Fassier-Duval Rod Failure: Is It Related to Positioning in the Distal Epiphysis?

BACKGROUND: The relationship between Fassier-Duval (FD) rod placement and rod failure rates has not previously been quantified. METHODS: Retrospective review was conducted on patients with osteogenesis imperfecta treated with FD rods between 2005 and 2017. Age at first surgery, sex, Sillence type of osteogenesis imperfecta, bisphosphonate treatment, location of rod (side of body and specific bone), and dates of surgeries, radiographs, and rod failures were collected. C-arm images determined rod fixation within the distal epiphysis at the time of surgery. C-arm variables included rod deviation (percent deviation from the midline of the distal epiphysis) and anatomical direction of deviation (anterior/posterior and medial/lateral). X-ray images were examined for rod failure, which was defined as bending, pulling out of the physis, protrusion out of the bone, and/or failure to telescope. Cox proportional hazards regression models were used to compare failure rates with location of placement within the distal epiphysis allowing for clustering of the data by side (left or right) and bone (femur or tibia). RESULTS: The cohort was 13 patients (11 female individuals and 2 male individuals) with a total of 66 rods and 75 surgeries. Mean time from the first surgery to the last follow-up visit was 8.9 years (SD=5 y). There was a 7% increase in hazard of failure per 1-mm increase in antero-posterior (AP) deviation [hazard ratio (HR), 1.07; 95% confidence interval (CI), 1.01-1.14; P=0.029)]. Similarly, there was a 9% increase in hazard of failure for every 1-mm increase in lateral deviation (HR, 1.09; 95% CI, 1.01-1.18; P=0.019). A 12% increase in hazard of failure per 10% increase in deviation from the midline for both AP and lateral radiograph views was also found, although this was only statistically significant for lateral deviation on the AP radiograph view (HR, 1.12; 95% CI, 1.01-1.25; P=0.030). CONCLUSIONS: FD rod placement within the distal epiphysis has significant impact on increasing rod survival. LEVEL OF EVIDENCE: Level III-therapeutic study.

Fassier-Duval Rods are Associated With Superior Probability of Survival Compared With Static Implants in a Cohort of Children With Osteogenesis Imperfecta Deformities.

BACKGROUND: The survival of Fassier-Duval (FD) telescoping rods as compared with static implants in children affected by osteogenesis imperfecta is not well characterized. The purpose of this study was to compare risk of lower extremity implant failure in FD rods versus static implants. METHODS: Data were retrospectively collected from patients with osteogenesis imperfecta who underwent surgical treatment using either FD rods or static implants (Rush rods, flexible nails, or Steinmann pins) between 1995 and 2015. The timing of implant failure was the primary outcome variable of interest. Comparisons were limited to limbs with no previous history of implants. Cox-proportional hazards regression analyses were used to compare the hazard of implant failure across implants. Negative binomial regression analyses were used to compare the incidence of surgical procedures in the 2 implant groups. RESULTS: The final cohort consisted of 64 limbs (n=21 patients). The static implant group (n=38) consisted of 24 Rush rods (63%), 14 flexible nails (37%), and 2 Steinmann pins (5%). The hazard of implant failure in the static implant group was 13.2 times [95% confidence interval (CI), 2.5-69.6; P=0.0024] the hazard of implant failure in the FD rod group. The hazard of implant failure among females was 4.8 (95% CI, 1.4-16.7; P=0.0125) times the hazard of implant failure among males. The total surgery rate in the static implant group was 7.8 (95% CI, 1.8-33.0; P=0.0056) times the total surgery rate in the FD group. CONCLUSIONS: Among surgically naive limbs, FD rods were associated with significantly improved probability of survival compared with static implants. LEVEL OF EVIDENCE: Level II-retrospective study.

A polygenic burden of rare variants across extracellular matrix genes among individuals with adolescent idiopathic scoliosis.

Adolescent idiopathic scoliosis (AIS) is a complex inherited spinal deformity whose etiology has been elusive. While common genetic variants are associated with AIS, they explain only a small portion of disease risk. To explore the role of rare variants in AIS susceptibility, exome sequence data of 391 severe AIS cases and 843 controls of European ancestry were analyzed using a pathway burden analysis in which variants are first collapsed at the gene level then by Gene Ontology terms. Novel non-synonymous/splice-site variants in extracellular matrix genes were significantly enriched in AIS cases compared with controls (P = 6 × 10(-9), OR = 1.7, CI = 1.4-2.0). Specifically, novel variants in musculoskeletal collagen genes were present in 32% (126/391) of AIS cases compared with 17% (146/843) of in-house controls and 18% (780/4300) of EVS controls (P = 1 × 10(-9), OR = 1.9, CI = 1.6-2.4). Targeted resequencing of six collagen genes replicated this association in combined 919 AIS cases (P = 3 × 10(-12), OR = 2.2, CI = 1.8-2.7) and revealed a highly significant single-gene association with COL11A2 (P = 6 × 10(-9), OR = 3.8, CI = 2.6-7.2). Importantly, AIS cases harbor mainly non-glycine missense mutations and lack the clinical features of monogenic musculoskeletal collagenopathies. Overall, our study reveals a complex genetic architecture of AIS in which a polygenic burden of rare variants across extracellular matrix genes contributes strongly to risk.

Prevalence of Persistent Median Arteries in the Pediatric Population on Ultrasonography.

OBJECTIVES: To use real-time ultrasonography to estimate the prevalence of persistent median arteries in a cohort of pediatric orthopedic patients. METHODS: With Institutional Review Board approval, patients between the ages of 3 months and 19 years were recruited for this cross-sectional study. Variables of interest included demographics, underlying diagnoses, and the presence of a Doppler-confirmed median artery. Ultrasonographic examinations were performed on both upper extremities by a single investigator. Patient- and limb-level analyses were performed. A multivariable generalized logistic regression analysis was used to test the association between the prevalence of limb-specific persistent median arteries and age. A generalized estimating equation was used to adjust for the inclusion of multiple limbs from the same patient. RESULTS: A total of 135 patients (270 limbs) were evaluated. The patient-specific prevalence rate (persistent median arteries present in 1 or both limbs) was 26.7%. Among these patients (n = 36), a persistent median artery was present bilaterally in 55.6% (n = 20). The limb-specific prevalence rate (proportion of limbs with a persistent median artery) was 20.7%. After adjusting for race, for every 1-year increase in age, the odds of a persistent median artery decreased by 4.4%. After adjusting for age, African Americans were significantly more likely to present with a persistent median artery (odds ratio, 3.78; 95% confidence interval, 1.25-11.48). CONCLUSIONS: Ultrasonography can effectively visualize anatomic variants, such as persistent median arteries, in the pediatric population. The prevalence of persistent median arteries was higher than anticipated, especially among African American patients.

Clinical and Radiographic Outcomes After Submuscular Plating (SMP) of Pediatric Femoral Shaft Fractures.

BACKGROUND: To evaluate lower extremity strength and alignment among children who underwent submuscular plating (SMP). METHODS: Subjects who underwent SMP for a length unstable femoral fracture returned for isokinetic strength testing ≥2 years after surgery. Extensor and flexor strength deficits (percent difference) between the operative and nonoperative limbs were evaluated. Radiographic measurements of mechanical alignment [anatomic lateral distal femoral angle (aLDFA)] and qualitative measurements (The Pediatric Outcomes Data Collection Instrument, PODCI) were obtained from all subjects. The upper 97.5% confidence interval approach to establishing clinical equivalency was utilized to compare differences in strength and alignment between the operative and nonoperative limbs. An extensor strength deficit of >19% and an aLDFA discrepancy of >5 degrees were considered to be clinically significant. RESULTS: The average age at surgery of the 10 subjects included in the study was 8.7 years. The hardware was placed an average of 27.9 mm from the distal femoral physis and was removed 6.4 months postsurgery. Among all subjects, the median PODCI scores were ≥97 according to all subscales. There was no significant difference in extension torque between the operative versus nonoperative limbs at 60 degrees/s (P=0.5400), 120 degrees/s (P=0.4214), or 180 degrees/s (P=0.8166). More importantly, extension strength deficits between the operative and nonoperative limbs were not clinically significant at 60 degrees/s [upper 97.5% confidence interval (CI), 10.9%], 120 degrees/s (upper 97.5% CI, 11.0%), or 180 degrees/s (upper 97.5% CI, 10.7%). The difference in aLDFA between the operative and nonoperative limb was less than the predefined clinically significant threshold of 5 degrees for all subjects. CONCLUSIONS: SMP achieves satisfactory clinical and functional results. In this series, extensor strength deficits and/or lower extremity malalignment were not clinically meaningful. High patient satisfaction can be expected after implant removal. LEVEL OF EVIDENCE: Level IV-case series.

Outcomes of Isolated Varus Derotational Osteotomy in Children With Cerebral Palsy Hip Dysplasia and Predictors of Resubluxation.

BACKGROUND: The appropriate intervention for hip subluxation or dislocation in children affected by cerebral palsy (CP) remains controversial. The purpose of this retrospective study was to report radiographic and clinical outcomes following isolated femoral varus derotational osteotomy (VDRO) in children with CP hip dysplasia. Risk factors for resubluxation and avascular necrosis (AVN) were also examined. METHODS: A cohort of 100 patients (199 hips) with CP treated with isolated VDRO between 2003 and 2009 was reviewed. All but 1 patient received bilateral surgery. Patients were followed for an average of 5.4 years (range, 1.03 to 10.20 y). Anteroposterior pelvic radiographs were used to assess migration percentage (MP), Shenton's line, and presence of AVN. Resubluxation was defined as a postoperative break in Shenton's line. Radiographic outcomes and risk analysis was performed in the 91 subjects (179 hips) with radiographic follow-up >1 year. RESULTS: Significant improvement was observed in MP, and all hips had a reconstituted Shenton's line following surgery. Over the course of follow-up, 16% of hips were noted to have a repeat break in Shenton's line. Univariate risk analysis showed preoperative MP, Gross Motor Function Classification System (GMFCS) level, and age at surgery were risk factors for a recurrent line break. Preoperative MP and GMFCS level were found to be predictors of resubluxation in multivariate analysis. AVN was detected in 10 hips (5.7%). GMFCS level V patients were more at risk for resubluxation, but less at risk for AVN when compared with ambulatory (GMFCS I/II/II) patients and GMFCS level IV patients. CONCLUSIONS: Performing a VDRO without additional procedures provided a stable and concentrically reduced hip joint in this population of children with CP. Attention should be paid to initial ambulatory status during the postoperative period. Concomitant procedures such as pelvic osteotomy should be considered for patients of GMFCS level IV and V, as these patients were more at risk for recurrent subluxation. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Rare variants in FBN1 and FBN2 are associated with severe adolescent idiopathic scoliosis.

Adolescent idiopathic scoliosis (AIS) causes spinal deformity in 3% of children. Despite a strong genetic basis, few genes have been associated with AIS and the pathogenesis remains poorly understood. In a genome-wide rare variant burden analysis using exome sequence data, we identified fibrillin-1 (FBN1) as the most significantly associated gene with AIS. Based on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS cases and 669 controls. In individuals of European ancestry, rare variants in FBN1 and FBN2 were enriched in severely affected AIS cases (7.6%) compared with in-house controls (2.4%) (OR = 3.5, P = 5.46 × 10(-4)) and Exome Sequencing Project controls (2.3%) (OR = 3.5, P = 1.48 × 10(-6)). Scoliosis severity in AIS cases was associated with FBN1 and FBN2 rare variants (P = 0.0012) and replicated in an independent Han Chinese cohort (P = 0.0376), suggesting that rare variants may be useful as predictors of curve progression. Clinical evaluations revealed that the majority of AIS cases with rare FBN1 variants do not meet diagnostic criteria for Marfan syndrome, though variants are associated with tall stature (P = 0.0035) and upregulation of the transforming growth factor beta pathway. Overall, these results expand our definition of fibrillin-related disorders to include AIS and open up new strategies for diagnosing and treating severe AIS.

Does Strict Adherence to the Ponseti Method Improve Isolated Clubfoot Treatment Outcomes? A Two-institution Review.

BACKGROUND: Despite being recognized as the gold standard in isolated clubfoot treatment, the Ponseti casting method has yielded variable results. Few studies have directly compared common predictors of treatment failure between institutions with high versus low failure rates. QUESTIONS/PURPOSES: We asked: (1) is the provider's rigid adherence to the Ponseti method associated with a lower likelihood of unplanned clubfoot surgery, and (2) at the institution that did not adhere rigidly to Ponseti's principles, are any demographic or treatment-related factors associated with increased likelihood of unplanned clubfoot surgery? METHODS: After institutional review board approval, a consecutive series of patients with a diagnosis of isolated clubfoot who underwent treatment between January 2003 and December 2007 were identified. At Institution 1, 91 of 133 patients met the eligibility criteria and were followed for a minimum of 2 years compared with 58 of 58 patients at Institution 2. At Institution 1, 16 providers managed care using a conservative casting approach based on the Ponseti method. However, treatment was adapted by the provider(s). At Institution 2, one orthopaedic surgeon managed care with strict adherence to the Ponseti method. Surgical indications at both institutions included the presence of a persistent equinovarus foot position while standing. A chart review was used to collect data related to proportion of patients undergoing unplanned additional treatment for deformity recurrences after Ponseti casting, demographics, and treatment patterns. RESULTS: The proportion of subjects who underwent unplanned major surgical intervention was greater (odds ratio [OR], 51.1; 95% CI, 6.8-384.0; p < 0.001) at Institution 1 (60 of 131, 47%) compared with Institution 2 (two of 91, 2%). There was no difference (p = 0.200) in the proportion of patients who underwent additional casting, repeat tendo Achilles lengthening, and/or anterior tibialis tendon transfer only (minor recurrence) at Institution 1 (nine of 131, 7%) compared with Institution 2 (11 of 91, 13%). At Institution 1, an increase in the number of revision casts (multiple vs no casts, hazard ratio [HR] = 3.9; 95% CI, 2.0-7.6; p < 0.001) and an increase in the number of cast-related complications (multiple vs no complications, HR = 2.8; 95% CI, 1.2-6.7; p = 0.019) were associated with increased risk of major surgery in the multivariate analysis. CONCLUSIONS: Rigid commitment to the Ponseti method in the conservative treatment of patients with isolated clubfoot was associated with a lower risk of subsequent unplanned surgical intervention. In addition, clubfoot treatment programs that use a care model that prioritizes continuity in care and dedication to the Ponseti method may decrease the proportion of patients who undergo unplanned surgical intervention. LEVEL OF EVIDENCE: Level III, therapeutic study.

Incidence of Deep Vein Thrombosis and Pulmonary Embolism in the Elective Pediatric Orthopaedic Patient.

BACKGROUND: Although venous thromboembolism (VTE) has been well studied in the pediatric trauma population, rates of VTE associated with elective pediatric orthopaedic procedures have not been addressed in current literature. The purpose of this retrospective study was to identify the incidence of VTE in the elective pediatric orthopaedic surgical population and delineate subsets of this population at greatest risk. This study may provide valuable data to begin the process of resolving the controversy surrounding deep vein thrombosis prophylaxis in the pediatric orthopaedic population. METHODS: The Pediatric Health Information System was queried for patients admitted on an ambulatory or inpatient basis, aged below 18 years, from January 2006 to March 2011 during which an elective orthopaedic surgery was the principal procedure performed. Patients with diagnoses or procedures related to infection, trauma, malignancy, or coagulopathies were excluded. Patients admitted through the emergency department or whose orthopaedic procedure was not performed on the admission date were excluded. Age, sex, ethnicity, race, admission year, and all procedures/diagnoses were recorded. The presence of VTE at the index admission or any subsequent readmission within 90 days was recorded. All criteria were coded using ICD-9-CM codes. Generalized logistic regression analyses were used to identify factors related to VTE. RESULTS: A total of 143,808 admissions (117,676 patients) matched the inclusion criteria. Thirty-three had a VTE during the index admission with an additional 41 at subsequent readmissions, for a total incidence of 0.0515% by admission and 0.0629% by patient. In the multivariable model, variables significantly (P<0.05) related to VTE included increasing age, admission type, diagnosis of metabolic conditions, obesity, and/or syndromes, and complications of implanted devices and/or surgical procedures. No procedure variables were significantly related to VTE in the multivariable model. CONCLUSIONS: The incidence of VTE in this cohort of pediatric patients undergoing elective orthopaedic surgery was 0.0515%. In children, underlying diagnosis seems to be a stronger predictor of VTE than procedures performed. Diagnosis with a metabolic condition, syndrome, and/or obesity, complications of implanted devices and/or surgical procedures, older age, and admission as an inpatient were significantly related to the development of a VTE. LEVEL OF EVIDENCE: Level IV­case series.

Are copy number variants associated with adolescent idiopathic scoliosis?

BACKGROUND: Adolescent idiopathic scoliosis (AIS) is a complex genetic disorder that causes spinal deformity in approximately 3% of the population. Candidate gene, linkage, and genome-wide association studies have sought to identify genetic variation that predisposes individuals to AIS, but the genetic basis remains unclear. Copy number variants are associated with several isolated skeletal phenotypes, but their role in AIS, to our knowledge, has not been assessed. QUESTIONS/PURPOSES: We determined the frequency of recurrent copy number rearrangements, chromosome aneuploidy, and rare copy number variants in patients with AIS. METHODS: Between January 2010 and August 2014, we evaluated 150 patients with isolated AIS and spinal curvatures measuring 10° or greater, and 148 agreed to participate. Genomic copy number analysis was performed on patients and 1079 control subjects using the Affymetrix(®) Genome-wide Human SNP Array 6.0. After removing poor quality samples, 143 (97%) patients with AIS were evaluated for copy number variation. RESULTS: We identified a duplication of chromosome 1q21.1 in 2.1% (N = 3/143) of patients with AIS, which was enriched compared with 0.09% (N = 1/1079) of control subjects (p = 0.0057) and 0.07% (N = 6/8329) of a large published control cohort (p = 0.0004). Other notable findings include trisomy X, which was identified in 1.8% (N = 2/114) of female patients with AIS, and rearrangements of chromosome 15q11.2 and 16p11.2 that previously have been associated with spinal phenotypes. Finally, we report rare copy number variants that will be useful in future studies investigating candidate genes for AIS. CONCLUSIONS: Copy number variation and chromosomal aneuploidy may contribute to the pathogenesis of adolescent idiopathic scoliosis. CLINICAL RELEVANCE: Chromosomal microarray may reveal clinically useful abnormalities in some patients with AIS.

Intra-familial tests of association between familial idiopathic scoliosis and linked regions on 9q31.3-q34.3 and 16p12.3-q22.2.

OBJECTIVE: Custom genotyping of markers in families with familial idiopathic scoliosis were used to fine-map candidate regions on chromosomes 9 and 16 in order to identify candidate genes that contribute to this disorder and prioritize them for next-generation sequence analysis. METHODS: Candidate regions on 9q and 16p-16q, previously identified as linked to familial idiopathic scoliosis in a study of 202 families, were genotyped with a high-density map of single nucleotide polymorphisms. Tests of linkage for fine-mapping and intra-familial tests of association, including tiled regression, were performed on scoliosis as both a qualitative and quantitative trait. RESULTS AND CONCLUSIONS: Nominally significant linkage results were found for markers in both candidate regions. Results from intra-familial tests of association and tiled regression corroborated the linkage findings and identified possible candidate genes suitable for follow-up with next-generation sequencing in these same families. Candidate genes that met our prioritization criteria included FAM129B and CERCAM on chromosome 9 and SYT1, GNAO1, and CDH3 on chromosome 16.

Variability in spinal surgery outcomes among children's hospitals in the United States.

BACKGROUND: We performed a retrospective cohort study of 7637 spinal fusion surgical cases from 2004 to 2006 at 38 children's hospitals participating in the Pediatric Health Information System database to evaluate the variability of in-hospital outcomes by patient factors and between facilities in children who underwent spinal surgery. METHODS: Outcomes were stratified by whether children did or did not have neurological impairment. Multilevel multivariate logistic regression models were used to determine patient and hospital factors associated with in-hospital infections, surgical complications, and length of stay (LOS)≥10 days. RESULTS: Neurologically impaired (NI) children (N=2117 out of 7637) represented 28% of the cases. The interhospital interquartile range of LOS for NI children was 6 to 8 days (median 7 d) and for non-neurologically impaired (NNI) children was 5 to 6 days (median 5 d). Children with NI had roughly 6 times higher rates of in-hospital infection and 3 times higher complication rates: major interhospital variation was seen for both of these outcomes. Hospital rates of infection ranged from 0% to 27% (median 10%) for NI and from 0% to 14% (median 2%) for NNI children. Complication rates ranged from 0% to 89% (median 33%) for NI and from 3% to 68% (median 9%) for NNI children. The following factors were associated with a LOS≥10 days: in-hospital infection (P<0.0001), surgical complication (P<0.0001), and anterior/posterior versus posterior-only surgery (P<0.0001). Hospital case volume was not associated with infection, surgical complication, or LOS≥10 days. CONCLUSIONS: Substantial variation exists in reported outcomes for children undergoing spinal surgery in children's hospitals within the United States. Further study is needed to characterize hospital-level factors related to surgical outcome to direct future quality improvement.

The role of serial casting in early-onset scoliosis (EOS).

BACKGROUND: Serial casting has demonstrated efficacy for idiopathic early-onset scoliosis (EOS). Results of casting in nonidiopathic (syndromic and congenital) EOS patients have not previously been well described. METHODS: A total of 53 patients underwent serial casting for EOS from 2005 to 2010 at a single institution. Deformity was classified as idiopathic or nonidiopathic. Diagnosis, time in cast, number of casts, use of bracing, complications, and outcomes were recorded. Radiographic measures included Cobb angle and thoracic height (T1-T12). Thoracic height velocity was calculated and compared with established norms. RESULTS: A total of 36 patients, 19 idiopathic and 17 nonidiopathic (14 syndromic, 3 congenital), completed cast treatment and had >6-month follow-up and were therefore included. Of those, 17% (6/36) experienced resolution of their deformity, 53% (19/26) are currently in braces, and 31% (11/36) had undergone surgery. Surgery occurred on average at age 5.6 years and was delayed by an average of 2.1 years from time of first cast. A 19% complication was observed. There was no statistical difference in the rate of resolution of deformity between idiopathic (5/19) and nonidiopathic (1/17) patients (P=0.182), although there exists a trend toward greater curve correction in idiopathic patients. Surgery occurred in fewer patients (2/19) in the idiopathic group compared with the nonidiopathic group (9/17) (P=0.006). Significant improvements in Cobb angle was observed in the idiopathic group (12.2 degrees) during casting (P=0.003). Nonidiopathic patients did not maintain the correction gained during casting at the time of final follow-up. T1-T12 height increased across all study patients regardless of etiology during the period of casting at similar velocity to established norms of 1.4 cm/y for this age group. CONCLUSIONS: Serial casting offers modest deformity correction in idiopathic deformities compared with nonidiopathic deformities. Thoracic height growth continued throughout the casting period at normal velocity. Serial casting maintained normal longitudinal thoracic growth in all patients with EOS in this cohort. Although many required surgery, the increased thoracic height may have positive implications on ultimate pulmonary function. LEVEL OF EVIDENCE: Therapeutic level III.

A multidisciplinary approach improves infection rates in pediatric spine surgery.

BACKGROUND: Surgical site infections (SSI) associated with elective pediatric spinal surgery are a commonly reported complication, increasing hospital length of stay, readmissions, operations, and financial costs. In July 2007, a multidisciplinary task force, designated Target Zero, was created to address this issue and establish prevention protocols at our institution. METHODS: A consecutive series of 394 patient charts from April 2006 to September 2008 were retrospectively reviewed to identify patients who developed an SSI secondary to elective spinal surgery. Four cohorts were evaluated; high-risk (HR) and low-risk (LR) patients who underwent surgery before (April 2006 to June 2007) and after (July 2007 to September 2008) Target Zero initiation. The definition of HR included diagnoses of cerebral palsy, spina bifida, muscle disease, paralytic deformities, and vertebral column resections. Patients were followed for 1 year to meet The Center for Disease Control-National Health Safety Network's definition of an SSI with an implantable device. Overall infection rates were determined for each group and compared statistically. RESULTS: A total of 192 patients (70 HR and 122 LR) underwent surgery before, and 202 patients (92 HR and 110 LR) underwent surgery after Target Zero initiation. Overall infection rates were reduced from 7.8% to 4.5% (P=0.203), 12.9% to 6.5% (P=0.183), and 4.9% to 2.7% (P=0.505) for all patients, HR patients, and LR patients, respectively. The relative risk reduction was 43.0% for all patients, 49.3% for HR patients, and 44.6% for LR patients. CONCLUSIONS: Although decreases in overall infection rates were not statistically significant, the results from Target Zero were shown to be clinically meaningful with a relative risk reduction approaching 50% overall and in defined subgroups. Based on the number needed to treat analysis, 1 infection in every 16 patients within the HR group, and 1 in 30 overall, was prevented up to 1 year postoperatively. This study is the first to document the effectiveness of a multidisciplinary team implementing protocols for decreasing infection rates in pediatric spine surgery.

Lower extremity physeal fractures and post-traumatic surgical deformities; National Trauma Data Bank and institutional cohorts.

Background: Pediatric lower extremity physeal fractures carry a risk of developing deformities. Most epidemiological evidence is over 25 years old, single institution, and lacks follow-up, while recent studies report variable results. Understanding their epidemiology and deformity risk is important for patient counseling and follow-up. Methods: The National Trauma Data Bank (NTDB) from 2016 was queried to describe the modern epidemiology of physeal fractures. This was contrasted with our 10-year experience of surgically treated deformities. Basic descriptive statistics, Chi-square analysis, prevalence ratios and multivariable linear regression were used to interpret results. Results: The NTDB contained 22,048 non-physeal and 1,929 physeal fractures of the femur, tibia, and fibula. Physeal fracture prevalence rose after 8 years of age but decreased for girls 2 years sooner than boys. Salter Harris (SH) type 2 fractures predominated. Physeal fractures were more commonly associated with lower energy mechanisms of injury. Distal tibia fractures were more prevalent in the NTDB cohort, while distal femur and SH-1 fractures were more prevalent in the operative cohort. Over 10 years, only 52 (5.3%) of the deformity-correcting surgeries at our institution were for physeal fracture sequelae. Age at injury and intraarticular fractures were associated with shorter times from injury to deformity correction. Conclusion: Lower extremity physeal fractures are uncommon. Fracture pattern prevalence differs from an operative cohort. Proximal tibia physeal fractures appear to be an underappreciated source of deformity. The risk of developing deformity requiring operative intervention appears to be low and is generally treated within 2 years of initial injury.

GLP-1 receptor agonists and cardiovascular outcomes in patients with type 2 diabetes: Clinical evidence and best practice.

ABSTRACT: Cardiovascular disease (CVD) is a major cause of death and disability among people with type 2 diabetes (T2D), presenting a significant impact on longevity, patient quality of life, and health care costs. In the United States, attainment of recommended glycemic targets is low and T2D-related cardiovascular complications remain a significant burden. Many glucose-lowering treatment options are available, but glucagon-like peptide-1 receptor agonists (GLP-1RAs) and sodium-glucose cotransporter-2 (SGLT-2) inhibitors are recommended in recent guidelines as the preferred add-on therapy to metformin to improve glycemic control. This is particularly the case for patients with T2D and established atherosclerotic CVD, at high risk of atherosclerotic CVD, and/or with chronic kidney disease. Recommendations were based on GLP-1RA and SGLT-2 inhibitor cardiovascular outcomes trials (CVOTs), which consistently showed that these agents pose no additional cardiovascular risk compared with placebo. Three GLP-1RAs (liraglutide, dulaglutide, and subcutaneous semaglutide) demonstrated significantly lower major adverse cardiovascular events versus placebo and are now approved for this indication. However, to realize improvement in outcomes in the clinical setting, organized, systematic, and coordinated approaches to patient management are also needed. For example, nurse-led diabetes self-management education and support programs have been shown to be effective. This article explores T2D management with emphasis on cardiovascular risk and CVOTs performed to date and reviews the clinical experience with GLP-1RAs for managing hyperglycemia and their impact on cardiovascular risk. In addition, practical guidance is given for key health care providers involved in the care of patients with T2D with cardiovascular risk outside of diabetes clinics/endocrinology centers.

Twelve-week standard of care protocol longer than median time to normalization among IIc hips treated with Pavlik harness.

Best treatment protocols for infants with developmental dysplasia of the hip (DDH) are poorly defined. This study estimates the time to normalization among Graf IIc hips undergoing Pavlik harness treatment. Following institutional review board approval, patients referred for DDH evaluation at a pediatric institution between 2009 and 2018 (n = 1424 hips/712 patients) were identified. We isolated all Graf IIc hips that underwent Pavlik harness treatment (n = 132 hips/n = 106 patients). Demographic and outcome measures were collected. Normalization was defined as alpha angle greater than or equal to 60° and femoral head coverage greater than or equal to 50%. Kaplan-Meier and Cox proportional hazards regression analyses modeled time to normalization and identified factors associated with earlier normalization. Median time to normalization was 7.0 weeks. At 12 weeks standard treatment, 85.8% [95% confidence interval (CI): 80.2-91.9%] had normalized. Greater femoral head coverage [hazard ratio (HR) per 1% increase: 1.03; 95% CI: 1.01-1.05; P = 0.0068] and hip stability at treatment initiation (HR unstable vs. stable: 0.64; 95% CI: 0.44-0.93; P = 0.0192) were associated with longer time to normalization. Some patients may not need 12 weeks of Pavlik bracing, particularly those with stable presentation who normalize before week 12. Shorter treatment lengths offer benefit without sacrificing long-term outcomes. Findings reinforce growing evidence that femoral head coverage should be a more significant consideration during diagnosis and instability is a concerning finding on examination.

Identification of susceptibility loci for scoliosis in FIS families with triple curves.

The triple curve pattern (three lateral curvatures of equal severity) has been recognized as a distinct and unique clinical subtype of scoliosis. As part of a large study of familial idiopathic scoliosis (FIS), a subset of five families with a triple curve pattern (at least one member of each family having a triple curve) was evaluated to determine if this curve pattern was linked to any of the markers previously genotyped as part of the STRP-based previous linkage screen. Model independent linkage analysis (SIBPAL, v4.5) of the initial genomic screen identified candidate regions on chromosomes 6 and 10 when FIS was analyzed both as qualitative and quantitative traits in single- and multipoint linkage analyses. Additional fine mapping analyses of this subgroup with SNPs corroborated the findings in these regions (P < 0.001). These regions have been previously linked to FIS, however, this is the first time these regions have been implicated in a clinically well-defined subgroup and may suggest a unique genetic etiology for the formation of a triple curve.

Evaluation of GPR50, hMel-1B, and ROR-alpha melatonin-related receptors and the etiology of adolescent idiopathic scoliosis.

BACKGROUND: Adolescent idiopathic scoliosis (AIS) is the most common spinal deformity in children. Studies have shown low melatonin levels resulting from pinealectomy in chickens and mice result in the development scoliosis, whereas supplementation with melatonin after the pinealectomy prevented it. The mere characterization of low melatonin levels is not sufficient to explain the development of idiopathic scoliosis in primates and humans, but we hypothesize that a mutation in melatonin-related receptors may be involved with the development of scoliosis. METHODS: The coding, splice-site, and promoter regions of 3 melatonin-related receptors (hMel-1B, RORalpha, and GPR50) were evaluated by DNA sequencing for variants associated with the phenotype of adolescent idiopathic scoliosis. An initial screening of 50 scoliosis patients with adolescent idiopathic scoliosis was compared with 50 controls by DNA sequencing of the 3 receptors. Additional cases and controls were evaluated when genetic variants were observed (for a total of 885 individuals). RESULTS: No significant differences were found in the hMel-1B and RORalpha receptors. We found 2 cSNPs in GPR50 (rs561077 and rs13440581) in the initial 50 patients. To evaluate the significance of these cSNPs, an additional 356 patients and 429 controls were analyzed. When the combined groups were analyzed, no significant associations were observed. CONCLUSIONS: Despite the observed relationship between melatonin and scoliosis, there is no significant association between mutations found in any known melatonin-related receptors with adolescent idiopathic scoliosis. The strong evidence of a melatonin-related cause for the development of idiopathic scoliosis still encourages research into undiscovered melatonin-related receptors, melatonin-related hormones, and the catalytic enzymes for the serotonin-melatonin pathway. CLINICAL RELEVANCE: This investigation is a genetic testing of the remaining currently known melatonin-related receptors that have not been analyzed earlier for association with AIS. Given the support in the literature of a relationship between melatonin and AIS, we have shown no mutations in any of the known melatonin-related receptor in patients with AIS.

Effectiveness of an anesthetic continuous-infusion device in children with cerebral palsy undergoing orthopaedic surgery.

BACKGROUND: Effective postoperative pain management is a vital component of orthopaedic surgical care in the pediatric population. In children with cognitive impairments pain management can be difficult, making these children vulnerable to ineffectively managed postoperative pain. This prospective, randomised study evaluated the use of a local anesthetic continuous infusion device (pain pump) to manage postoperative pain in children with cerebral palsy (CP) undergoing lower extremity orthopaedic surgical procedures. METHODS: Children with a diagnosis of CP who were undergoing select orthopaedic outpatient procedures were enrolled in this study. Postoperatively, patients were randomised to receive either a pain pump in addition to oral analgesics or oral analgesics only. Patient's parents were asked to record the amount of medication administered and assess pain intensity with the use of a modified visual analogue scale for 3 days postoperatively. Parents also completed an overall pain management satisfaction questionnaire at the end of the study participation period. RESULTS: Fifty-four patients were enrolled in this study and data were analyzed on 37 patients. The mean daily pain intensity in the pain pump group was significantly lower for the day of surgery and for 2 days postoperatively, and there was an overall significant difference between the groups (P<0.0001). The amount of analgesic medication administered was significantly lower for the first 2 postoperative days, but there was not a significant difference between the 2 groups overall (P=0.29). Parent satisfaction with both pain management techniques was high and responses were similar between the 2 groups. CONCLUSIONS: Children with CP present unique postoperative pain management concerns that can be effectively addressed through the use of multimodal analgesic techniques. This study found that the pain pump is an effective pain management technique that significantly reduces pain intensity in children with CP after lower extremity orthopaedic procedures. LEVEL OF EVIDENCE: Therapeutic Level II.