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We successfully performed a hematopoietic stem cell apheresis on the smallest allogeneic donor reported to date, a 6.1 kg female. After placement of a dialysis catheter in the left femoral vein, the COBE Spectra was primed with one unit of paternal whole blood. Heparin and anticoagulant citrate dextrose, solution A (ACD-A) were slowly administered to the patient. Ionized calcium levels were checked hourly and calcium gluconate was given for hypocalcemia. Coagulation parameters were checked throughout the procedure. We collected 4.4 × 106 CD34+ cells/kg (recipient). The donor tolerated the procedure well and was discharged the following day. Five months later, the child manifests no obvious late effects. Bone Marrow Transplantation (2000) 26, 339–341. [ABSTRACT FROM AUTHOR]

Purpose: Cerebral Palsy (CP) often presents with a sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the oblique pelvis, termed rib-on-pelvis deformity (ROP). ROP can result in costo-iliac impingement, or pain associated with ROP, and can also adversely affect breathing and sitting balance. The goal of this study was to evaluate whether CP patients with ROP have worse health-related quality of life (HRQOL) before surgery and a greater improvement in HRQOL after surgery., Methods: A retrospective analysis of a prospectively collected, multicenter, international registry was performed for all nonambulatory patients with CP treated with spinal fusion with at least two-year follow-up. HRQOL was measured via the Caregiver Priorities & Child Health Index of Life with Disabilities (CPCHILD) questionnaire domains (0 = most disability, 100 = least disability). ROP was defined as having a rib distal to the superior portion of the iliac crest on preop upright radiographs. The ROP group and control group without ROP were compared regarding six domain scores and total score of CPCHILD. Multiple linear regression was used to control for curve apex location, major coronal Cobb angle, type of tone, and pelvic obliquity., Results: 340 patients met inclusion criteria (52% female, mean age 14.0 years). The mean major coronal Cobb angle was 81 degrees and mean pelvic obliquity was 22 degrees. 176 patients (51.8%) had ROP while 164 patients (48.2%) did not. ROP was independently associated with worse preoperative Positioning/Transfers/Mobility (PTM), Comfort & Emotions (C&E), and total CPCHILD score via the CPCHILD questionnaire (p < 0.05). Patients with preoperative ROP experienced a greater improvement in the C&E and PTM domains as well as total CPCHILD score than patients without ROP (p < 0.05)., Conclusion: CP patients with rib-on-pelvis deformity experience more pain and worse HRQOL than patients without this deformity. These patients experienced a greater improvement in HRQOL after spinal fusion measured via the CPCHILD questionnaire., (© 2024. The Author(s), under exclusive licence to Scoliosis Research Society.)

Purpose: Pulmonary function and quality of life (QOL) are important outcome measures for patients with early-onset scoliosis (EOS) undergoing rib-based growing system (RBGS) implantation. The Assisted Ventilation Rating (AVR) measures ventilator requirements in this population. A higher, more severe, score implies negative changes in QOL. The EOS Questionnaire (EOSQ) is a validated outcome measure. Paired measurements for both ratings were compared to clarify correlation between ventilator status and QOL. Secondary analysis aimed at defining QOL between more broad subgroups defined by ventilator use., Methods: AVR and EOSQ scores were extracted from the Pediatric Spine Study Group database for patients 10 years of age and under. Instances were excluded if the time between AVR and EOSQ assessment was greater than 6 months. Scores were compared using Spearman correlation coefficient. Subgroup analysis included control for age, gender, and etiology. Secondary analysis was performed for broad functional grouping using ranked analysis of variance for repeated measures using median scores., Results: Two thousand five hundred and forty-two instances of paired EOSQ and AVR in 329 patients were analyzed. A statistically significant weak correlation between AVR and EOSQ was identified in Child's Health Related QOL and Family Impact sections, in nine subsets. Subgroup analysis showed little variation, except increased correlation in female patients to near moderate level. Analysis of variance for demonstrated decreased medians for all subdomains when comparing those mechanically ventilated to patients who did not require ventilation., Conclusion: Ventilator status tracks with QOL were measured by EOSQ. A more severe AVR is negatively correlated with most domains of the EOSQ for patients with EOS who have undergone RBGS implantation. The strength of this correlation is weak, and so AVR alone may be insufficient to precisely determine QOL in this population., Level of Evidence: Level-III, Retrospective., (© 2022. The Author(s), under exclusive licence to Scoliosis Research Society.)

Study Design: Cross-sectional reliability study., Objective: The deformity angular ratio (DAR) is a means of quantifying magnitude of the coronal (C-DAR) and sagittal (S-DAR) plane of deformity curves to produce a total DAR (T-DAR). It has been shown to predict the risk of spinal cord monitoring alerts and actual neurologic deficits. We sought to assess the reliability of determining the C-DAR and S-DAR among pediatric spinal deformity surgeons., Methods: Twelve preoperative anterior-posterior (AP) and lateral X-rays from the Fox multi-center pediatric deformity study were de-identified and sent to7 pediatric spinal deformity surgeons. Each surgeon measured: coronal/sagittal Cobb angles, upper/lower endplate vertebrae (UEV/LEV), apices, and number of vertebrae included in the main curve. The C-DAR and S-DAR were then calculated by dividing the Cobb angles by the number of vertebrae included in the curve. Intra- and interobserver reliability was calculated using interclass correlation (ICC)., Results: The mean C-DAR was 14.9 (range 1.3-51.5) with a mean Cobb angle of 88.8° (range 15.0-163.0) over a mean of 7.5 (range 2.0-14.0) levels. The mean S-DAR was 8.6 (range 1.0-19.6), with a mean Cobb angle of 68.0° (range 10.0-137.0) over a mean of 7.5 (range 3-11) levels. The intraobserver reliability of the C-DAR was ICC = 0.908 (range 0.846-0.960) and the S-DAR 0.914 (range 0.815-0.961). The interobserver reliability of the C-DAR was ICC = 0.868 (range 0.846-0.938), and the S-DAR was ICC = 0.848 (range 0.815-0.961). Despite poor reliability among UEV, LEV, and apex selection (aggregated range 0.340-0.724), the C-DAR and S-DAR were demonstrated to be reliable in our study CONCLUSIONS: Reliability was good to excellent for C-DAR and S-DAR, despite poor to moderate reliability among UEV, LEV, and apex selection. These data support the use of the C-DAR, S-DAR, and combined T-DAR as a means of quantifying deformity magnitude.

Study Design: Prospective cohort., Objective: To compare preoperative hemoglobin levels to postoperative hemoglobin levels in patients with early-onset scoliosis (EOS)., Summary of Background Data: Elevated hemoglobin (Hgb) may be a marker for preoperative hypoxia in patients with EOS and thoracic insufficiency syndrome (TIS). The changes in Hgb level after treatment may be a surrogate marker for improved oxygenation., Methods: Because normal levels of Hgb vary with patient age, Hgb z scores were calculated by dividing age-adjusted mean Hgb levels by the age-adjusted standard deviation. Elevated Hgb was defined by a hemoglobin z score >1. Patients with a baseline Hgb value measured before initial implantation with at least one follow-up measurement, at 6, 12, or 18 months, were included in longitudinal analysis. Change in Hgb z score as well as change in curve magnitude over time was assessed using piecewise linear mixed modeling for patients with elevated Hgb and those without., Results: Two hundred sixty-seven patients with EOS were treated surgically over the study period. Average age at initial implantation was 6.8 years. Forty-eight (18%; 95% confidence interval = 13.7%, 23.2%) subjects had an elevated Hgb (z score > 1) level before implantation procedure. Hgb levels decreased in subjects with elevated Hgb from implantation to 6 months (p < .001) with no change in Hgb from 6 to 12 months (p = .46) or from 12 to 18 months (p = .59), but an overall decrease from preoperative to 18 months (p < .001). There was no change in Hgb levels for subjects without elevated Hgb from implantation to 6 months (p = .94), from 6 to 12 months (p = .61), or from 12 to 18 months (p = .78)., Conclusions: In some patients with EOS and TIS, there appears to be significant positive impact on oxygenation from distraction instrumentation as evidenced by a meaningful proxy measurement: improvement in abnormal preoperative Hgb levels after surgery., Level of Evidence: III., (Copyright © 2018 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

179 patients with congenital rib fusions treated with rib-based or spine-based constructs and minimum 2-year follow-up were reviewed. 19 patients were treated with proximal spine-based and 160 with proximal rib-based devices. Patients treated with growing rods achieved equivalent SAL to those treated with rib-based devices, while also achieving greater improvement to their spine deformity, as measured by postoperative kyphosis, and Cobb angle.

Study Design: Retrospective review., Objectives: To describe clinical characteristics and infection rates in modern vertical expandable prosthetic titanium rib (VEPTR) surgery., Summary of Background Data: Prior studies have demonstrated infection rates from 10% to 30% with VEPTR surgery., Methods: A retrospective query was done on an institutional review board-approved, multicenter prospectively collected database for patients implanted with VEPTR from 2007 to 2013 at eight sites. This identified 213 patients with appropriate data for analysis. Average follow-up was 4.1 years (range 1.7-6.3). Data collected included a Classification of Early-Onset Scoliosis (C-EOS) diagnosis, American Society of Anesthesiologists Physical Status (ASA-PS), major Cobb angle, construct type, clinical symptoms, and microbiology. The distribution of infection rates across all the study sites was compared. The exact p value was estimated by Monte Carlo simulation., Results: Overall, 18% (38/213) of patients implanted with VEPTR developed infection requiring operative debridement. There were significantly different infection rates among the sites, ranging from 2.9% to 42.9% (p = .029). The average time to infection was 70 days (range 8-236) after the infecting procedure. The majority of infections were due to gram-positive bacteria (80%, 44/55), the most prevalent being methicillin-sensitive Staphylococcus aureus (45%, 25/55). There were 20 patients (53%, 20/38) with either partial or complete implant removal to resolve infection; however, only 3 of 38 (8%) of these resulted in abandonment of VEPTR treatment. There was no difference in infection rate across the primary C-EOS diagnosis categories (p = .21) or based on ASA score (p = .53). After controlling for study site, the odds ratio of an infection following an implant procedure versus an expansion was 2.8 (p = .002). There was no difference in the odds ratio of an infection between the other procedure types (implant, expansion, exchange/revision)., Conclusions: There were significant differences in infection rates between sites. The variability in infection rate indicates a need for guided efforts to standardize best practices for infection control in VEPTR surgery., Level of Evidence: III, therapeutic study., (Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)