, helping to reinforce efforts toward continual improvement in patient care.

Evaluation and management of adolescent idiopathic scoliosis: a review.

Adolescent idiopathic scoliosis (AIS) is a 3D spinal deformity affecting children between the ages of 11 and 18, without an identifiable etiology. The authors here reviewed the available literature to provide spine surgeons with a summary and update on current management options. Smaller thoracic and thoracolumbar curves can be managed conservatively with observation or bracing, but corrective surgery may be indicated for rapidly growing or larger curves. The authors summarize the atypical features to look for in patients who may warrant further investigation with MRI during diagnosis and review the fundamental principles of the surgical management of AIS. Patients with AIS can be managed very well with a combination of conservative and surgical options. Outcomes for these children are excellent with sustained longer-term results.

The Role of Age and Initial Deformation on Final Cranial Asymmetry in Infants with Plagiocephaly Treated with Helmet Therapy.

BACKGROUND/AIMS: To characterize the impact of cranial asymmetry and age at initiation of therapy on final cranial asymmetry in infants with deformational plagiocephaly treated with helmet orthotics. METHODS: We conducted a single-center retrospective review of 45 pediatric patients <12 months of age with deformational plagiocephaly who underwent STARband cranial orthotic helmet treatment. Cranial asymmetry was measured using a 3-dimensional laser surface scanner and defined as a cranial vault asymmetry index (CVAI) >3.5%. RESULTS: Twenty-one patients (47%) were <6 months of age at the start of helmet therapy. A greater initial CVAI or age at therapy initiation correlated with a greater final CVAI (univariate analysis, r = 0.53, p < 0.001; r = 0.42, p = 0.004). Subgroup analysis of 18 patients beginning therapy at 4-5 months of age and 20 patients beginning therapy at 6-8 months of age revealed that only a change in the initial CVAI was associated with a change in the final CVAI (r = 0.43, p = 0.007). CONCLUSION: In a comparison of patients who initiated helmet therapy at 4-5 and 6-8 months of age, only cranial asymmetry at the outset of therapy was correlated with final cranial asymmetry (r = 0.43, p = 0.007).

PSO without neuromonitoring: analysis of peri-op complication rate after lumbar pedicle subtraction osteotomy in adults.

PURPOSE: A decompensated sagittal imbalance has been associated with worsening health-related quality of life outcomes and increasing pain. Significant improvement in sagittal balance can be achieved using a pedicle subtraction osteotomy (PSO); however, this procedure has a high complication profile. A lumbar PSO has the advantages of direct visualization of all neural structures and safer retraction of the thecal sac. Sacrificing neuromonitoring may reduce anaesthesia time and lead to improved cost effectiveness of the surgery. However, there are no studies analyzing the complication rate of lumbar PSO without the use of neuromonitoring. We hypothesize that the neurologic complication rate remains comparable to published studies even without the use of intraoperative neuromonitoring. METHODS: The medical records of all patients of a single institution who received a lumbar PSO for sagittal imbalance between July 2012 and June 2014 were retrospectively analyzed. All surgeries were performed by the first author and without the use of intraoperative neuromonitoring. We analyzed the 30-day complication rate. RESULTS: Twenty-two patients were included in this study. The average age at surgery was 67 years. 16 patients had at least one previous lumbar surgery. The average fusion length was seven segments. All patients were instrumented to the sacrum and 19 patients additionally received spinopelvic instrumentation. The average amount of focal correction was 28°. The average surgical time was 362 min and blood loss was 2302 mL. A total of eight patients with complications were identified. There was a new neurologic postoperative deficit in two patients presenting as a foot drop. Five patients had an unplanned revision surgery. CONCLUSION: Historically, PSOs are associated with a high surgical complication rate and our results show comparable outcomes and complications to those previously reported. In our series, the absence of neuromonitoring in lumbar PSOs does not appear to increase the risk of neurological injury.

Morphometric analysis of lumbar pedicles in patients with spinal dysraphism.

BACKGROUND: In patients with open neural tube defects, the incidence of scoliosis and requirement for spinal fusions are increased. Historically, there has been no standardized measurement of vertebral morphometry in these patients. However, anecdotally, patients with open neural tube defects have a more medially oriented lumbar pedicle trajectory than the average population. METHODS: A single-institution retrospective review of patients with open neural tube defects was conducted. The demographic parameters and functional and anatomical levels of the defects were noted. CT and MRI scans of the lumbar spine were analyzed; the pedicles from L 1 to S 1 were measured for width (W), length (L) and midline angle (α). The measurements were compared bilaterally, at each level, and with data from previously published reports. RESULTS: 16 scans of pediatric patients (mean = 3.0 ・} 4.3; age range = 7 days to 14.4 years; 7 males, 9 females) with a diagnosis of either myelomeningocele or lipomyelomeningocele were assessed. Most defects occurred in the lumbar region, with L 2 and L 5 accounting for 37.5% each. All angles demonstrated a quadratic increase from L 1 to S 1 (means: L 1 = 28.3 ・} 5.24° ; L 2 = 29.1 ・} 6.2°; L 3 = 33.2 ・} 6.0°; L 4 = 36.8 ・} 5.6°; L 5 = 43.8 ・} 5.9°; S 1 = 52.0 ・} 3.6°) and were more medially angulated than those reported previously; no significant difference existed between right and left measurements (W = 0.65 ≤ p ≤ 0.94; L = 0.91 ≤ p ≤ 1; α = 0.24 ≤p ≤0.86). CONCLUSIONS: Patients with open neural tube defects had more medially angled pedicle trajectories in the lumbar spine when compared to previously reported values.