Clinical Relevance of Painful Congenital Early-onset Scoliosis: A Magnetic Resonance Image-based Study.

BACKGROUND: Back pain, as a clinical marker in scoliosis, has been associated with underlying pathology for many years, warranting further magnetic resonance imaging (MRI). Failures of segmentation, mixed defects, female gender, rib anomalies, congenital thoracic anomalies, and neurocutaneous markers are known risk factors for abnormal MRI pathology findings in patients with congenital early-onset scoliosis (Congenital-EOS). Yet, back pain has not been evaluated as a risk factor for underlying MRI pathology in patients with Congenital-EOS. This study aimed to assess back pain as a risk factor for underlying pathology in Congenital-EOS using MRI as a diagnostic tool. METHODS: A retrospective database review from the Pediatric Spine Study Group (PSSG) of all patients with Congenital-EOS who reported a back pain complaint, and underwent a spinal MRI study before surgical intervention was performed. Patients were divided into those with an underlying MRI pathology and those without. Demographics were compared between groups. RESULTS: From a total of 2355 patients with Congenital-EOS registered in PSSG, 107 patients reported a back pain complaint, with only 42 patients fulfilling the inclusion criteria (being evaluated with an MRI study). Overall group mean age was 8.1±4.5 years, with 25 of the 42 patients (60%) being females. Twenty-four of 42 patients (57%) had a comorbidity reported such as cardiac problems, musculoskeletal complaints, neurological deficits/myelopathy, gastrointestinal symptoms, developmental delay, respiratory problems, craniofacial abnormalities, and chromosomal conditions. An underlying MRI pathology was found in 21 of 42 patients with Congenital-EOS (50%) with back pain. The underlying MRI pathologies found were tethered spinal cord, spinal canal stenosis, syringomyelia, Arnold-Chiari malformation, and arachnoid cyst. CONCLUSIONS: Abnormal MRI findings are common in patients with Congenital-EOS who report back pain. Gender, age, major coronal curve angle, thoracic or lumbar predominance deformity, and comorbidities type or amount were not associated with abnormal MRI findings. LEVEL OF EVIDENCE: Level II-Prognostic study.

Unusual Vascular Distribution in the Third Segment of the Axillary Artery.

The third segment of the axillary artery (TSAA) is the main vascular supply to the muscles of the upper limb. Numerous studies have reported atypical branching patterns of the TSAA, which can complicate operative interventions involving structures supplied by this segment of the artery. Our current study evaluated a previously undescribed branching pattern in the TSAA, in which the subscapular artery gave rise to an unusual posterior humeral circumflex artery, and a second subscapular artery. In addition, a third variant was found in the origin of the thoracodorsal artery: two collateral horizontal arteries supplying the deep medial surface of the latissimus dorsi muscle. Vascular anatomical variants may affect the classical upper limb interventions requiring modification of the traditional surgical approaches. This case report aims to evaluate these variants from a clinical perspective regarding the management of upper limb trauma, axillary, breast, and muscle flap surgery.

Orthopaedic trauma epidemiology after Hurricane Maria in the Puerto Rico Trauma Centre.

PURPOSE: Hurricane Maria is the most devastating natural phenomenon in the recent history of Puerto Rico. Due to its destructive path through the island, the Puerto Rico Trauma Center (PRTC) remained the only hospital managing orthopaedic trauma in the immediate post-disaster period. We investigated the impact of this hurricane on the orthopaedic trauma epidemiology in the PRTC. METHODS: We evaluated the admissions by the orthopaedic surgery service in terms of demographics, mechanisms of injury, and orthopaedic diagnoses for two months after the impact of Hurricane Maria (HM) on September 20, 2017. We compared our study group with the same two month period for two years prior (2015 and 2016) and after (2018 and 2019) as control periods. A p value of < 0.05 was considered statistically significant. RESULTS: We included 384 admissions from September 20 to November 20, 2017. The majority were males (63%) and had an average age of 54 years. The most-reported mechanism of injury was fall from standing height (FFSH), showing a significant increment compared with the control periods. Contrarily, motor vehicle accidents (MVA) showed a significant reduction. Among the orthopaedic diagnoses, the hip + pelvis category showed a significant decline within the study group. CONCLUSIONS: This study highlighted the impact of HM on the orthopaedic trauma epidemiology at the PRTC. Our findings provide valuable evidence to healthcare institutions to better prepare to manage the potential changes in the orthopaedic trauma epidemiology after a major atmospheric event.

Associated factors of patients with spinal stenosis who undergo reoperation after a posterior lumbar spinal fusion in a Hispanic-American population.

PURPOSE: The aim of this study was to evaluate the associated factors of patients with LSS who undergo reoperation after a PLSF in a Hispanic-American population. METHODS: A retrospective single-center review was performed from all non-age-related Hispanic-Americans with LSS who underwent one or two-level PLSF from 2008 to 2018. Baseline characteristics were analyzed between the reoperation and no-reoperation group using a bivariate and multivariate analyses. RESULTS: Out of 425 patients who underwent PLSF, 38 patients underwent reoperation. At a two-year follow-up, the reoperation rate was 6.1% (26/425), mostly due to pseudoarthrosis (39.5%), recurrent stenosis (26.3%), new condition (15.8%), infection (10.5%), hematoma (5.3%), and dural tear (2.6%). Patients who underwent reoperation were more likely to have a preoperative history of epidural steroid injection (ESI) (OR 5.18, P = 0.009), four or more comorbidities (OR 2.69, P = 0.028), and operated only with a posterolateral fusion without intervertebral fusion (OR 2.15, P = 0.032). Finally, the multivariable analysis showed that ESI was the only independent associated factor in patients who underwent reoperation after a PLSF in our group. CONCLUSION: Among this population who underwent surgery, a reoperation rate at two years of follow-up was less than ten percent. Our study did not find any associated factor inherent to Hispanic-Americans, as ethnic group, who were reoperated after LSS.

Incidence of complications in the management of non-ambulatory neuromuscular early-onset scoliosis with a rib-based growing system: high- versus low-tone patients.

PURPOSE: The purpose of this study is to evaluate whether patients with high-tone neuromuscular early-onset scoliosis have different surgical outcome and complication rate, when compared to patients with low-tone neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. METHODS: This is a retrospective cohort study of 67 neuromuscular early-onset scoliosis patients, collected from a multicenter database, treated with a rib-to-pelvis rib-based dual growing system. All patients were divided into two groups: high tone and low tone. Pre-, intra- and postoperative data were compared between both groups. Complications were reported by a standardized system. RESULTS: Twenty-six high-tone and 41 low-tone patients were found homogeneous regarding gender, age at surgery, weight, height, estimated blood loss and surgery time. High-tone group (19/26 = 73.1%) experiences more postoperative complications than low-tone group (22/41 = 53.7%). Most common complications were infection, device migration, death and hardware failure. Permanent abandonment of rib-based growing technique and device removal was required in 21% of high-tone patients (P < 0.001). None of the low-tone patients required abandonment. CONCLUSION: High-tone patients had more complications than those with low tone in management of neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. A different surgical approach may be required to treat the high-tone neuromuscular early-onset scoliosis.

Are Constant Pain, Night Pain, or Abnormal Neurological Examination Adequate Predictors of the Presence of a Significant Pathology Associated With Pediatric Back Pain?

INTRODUCTION: Previous studies have suggested that most cases of pediatric back pain do not have an identifiable cause. No reliable sign or indication differentiates between a benign or serious cause of the symptom. Constant pain, night pain, and abnormal neurological examination have been suggested as adequate predictors of an identifiable cause, when plain radiographs could not explain the symptoms. The purpose of this study is to determine the sensitivity, specificity, and likelihood ratios of constant pain, night pain, and abnormal neurological examination to predict the presence of an underlying positive finding as a cause of back pain. METHOD: From 2010 to 2016, all patients who presented with a chief complaint of back pain were included in the study. Magnetic Resonance Image was performed to all patients presenting with back pain without identifiable cause lasting >4 weeks. Patients who presented with spondylolysis were treated accordingly base on radiographic findings and were excluded as study protocol. RESULTS: A total of 388 patients were evaluated during the study period. The mean age of the subjects was 14.5 years; 69.7% being female. An underlying pathologic condition was identified in 56 of 132 (42%) of patients with constant pain, 61 of 162 (38%) with night pain, and 8 of 9 (89%) with abnormal neurological examination. Probability to have an underlying pathology correlated directly with the amount of clinical markers. DISCUSSION: An abnormal neurological examination was found as a strong predictor for an underlying pathologic condition. Further imaging of a pediatric patient with back pain without clear explanation for their symptoms on plain radiographs should not be limited to constant pain, or night pain because clinicians could be missing important diagnosis. Therefore, the clinician cannot be assured by absence of these clinical markers, that there is no underlying spinal pathology. LEVEL OF EVIDENCE: Level III.

Impact of patient position on coronal Cobb angle measurement in non-ambulatory myelodysplastic patients.

OBJECTIVE: The purpose of this study was to assess the impact of patient position on the magnitude of the coronal Cobb angle measurements in relation to the change of position using plain radiograph on non-ambulatory children with myelodysplasia. Whole-spine radiographs with the patient sitting generally are preferred for the diagnosis and monitoring of progression of scoliosis in neuromuscular patients. Supine, supine traction, and sitting push-up positions have been used as substitutes, although there is no general consensus validating if these positions correlate with the sitting position. The magnitude of the Cobb angles in neuromuscular scoliosis may vary greatly depending on the position of the patient. METHODS: Radiographs of 39 myelodysplastic, non-ambulatory children were evaluated to assess the impact of change in positions (unsupported sitting, sitting push-up, supine, and supine traction) on coronal Cobb angle measurement using plain whole-spine radiographs. RESULTS: The mean difference in thoracic Cobb angle measurements between sitting and all other positions ranged from 6° to 12°. At the lumbar level, the Cobb angles ranged from 12° to 16°. CONCLUSIONS: Statistically significant differences in the Cobb angle measurements were identified between plain radiographs of the whole spine with the patient in the unsupported sitting position compared to sitting push-up, supine, and supine traction positions. The data support that the magnitude of the Cobb angles in neuromuscular scoliosis varies greatly depending on the position of the patient. LEVEL OF EVIDENCE: III.

Vitamin D levels and fracture risk among Hispanic children.

PURPOSE: There is a lack of knowledge about whether low vitamin D levels increase the risk of pediatric low-energy fractures among Hispanic population. The objective of this study is to determine whether there is a direct relationship between low vitamin D levels and the incidence of low-energy fractures in Hispanic children. METHOD: Cases included all consecutive patients evaluated with low-energy fractures in the pediatric orthopedic clinic. The control group consisted of all pediatric patients evaluated, without fractures, who had bone and joint pain complaints in the general pediatric clinic. The main focus was to compare cases and controls in relation to their vitamin D levels. Cases and controls were compared using t tests for means of quantitative variables and Chi-square tests. RESULTS: A total of 201 subjects, distributed as cases (n = 107) and controls (n = 94), were included in this study. One hundred twelve (55.7%) of the total study population were males. The mean age for the study population was 8.6 years old ranging from 1 year to 18 years, and standard deviation = 4.0 years. The median age for the study population was 9 years. The mean vitamin D level for the cases was 32.6 ng/dl (SD = 10.9); the mean vitamin D level for controls was 32.3 ng/dl (SD = 13.4). This difference was not statistically significant (t = 0.18, 95% CI - 3.2 to 3.9; p = 0.854). CONCLUSION: A direct relationship between low vitamin D levels and fracture risk in a Hispanic pediatric population was not established. LEVELS OF EVIDENCE: III.

Epidemiology of pediatric bone fractures across age groups: Analysis of a pediatric population at an outpatient clinic in the Southwest region of Puerto Rico.

BACKGROUND: Background: Gender disparities in pediatric fracture events has been well-documented however, less is known about racial disparities. The aim of this study is to investigate the epidemiology of pediatric bone fractures in a Hispanic population. METHODS: This is a retrospective cross-sectional analysis of patient's records from a Pediatric Orthopeadic outpatient clinic in the Southwest region of Puerto Rico during a 1-year span (August 2014 ­ August 2015). Differences in sex, BMI, health insurance coverage, parental educational level and employment status, mechanism of fracture, fracture site, and seasonality were investigated among three age classes (pre-school children, school children and adolescents). RESULTS: The sample consited of 243 subjects (0-21 y/o) sustaining a fracture of any extremity within the study period. Boys were at a higher risk of having a fracture event and as age increased, the male/female ratio also increased. The mean BMI was 18.0 kg/m2, with the highest in the school children age group (20.3 kg/m2). Slighlty more than half of the subjects (55.7%) benefitted from public health insurance coverage. The typical father was a laborer with a high school diploma, while the mother had a high school diploma but was unemployed There was a tendency for the children to suffer a bone fracture while at school (49.4%), followed by fractures at home (34.6%). In both the pre-school and school children groups, the upper extremity was more frequently involved (66.7% and 63.9%, respectively) in fracture events. Finally, there was very little variation in the seasonality of fracture events. CONCLUSION: This study provided valuable epidemiological information about pediatric bone fractures within a Hispanic population. It may contribute to the development and implementation of educational and preventive strategies appropriate to age and sex-differences.

One-step (standard) versus two-step surgical approach in adolescent idiopathic scoliosis posterior spinal fusion: Which is better?

STUDY DESIGN: A retrospective cohort study with IRB approval. INTRODUCTION: Significant blood loss remains an important concern in terms of the performance of the posterior spinal fusion in adolescent idiopathic scoliosis. Several strategies have been reported to minimize blood loss during surgery. In order to address the need to minimize blood loss without sacrificing the quality of the fusion, in our hospital, we adopted a two-step surgical approach. This surgical approach consist of the exposure and instrumentation of the lumbar region prior to and followed by an extension of the surgical incision to the thoracic region for its subsequent instrumentation. The main purpose of this study was to compare a two-step surgical approach with the one-step (standard) approach. METHODS: This study was a review of all the data on consecutive posterior spinal fusion surgeries performed by a specific two-surgeon team during 2004-2013. Demographics, surgical variables, radiographic findings, and outcomes regarding blood loss, morbidity, and the duration of the procedure were evaluated. RESULTS: Eighty-five patients underwent the standard surgical exposure, and 41 patients underwent the two-step surgical technique. With the exception of BMI, neither group showed any statistically preoperative variable significant differences. None of the postoperative outcome variables were statistically significant between both surgical approaches. CONCLUSIONS: No differences were detected in terms of using a two-step surgical approach versus the one-step standard surgical approach regarding perioperative blood loss, surgical time, or complications. LEVEL OF EVIDENCE: Level of evidence III.

Reliability analysis of Cobb angle measurements of congenital scoliosis using X-ray and 3D-CT images.

PURPOSE: Therapeutic decisions for congenital scoliosis rely on Cobb angle measurements on consecutive radiographs. There have been no studies documenting the variability of measuring the Cobb angle using 3D-CT images in children with congenital scoliosis. The purpose of this study was to compare the reliability and measurement errors using X-ray images and those utilizing 3D-CT images. MATERIALS AND METHODS: The X-ray and 3D-CT images of 20 patients diagnosed with congenital scoliosis were used to assess the reliability of the digital 3D-CT images for the measurement of the Cobb angle. Thirteen observers performed the measurements, and each image was analyzed by each observer twice with a minimum interval of 1 week between measurements. The analysis of intraobserver variation was expressed as the mean absolute difference (MAD) and standard deviation (SD) between measurements and the intraclass correlation coefficient (IaCC) of the measurements. In addition, the interobserver variation was expressed as the MAD and interclass correlation coefficient (IeCC). RESULTS: The average MAD and SD was 4.5° and 3.2° by the X-ray method and 3.7° and 2.6° by the 3D-CT method. The intraobserver and interobserver intraclass ICCs were excellent in both methods (X-ray: IaCC 0.835-0.994 IeCC 0.847, 3D-CT: IaCC 0.819-0.996 IeCC 0.893). There was no significant MAD difference between X-ray and 3D-CT images in measuring each type of congenital scoliosis by each observer. CONCLUSIONS: Results of Cobb angle measurements in patients with congenital scoliosis using X-ray images in the frontal plane could be reproduced with almost the same measurement variance (3°-4° measurement error) using 3D-CT images. This suggests that X-ray images are clinically useful for assessing any type of congenital scoliosis about measuring the Cobb angle alone. However, since 3D-CT can provide more detailed images of the anterior and posterior components of malformed vertebrae, the volume of information that can be obtained by evaluating them has contributed greatly to the development of strategies for the surgical treatment of congenital scoliosis.

Evaluation of a systematic approach to pediatric back pain: the utility of magnetic resonance imaging.

BACKGROUND: Several studies have suggested that back pain in the majority of pediatric patients does not have an identifiable cause. Many children undergo extensive diagnostic workup that ultimately results in a nonconfirmative diagnosis. The purpose of this study was to (1) describe the prevalence of back pain seen in a pediatric orthopaedic clinic; (2) evaluate the efficacy of a systematic approach dependent on magnetic resonance imaging (MRI) in the diagnosis of pediatric back pain; and (3) analyze sensitivity, specificity, positive predictive value, and negative predictive value of various clinical signs and symptoms. METHODS: For a 24-month period, all patients that presented with a chief complaint of back pain were prospectively enrolled in this study and evaluated in a systematic approach which utilized MRI for patients with constant pain, night pain, radicular pain, or abnormal neurological examination after an initial history, physical examination, and negative radiographic examination. RESULTS: The prevalence of chief complaint of back pain was 8.6% (261/3042 patients). Of the 261 patients, 34% had an identifiable pathology following the systematic approach. In 8.8% of patients, the diagnosis was established with the history, physical examination, and plain radiographs. MRI yielded a definitive diagnosis in another 25% of patients. It is noteworthy that of the 89 patients with a confirmed pathology, 26% were identified with plain radiographs and 74% with MRI. CONCLUSIONS: A systematic approach to diagnose pediatric back pain demonstrated that 34% of pediatric patients that present to an outpatient orthopaedic clinic complaining of back pain will have identifiable pathology. The diagnostic yield increased from 8.8% with the history, physical examination, and plain radiographs to 22% with the TCN Bone Scan to 36% with the use of the MRI. The clinician should be aware that the presences of lumbar pain or constant pain are red flags for the presence of underlying pathology. LEVEL OF EVIDENCE: Level III.

Osteogenesis imperfecta type V: marked phenotypic variability despite the presence of the IFITM5 c.-14C>T mutation in all patients.

BACKGROUND: Osteogenesis imperfecta (OI) type V is an autosomal dominant bone fragility disorder that we had described a decade ago. Recent research has shown that OI type V is caused by a recurrent c.-14C>T mutation in IFITM5. In the present study, we assessed all patients diagnosed with OI type V at our institutions for the presence of the IFITM5 mutation. METHODS: IFITM5 exon 1 was analysed by Sanger sequencing in genomic DNA from 42 patients with OI type V (age: 2-67 years; 18 female). RESULTS: The c.-14C>T mutation of IFITM5 was detected in all individuals. Indicators of disease severity varied widely: Height z-scores (n=38) ranged from -8.7 to -0.1, median -3.5. Median final height was 147 cm in men (N=15) and 145 cm in women (N=10). Lumbar spine areal bone mineral density z-scores in the absence of bisphosphonate treatment (n=29) were between -7.7 and -0.7, median -5.3. Scoliosis was present in 57%, vertebral compression fractures in 90% of patients. CONCLUSIONS: Even though the disease-causing mutation is identical among patients with OI type V, the interindividual phenotypic variability is considerable.

The efficacy of rib-based distraction with VEPTR in the treatment of early-onset scoliosis in patients with arthrogryposis.

BACKGROUND: Although arthrogryposis multiplex congenital (AMC) is a rare condition, rapidly progressive scoliosis is common in children with AMC. Only a limited number of studies characterize the nature of these curves, and even fewer describe surgical outcomes. To determine efficacy or rib-based distraction in these patients, we reviewed the outcomes of the use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device in what we believe is the first study to report this. METHODS: Search of the Chest Wall Spinal Deformity Study Group database identified 10 children with AMC and early-onset scoliosis who were treated with the VEPTR device at 6 different pediatric health centers. The 7 female and 3 male patients had their initial surgery at an average age of 5 years. Mean follow-up was 4.2 years. RESULTS: The most common curve was from T5 to L2. After initial VEPTR insertion, the scoliosis decreased from a mean of 67 to 43 degrees (37% correction) and kyphosis from 65 to 48 degrees (29% correction). The mean proximal junctional kyphosis after initial insertion was 33 degrees. At final follow-up, scoliosis and kyphosis were 55 degrees (17% correction) and 62 degrees (8% correction), respectively. Spinal growth during the treatment period showed a mean T1-S1 increase of 4.2 cm or approximately 1 cm/y. In the 62 procedures performed over the course of the study period, 6 complications occurred in 4 patients: 3 infections, 2 rib failures, and 1 implant failure. Six patients had proximal junctional kyphosis of ≥45 degrees at the last follow-up. CONCLUSIONS: In children with AMC, rib-based distraction using the VEPTR is an effective treatment method for controlling scoliosis and kyphosis and maintaining thoracic growth, but proximal junctional kyphosis remains a problem.

Lower Limb Deep Venous Thrombosis After Posterior Spinal Fusion as an Initial Manifestation of Factor V Leiden Mutation in a Pediatric Patient: A Case Report.

Deep venous thrombosis (DVT) is a serious condition in which a blood clot forms in a deep vein, usually of the lower extremity. In pediatric orthopedic surgery, the incidence of thrombotic events is rare. This is a case presentation of a 12-year-old female patient without previous events or a family history of thrombotic events who underwent a posterior spinal fusion due to severe adolescent idiopathic scoliosis. The patient developed a DVT due to an underlying Factor V Leiden mutation. The purpose of this case report is to create awareness, facilitate the diagnosis and management, and aid in future interventions and clinical outcomes.

Patient Perception of Robotic-Assisted Total Joint Arthroplasty in a Hispanic Population.

Background: Robotic-assisted orthopaedic surgery has become popular and widely available, mainly for total joint arthroplasty. However, there has been a persistent concern regarding access to robotic-assisted surgery and the utilization rate of total joint arthroplasty among minority groups. As an imperative effort to close the gap regarding health inequalities, we assessed the knowledge and perspective of Hispanics regarding robotic-assisted orthopaedic surgery. Methods: A 28-item questionnaire was established to evaluate Hispanics' perceptions of robotic-assisted orthopaedic surgery. Participants answered questions about demographic features, knowledge about robotic-assisted orthopaedic surgery, and preferences regarding manual vs robotic-assisted procedures. Results: A total of 580 questionnaires were analyzed in our study, with an average age of participants of 49.1 years. Only 44.2% of the participants were familiar with robotic-assisted orthopaedic surgery. Fifty-three percent of the respondents preferred robotic-assisted surgery over conventional procedures, with many participants believing that robotic-assisted surgery leads to better outcomes (54.7%) and faster recovery (53.1%). Conclusions: Knowledge about specific factors such as clinical outcomes and costs may influence the perception and preference of Hispanics toward robotic-assisted orthopaedic surgery. Therefore, patient education may play a crucial role in the informed decision-making process in Hispanics when opting between robotic-assisted or traditional orthopaedic surgery.

Opioid-Sparing Nonsteroid Anti-inflammatory Drugs Protocol in Patients Undergoing Intramedullary Nailing of Tibial Shaft Fractures: A Randomized Control Trial.

INTRODUCTION: Nonsteroidal anti-inflammatory drugs (NSAIDs) are effective analgesics commonly used in fracture management. Although previously associated with delayed fracture healing, multiple studies have demonstrated their safety, with minimal risks of fracture healing. Given the current opioid crisis in the United States, alternate pain control modalities are essential to reduce opioid consumption. This study aims to determine whether the combination of oral acetaminophen and intravenous ketorolac is a viable alternative to opioid-based pain management in closed tibial shaft fractures treated with intramedullary nailing. METHODS: We conducted a randomized controlled trial evaluating postoperative pain control and opioid consumption in patients with closed tibial shaft fractures who underwent intramedullary nailing. Patients were randomized into an NSAID-based pain control group (52 patients) and an opioid-based pain control group (44 patients). Visual analog scale (VAS) scores and morphine milligram equivalents (MMEs) were evaluated at 12-hour postoperative intervals during the first 48 hours after surgery. Nonunion and delayed healing rates were recorded for both groups. RESULTS: A statistically significant decrease in MMEs was noted at every measured interval (12, 24, 36, and 48 hours) in the NSAID group compared with the opioid group ( P -value 0.001, 0.001, 0.040, 0.024, respectively). No significant change in visual analog scale scores was observed at 12, 36, and 48 hours between both groups ( P -value 0.215, 0.12, and 0.083, respectively). A significant decrease in VAS scores was observed at the 24-hour interval in the NSAID group compared with the opioid group ( P -value 0.041). No significant differences in union rates were observed between groups ( P -value 0.820). DISCUSSION: Using an NSAID-based postoperative pain protocol led to a decrease in opioid consumption without affecting pain scores or union rates. Owing to the minimal risk of short-term NSAID use, their role in the perioperative management of tibia shaft fractures is justified, especially when they reduce opioid consumption markedly. LEVEL OF EVIDENCE: Therapeutic Level I.

Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

BACKGROUND: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history. METHODS: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed. RESULTS: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points). CONCLUSIONS: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Homozygous TRPV4 Mutation Broadens the Phenotypic Spectrum of Congenital Spinal Muscular Atrophy and Arthrogryposis: A Case Report.

Transient receptor potential vanilloid 4 (TRPV4) mutations are known to cause inherited axonal neuropathies and skeletal dysplasia. TRPV4 mutations are associated with distal hereditary motor neuropathies (dHMN), which distinctly involve motor deficits. A 1 ½-year-old boy presented at the clinic with diminished lower limb movement and ambulatory limitations. The patient was born with bilateral knee arthrogryposis and bilateral talipes equinovarus, which required surgical intervention. A gross neurologic exam was unremarkable, with normal vision and hearing. A bone survey radiograph showed no evidence of skeletal dysplasia. Genetic tests revealed a homozygous mutation in the TRPV4 gene (c.281C>T; p.S94L), leading to the diagnosis of congenital spinal muscular atrophy and arthrogryposis (CSMAA). Hence, this presents the first case of CSMAA caused by a TRPV4 mutation (p.S94L), with a different presentation from the one previously described in the literature, thus broadening the phenotypic variability and clinical spectrum of TRPV4 mutations.

Predictors of reoperation in hispanic-americans with recurrent lumbosacral disc herniation following primary hemilaminectomy and discectomy surgery.

Background: Multiple risk factors for recurrent lumbosacral disc herniation (rLDH) have been evaluated. However, it has been difficult to establish a consensus due to conflicting results. Therefore, the aim of our study was to evaluate the predictors of reoperation in Hispanic-Americans with rLDH following primary hemilaminectomy and discectomy surgery. Methods: A retrospective case-control study of 451 Hispanic-Americans with lumbosacral disc herniation (LDH) was conducted. The sample was divided into two groups: reoperated (cases) and non-reoperated (controls). Preoperative, operative, and postoperative variables of initial surgery were compared between the two groups. Results: The reoperation rate was 11.5%, with a mean interval between primary surgery and reoperation of 3.32 years ± 2.07. Analysis of preoperative variables identified a higher rate of reoperation in patients who were unemployed (cases: 48.1%, controls: 17.1%, p=0.001). A significant difference was also seen regarding the presence of gastrointestinal disease (cases: 11.5%, controls: 4.3%, p=0.038). However, there were no differences in the sociodemographic factors, preoperative physical exam, preoperative management, radiological parameters, or operative data. Those patients with persistent postoperative lower extremity pain, radiculopathy, low back pain, and buttock pain demonstrated a higher correlation with rLDH. Multivariable logistic regression analysis identified a significant difference only in work status (employed; OR and 95% CI [0.60 (0.55, 0.67)], p=0.002) and presence of postoperative low back pain (OR and 95% CI [2.17 (1.13, 4.29)], p=0.014). Conclusions: Patients who required reoperation due to rLDH were more frequently unemployed and/or suffered postoperative low back pain after primary hemilaminectomy and discectomy surgery.