BACKGROUND:The association between congenital talipes equinovarus (CTEV) and developmental dysplasia of the hip (DDH) remains contested. Some guidelines consider idiopathic clubfoot a DDH risk factor warranting routine hip ultrasonography, while others do not. This study aimed to determine the prevalence of DDH in a large cohort of infants with idiopathic CTEV and assess whether routine screening is justified. METHODS:A retrospective review of prospectively collected data was conducted at a single tertiary pediatric orthopaedic center. Children treated for idiopathic CTEV between July 2000 and November 2023 with a minimum 2-year follow-up were included. Teratologic and syndromic cases were excluded. All patients underwent routine hip ultrasonography at 6 weeks of age, classified by the Graf method. Hips graded Graf type 2B or higher received treatment. RESULTS:A total of 515 patients (766 feet; 72% male) were included. Mean age at initiation of Ponseti casting was 14 days (SD: 14.2); mean follow-up was 7.3 years (SD: 4.1). DDH was identified in 4 patients (0.78%): 1 male (0.27%) and 3 females (2.05%). All 4 were classified as Graf type 2B or 2C; no frank dislocations were observed. DDH was successfully managed concurrently with CTEV treatment in all cases, with no recurrence. CONCLUSIONS:In this largest single-center series to date, the prevalence of DDH in idiopathic CTEV was comparable to population norms, indicating that idiopathic clubfoot is not an independent risk factor for DDH. These findings do not support routine hip ultrasound screening based on a clubfoot diagnosis alone in selective screening systems, though no change is warranted in universal screening systems. This may not apply to syndromic clubfoot, which was not evaluated here. LEVELS OF EVIDENCE:Level III-retrospective review of a prospective cohort.

PurposeThe use of intramedullary nails (IMNs) for deformity correction and limb lengthening has become increasingly established. However, performing acute deformity correction over an IMN remains technically demanding. The purpose of this study was to describe the application of 3D virtual surgical planning and patient specific 3D-printed cutting guides for the management of complex lower-limb deformities in young patients.MethodsThis retrospective, single-center study included 21 patients (mean age: 12 years) who underwent surgery using IMNs for complex lower-limb deformities correction between May 2019 and June 2024. Virtual 3D femoral and tibial models were created for preoperative planning and used to design patient-specific cutting guides for the surgical correction. All patients were evaluated for preoperative and postoperative anteroposterior and lateral deformity angles, time to osteotomy union, and complications.ResultsTwenty-one patients underwent correction of 23 extremities. According to an a priori classification, an excellent correction (residual angulation ≤5° in both planes) was achieved in 19/23 extremities (83%) and an acceptable correction (6–10° residual angulation, within the recognized tolerance for the growing skeleton) in 4/23 extremities (17%), no extremity demonstrated residual deformity >10°. Among the 20 patients available for follow-up, the mean time to radiographic union was 8.5 weeks (range, 4.5–18 weeks), excluding the cases treated with a PRECICE lengthening nail for combined limb deformity correction and bone lengthening. No major intraoperative or early postoperative complications occurred, two extremities (8.7%) developed delayed union that resolved without intervention, one planned secondary procedure was performed (4.3%), and one patient (4.8%) was lost to follow-up.ConclusionThree-dimensional virtual planning and patient-specific cutting guides represent effective adjuncts for correcting complex lower-limb deformities using intramedullary nails in pediatric patients. This approach enhances the accuracy of preoperative planning and facilitates more precise and controlled surgical execution.

Preoperative planning is crucial for successful surgical outcomes. 3D printing technology has revolutionized surgical planning by enabling the creation and manufacturing of patient-specific models and instruments. This review explores the applications of 3D printing in pediatric orthopedics, focusing on image acquisition, segmentation, 3D model creation, and printing techniques within specific applications, including pediatric limb deformities, pediatric orthopedic oncology, and pediatric spinal deformities. 3D printing simultaneously enhances surgical precision while reducing operative time, reduces complications, and improves patient outcomes in various pediatric orthopedic conditions. 3D printing is a transformative technology in pediatric orthopedics, offering significant advantages in preoperative planning, surgical execution, and postoperative care.

Purpose: To identify and characterize events of deterioration in intraoperative neuromonitoring data during correction procedures for thoracic and lumbar abnormal spinal curvature in young patients. Methods: Records of 1,127 cases were retrospectively reviewed to identify events with deterioration of the neuromonitoring data. General etiological and demographic variables were summarized, and neuromonitoring events were studied and characterized. Results: Adolescent idiopathic cases were associated with female dominance and older age. Nonadolescent idiopathic cases were associated with a higher rate of neuromonitoring events. The neuromonitoring events evolved during the different procedural stages, were primarily reflected in the motor-evoked potential data and affected a range of neural structures to varying degrees. Most of the events were resolved, partially or completely, following a corresponding intervention by the surgical team, before the end of the procedure. Significant immediate weakness of the lower extremities was demonstrated in patients with unresolved neuromonitoring events, most of them were nonadolescent idiopathic patients. Conclusions: Neurophysiological monitoring enables the intraoperative assessment of the integrity of neural pathways and allows the detection of surgery-related impending neural injuries. Neuromonitoring contributes to intraoperative decision making, either when data are uneventful and allow confident continuation or when data deteriorate and lead to corresponding intervention. Further awareness should be paid to the vulnerable characteristics of the patient, surgery course, and neuromonitoring data. Proper interpretation of the neuromonitoring data, together with corresponding intervention by the surgeon when necessary, has the potential to reduce postoperative neurological insults and improve clinical outcomes.

Introduction:Patellar instability and dislocation pose complex clinical and surgical challenges, especially in children. Congenital (fixed) and obligatory (habitual) dislocations present significant anatomical and etiological complexity, frequently leading to deformities and functional impairments, which can range from walking difficulties to sports limitations. Conservative treatment is often inadequate. Technique:We describe a surgical technique for treating congenital or obligatory patellar dislocations in patients with various underlying diagnoses-including Down syndrome, nail-patella syndrome, and skeletal dysplasia-that involves extensive subperiosteal quadriceps realignment, distal realignment (Roux-Goldthwait or tibial tuberosity transfer), and optional medial plication. This modified 4-in-1 technique follows the principles described in 1976 by Stanisavljevic, which involves subperiosteal quadriceps mobilization, thus minimizing muscle damage, bleeding, and postoperative muscular adherences. Results:In 24 patients treated at our institution between 2002 and 2021 (35 knees; age range = 5.5-16.8 years; 13 girls, 11 boys), with a mean follow-up of 8.2 years (2.4-20 years), we achieved satisfactory improvements in patellar stability, range of motion, and quality of life with a modified 4-in-1 Stanisavljevic technique. A total of 9 patients (7 with obligatory dislocations and 2 with congenital dislocations) could engage in recreational or competitive sports. The average postoperative pediatric International Knee Documentation Committee (pedi-IKDC) score was 78.45 ± 22.3 (range = 0-100); a patient with DiGeorge syndrome and 1 with multiple epiphyseal dysplasia had scores of 35 and 48, respectively. Discussion:We found at our institution that a modified 4-in-1 Stanisavljevic technique produced favorable outcomes in patellar stability, range of motion, and quality of life in pediatric patients with congenital or obligatory patellar dislocation. More study is warranted to determine the procedure's overall benefits for children with obligatory or congenital dislocations of complex etiology.

BACKGROUND:Magnetically controlled growing rod (MCGR) treatment for early-onset scoliosis (EOS) has become a widely utilized method of deformity control, but long-term follow-up reports are sparse. We evaluated the outcomes of a large group of consecutive pediatric patients diagnosed with EOS who were treated by MCGR to the endpoint of definitive spinal fusion in a single center. We hypothesized that lessons learned from treating a large volume of MCGR patients would lower the complication rate in comparison to what was previously reported by other studies.METHODS:The records of 48 EOS patients with varied etiologies who were treated by MCGR between 2012 and 2022 and reached the end of treatment were analyzed retrospectively for baseline and final radiographic measurements and surgery-related complications.RESULTS:The mean percent of improvement in the major coronal deformity was 51.7±25.0% and the mean T1 to T12 total growth was 52.9±21.9 mm. The total complication rate was 22.9% of which 12.5% required a single unplanned surgery, 6.3% required multiple unplanned surgeries, and 4% did not require any unplanned surgery.CONCLUSION:MCGR treatment provides adequate control of EOS, enabling satisfactory growth of the thoracic spine. The complication rate in a single large volume center is moderate.LEVEL OF EVIDENCE:IV.

Background Magnetic intramedullary lengthening nailing has demonstrated benefits over external fixation devices for femoral bone lengthening. These include avoiding uncomfortable external fixation and associated pin site infections, scarring, and inhibition of muscle or joint function. Despite this, little has changed in the field of biologically enhanced bone regeneration. Venting the femoral intramedullary canal at the osteotomy site before reaming creates egress for bone marrow during reaming. The reamings that are extruded from vent holes may function as a prepositioned bone graft at the distraction gap. The relationship between venting and the consolidation of regenerating bone remains unclear. Questions/purposes (1) Do bone marrow reamings extruded through venting holes enhance the quality of bone regeneration and improve healing indices and consolidation times? (2) Is venting associated with a higher proportion of complications than nonventing? Methods We performed a retrospective study of femoral lengthening performed at one hospital from December 2012 to February 2022 using a magnetic intramedullary lengthening nail with or without venting at the osteotomy site before reaming. This was a generally sequential series, in which the study groups were assembled as follows: Venting was performed between July 2012 and August 2016 and again from November 2021 onward. Nonventing was used between October 2016 and October 2021 because the senior author opted to create drill holes after the reaming procedure to avoid commitment to the osteotomy level before completing the reaming procedure. Outcomes were evaluated based on bone healing time, time to achieve full weightbearing, and complications. Sixty-one femoral lengthening procedures were studied (in 33 male and 28 female patients); two patients were excluded because of implant breakage. The mean age was 17 ± 5 years. The mean amount of lengthening was 55 ± 13 mm in the venting group and 48 ± 16 mm in the nonventing group (mean difference 7 ± 21 [95% CI 2 to 12]; p = 0.07). The healing index was defined as the time (in days) required for three cortices to bridge with new bone formation divided by the length (in cm) lengthened during the clinical protocol. This index signifies the bone formation rate achieved under the specific conditions of the protocol. Full weightbearing was allowed upon bridging the regenerated gap on three sides. Consolidation time was defined as the total number of days from the completion of the lengthening phase until adequate bone union (all three cortices healed) was achieved and full weightbearing was permitted. This time frame represents the entire healing process after the lengthening is complete divided by the amount of lengthening achieved (in cm). Patient follow-up was conducted meticulously at our institution, and we adhered to a precise schedule, occurring every 2 weeks during the distraction phase and every 4 weeks during the consolidation phase. There were no instances of loss to follow-up. Every patient completed the treatment successfully, reaching the specified milestones of weightbearing and achieving three cortexes of bone bridging. Results The mean healing index time in the venting group was faster than that in the nonventing group (21 ± 6 days/cm versus 31 ± 22 days/cm, mean difference 10 ± 23 [95% CI 4 to 16]; p = 0.02). The mean consolidation time was faster in the venting group than the nonventing group (10 ± 6 days/cm versus 20 ± 22 days/cm; mean difference 10 ± 23 [95% CI 4 to 15]; p = 0.02). No medical complications such as deep vein thrombosis or fat or pulmonary embolism were seen. Two patients had lengthy delays in regenerate union, both of whom were in the nonventing group (healing indexes were 74 and 62 days/cm; consolidation time was 52 and 40 days/cm). Conclusion Femoral lengthening with a magnetic intramedullary lengthening nail healed more quickly with prereaming venting than with nonventing, and it allowed earlier full weightbearing without any major associated complications. Future studies should evaluate whether there is a correlation between the number of venting holes and improvement in the healing index and consolidation time. Level of Evidence Level III, therapeutic study.

Introduction: The field of pediatric spine surgery has encountered major changes and evolutions lately, with new treatment options available and the development of enabling technologies. This article aims to summarize the most relevant recent literature. Materials and methods: The five most relevant topics were selected and assigned to one or two authors who performed a comprehensive Pubmed database search for articles published in the last 4 years (2021–2024). Only studies with a high level of evidence or clinical relevance were reported. Results: Thirty-nine articles were selected and analyzed, covering the following subjects: treatment options in tweeners, the impact of new medical treatments in pediatric spine practice, the emergence of new surgical techniques, the development of enabling technologies in scoliosis surgery, and recent relevant randomized controlled trials. Discussion: Many new surgical concepts and techniques have been developed lately, but their results need to be further assessed on specific subgroups of patients. Numerous significant medical improvements have been reported in the last 5 years, affecting positively the management of syndromic and neuromuscular patients.

Background: The COVID-19 pandemic has affected healthcare worldwide since December 2019. We aimed to identify the effect of the COVID-19 pandemic on outpatient clinic and surgical volumes and peri-operative complications for pediatric spinal deformities patients. Methods: In this multi-center retrospective study, outpatient visits (in-person and virtual care) and pediatric spine surgeries volumes in four high-volume pediatric spine centers were compared between March and December 2019 and the same period in 2020. Peri-operative complications were collected and compared in the same periods. Descriptive statistics were calculated, and comparative analyses were performed. Results: During the 2020 study period, the outpatient visit (in-person and virtual care) volume decreased during local lockdown periods by 71% for new patients (p < 0.001) and 53% for returning patients (p = 0.03). Overall, for 2020, there was a 20% reduction in new patients (p = 0.001) and 21% decrease in returning patients (p < 0.001). During the pandemic, there was also 20% less overall surgical volume of adolescent idiopathic scoliosis (AIS) patients undergoing primary posterior spinal fusion, with a 70% reduction during lockdown times (p < 0.001). Complication rate and profile were similar between periods. Conclusion: There was a significant decrease in outpatient pediatric spine outpatient visits, particularly new patients, which may increase the proportion of pediatric patients with spinal deformities that present late, meeting surgical indication. This, in combination with the reduction in surgical volume of AIS over the first year of the pandemic, could result in an extended waitlist for surgeries during years to come. Complication rate was similar for both periods, suggesting it is safe to continue elective pediatric spine surgery even in a time of a pandemic. Level of evidence: level IV.

Purpose: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy. Methods: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits. Results: Twenty-eight patients (mean follow-up: 16 months (range 2–41)) were included. The mean age at surgery was 60 months (range 29–96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded. Conclusion: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2.

The purpose of this study is to present the outcomes all patients with osteogenesis imperfecta (OI) who underwent cementless posterior spinal fusion for the treatment of severe spine deformity in our institution. All patients with OI who underwent surgical correction of their spine deformity in our institution between 2003 and 2020 were enrolled. The collected data included demographics, operative and follow-up findings, medical history, bisphosphonate therapy, HGT protocol, pre- and post-HGT and postoperative scoliosis and kyphosis curve measurements, hospitalization length, complications, and revision surgeries. General treatment strategies included cessation of bisphosphonate therapy around the surgery, 30-day HGT protocol, titanium rods, cementless screw technique, and a high implant density policy. Eleven consecutive patients with OI who underwent surgery for spine deformity in our institution were identified. The mean age at surgery was 15.6 ± 2.3. Mean follow-up period was 6.6 ± 5.8 years. The mean pre- and postoperative scoliosis curves were 85.4 ± 19.3° and 43.1 ± 12.5°, respectively, representing a 49.5% correction rate. Five patients underwent HGT and achieved a mean correction of 27.6 ± 7.1° (31.6%) preoperatively. Implant density ratio was 1.5 (screw or hook/level). Mean postoperative hospitalization length was 5.9 ± 1.6 days. One patient had deep wound infection which resolved following treatment according to our protocol for surgical site infection, and one patient had skull penetration by one of the halo pins. Surgical treatment of severe spine deformity in OI patients with cementless posterior spinal fusion is safe and effective after applying a specific preoperative strategy.

Background Emergence of new treatments for spinal muscular atrophy type 1 (SMA1) has led to dramatic improvements in respiratory failure and survival. However, these "treated" patients sustain major problems in other organ systems, which may directly or indirectly affect their respiratory function. We observed three main nonrespiratory manifestations in these patients comprised of facial deformities, feeding problems, and spinal deformities. Objective To investigate these three main sequelae in nusinersen-treated SMA1 patients. Methods Data on nusinersen-treated SMA1 patients were prospectively collected throughout a 3-year period, with special focus upon nonrespiratory features of the disease. Results Twenty nusinersen-treated SMA1 patients were included (eight males, median age 13.5 months, interquartile range: 4-56.2 months), among whom 17 survived after 3 years of follow-up. At follow-up, 15 (88%) patients were diagnosed with facial weakness, hypoplasia, or deformity. All but one patient (94%) were fed invasively by percutaneous endoscopic gastrostomy or nasogastric tube feeding. Four patients (25%) had maintained oral feeding in parallel to gastrostomy feeding and had clinical and radiologic evidence of aspirations. Fifteen (88%) patients were diagnosed with scoliosis, of whom seven had undergone or were scheduled to undergo corrective surgery. Conclusions Nusinersen-treated SMA1 patients may sustain facial deformities, feeding problems, and severe scoliosis, all of which affect their respiratory system. Strict surveillance of these complications is essential to avoid further respiratory morbidity.

PURPOSE:Several hexapod external fixators are used in the treatment of bone fracture and deformity corrections. One characteristic of all of them is the requirement for manual adjustment of the fixator struts. The purpose of this study was to introduce a novel robotic system that executes automatic adjustment of the struts. METHODS:Ten patients were treated for various bone deformities using a hexapod external fixator with the Auto Strut system. This new system automatically adjusts the fixator struts according to a hexapod computer-assisted correction plan. During each visit, the progress of the correction was assessed (clinically and radiographically) and reading of the strut scale numbers was performed and compared with the original treatment plan. RESULTS:All patients completed treatment during the follow-up period, achieving all planned correction goals, except from one patient who switched to manual struts due to personal preference. The device alarm system was activated once with no device-related adverse events. Duration of distraction ranged between ten and 90 days with a distraction index ranging between eight and 15 days/cm. Regenerate consolidation time between one and seven months. In total, 48 struts of eight patients were recorded and analyzed. In all, 94% of the final strut number readings presented a discrepancy of 0 mm to 1 mm between planned and actual readings, indicating high precision of the automatic adjustment. CONCLUSION:This study presents preliminary results, showing that Auto Strut can successfully replace the manual strut adjustment providing important advantages that benefit the patient, the caregiver and the surgeon. LEVEL OF EVIDENCE:Level II.

The intramedullary magnetic IM nail enables bone graduated distraction. Proximal femur osteotomies for ante grade IM lengthening nails have a tendency towards varus-procurvatum malalignment. We examined the effect of the level of the osteotomy and of trochanteric versus piriformis entry points on the neck shaft angle (NSA) during lengthening with the PRECICE IM magnetic nail. A novel parameter, the osteotomy level coefficient (OLC), was introduced as a guide to determine the level of an osteotomy at the proximal femur. The OLC was defined as the ratio between the distances from the tip of the greater trochanter to the osteotomy divided by the full length of the femur. A retrospective review of all femoral lengthening procedures with the PRECICE ante grade IM lengthening nail between 2013 and 2018 was carried out. 31 femurs were lengthened in 30 patients (16 males and 14 females, mean age at surgery years 17.1. The average amount of lengthening was 4.4 cm. Trochanteric entry points were used in 24 femurs, and piriformis entry points in seven femurs. The OLC values ranged from 0.16 to 0.34 (average 0.25). The average follow-up period was 10.15 months. The distraction index average 10.5 days/cm (Range 8.6–11.9), Consolidation index 32.1 days/cm (14.3–51.9). The average post-operative NSA was significantly reduced from 133.5º to 128.5º [t (31) = 5.57, p = 0.000]. There was no correlation between the OLC and the change in the NSAs. The trochanteric entry point showed a greater tendency to reduce the NSA (Mdif = − 6, SD = 4.8) compared to the piriformis entry point (Mdif =  − 0.86, SD = 2.27) [t (31) = -3.96, p = 0.001]. Proximal femur lengthening with the PRECICE IM nail significantly reduced the NSA and might cause Varus deformity. The level of osteotomy by OLC did not influence the amount of NSA reduction. The trochanteric entry points have a greater tendency to reduce the NSA compared to the piriformis entry points.

PURPOSE:To evaluate the AOSpine Thoracolumbar Spine Injury Classification System and if it is reliable and reproducible when applied to the paediatric population globally. METHODS:A total of 12 paediatric orthopaedic surgeons were asked to review MRI and CT imaging of 25 paediatric patients with thoracolumbar spine traumatic injuries, in order to determine the classification of the lesions observed. The evaluators classified injuries into primary categories: A, B and C. Interobserver reliability was assessed for the initial reading by Fleiss's kappa coefficient (kF) along with 95% confidence intervals (CI). For A and B type injuries, sub-classification was conducted including A0-A4 and B1-B2 subtypes. Interobserver reliability across subclasses was assessed using Krippendorff's alpha (αk) along with bootstrapped 95% CIs. A second round of classification was performed one-month later. Intraobserver reproducibility was assessed for the primary classifications using Fleiss's kappa and sub-classification reproducibility was assessed by Krippendorff's alpha (αk) along with 95% CIs. RESULTS:In total, 25 cases were read for a total of 300 initial and 300 repeated evaluations. Adjusted interobserver reliability was almost perfect (kF = 0.74; 95% CI 0.71 to 0.78) across all observers. Sub-classification reliability was substantial (αk= 0.67; 95% CI 0.51 to 0.81), Adjusted intraobserver reproducibility was almost perfect (kF = 0.91; 95% CI 0.83 to 0.99) for both primary classifications and for sub-classifications (αk = 0.88; 95% CI 0.83 to 0.93). CONCLUSION:The inter- and intraobserver reliability for the AOSpine Thoracolumbar Spine Injury Classification System was high amongst paediatric orthopaedic surgeons. The AOSpine Thoracolumbar Spine Injury Classification System is a promising option as a uniform fracture classification in children. LEVEL OF EVIDENCE:III.

Patients treated with magnetically controlled growing rods for spinal deformity in the growing spine were followed to definitive fusion. We showed successful control of the deformity. We have further defined the rate of surgical and implant related complications. Congenital scoliosis, rod diameter, and initial kyphosis were associated with higher complication rate. Study Design. A multicenter retrospective review of consecutive series of patients. Objective. Long-term experience with using the magnetically controlled growing rods (MCGR) to treat patients with deformity in the growing spine to the conclusion of treatment with posterior spine fusion. Summary of Background Data. MCGR treatment for growing spine gained popularity with paucity of long-term follow up data. We hypothesized that final fusion might be more effective in bringing additional correction of the spine deformity after treatment with MCGR than that reported after traditional growing rods (TGR) due to less scarring and auto-fusion. Methods. Retrospective review of 47 patients with varied etiology, treated between 2011 and 2017 which graduated treatment were followed in five academic medical centers for average of 50 months (range, 10–88). Results. The initial mean coronal deformity of 69.6° (95% CI 65–74) was corrected to 40° (95% CI 36–40) immediately after the MCGR implantation but progressed to 52.8° (95% CI 46–59) prior to the final surgery (P < 0.01). Nevertheless, thoracic spine growth (T1–T12 height) improved from 187.3 mm (95% CI 179–195) following index surgery to 208.9 mm (95% CI 199–218) prior to final fusion (P < 0.01). Significant correction and spinal length were obtained at final fusion, but metallosis was a frequent observation (47%, 22/47). The average growth rate was 0.5 mm/month (95% CI 0.3–0.6). The overall complication rate within our cohort was 66% (31/47) with 45% (21/47) of unplanned returns to the operating theater. 32% (15/47) of the patients had an implant related complication. Unplanned surgery was highly correlated with thoracic kyphosis greater than 40° (OR 5.42 95% CI 1.3–23). Conclusion. Treatment of growing spine deformities with MCGR provides adequate control of spine deformity it is comparable to previously published data about TGR. The overall high complications rate over time and specifically implant related complications. Level of Evidence: 4

Abstract Purpose Scoliosis, chest wall deformities and pulmonary involvement are common features of Marfan syndrome (MFS). We aimed to assess the impact of spinal and chest wall deformities on pulmonary function in paediatric MFS patients with a surgically managed spinal deformity. Methods In this multicentre retrospective study, spirometry, lung volumes and radiographic imaging were performed on 26 MFS patients between the ages of seven and 18 years who were undergoing planned spinal fusion surgery for spinal deformity. A correlation analysis assessed the relationship between radiographic measurements of spinal and chest wall deformities and predicted total lung capacity (TLC), forced vital capacity (FVC) and the ratio between forced expiratory volume in one second and FVC (FEV1/FVC). Results In total, 18 patients (70%) had impaired pulmonary function. Thoracic kyphosis (mean 19.3°; -32° to 54°) had a strong positive correlation with FEV1/FVC (r = 0.65; p < 0.001). Significant decrease in FEV1/FVC below 80% occurred at kyphosis under 15° (p = 0.004). Kyphosis had a moderate negative correlation with FVC (r = -0.43; p = 0.03). Chest wall deformity had a strong negative correlation with FEV1/FVC (r = -0.61; p = 0.001). The magnitude of the thoracic curve (mean 55.2°; 28° to 92°) had a significant moderate negative correlation with TLC (r = -0.45; p = 0.04). Conclusion In MFS, three factors correlate with decreased pulmonary function measures: hypokyphosis, increasing chest wall deformity and increasing coronal curve magnitude. Hypokyphosis and increased chest wall deformity correlated with diminished FEV1/FVC; increasing thoracic spinal curvature with diminished TLC. Further analysis with a larger cohort will help better define the relationship between these deformities and pulmonary function in this unique population. Level of Evidence IV

Tricho-rhino-phalangeal syndrome (TRPS) is characterized by craniofacial and skeletal malformations including short stature, cone-shaped phalangeal epiphyses and Perthes-like changes of the hip. We describe the response to growth hormone (GH) treatment in a boy with TRPS. The patient presented at age 3.5 years for evaluation of short stature (-3.2SD). On physical examination, the characteristic facial phenotype of TRPS was noted. Radiographs showed cone-shaped phalangeal epiphyses and bilateral small and fragmented femoral heads. The diagnosis was confirmed by Sanger sequencing of the TRPS1 gene. Two GH stimulation tests revealed GH deficiency, and GH treatment was initiated. Subsequently, growth velocity improved, as did the radiographic appearance of the femoral epiphyses, as seen on sequential pelvis radiographs. This observation suggests the possibility of a beneficial effect of GH treatment on both height and epiphyses status in TRPS patient with GH deficiency. Further studies are needed to support the observation.

Abstract Introduction Progressive neuromuscular spinal deformities with pelvic obliquity and loss of sitting balance are typical features of severely affected patients with cerebral palsy. The pelvis represents the key bone between the spine and the lower extremity when it comes to deciding whether and when to operate and if spine or hip surgery first is beneficial. The pelvis can be looked at as the lowest vertebra and as the rooftop of the lower extremities. Biomechanical considerations To allow for a normal spinal shape, the pelvis needs to be horizontal in the frontal plane and mildly anterior tilted in the sagittal plane, less for sitting and more for standing. Any abnormal pelvic position requires spinal compensation and challenges the equilibrium control of the individual. Both anatomical neighbourhoods – the spine and the hip joints — have to be considered when spinal deformities, hip instability and contractures evolve, in conservative therapy (bracing, physiotherapy, seating in the wheelchair) and when surgical interventions are weighed out against each other. Surgical considerations Multiple anatomical factors such as sagittal profile and pelvic orientiation, pelvic transverse plane asymmetries and lumbosacral malformations have to be considered in case the pelvis is instrumented with sacral and iliac screws. Rotational deformities and asymmetries of the pelvic bones make the safe insertion of long screws challenging. Advantages of primary pelvic fixation include correction of pelvic obliquity, especially considering the lever arm of the whole spinal construct. The risk of revision surgery due to progression of distal curves is also reduced. Disadvantages of pelvic fixation include the complexity of the additional intervention, which may result in longer operating times, increased risk of blood loss, infection and hardware malpositioning.

INTRODUCTION:Progressive neuromuscular spinal deformities with pelvic obliquity and loss of sitting balance are typical features of severely affected patients with cerebral palsy. The pelvis represents the key bone between the spine and the lower extremity when it comes to deciding whether and when to operate and if spine or hip surgery first is beneficial. The pelvis can be looked at as the lowest vertebra and as the rooftop of the lower extremities.BIOMECHANICAL CONSIDERATIONS:To allow for a normal spinal shape, the pelvis needs to be horizontal in the frontal plane and mildly anterior tilted in the sagittal plane, less for sitting and more for standing. Any abnormal pelvic position requires spinal compensation and challenges the equilibrium control of the individual. Both anatomical neighbourhoods - the spine and the hip joints - have to be considered when spinal deformities, hip instability and contractures evolve, in conservative therapy (bracing, physiotherapy, seating in the wheelchair) and when surgical interventions are weighed out against each other.SURGICAL CONSIDERATIONS:Multiple anatomical factors such as sagittal profile and pelvic orientiation, pelvic transverse plane asymmetries and lumbosacral malformations have to be considered in case the pelvis is instrumented with sacral and iliac screws. Rotational deformities and asymmetries of the pelvic bones make the safe insertion of long screws challenging. Advantages of primary pelvic fixation include correction of pelvic obliquity, especially considering the lever arm of the whole spinal construct. The risk of revision surgery due to progression of distal curves is also reduced. Disadvantages of pelvic fixation include the complexity of the additional intervention, which may result in longer operating times, increased risk of blood loss, infection and hardware malpositioning.