CATSHL syndrome, a new family and phenotypic expansion.

We report the case of a 12-year-old girl and her father who both had marked postnatal tall stature, camptodactyly and clinodactyly, scoliosis and juvenile-onset hearing loss. The CATSHL (CAmptodactyly - Tall stature - Scoliosis - Hearing Loss syndrome) syndrome was suspected, and molecular analysis revealed a hitherto unreported, monoallelic variant c.1861C>T (p.Arg621Cys) in FGFR3. This variant affects the same residue, but is different than, the variant p.Arg621His reported in the two families with dominant CATSHL described so far. Interestingly, peg-shaped incisors were observed in the proband, a feature never reported in CATSHL but typical of another FGFR3-related condition, LADD (Lacrimo - Auricolo - Dento - Digital) syndrome. The FGFR3 p.Arg621Cys variant seems to be a newly identified cause of CATSHL syndrome with some phenotypic overlap with the LADD syndrome.

Neuropathy due to bi-allelic SH3TC2 variants: genotype-phenotype correlation and natural history.

Recessive SH3TC2 variants cause Charcot-Marie-Tooth disease type 4C (CMT4C). CMT4C is typically a sensorimotor demyelinating polyneuropathy, marked by early onset spinal deformities, but its clinical characteristics and severity are quite variable. Clear relationships between pathogenic variants and the spectrum of disease manifestations are to date lacking. Gene replacement therapy has been shown to ameliorate the phenotype in a mouse model of CMT4C, emphasizing the need for natural history studies to inform clinical trial readiness. Data, including both genetic information and clinical characteristics, were compiled from the longitudinal, prospective dataset of the Inherited Neuropathy Consortium, a member of the Rare Diseases Clinical Research Network (INC-RDCRN). The Charcot Marie Tooth Neuropathy Score (CMTNS), Examination Score (CMTES) and the Rasch-weighted CMTES (CMTES-R) were used to describe symptoms, neurological examinations and neurophysiological characteristics. Standardized response means were calculated at yearly intervals and a mixed model for repeated measures was used to estimate the change in CMTES and CMTES-R over time. Fifty-six individuals (59% female), median age 27 years (range 2-67 years) with homozygous or compound heterozygous variants in SH3TC2 were identified, including 34 unique variants, 14 of which have not previously been published. Twenty-eight participants had longitudinal data available. While there was no significant difference in the CMTES in those with protein truncating versus non-protein truncating variants, there were significant differences in the mean ulnar nerve compound muscle action potential amplitude, the mean radial sensory nerve action potential amplitude, and in the prevalence of scoliosis, suggesting the possibility of a milder phenotype in individuals with one or two non-protein-truncating variants. Overall, the mean value of the CMTES was 13, reflecting moderate clinical severity. There was a high rate of scoliosis (81%), scoliosis surgery (36%), and walking difficulty (94%) among study participants. The CMTES and CMTES-R appeared moderately responsive to change over extended follow-up, demonstrating a standardized response mean of 0.81 standard deviation units or 0.71 standard deviation units, respectively, over 3 years. Our analysis represents the largest cross-sectional and only longitudinal study to date, of the clinical phenotype of both adults and children with CMT4C. With the promise of upcoming genetic treatments, these data will further define the natural history of the disease and inform study design in preparation for clinical trials.

The role of scoliosis on temporomandibular joint disease: a cross-sectional study based on ultrasonography.

AIM: To investigate the relationship between temporomandibular disorders (TMD) and the stomatognathic system and spine through a multidisciplinary approach, utilising ultrasound to assess the temporomandibular joint. MATERIALS AND METHODS: Between October 2020 and January 2021, 50 patients aged 12-18 years with adolescent idiopathic scoliosis and 50 healthy individuals were enrolled. All participants underwent clinical examinations based on the Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) protocol. Additionally, ultrasound evaluations were performed of the temporomandibular joint and masseter muscles. Subsequently, the data from both groups were compared and analysed. RESULTS: The mean age of participants was 14.69 ± 2.73 years in the scoliosis group (SG) and 14.68 ± 1.81 years in the control group (CG). Among scoliosis patients, a moderate negative correlation was observed between the Cobb angle (indicative of curvature severity) and mouth opening (p=0.023; r=-0.320). Furthermore, the incidence of TMD was significantly higher in the scoliosis group compared to the control group (p<0.001). Additionally, it was found that a 1-unit increase in joint space elastography value led to a 4.81-fold higher likelihood of diagnosing disc displacement with reduction (p=0.009; 95% CI: 1.47-15.73). CONCLUSION: This pioneering study, the first of its kind to explore the connection between scoliosis and ultrasound-based temporomandibular joint screening, suggests that scoliosis may be a predisposing factor for TMD. Moreover, the present findings underscore the importance of joint elastography as a valuable quantitative tool in TMD diagnosis.

Profound hypoxemia and hypotension during posterior spinal fusion in a spinal muscular atrophy child with severe scoliosis: a case report.

BACKGROUND: Anesthesia for spinal muscular atrophy (SMA) patients undergoing spinal deformity surgery is challenging. We report an unusual case of an SMA girl who developed severe intraoperative hypoxemia and hypotension during posterior spinal fusion related with surgical positioning. CASE PRESENTATION: A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position. CONCLUSIONS: Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.

Assessment of malalignment at early stage in adolescent idiopathic scoliosis: a longitudinal cohort study.

INTRODUCTION: Our objective was to assess abnormalities of the odontoid-hip axis (OD-HA) angle in a mild scoliotic population to determine whether screening for malalignment would help predict the distinction between progressive and stable adolescent idiopathic scoliosis (AIS) at early stage. MATERIALS AND METHODS: All patients (non-scoliotic and AIS) underwent a biplanar X-ray between 2013 and 2020. In AIS, inclusion criteria were Cobb angle between 10° and 25°; Risser sign lower than 3; age higher than 10 years; and no previous treatment. A 3D spine reconstruction was performed, and the OD-HA was computed automatically. A reference corridor for OD-HA values in non-scoliotic subjects was calculated as the range [5th-95th percentiles]. A severity index, helping to distinguish stable and progressive AIS, was calculated and weighted according to the OD-HA value. RESULTS: Eighty-three non-scoliotic and 205 AIS were included. The mean coronal and sagittal OD-HA angles in the non-scoliotic group were 0.2° and -2.5°, whereas in AIS values were 0.3° and -0.8°, respectively. For coronal and sagittal OD-HA, 27.5% and 26.8% of AIS were outside the reference corridor compared with 10.8% in non-scoliotic (OR = 3.1 and 3). Adding to the severity index a weighting factor based on coronal OD-HA, for thoracic scoliosis, improved the positive predictive value by 9% and the specificity by 13%. CONCLUSION: Analysis of OD-HA suggests that AIS patients are almost three times more likely to have malalignment compared with a non-scoliotic population. Furthermore, analysis of coronal OD-HA is promising to help the clinician distinguish between stable and progressive thoracic scoliosis.

Finite element analysis of biomechanical effects of percutaneous cement discoplasty in scoliosis.

OBJECTIVE: To investigate the effect of bone cement on the vertebral body and biomechanical properties in percutaneous cement discoplasty (PCD) for degenerative lumbar disc disease. METHODS: Three-dimensional reconstruction of L2 ~ L3 vertebral bodies was performed in a healthy volunteer, and the corresponding finite element model of the spine was established. Biomechanical analysis was performed on the changes in stress distribution in different groups of models by applying quantitative loads. RESULTS: Models with percutaneous discoplasty (PCD) showed improved stability under various stress conditions, and intervertebral foraminal heights were superior to models without discoplasty. CONCLUSION: Cement discoplasty can improve the stability of the vertebral body to a certain extent and restore a certain height of the intervertebral foramen, which has a good development prospect and potential.

Percutaneous kyphoplasty through unilateral puncture on the convex side for the treatment of painful osteoporotic vertebral compression fracture with scoliosis.

PURPOSE: To assess the clinical safety, accuracy, and efficacy of percutaneous kyphoplasty (PKP) surgery using an enhanced method of unilateral puncture on the convex side for the treatment of painful osteoporotic vertebral compression fractures (P-OVCF) with scoliosis. METHODS: Clinical and radiographic data of P-OVCF patients with scoliosis who underwent PKP via unilateral puncture on the convex side from January 2018 to December 2021 were retrospectively analyzed. This technique's detailed surgical steps and tips were described. The local kyphosis angle (LKA), scoliosis Cobb angle (SCA), and local scoliosis Cobb angle (LSCA) were measured using X-ray and compared at pre-operation, post-operation, and the last follow-up. The width of pedicle (POW), inner inclination angle (IIA), lateral distance (LD), and puncture course length (PCL) were measured on the axial computed tomography image and compared between two sides. Postoperative computed tomography was employed to evaluate the condition of cement distribution and puncture. Clinical outcomes were evaluated using the Oswestry Disability Index (ODI) and Visual Analog Scale (VAS) for back pain (BP). RESULTS: Thirty-six patients, 23 women and 13 men, with an average age of 76.31 ± 6.28 years were monitored for 17.69 ± 4.70 months. The median surgical duration of single vertebrae was 35 min. The volume of bone cement for single vertebrae was 3.81 ± 0.87 ml and the proportion of sufficient cement distribution of the patients was 97.22. LKA was considerably improved from pre-operation to post-operation and sustained at the last follow-up. SCA and LSCA were not significantly modified between these three-time points. IIA, PCL, and LD were lower on the convex side than on the concave side. POW was considerably wider on the convex side. The ODI and VAS-BP scores were significantly improved after surgery and sustained during the follow-up. CONCLUSIONS: Combining with the proper assessment of the pre-injured life status of patients, PKP surgery using unilateral puncture on the convex side for the treatment of P-OVCF with scoliosis can achieve safe, excellent clinical, and radiographic outcomes.

[Facilitating and Hindering Factors in the Implementation of Medical Rehabilitation of Adolescents with Scoliosis before and during the SARS-CoV-2 Pandemic: A Qualitative Study]. / Förderliche und hinderliche Faktoren für die Durchführung der medizinischen Rehabilitation bei Jugendlichen mit Skoliose vor und während der SARS-CoV-2-Pandemie: Eine qualitative Studie.

PURPOSE: Based on the experience during the SARS-CoV-2 pandemic, the study aimed to derive facilitating and hindering factors in the implementation of medical rehabilitation during future pandemics in adolescents with scoliosis. METHODS: A qualitative study design with guided expert interviews was chosen. Twelve interviews with adolescents and seven interviews with physiotherapists were conducted. The evaluation was carried out using qualitative content analysis according to Mayring and inductive categorization. RESULTS: For 83.3% of the adolescents a therapy considering individual patient needs was a facilitator. Good information management (91.7%), continued availability of leisure activities (66.7%), and a high perception of safety (100%) were facilitating. 71.4% favoured outdoor therapy. The increased exertion caused by wearing a mask (91.7%) and the shortened therapy times (66.7%) were seen as barriers. For 75.0% of the adolescents, social contacts were more difficult. All therapists stated that communication between patients and therapists was more difficult. CONCLUSION: For successful rehabilitation during the SARS-CoV-2 pandemic, depending on weather conditions, therapy should be outside, as there is no need to wear mouth-nose protection outdoors. In addition, the physiotherapeutic treatment (Schroth therapy) should be carried out without mask due to the intensity of the exercise. Social contacts among all adolescents should be supported as much as possible. The leisure activities should provide a wide choice and enough free places. Continuation of certain hygienic measures could help to reduce the incidence of illnesses (e. g., gastrointestinal infections) in the future. These were barely noticed in rehabilitation facilities during the pandemic.

Spinal disorders and mastication: The potential relationship between adolescent idiopathic scoliosis and alterations of the chewing patterns.

OBJECTIVE: To evaluate mastication in a group of patients with adolescent idiopathic scoliosis (AIS) with a control group, by means of the prevalence of reverse chewing cycles (RCCs). MATERIAL AND METHODS: This study included a group of patients (N = 32; F = 24; M = 8; mean age ± SD = 14 ± 3 years) with a confirmed diagnosis of AIS and a group of control subjects (N = 32; F = 24; M = 8; mean age ± SD = 13 ± 6 years) without spinal disorders. Mastication was recorded with both a hard and a soft bolus, following a standardized protocol, and the prevalence of RCCs was compared between the groups. RESULTS: The prevalence of RCCs was significantly higher in the AIS group, with both a soft and a hard bolus, compared to the control group (P < .001). CONCLUSION: The results of this study indicate that the presence of AIS influences mastication, one of the main functions of the stomatognathic system. A multidisciplinary approach to these patients may be relevant in providing the best possible treatment outcomes.

Can hydroxyapatite charged collagen sponge help reduce perioperative blood loss in adolescent idiopathic scoliosis surgery? Preliminary results in 68 patients.

PURPOSE: Patient blood management has been recently emphasized to avoid perioperative blood transfusion in AIS surgery. Hydroxyapatite charged collagen sponge (HCS) is a bone substitute material made of collagen and ceramized hydroxyapatite, with associated haemostatic properties. The goal of this study was to assess the impact of HCS in the perioperative blood loss in AIS surgery. METHODS: After IRB approval, all AIS patients undergoing primary correction were prospectively included over a 15-month period. Patients receiving HCS at the end of the procedure were compared to a control group (matched for age, gender, and fusion levels) without any haemostatic agent or bone substitute. The same perioperative blood saving strategies were used in both groups. Two subfascial drains were used for 48 h in all patients. Perioperative blood loss and transfusion rates were analysed. RESULTS: A total of 34 patients were included in each group. No difference in drainage volume was observed at day 1, but the reduction was statistically different at day 3 (1135 mL [800-1640] versus 930 [480-1510], p = 0.028, 0.63 ml/Kg/h [0.4-0.92] versus 0.46 [0.29-0.7], p = 0.042). Multivariate analysis found that the use of HCS was associated with a decrease in the postoperative blood loss (OR = 1.17 [1.10-1.25]). The transfusion rate was lower in the HCS group [0 (0% vs. 3(8.8%), p = 0.076)]. No infection occurred, and no complication was reported. CONCLUSION: With 27% reduction in drain volume, hydroxyapatite charged collagen sponge can be considered as a blood salving strategy in AIS surgery. The role of the biomaterial in fusion rate still needs to be further assessed.

Fusion rates based on type of bone graft substitute using minimally invasive scoliosis surgery for adolescent idiopathic scoliosis.

BACKGROUND: Minimally invasive scoliosis surgery (MISS) is currently introduced on novel technique for surgical treatment of adolescent idiopathic scoliosis (AIS). This study is aimed to evaluate the efficacy of facet fusion in MISS compared to posterior fusion in conventional open scoliosis surgery (COSS) and compare facet fusion rates based on three bone graft substitutes in MISS for adolescent idiopathic scoliosis (AIS). METHODS: Eighty six AIS patients who underwent scoliosis surgery were divided into two groups: the COSS group and the MISS group. COSS was performed through posterior fusion with allograft. MISS was applied via facet fusion with three bone graft substitutes. The MISS group was further divided into three subgroups based on graft substitute: Group A (allograft), Group B (demineralized bone matrix [DBM]), and group C (demineralized cancellous bone chips). Fusion rate was measured using conventional radiographs to visualize loss of correction > 10°, presence of lysis around implants, breaks in fusion mass, and abnormal mobility of the fused segment. RESULTS: The fusion rates showed no significant difference in COSS and MISS groups (p = 0.070). In the MISS group, the fusion rates were 85, 100, and 100% in groups A, B, and C, respectively, with no significant difference (p = 0.221). There were no statistical differences between groups A, B, and C in terms of correction rate, fusion rate, and SRS-22 scores (p > 0.05). CONCLUSIONS: The facet fusion in MISS showed comparable to posterior fusion in COSS with regard to radiological and clinical outcomes. Furthermore, the type of graft substitute among allograft, DBM, and demineralized cancellous bone chips did not affect facet fusion rate or clinical outcomes in MISS. Therefore, MISS showed comparable fusion rate (with no influences on the type of graft substitute) and clinical outcomes to those of COSS in the surgical treatment of AIS.

Carbon fibre instrumentation for scoliosis surgery in children with spinal cord intramedullary tumours: a novel technical note.

INTRODUCTION: Scoliosis in children is the most common spinal deformity seen by general practitioners, paediatricians and spinal surgeons. Progressive scoliosis can result in the development of a worsening deformity and cosmesis. Patients usually present with aesthetic concerns. Progressive scoliosis that fails conservative management may require or be offered surgical intervention. Intramedullary tumours may be associated with scoliosis. Management of patients with these dual pathologies can be challenging. Classical scoliosis instrumentation utilising titanium implants impairs post-operative MRI evaluation with metal artefacts. Carbon fibre instrumentations has the potential to reduce the imaging metal artefacts but has not been described in scoliosis correction. METHODS: Surgical technical note describing correction of scoliosis in two adolescents' with intradural tumours utilising carbon fibre implants. RESULTS: We developed a hybrid approach where we initially used titanium implants to manipulate the deformity then replaced the construct with carbon fibre implants in the same setting to maintain the deformity correction with good follow up outlook. CONCLUSION: Our technique is robust, safe and replicable. It enabled appropriate post-operative MRI evaluation of the neural structures with a reduced risk of metal artefacts.

Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome.

BACKGROUND: STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. METHOD: Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. RESULT: Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. CONCLUSION: Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.

Bethlem Myopathy (Collagen VI-Related Dystrophies): A Retrospective Cohort Study on Musculoskeletal Pathologies and Clinical Course.

BACKGROUND: Collagen VI-related myopathies with pathologic COL6A1, COL6A2, and COL6A3 variants manifest as a phenotypic continuum of rare disorders, including Bethlem myopathy (BM), characterized by early onset muscle weakness, proximal joint contractures, and distal joint laxity. Herein we discuss the concomitant orthopedic manifestations of BM, potential management strategies, and patient outcomes. METHODS: An IRB-approved retrospective cohort study (n=23) from 2 pediatric institutions with a confirmed diagnosis of BM. Charts were reviewed for demographic data, age of disease presentation and diagnosis, COL6 genotype, diagnosis method, ambulation status, need for assistance, musculoskeletal abnormalities, other systemic comorbidities, advanced imaging and screening diagnostics, previous surgical interventions, and progression of the disease. RESULTS: The mean age was 11.65 years (range 3 to 19 y). Mean age at initial presentation with symptoms was 4.18 years old, whereas diagnosis was delayed until 8.22 years old on average. Muscle weakness was the most common presenting symptom (65.2%), and 73.9% of patients required some use of assistive or mobility devices. Overall, 30.4% of patients were diagnosed with scoliosis; 57.1% required operative intervention for their scoliosis; 43.5% of patients had acetabular dysplasia; 10% required open reduction of a dislocated hip; 10% required closed reduction with hip spica application; 10% required bilateral periacetabular osteotomies for instability; 91.3% of patients developed foot and ankle deformities; 33.3% of patients underwent posteromedial-lateral equinovarus releases; 28.6% required an Achilles tendon lengthening, and 86.9% of patients had muscle tendon contractures, the most common locations being the ankle (55%) and elbow (40%). CONCLUSION: Although often less severe than other more common neuropathies and myopathies like Charcot-Marie-Tooth disease and Duchenne muscular dystrophy, BM does lead to progressive musculoskeletal deformity and disability. Its relative rarity makes it less familiar to providers and likely contributes to delays in diagnosis. Scoliosis, hip dysplasia, and equinus and varus ankle deformities are the most common musculoskeletal deformities. Physicians and surgeons should appropriately counsel patients and families about the clinical course of this disorder and the potential need for mobility assistance or surgical procedures. LEVEL OF EVIDENCE: III, Prognostic. study.

A Stop-gain Variant c.220C>T (p.(Gln74*)) in FLNB Segregates with Spondylocarpotarsal Synostosis Syndrome in a Consanguineous Family.

Spondylocarpotarsal synostosis (SCT) syndrome is a very rare and severe form of skeletal dysplasia. The hallmark features of SCT are disproportionate short stature, scoliosis, fusion of carpal and tarsal bones, and clubfoot. Other common manifestations are cleft palate, conductive and sensorineural hearing loss, joint stiffness, and dental enamel hypoplasia. Homozygous variants in FLNB are known to cause SCT. This study was aimed to investigate the phenotypic and genetic basis of unique presentation of SCT syndrome segregating in a consanguineous Pakistani family. Three of the four affected siblings evaluated had severe short stature, short trunk, short neck, kyphoscoliosis, pectus carinatum, and winged scapula. The subjects had difficulty in walking and gait problems and complained of knee pain and backache. Roentgenographic examination of the eldest patient revealed gross anomalies in the axial skeleton including thoracolumbar and cervical fusion of ribs, severe kyphoscoliosis, thoracic and lumbar lordosis, coxa valga, fusion of certain carpals and tarsals, and clinodactyly. The patients had normal faces and lacked other typical features of SCT like cleft palate, conductive and sensorineural hearing loss, joint stiffness, and dental enamel hypoplasia. Whole exome sequencing (WES) of two affected siblings led to the discovery of a rare stop-gain variant c.220C>T (p.(Gln74*)) in exon 1 of the FLNB gene. The variant was homozygous and segregated with the malformation in this family. This study reports extensive phenotypic variability in SCT and expands the mutation spectrum of FLNB.

Serum metal ion levels following spinal deformity surgery: a case-control study of 182 individuals.

PURPOSE: The aim of this study is to evaluate the levels of chromium (Cr), cobalt (Co), and titanium (Ti) after instrumented fusion for scoliosis. METHODS: Serum samples were collected at median 2.24 (range 0.1-38.8) years after fusion surgery for scoliosis in 91 individuals, of which 71 had been treated with steel implants and 20 with titanium implants. 91 sex and age-matched non-surgically treated individuals with scoliosis were used as controls. Levels of Cr, Co, and Ti were measured. RESULTS: In the 91 surgically treated individuals median levels of Cr were 0.54 µg/l vs 0 µg/l in the 91 controls, p < 0.001. Corresponding results for Co were 0.29 µg/l vs. 0.24 µg/l, p = 0.19, and for Ti were 0 µg/l vs. 0 µg/l, p < 0.001. In the individuals with steel implants and their corresponding controls median Cr levels were 0.63 µg/l vs. 0.00 µg/l, p < 0.001 and Co levels 0.27 µg/l vs. 0.23 µg/l, p = 0.36. No Ti was detected. In the individuals with titanium implants, median Cr levels were 0 µg/l vs. 0 µg/l in their corresponding controls, p = 0.38. Corresponding results for Co was 0.39 µg/l vs. 0.31 µg/l, p = 0.27 and for Ti 4.31 µg/l vs. 0 µg/l, p < 0.001. In the individuals with steel implants a negative correlation between implant time in situ and levels of Cr was found ([Formula: see text] = - 0.52, p < 0.001) but not with Co ([Formula: see text] = - 0.14, p = 0.23). Ti was not detected. In the individuals with titanium implants, there was no correlation between implant time in situ and levels of Cr ([Formula: see text] = 0.36, p = 0.12), Co ([Formula: see text] = - 0.12, p = 0.60) or Ti ([Formula: see text] = 0.22, p = 0.35). CONCLUSION: The use of stainless steel and titanium implants in spinal fusion surgery is associated with elevated metal ion concentrations several years after surgery.

A comparison of the inflammatory host response to particulate debris adjacent to unlocked and locked screws of a growth guidance system for early onset scoliosis.

PURPOSE: The SHILLA™ Growth Guidance system is a stainless-steel rod and screw system used for Early Onset Scoliosis which incorporates a unique flanged set screw designed to capture the rod, while allowing it to slide as the patient grows. Concomitant with this design is the potential for generation of wear debris and for an inflammatory host response. We hypothesized that the magnitude of the host response adjacent to the unlocked screws and rods would be greater than the host response to the locked rod/screws. METHODS: Seven tissue samples adjacent to locked (3) and unlocked screws (4) from three SHILLA patients (mean implantation time of 19 post-operative months) with infantile idiopathic scoliosis were obtained as part of an explant analysis protocol during a PMDA-approved clinical trial in Japan. Gross appearance, high-resolution radiographs, and histology were assessed. ISO Standard 10993 Part 6 was used to assess the host response. RESULTS: All three locked screw had no metallosis. In contrast, metallosis for unlocked screw tissue samples were rated as "ubiquitous" (2/4), "focal" (1/4), or "absent" (1/4). Microscopic metallic debris was found intracellularly and within interstices of fibrous connective tissues more frequently adjacent to unlocked screws compared to locked screws. Cell type and population scoring consistently showed a modestly larger inflammatory response (macrophages) in the unlocked tissue samples. CONCLUSIONS: The peri-prosthetic tissue response to the unlocked rods/screws had a higher reactivity grade (slight reaction, Δ = 4.0) per ISO 10993 Part 6 compared to the locked screws in three patients with the SHILLA™ Growth Guidance scoliosis system.

Does the use of intrawound povidone-iodine irrigation and local vancomycin powder impact surgical site infection rate in adolescent idiopathic scoliosis surgery?

PURPOSE: Surgical site infection (SSI) is a major complication after adolescent idiopathic scoliosis (AIS) surgery, with an incidence ranging from 0.5 to 7%. Intraoperative wound decontamination with povidone-iodine (PVP-I) irrigation and/or vancomycin powder in adult spinal surgery has gained attention in the literature with controversial results. The aim of this study was to investigate the impact of using intrawound PVP-I irrigation and local vancomycin powder (LVP) on the incidence of early SSI in AIS surgery. METHODS: All AIS patients who underwent posterior spinal fusion between October 2016 and December 2019 were retrospectively reviewed. The incidence of early SSI was reported and compared between 2 groups defined by the treating spinal surgeons' preferences: group 1-intrawound irrigation with 2L of PVP-I and application of 3 g LVP before closure and control group 2-patients that did not receive either of these measures. RESULTS: Nine early cases of SSI (2.9%) were reported among the 307 AIS posterior spinal fusion patients. Incidence of SSI in group 1 (2/178 = 1.1%) was significantly lower than in group 2 (7/129 = 5.4%; p = 0.04). There were no adverse reactions to the use of PVP-I and LVP in our study. At latest follow-up, rate of surgical revision for mechanical failure with pseudarthrosis was significantly lower in group 1 (2/178 = 1.1%) than in group 2 (9/129 = 7.0%; p = 0.01). CONCLUSION: Intraoperative use of intrawound PVP-I irrigation and vancomycin powder is associated with a significant reduction of early SSI after AIS spine surgery. LEVEL OF EVIDENCE IV: Retrospective study.

Efficacy of polyester bands placed under the transverse vertebral process for the correction of adolescent idiopathic scoliosis : A case series of 105 patients with a minimum of 24 months follow-up.

PURPOSE: To report an original technique for the surgical correction of adolescent idiopathic scoliosis (AIS) based on hybrid construct using polyester bands placed under the thoracic transverse processes. METHODS: We reviewed 105 patients operated between 2013 to 2017 for AIS with a minimum of 2 years follow-up. Clinical and radiological data, including 2D measurements of scoliosis parameters preoperatively, and at 3- and 24-months postoperatively were analyzed. Radiological analysis was performed by two independent observers using KEOPS (Smaio, Lyon-France, CE 2014) database. RESULTS: Mean age was 14.6 ± 2 years. Mean follow-up was 23.7 ± 1.2 months. Most curves were classified as Lenke-1 (78%). The average number of vertebra involved in the construct was 10.9 ± 1.3 (range 7-13). A mean of 12.8 implants were used per construct (mean implant density 0.59). The number of subtransverse bands placed averaged 3 (range 1-5 bands). The mean frontal Cobb angle decreased from 57.9 to 21.2 ° (p < 0.05) postoperatively. The average T4-T12 kyphosis increased from 20.2°to 32.4° (p < 0.05) postoperatively and no loss of correction was observed at 2 years follow-up. We encountered one case of transverse process fracture intraoperativley. No neurological complications were observed. In the postoperative period, 4 cases were diagnosed with distal junctional failure, three of which required an extension of the construct, while the fourth case was treated by bracing. CONCLUSIONS: The use of subtransverse band is an efficient and safe method for the surgical correction of AIS. This technique incorporates technical ease (minimal risk of neurological injury), provides good frontal correction and restores thoracic kyphosis. Results are maintained over a two-year period.

Global alignment taking into account the cervical spine with odontoid hip axis angle (OD-HA).

INTRODUCTION: Global alignment analysis is of upmost importance in adult spinal deformity patients (ASD). Numerous parameters exist in the literature to measure global alignment based upon C7 or T1. One common limitation of these parameters is that they neglect the cervical segment which is essential in spinal compensatory mechanisms and in horizontal gaze preservation. A recent stereoradiography analysis of asymptomatic subjects introduced a new 3D parameter (ODHA), defined as the angle between the vertical reference line and the line joining the odontoid tip (OD) to hip axis center (HA). Thus, the goal of this study was to analyze 3D global alignment of ASD patients using the new parameter odontoid hip axis angle and its relationship to other spinal parameters. METHODS: In this prospective study, 90 adult patients with lumbar scoliosis (Cobb > 20°) were included. All subjects underwent low dose biplanar X-rays with 3D spinal reconstructions. Based on published normative values of ODHA, we defined abnormally high value as mean ODHA of asymptomatic subject + 2SD (i.e., ODHA > 6.1°). Values of 3D radiographic parameters and ODI were compared between patients with ODHA > 6.1° and < 6.1°. RESULTS: Mean ODHA was 5+/- 3.6° (0.4° to 18.6°). 22 patients had abnormally high ODHA. They were older than the 68 other patients (68+/- 9y vs 53+/- 14y, p = 0.001), without any significant difference in terms of sex, BMI and rate of rotatory subluxation (54% vs 62%, p = 0.06). However, coronal and sagittal deformity was more important in patients with abnormal ODHA (larger Cobb angle, coronal malalignment, pelvic tilt and lower lumbar lordosis). Patients with abnormal ODHA had significantly worst ODI (50+/- 23 vs 30+/- 18, p = 0.0005). CONCLUSION: Extreme values of ODHA are observed in significantly older patients with significant functional impairment. In addition, in these patients with sagittal malalignment with loss of lumbar lordosis, who recruit compensatory mechanisms such as pelvic retroversion, the cervical area is also involved with a posture in cervical hyperlordosis to maintain the head over the pelvis. Thus, ODHA is an interesting parameter allowing a more comprehensive alignment measurement taking into account the mechanisms of compensation of the cervical spine to the pelvis.