Despite guidelines to fuse both thoracic and thoracolumbar/lumbar (TH/L) curves in patients with structural curves in both regions, a thoracic-only fusion allows preservation of lumbar motion ...segments. The purpose of this study was to assess the 2-year postoperative three-dimensional (3D) radiographic and clinical outcomes of patients with double or triple major (thoracic curves >TH/L curves) structural curves who underwent a thoracic-only fusion.
A prospective adolescent idiopathic scoliosis registry was queried for double or triple major curves undergoing thoracic-only posterior fusion and a minimum 2-year follow-up. 3D reconstructions were generated from bi-planar radiographs. Paired sample t tests were used to assess differences in the coronal, sagittal, and axial planes pre and postoperatively, as well as Scoliosis Research Society Questionnaire-22 scores. Pearson correlations were utilized to identify variables related to spontaneous lumbar derotation.
Twenty-two patients met the inclusion criteria. Both thoracic 61 ± 10 degrees to 20 ± 9 degrees ( P < 0.001) and lumbar curves 41 ± 7 degrees to 22±7 degrees ( P < 0.001) had significant coronal improvement and T5 to T12 kyphosis improved from 7 ± 14 degrees to 23 ± 8 degrees ( P < 0.001). The thoracic apical translation was significantly improved postoperatively (4.7 ± 1.5 to 0.5 ± 1 cm, P < 0.001), but the lumbar apical translation was unchanged (-1.7 ± 0.6 to -1.7±0.8 cm, P = 0.94). Scoliosis Research Society Questionnaire-22 scores significantly improved by 2 years postoperative.
Unlike the 3D correction observed in nonstructural TH/L curves after thoracic-only fusion, patients with double or triple major curves demonstrated only spontaneous coronal correction of the lumbar curve, whereas the sagittal and axial planes were not significantly improved. These radiographic parameters did not negatively affect subjective or clinical outcomes at minimum 2-year follow-up.
Level IV-therapeutic.
The extracellular matrix comprises a network of macromolecules such as collagens, proteoglycans and glycoproteins. VWA1 (von Willebrand factor A domain containing 1) encodes a component of the ...extracellular matrix that interacts with perlecan/collagen VI, appears to be involved in stabilizing extracellular matrix structures, and demonstrates high expression levels in tibial nerve. Vwa1-deficient mice manifest with abnormal peripheral nerve structure/function; however, VWA1 variants have not previously been associated with human disease. By interrogating the genome sequences of 74 180 individuals from the 100K Genomes Project in combination with international gene-matching efforts and targeted sequencing, we identified 17 individuals from 15 families with an autosomal-recessive, non-length dependent, hereditary motor neuropathy and rare biallelic variants in VWA1. A single disease-associated allele p.(G25Rfs*74), a 10-bp repeat expansion, was observed in 14/15 families and was homozygous in 10/15. Given an allele frequency in European populations approaching 1/1000, the seven unrelated homozygote individuals ascertained from the 100K Genomes Project represents a substantial enrichment above expected. Haplotype analysis identified a shared 220 kb region suggesting that this founder mutation arose >7000 years ago. A wide age-range of patients (6-83 years) helped delineate the clinical phenotype over time. The commonest disease presentation in the cohort was an early-onset (mean 2.0 ± 1.4 years) non-length-dependent axonal hereditary motor neuropathy, confirmed on electrophysiology, which will have to be differentiated from other predominantly or pure motor neuropathies and neuronopathies. Because of slow disease progression, ambulation was largely preserved. Neurophysiology, muscle histopathology, and muscle MRI findings typically revealed clear neurogenic changes with single isolated cases displaying additional myopathic process. We speculate that a few findings of myopathic changes might be secondary to chronic denervation rather than indicating an additional myopathic disease process. Duplex reverse transcription polymerase chain reaction and immunoblotting using patient fibroblasts revealed that the founder allele results in partial nonsense mediated decay and an absence of detectable protein. CRISPR and morpholino vwa1 modelling in zebrafish demonstrated reductions in motor neuron axonal growth, synaptic formation in the skeletal muscles and locomotive behaviour. In summary, we estimate that biallelic variants in VWA1 may be responsible for up to 1% of unexplained hereditary motor neuropathy cases in Europeans. The detailed clinical characterization provided here will facilitate targeted testing on suitable patient cohorts. This novel disease gene may have previously evaded detection because of high GC content, consequential low coverage and computational difficulties associated with robustly detecting repeat-expansions. Reviewing previously unsolved exomes using lower QC filters may generate further diagnoses.
STUDY DESIGN.Retrospective review of a prospectively collected multicenter database.
OBJECTIVE.The aim of this study was to investigate factors associated with low preoperative SRS pain scores.
...SUMMARY OF BACKGROUND DATA.The prevalence of preoperative pain in patients with adolescent idiopathic scoliosis (AIS) has become increasingly evident and is a primary concern for patients and families. Greater preoperative pain is associated with more postoperative pain; however, less is understood about what contributes to preoperative pain.
METHODS.A prospectively collected, multicenter database was queried for patients with AIS. Patients were divided into 2 cohorts based on preoperative SRS pain scores≤ 3 (Pain cohort), 4 to 5 (No Pain cohort). Univariate analysis was performed identifying which factors were associated with a low preoperative SRS score and used for a CART analysis.
RESULTS.Of 2585 patients total, 2141 (83%) patients had SRS pain scores of 4 to 5 (No Pain) and 444 (17%) had SRS pain scores ≤3 (Pain). Female sex, older age, greater % body mass index, larger lumbar curves, greater T5–12 kyphosis, and lower mental health scores were associated with greater preoperative pain. In multivariate CART analysis, lower mental health SRS scores (P = 0.04) and older age (P = 0.003) remained significant, with mental health scores having the greatest contribution. In subdividing the mental health component questions, anxiety-related questions appeared to have the greatest effect followed by mood/depression (SRS Question 13OR 2.04; Q16OR 1.35; Q7OR 1.31; Q3OR 1.20).
CONCLUSION.Anxiety and mood are potentially modifiable risk factors that have the greatest impact on pre- and postoperative pain. These results can be used to identify higher-risk patients and develop preoperative therapeutic protocols to improve postoperative outcomes.Level of Evidence3
: To determine the efficacy of powered intracapsular tonsillectomy (PIT, e.g. regrowth rate) in children who underwent PIT at three different institutions. We also wanted to determine if the trend to ...greater safety through reduced bleeding and re-admission for dehydration, noted in our initial reports, would become statistically significant in a larger sample.
: Multi-center retrospective case series.
: We retrospectively reviewed all charts’ of children who underwent PIT at three different institutions: the Children's Hospital at the Cleveland Clinic, Alfred I. DuPont Hospital for Children, and the New York Otolaryngology Institute. For comparison, we reviewed the outpatient and inpatient records of all children who underwent conventional tonsillectomy performed by the same surgeons at the Children's Hospital at the Cleveland Clinic and Alfred I. DuPont Hospital for Children during the same period. No comparison group was available for the New York Otolaryngology Institute group. Three outcome measures were recorded: regrowth, bleeding and re-admission for dehydration rates. All statistical analyses were performed using SAS, and
P < 0.05 was considered statistically significant.
: We identified 870 children that underwent PIT at three different institutions. In addition, 1121 children underwent conventional tonsillectomy at two of the three institutions. The mean follow-up for the PIT group was 1.2 years (range, 0.1–2.6 years) and 1.5 years (range, 0.1–3.0 years) for the conventional tonsillectomy group. The incidence of and 95% CI for the outcome measures were as follows regrowth 0.5% (0%, 1.4%), delayed post-operative bleeding 0.7% (0%, 1.9%), re-admission for dehydration 1.3% (0.05%, 2.6%), and overall major complications 0.46% (0.009%, 0.9%). When comparing conventional tonsillectomy to PIT, the bleeding rate, re-admission for dehydration, and the overall incidence of major complications were significantly lower in the PIT group (
P = 0.001,
P = 0.002, and
P < 0.001, respectively).
PIT is a safe and effective technique in the management of obstructive sleep disordered breathing in children. PIT has the advantages of decreased pain, dehydration and post-operative bleeding, and with a mean follow-up of 1.2 years, a low incidence of tonsillar regrowth thus far.
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.
Graphic abstract
These slides can be retrieved under Electronic Supplementary Material.
Surgical site infection (SSI) rates in pediatric spinal deformity surgery for cerebral palsy (CP) patients are higher than that in idiopathic scoliosis. The use of vancomycin powder is associated ...with decreased risk of SSI in neuromuscular patients. Prior studies in adult and pediatric early-onset scoliosis patients have shown that vancomycin powder alters microbacterial profile in patients that develop SSI. However, the effects of topical vancomycin powder on microbiology in spinal deformity surgery for CP patients has not been studied.
An international multicenter database of CP neuromuscular scoliosis patients was used in this retrospective cohort study. All patients that underwent posterior spinal instrumented fusion for CP neuromuscular scoliosis from 2008 to 2019 were queried, and 50 cases complicated by postoperative SSI were identified. Intraoperative antibiotic details were documented in 49 cases (98.0%). Microbiology details were documented in 45 cases (91.8%). Microbiology for patients that received topical vancomycin powder were compared with patients that did not. A multivariate regression model was used to control for potential confounders.
There were 45 patients included in this study. There were 27 males (60.0%) and 18 females (40.0%). Mean age at surgery was 14.8±2.4 years. There were 24 patients that received topical vancomycin powder (53.3%). The mean time from index surgery to SSI was 4.3±11.3 months.On univariate analysis of microbiology cultures by vancomycin powder cohort, there were no significant differences in culture types. Proteus spp. trended on significance with association with vancomycin powder use (P=0.078). When controlling for potential confounders on multivariate analysis, intraoperative topical vancomycin powder was associated with increased risk for proteus infection (adjusted odds ratio: 262.900, 95% confidence interval: 1.806-38,267.121, P=0.028).
In CP patients undergoing pediatric spinal deformity surgery, the use of vancomycin powder was independently associated with increased risk for proteus infections. Further study into antibiotic regimens for spinal deformity surgery in the CP population should be performed.
Level III-retrospective cohort study.
OBJECTIVE:As a proxy for undiagnosed HIV, the Centers for Disease Control and Preventionʼs National HIV Behavioral Surveillance (NHBS) monitors participants who report being unaware of their ...infection, defined as self-reporting an HIV-negative or unknown status during the interview but testing positive for HIV infection. We validated the NHBS measure of awareness among MSM in 2014.
DESIGN:We tested dried blood spots from MSM who reported being unaware of their infection for seven antiretrovirals (ARVs). MSM unaware with at least one ARV detected were defined as misreporters.
METHODS:Weighted percentages and 95% confidence intervals were calculated to compare characteristics among misreporters, nonmisreporters, and those who self-reported as HIV-positive. Viral load was quantified with a validated assay using dried blood spots.
RESULTS:Of 1818 HIV-positive MSM, 299 (16%) self-reported as HIV-negative or unknown infection status. Of these 299, 145 (49%) were considered misreporters based on ARV detection. Among the unaware, misreporters were more likely than nonmisreporters to be older and have health insurance. Compared with self-reported HIV-positive MSM, misreporters were more likely to be black, be bisexual, and have perceived discrimination. Of 138 misreporters with viral load data, 116 (84%) had an undetectable viral load.
CONCLUSION:ARV testing revealed that half of MSM classified as unaware of their infection misreported their status. Although off-label preexposure prophylaxis use might explain the presence of ARVs, it is unlikely as many misreporters were virally suppressed, suggesting they were on HIV therapy. Biomarker validation of behavioral data can improve data quality and usefulness in NHBS and other studies.
Purpose
To evaluate the three-dimensional (3D) characteristics of spine deformity in patients with non-idiopathic scoliosis compared with those observed in patients with adolescent idiopathic ...scoliosis (AIS).
Methods
A retrospective chart review was conducted to identify patients with non-idiopathic scoliosis. Twenty-eight patients with neural axis (NA) abnormalities (Chiari 1, syrinx) and 20 patients with connective tissue disorder (CTD) (Marfan’s, Beal’s, Ehlers-Danlos syndrome, mixed) were identified. The 3D parameters of the coronal, sagittal, and axial plane were compared with 284 AIS patients with a similar range of coronal deformity.
Results
The average coronal curve was similar between all three groups (AIS 48 ± 15°, CTD 43 ± 22°, and NA 49 ± 18°;
p
= 0.4). The NA patients had significantly greater 3D thoracic kyphosis (20 ± 18° vs 10 ± 15°,
p
= 0.001) and less thoracic apical vertebral rotation (− 5 ± 18° vs − 12 ± 10°,
p
= 0.003) when compared with AIS. The CTD group’s 3D thoracic kyphosis (
p
= 0.7) and apical vertebral rotation (
p
= 0.09) did not significantly differ from AIS. Significant negative correlations were found in all three groups between thoracic kyphosis and coronal curve magnitude (AIS
r
= − 0.49, CTD
r
= − 0.772, NA
r
= −0.677, all
p
< 0.001).
Conclusions
Scoliotic patients with NA abnormalities have a more kyphotic, less-rotated 3D profile than patients with AIS, while scoliosis patients with CTD have 3D features similar to AIS. Irrespective of the underlying diagnosis, however, greater scoliotic curves were associated with a greater loss of intersegmental kyphosis, suggesting a similar biomechanical pathophysiology for curve progression.
Waist circumference (WC) and waist-to-hip ratio (WHR) are surrogate measures of central adiposity that are associated with adverse cardiovascular events, type 2 diabetes and cancer independent of ...body mass index (BMI). WC and WHR are highly heritable with multiple susceptibility loci identified to date. We assessed the association between SNPs and BMI-adjusted WC and WHR and unadjusted WC in up to 57 412 individuals of European descent from 22 cohorts collaborating with the NHLBI's Candidate Gene Association Resource (CARe) project. The study population consisted of women and men aged 20-80 years. Study participants were genotyped using the ITMAT/Broad/CARE array, which includes ∼50 000 cosmopolitan tagged SNPs across ∼2100 cardiovascular-related genes. Each trait was modeled as a function of age, study site and principal components to control for population stratification, and we conducted a fixed-effects meta-analysis. No new loci for WC were observed. For WHR analyses, three novel loci were significantly associated (P < 2.4 × 10(-6)). Previously unreported rs2811337-G near TMCC1 was associated with increased WHR (β ± SE, 0.048 ± 0.008, P = 7.7 × 10(-9)) as was rs7302703-G in HOXC10 (β = 0.044 ± 0.008, P = 2.9 × 10(-7)) and rs936108-C in PEMT (β = 0.035 ± 0.007, P = 1.9 × 10(-6)). Sex-stratified analyses revealed two additional novel signals among females only, rs12076073-A in SHC1 (β = 0.10 ± 0.02, P = 1.9 × 10(-6)) and rs1037575-A in ATBDB4 (β = 0.046 ± 0.01, P = 2.2 × 10(-6)), supporting an already established sexual dimorphism of central adiposity-related genetic variants. Functional analysis using ENCODE and eQTL databases revealed that several of these loci are in regulatory regions or regions with differential expression in adipose tissue.
Traditionally, 2-year follow-up data have been the established standard for reporting clinical outcomes following spinal deformity surgery. However, previous studies indicate that 2-year follow-up ...does not represent long-term outcomes. Currently, there is no clear data that demonstrate a difference in outcomes between the 1 and 2 years postoperative time-periods following posterior spinal fusions (PSF) for adolescent idiopathic scoliosis (AIS).
A multi-center, prospective database was queried for AIS patients treated with PSF. Clinical outcome scores, assessed by SRS-22, coronal and sagittal radiographic parameters were assessed at time periods: 6 months, 1 year, and 2 years post operatively. Complications and reoperation rates were also assessed. Statistical analysis compared outcomes variables across time-points to assess for significant differences.
694 patients (82.6% female, mean age at surgery 14.9 ± 2.13 years) were identified. Between post-operative year 1 and 2, significant difference in SRS-22 domain scores were present for function domain (4.5 vs. 4.6; p < 0.001), mental health domain (4.3 vs. 4.2; p < 0.001), and total domain score (4.4 vs. 4.4; p = 0.03) but were below the minimal clinically important difference threshold. New complication development was significantly higher within the first year following surgery than the 1-2 year follow-up period (p < 0.001) with greater complication severity within the 0-1 year follow-up period (P = 0.03).
There are no clinically important changes in SRS-22 domain scores between 1 and 2 years following PSF for AIS, with higher complications in the first year following PSF. Two-year follow-up data provide little added clinical information while under-estimating the cumulative complication and reoperation rates at long-term follow-up.
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