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Background: Congenital myopathies (CMs) are complex conditions often associated with early-onset scoliosis (EOS). The purpose of this study was to investigate radiographic outcomes in CM patients undergoing EOS instrumentation as well as complications. Secondarily, we sought to compare these patients to a population with higher prevalence, cerebral palsy (CP) EOS patients., Methods: This is a retrospective study of a prospectively collected multicenter registry. The registry was queried for EOS patients with growth-sparing instrumentation (vertical expandable prosthetic titanium ribs, magnetically controlled growing rods, traditional growing rod, or Shilla) and a CM or CP diagnosis with minimum 2 years follow-up. Outcomes included major curve magnitude, T1-S1 height, kyphosis, and complications., Results: Sixteen patients with CM were included. Six (37.5%) children with CM experienced 11 complications by 2 years. Mean major curve magnitude for CM patients was improved postoperatively and maintained at 2 years (P<0.01), with no significant increase in T1-S1 height or maximum kyphosis(P>0.05). Ninety-seven patients with CP EOS were included as a comparative cohort. Fewer CP patients required baseline respiratory support compared with CM patients (20.0% vs. 92.9%, P<0.01). Fifty-four (55.7%) CP patients experienced a total of 105 complications at 2 years. There was no evidence that the risk of complication or radiographic outcomes differs between cohorts at 2 years, though CP EOS patients experienced significant improvement in all measurements at 2 years., Conclusions: EOS CM children face a high risk of complication after growing instrumentation, with similar curve correction and risk of complication to CP patients., Level of Evidence: Level III., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population., Methods: A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation., Results: Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (±4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced ≥6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following., Conclusions: This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Early-onset scoliosis (EOS) is a complex, heterogeneous condition involving multiple etiologies, genetic associations, and treatment plans. In 2014, Williams and colleagues proposed a classification system of EOS (C-EOS) that categorizes patients by etiology, Cobb angle, and kyphosis. Shortly after, Smith and colleagues validated a classification system to report complications of growth-friendly spine instrumentation. Severity refers to the level of care and urgency required to treat the complication, and can be classified as device-related or disease-related complications. The purpose of this study was to investigate if C-EOS can be used as a reliable predictor of Smith complications to better risk stratify these young, surgical patients., Methods: This study queried retrospective data from a large multicenter registry with regard to growth-friendly spine instrumentation in the EOS population. One-hundred sixteen patients were included in final data analysis to investigate the outcomes of their growth-friendly procedures according to the Smith complication classification system., Results: There were 245 Smith complications distributed among 116 patients included in this study (2.1 complications per patient). The majority of the complications were device related requiring at least one unplanned trip to the operating room (Smith Device Complication IIA or IIB; 111 complications). There were no complications that caused abandonment of growth-friendly instrumentation (Smith Complication III) and no mortalities (Smith Complication IV). The most severely affected EOS group was the hyperkyphotic syndromic group with a major curve angle >50 degrees (S3+), with 3.4 complications per patient. The least affect EOS group was the hyperkyphotic congenital group with a major curve angle between 20 and 50 degrees, with 0.3 complications per patient., Conclusions: The C-EOS simplifies a complex pathology and the Smith complication classification scheme creates a language to discuss treatment of known complications of growth-friendly spine surgery. Although there is an association between more advanced C-EOS and Smith complications, there does not appear to be a correlation that can satisfy a risk stratification at this time., Level of Evidence: Level II.

Objectives: A phase I trial was conducted to determine the maximally tolerated dose (MTD) and dose-limiting toxicities (DLTs) of docetaxel, capecitabine, and carboplatin for first-line treatment of patients with metastatic esophageal and gastric cancers., Methods: Twenty-eight patients were treated over 5 dose levels in a 21-day cycle. Patients received carboplatin (AUC = 2) on days 1 and 8, docetaxel (35-40 mg/m2) on days 1 and 8, and capecitabine (500-2000 mg/m2) on days 1 to 10., Results: There were no DLTs in the first cycle of treatment. Dose reductions were required in 10 of 15 patients at the final dose level due to neutropenia, nausea, vomiting, diarrhea, dehydration, and hand/foot syndrome following a median of 3 cycles of treatment. Therefore, escalation beyond dose level 5 was not attempted. The MTD was docetaxel, 40 mg/m2 days 1 and 8; carboplatin, AUC = 2 days 1 and 8; and capecitabine, 1500 to 2000 mg/m2 days 1 to 10 in a 21-day cycle. Ten of 25 patients who could be evaluated (40%) responded and 8 of 14 patients treated at the final dose level responded (57%)., Conclusions: Cumulative gastrointestinal toxicities and neutropenia were the DLTs of docetaxel, capecitabine, and carboplatin. This combination represents an easily administered, active regimen for patients with metastatic gastric and esophageal cancers. Further evaluation of this regimen is indicated.

There is a clear need for standardized measures to assess health status that are valid and appropriate to the needs of children with orthopaedic problems. The Child Health Questionnaire and the American Academy of Orthopaedic Surgeons Pediatric Outcomes Data Collection Instrument, two new pediatric health status measures, were assessed for their ability to detect differences in health states in a pediatric orthopaedic population. The instruments have a range of scales designed to measure various aspects of physical and psychosocial health. Two hundred forty-two patients with wide-ranging diagnoses were enrolled in this cross-sectional study. The instruments exhibited ceiling effects in some domains but generally performed as they were intended in this large cohort. Using secondary factor analysis, it was shown that the domains of the instruments appropriately distinguish physical and psychosocial health. Several domains from each instrument discriminated between diagnosis groups and patients with varying numbers of comorbidities. Both of these measures show significant promise and have an important role in helping define the outcomes of children with orthopaedic problems.

New pressures of accountability brought on by a rapidly evolving system of health care financing have underscored the need for standardized, valid measures of patient outcome that reflect the effect of clinical intervention on all aspects of quality of life. In response, there has been a burgeoning interest in the area of outcomes assessment and measurement of quality of life after orthopaedic intervention in adults, but less attention has been focused on the assessment of broadly defined outcomes in children. In an effort to borrow from the broader adult experience in this area, the authors sought to examine whether the Medical Outcomes Study Short Form 36 (SF-36) or the EuroQol questionnaire, widely accepted adult health status measures, would be valid in this setting. These two measures were administered to 196 adolescent patients (10-18 years old) seeking orthopaedic evaluation. Tests of scale properties and construct validity show that these properties are maintained in this population, but neither instrument reflected known differences in health status among this cohort. Most importantly, both the SF-36 and the EuroQol exhibited serious ceiling effects (most respondents scored at the top of their scales), despite evidence indicating those patients often had suboptimal health status. Thus, neither the SF-36 nor the EuroQol is valid for use in this population. The assessment of pediatric health status demands outcomes measures specifically designed to reflect the unique needs of this population.

Concerns about the transmission of the human immunodeficiency virus (HIV) have driven the evolution of surgical transfusion practices including the use of preoperative erythropoietin (rhEPO). Although there is significant experience documenting the efficacy of preoperative rhEPO in reducing transfusion requirements for adult patients, there is little experience in the pediatric population. With 178 pediatric patients who underwent surgery for spinal deformity, a retrospective cohort study was performed using patient charts, administrative records, and blood bank computer data. Of these patients, 44% received erythropoietin and 55% did not. From the entire population, 17.5% were in the rhEPO treatment group that received homologous blood transfusion compared with 30.6% in the untreated group (p < 0.05). Among the children with idiopathic scoliosis, this effect was more pronounced, with 3.9% of rhEPO patients receiving blood transfusion compared with 23.5% of nontreated patients (p = 0.006). Additionally, rhEPO treatment was associated with a significantly decreased length of stay only for patients in the idiopathic group (9.3 vs. 6.7, p = 0.02). Use of preoperative erythropoietin in pediatric patients undergoing scoliosis surgery resulted in higher preoperative hematocrit levels. Significantly lower rates of transfusion were noted only in the idiopathic group, however. Although there is a possibility of erythropoietin "resistance" in the neuromuscular and congenital patients, alternative explanations for the lack of effect on transfusion rates may include underdosing and biases existent in this nonrandomized retrospective study.

Study Design: Radiographic analysis of a pediatric population with spondylolisthesis was performed to examine sagittal plane pelvic rotation and degree of slip over time., Objectives: To determine whether the degree of standing sagittal offset of L5 with respect to the acetabulum correlated with slip progression and symptoms., Summary of Background Data: The natural history of isthmic spondylolisthesis remains unclear. Attempts to predict slip progression in the clinical setting, and thus the possible need for eventual surgical intervention, remain imprecise. Predicting slip progression based on sagittal alignment of the L5 vertebra with respect to the acetabulum has been proposed by some investigators., Methods: Fifty-two children and adolescents were followed clinically and radiographically for an average of 5.6 years. Serial lateral standing radiographs that included the hips and lumbar spine were measured to compute a sagittal pelvic tilt index. The latter value is a ratio of relative distances from the center of S2 to the projection of L5 and the center of the femoral heads on the horizontal., Results: Of the 52 patients studied, 38 have remained asymptomatic without significant slip progression or change in sagittal pelvic tilt index ratio. Of the original group, 13 patients had significant symptoms and revealed a decrease in the sagittal pelvic tilt index over time. Eight of the 13 stabilized at the end of adolescence, whereas 5 had continued decrease in the sagittal pelvic tilt index ratio. These five required operative treatment for pain and progressive slip., Conclusions: The sagittal pelvic tilt index gives the examiner an objective measure of the stability of the lumbosacral junction by quantifying the relationship between S2, the center of the hip, and L5. A decreasing sagittal pelvic tilt index ratio in this preliminary series correlated with slip progression and risk of conservative treatment failure, whereas those patients with a stable sagittal pelvic tilt index did not progress and remained clinically asymptomatic.

We reviewed the results of motor evoked potential (MEP) and somatosensory evoked potential (SEP) monitoring during 116 operations on the spine or spinal cord. We monitored MEPs by electrically stimulating the spinal cord and recording compound muscle action potentials from lower extremity muscles and monitored SEPs by stimulating posterior tibial or peroneal nerves and recording both cortical and subcortical evoked potentials. We maintained anesthesia with an N2O/O2/opioid technique supplemented with a halogenated inhalational agent and maintained partial neuromuscular blockade using a vecuronium infusion. Both MEPs and SEPs could be recorded in 99 cases (85%). Neither MEPs nor SEPs were recorded in eight patients, all of whom had preexisting severe myelopathies. Only SEPs could be recorded in two patients, and only MEPs were obtained in seven cases. Deterioration of evoked potentials occurred during nine operations (8%). In eight cases, both SEPs and MEPs deteriorated; in one case, only MEPs deteriorated. In four cases, the changes in the monitored signals led to major alterations in the surgery. We believe that optimal monitoring during spinal surgery requires recording both SEPs and MEPs. This provides independent verification of spinal cord integrity using two parallel but independent systems, and also allows detection of the occasional insults that selectively affect either motor or sensory systems.

The purpose of this study is to determine the usefulness of the King classification in predicting decompensation in adolescent idiopathic scoliosis. Fifty-one patients were reviewed with a mean follow-up of 25 months. Five patients had Type 1 adolescent idiopathic scoliosis: four were treated with Zielke/Cotrel-Dubousset instrumentation or Zielke instrumentation alone. Correction was greater than 51% in these cases and there was no decompensation. Twenty-three patients had Type II scoliosis. Nineteen of whom were treated with Cotrel-Dubousset instrumentation; 3 with Zielke and Cotrel-Dubousset instrumentation, and 1 with Zielke. The best correction occurred with anterior/posterior instrumentation. Decompensation occurred in 9 patients, all of whom were treated with Cotrel-Dubousset instrumentation alone. Fourteen patients had Type III scoliosis. All were treated with Cotrel-Dubousset instrumentation with correction of 65%. Decompensation occurred in 4 patients, all of whom were fused to or beyond the stable vertebra. Four patients had Type IV scoliosis; all were fused short of the stable vertebra with Cotrel-Dubousset instrumentation, resulting in correction of 52% and no decompensation. Five patients had Type V instrumentation; four were treated with Cotrel-Dubousset instrumentation and 1 with Zielke. There was no relationship between level of fusion and decompensation. Based on this study, the authors contend that the King classification is a valuable tool in the selection of type of instrumentation and fusion level.