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.

Management and surgical outcomes of dystrophic scoliosis in neurofibromatosis type 1: a systematic review.

OBJECTIVE: Neurofibromatosis type 1 (NF1) dystrophic scoliosis is an early-onset, rapidly progressive multiplanar deformity. There are few studies on the surgical management of this patient population. Specifically, perioperative morbidity, instrument-related complications, and quality-of-life outcomes associated with surgical management have not been systematically evaluated. In this study, the authors aimed to perform a systematic review on the natural history, management options, and surgical outcomes in patients who underwent NF1 dystrophic scoliosis surgery. METHODS: A PubMed search for articles with "neurofibromatosis" and either "dystrophic" or "scoliosis" in the title or abstract was performed. Articles with 10 or more patients undergoing surgery for NF1 dystrophic scoliosis were included. Data regarding indications, treatment details, morbidity, and outcomes were summarized and analyzed with descriptive statistics. RESULTS: A total of 310 articles were identified, 48 of which were selected for full-text review; 30 studies describing 761 patients met the inclusion criteria. The mean age ranged from 7 to 22 years, and 99.7% of patients were younger than 18 years. The mean preoperative coronal Cobb angle was 75.2°, and the average correction achieved was 40.3°. The mean clinical follow-up in each study was at least 2 years (range 2.2-19 years). All patients underwent surgery with the intent of deformity correction. The scoliosis regions addressed were thoracic curves (69.6%) and thoracolumbar (11.1%) and lumbar (14.3%) regions. The authors reported on a variety of approaches: posterior-only, combined anterior-posterior, and growth-friendly surgery. For fixation techniques, 42.5% of patients were treated with hybrid constructs, 51.5% with pedicle screw-only constructs, and 6.0% with hook-based constructs. Only 0.9% of patients underwent a vertebral column resection. The nonneurological complication rate was 14.0%, primarily dural tears and wound infections. The immediate postoperative neurological deficit rate was 2.1%, and the permanent neurological deficit rate was 1.2%. Ultimately, 21.5% required revision surgery, most commonly for implant-related complications. Loss of correction in both the sagittal and coronal planes commonly occurred at follow-up. Five papers supplied validated patient-reported outcome measures, showing improvement in the mental health, self-image, and activity domains. CONCLUSIONS: Data on the surgical outcomes of dystrophic scoliosis correction are heterogeneous and sparse. The perioperative complication rate appears to be high, although reported rates of neurological deficits appear to be lower than clinically observed and may be underreported. The incidence of implant-related failures requiring revision surgery is high. There is a great need for multicenter prospective studies of this complex type of deformity.

Posterior-only Resection of Single Hemivertebrae With 2-Level Versus >2-Level Fusion: Can We Improve Outcomes?

BACKGROUND: The outcomes of congenital scoliosis (CS) patients undergoing hemivertebra (HV) resection surgery with a 2-level fusion versus a >2-level fusion are unclear. We hypothesized that CS patients undergoing HV resection and a >2-level fusion have decreased curve progression and reoperation rates compared with 2-level fusions. METHODS: Retrospective review of prospectively collected data from a multicenter scoliosis database. Fifty-three CS patients (average age 4.5, range 1.2 to 10.9 y) at index surgery were included. Radiographic and surgical parameters, complications, as well as revision surgery rates were tracked at a minimum of 2-year follow-up. RESULTS: Twenty-six patients had a 2-level fusion while 27 patients had a >2-level fusion with similar age and body mass index between groups. The HV was located in the lumbar spine for 69% (18/26) 2-level fusions and 30% (8/27) >2-level fusions ( P =0.006). Segmental HV scoliosis curve was smaller in 2-level fusions compared to >2-level fusions preoperatively (38 vs. 50 degrees, P =0.016) and at follow-up (25 vs. 34 degrees, P =0.038). Preoperative T2-T12 (28 vs. 41 degrees, P =0.013) and segmental kyphosis (11 vs. 23 degrees, P =0.046) were smaller in 2-level fusions, but did not differ significantly at postoperative follow-up (32 vs. 39 degrees, P =0.22; 13 vs. 11 degrees, P =0.64, respectively). Furthermore, the 2 groups did not significantly differ in terms of surgical complications (27% vs. 22%, P =0.69; 2-level fusion vs. >2-level fusion, respectively), unplanned revision surgery rate (23% vs. 22%, 0.94), growing rod placement or extension of spinal fusion (15% vs. 15%, P =0.95), or health-related quality of life per the EOS-Questionnaire 24 (EOSQ-24). Comparison of patients with or without the need for growing rod placement or posterior spinal fusion revealed no significant differences in all parameters analyzed. CONCLUSIONS: Two-level and >2-level fusions can control congenital curves successfully. No differences existed in curve correction, proximal junctional kyphosis or complications between short and long-level fusion after HV resection. Both short and long level fusions are viable options and generate similar risk of revision. The decision should be individualized by patient and surgeon.

Best Practice Guidelines for Surgical Site Infection in High-risk Pediatric Spine Surgery: Definition, Prevention, Diagnosis, and Treatment.

BACKGROUND: Prior "best practice guidelines" (BPG) have identified strategies to reduce the risk of acute deep surgical site infection (SSI), but there still exists large variability in practice. Further, there is still no consensus on which patients are "high risk" for SSI and how SSI should be diagnosed or treated in pediatric spine surgery. We sought to develop an updated, consensus-based BPG informed by available literature and expert opinion on defining high-SSI risk in pediatric spine surgery and on prevention, diagnosis, and treatment of SSI in this high-risk population. MATERIALS AND METHODS: After a systematic review of the literature, an expert panel of 21 pediatric spine surgeons was selected from the Harms Study Group based on extensive experience in the field of pediatric spine surgery. Using the Delphi process and iterative survey rounds, the expert panel was surveyed for current practices, presented with the systematic review, given the opportunity to voice opinions through a live discussion session and asked to vote regarding preferences privately. Two survey rounds were conducted electronically, after which a live conference was held to present and discuss results. A final electronic survey was then conducted for final voting. Agreement ≥70% was considered consensus. Items near consensus were revised if feasible to achieve consensus in subsequent surveys. RESULTS: Consensus was reached for 17 items for defining high-SSI risk, 17 items for preventing, 6 for diagnosing, and 9 for treating SSI in this high-risk population. After final voting, all 21 experts agreed to the publication and implementation of these items in their practice. CONCLUSIONS: We present a set of updated consensus-based BPGs for defining high-risk and preventing, diagnosing, and treating SSI in high-risk pediatric spine surgery. We believe that this BPG can limit variability in practice and decrease the incidence of SSI in pediatric spine surgery. LEVEL OF EVIDENCE: Not applicable.

Cerebral venous sinus thrombosis associated with spontaneous heparin-induced thrombocytopenia syndrome after total knee arthroplasty.

Spontaneous heparin-induced thrombocytopenia (HIT) syndrome, characterized by clinical and serologic features of HIT despite the absence of proximate heparin exposure, can be triggered by total knee arthroplasty (TKA). A 56-year-old female receiving aspirin thromboprophylaxis post-TKA presented with aphasia and thrombocytopenia on post-operative day 11. Imaging studies revealed cerebral venous sinus thrombosis (CVST) and intravenous bivalirudin was initiated. Her serum tested strong-positive for IgG anti-PF4/polyanion complexes and serotonin-release assay in the presence and absence of heparin; strong-positive IgG-specific chemiluminescent immunoassay; and moderate-positive latex immunoturbidimetric assay. Two 65 g doses of IVIG were administered. With the improvement of her platelet count, she was transitioned from bivalirudin to warfarin. At one-year follow-up, she remained free of recurrent thrombosis and neurologically stable with a normal platelet count. Previous reports of post-TKA spontaneous HIT syndrome include venous/arterial thrombosis and adrenal hemorrhage, and this report of CVST expands the clinical spectrum of this rare complication of orthopedic surgery.

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.

CT Findings of Pediatric Handlebar Injuries.

Direct bicycle handlebar injuries are a significant cause of chest and abdominal trauma and morbidity in the pediatric population. However, these injuries have been underemphasized. While blunt abdominal trauma has been described well, the literature is limited in reviewing trauma imaging specifically related to direct handlebar injuries in the pediatric population. Major chest injuries include lung contusions, pneumatoceles, and pneumothorax. In the abdomen, injuries to the pancreas, small bowel, mesentery, liver, and spleen are the more common abdominal injuries attributed to direct handlebar trauma. Traumatic abdominal wall hernias and groin injuries, which may be associated with vascular injuries, are other known injuries. The challenge is in both clinical and radiographic diagnosis. The physical findings are often underwhelming, and laboratory values in many studies are shown to be not very sensitive or specific. As a result, there is a risk of delay in imaging, diagnosis, and treatment of significant and sometimes life-threatening injuries. CT is considered the standard examination to delineate intra-abdominal trauma, with a reported sensitivity of 60%-88% and a specificity of 97%-99%. Moreover, CT helps in grading some types of injury and helps guide the surgical treatment course. It is important for radiologists who perform imaging in adults and children to be aware of the significance of direct handlebar injuries and their imaging findings. ©RSNA, 2020.

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.

Pyrimidine tract-binding protein 1 mediates pyruvate kinase M2-dependent phosphorylation of signal transducer and activator of transcription 3 and oncogenesis in anaplastic large cell lymphoma.

PKM2 (pyruvate kinase M2), a critical regulator of glycolysis, is phosphorylated by numerous growth factor receptors and oncogenic tyrosine kinases including NPM-ALK which is expressed in a subset of aggressive T-cell non-Hodgkin lymphomas known as anaplastic large cell lymphoma, ALK-positive. Our previous work demonstrated that phosphorylation of Y105-PKM2 by NPM-ALK regulates a major metabolic shift to promote lymphomagenesis. In addition to its role in metabolism, recent studies have shown that PKM2 promotes oncogenesis by phosphorylating nuclear STAT3 (signal transducer and activator of transcription 3) and regulating transcription of genes involved in cell survival and proliferation. We hypothesized that identification of novel PKM2 interactors could provide additional insights into its expanding functional role in cancer. To this end, immunocomplexes of FLAG-tagged PKM2 were isolated from NPM-ALK-positive ALCL (anaplastic large cell lymphoma) cells and subjected to liquid chromatography tandem mass spectrometry (LC-MS/MS) which led to the identification of polypyrimidine tract-binding protein (PTBP1) as a novel interactor of PKM2. The interaction between PTBP1 and PKM2 was restricted to the nucleus and was dependent on NPM-ALK mediated Y105 phosphorylation of PKM2. Stable shRNA-mediated silencing of PTBP1 resulted in a marked decrease in pY105-PKM2 and pY705-STAT3 which led to decreased ALCL cell proliferation and colony formation. Overall, our data demonstrate that PTBP1 interacts with PKM2 and promotes ALCL oncogenesis by facilitating PKM2-dependent activation of STAT3 within the nucleus.

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.