Cranial Suture Regeneration Mitigates Skull and Neurocognitive Defects in Craniosynostosis.

Craniosynostosis results from premature fusion of the cranial suture(s), which contain mesenchymal stem cells (MSCs) that are crucial for calvarial expansion in coordination with brain growth. Infants with craniosynostosis have skull dysmorphology, increased intracranial pressure, and complications such as neurocognitive impairment that compromise quality of life. Animal models recapitulating these phenotypes are lacking, hampering development of urgently needed innovative therapies. Here, we show that Twist1+/- mice with craniosynostosis have increased intracranial pressure and neurocognitive behavioral abnormalities, recapitulating features of human Saethre-Chotzen syndrome. Using a biodegradable material combined with MSCs, we successfully regenerated a functional cranial suture that corrects skull deformity, normalizes intracranial pressure, and rescues neurocognitive behavior deficits. The regenerated suture creates a niche into which endogenous MSCs migrated, sustaining calvarial bone homeostasis and repair. MSC-based cranial suture regeneration offers a paradigm shift in treatment to reverse skull and neurocognitive abnormalities in this devastating disease.

Review of susceptibility genes in developmental dysplasia of the hip: A comprehensive examination of candidate genes and pathways.

Developmental dysplasia of the hip (DDH) is one of the most prevalent skeletal deformities, primarily due to the incompatibility between the acetabulum and femoral head. It includes complete dislocation, partial dislocation, instability with femoral head subluxation, and a range of imaging abnormalities that reflect inadequate acetabular formation. Known risk factors for DDH include positive family history, sex, premature birth, non-cephalic delivery, oligohydramnios, gestational diabetes mellitus, maternal hypertension, associated anomalies, swaddling clothes, intrauterine space restriction, and post-term pregnancy. Various research designs have been employed in DDH studies to identify relevant genes, including candidate gene association studies (CGAS), genome-wide association studies (GWAS), restriction fragment length polymorphism (RFLP), and whole exome sequencing (WES). To date, multiple DDH-associated genes have been identified in various populations. Despite extensive research into the epidemiology, risk factors, and genes associated with DDH, its pathogenesis remains unclear. This study provides a comprehensive summary of DDH research designs and evidence for relevant gene mutations through a PubMed search.

Non-surgical correction of knee flexion deformity in persons with haemophilia: A staged multidisciplinary approach.

INTRODUCTION: Flexion deformity of the knee is a common complication following recurrent haemarthrosis in persons with haemophilia (PWH) on episodic factor replacement therapy, restricting independent mobility. There is limited literature on the comprehensive management of this condition. This report provides the outcome of a staged multidisciplinary approach for the correction of knee flexion deformity (KFD) even in limited resource settings. PATIENTS AND METHODS: The data of 49 consecutive PWH who were treated for KFD were analysed. The approach included graded physical therapy (PT), followed by serial casting and/or mobilisation under anaesthesia (MUA). MUA was done in carefully selected knees. Surgical correction was opted when non-surgical methods failed. RESULTS: Of the 49 patients (55 knees), with a median KFD of 40 degrees (range: 10-90), 26/55 (47%) were corrected by graded PT. With serial casting, 9/19 (47%) knees had their KFD corrected. MUA was done for 11 knees of which five achieved correction (45%). Surgical correction was required for only seven knees (12.7%). Following this approach, KFD improved from 40 degrees (range: 10-90) to 15 degrees (range: 0-40), with only minor loss of flexion from 105 (range: 60-155) to 90 degrees (range: 30-150). Out of 55 KFD, 46 (83.6%) KFD were corrected; non-surgical, 39 (70.9%) and surgery, seven (12.7%). The remaining patients (nine KFD; 16.4%) were able to achieve their functional goal despite not meeting the correction criteria. CONCLUSION: This study shows that in PWH, functionally significant KFD correction can be achieved in about 71%, through non-surgical methods, even without prophylactic factor replacement.

Early-onset and uncontrolled diabetes mellitus factors correlate with complications of Peyronie's disease.

BACKGROUND: Peyronie's disease (PD) is a connective tissue disorder that affects the penis and is characterized by abnormal collagen structure in the penile tunica albuginea, resulting in plaque formation and penile deformity. PD's overall prevalence is estimated at 3.2% to 8.9%, with rates as high as 20.3% among men with type 2 diabetes mellitus (DM). However, the characteristics of DM associated with PD complications remain unclear. AIM: To explore clinical associations between DM characteristics and PD complications. METHODS: We conducted a retrospective analysis of patients with DM and PD who presented at our institution between 2007 and 2022. We examined patients' clinical histories, DM- and PD-related clinical parameters, and complications. Penile deformities were assessed through physical examination, photographs, and penile Doppler ultrasound. Patients were categorized into subgroups based on age of DM onset: early (<45 years), average (45-65 years), and late (>65 years). OUTCOMES: Outcomes included effects of DM characteristics on PD development, progression, and severity. RESULTS: In total, 197 patients were included in the evaluation. Early-onset diabetes and elevated hemoglobin A1c (HbA1c) levels exhibited significant correlations with the early development of PD (ρ = 0.66, P < .001, and ρ = -0.24, P < .001, respectively). Furthermore, having DM at an early age was associated with the occurrence of penile plaque (ρ = -0.18, P = .03), and there were no significant differences in plaque dimensions (ρ = -0.29, P = .053). A rise in HbA1c levels after the initial PD diagnosis displayed positive correlations with the formation of penile plaque (ρ = 0.22, P < .006). CLINICAL IMPLICATIONS: These findings emphasize the need for comprehensive assessments and personalized treatment strategies for individuals with DM and PD. Enhanced management approaches can improve outcomes for those facing both challenges. STRENGTHS AND LIMITATIONS: Limitations include the single-site retrospective design with potential selection bias, inaccuracies in medical record data, and challenges in controlling confounding variables. CONCLUSIONS: This study highlights that early-onset diabetes and poor diabetes control, as indicated by a subsequent rise in HbA1c levels following PD diagnosis, are significantly correlated with the onset and severity of PD. Revealing the mechanisms behind these findings will help us develop better management strategies for individuals with DM and PD.

Role of epidural fat in the local milieu: what we know and what we don't.

PURPOSE: Traditionally, the epidural fat (EF) is known as a physical buffer for the dural sac against the force and a lubricant facilitating the relative motion of the latter on the osseous spine. Along with the development of the studies on EF, controversies still exist on vital questions, such as the underlying mechanism of the spinal epidural lipomatosis. Meanwhile, the scattered and fragmented researches hinder the global insight into the seemingly dispensable tissue. METHODS: Herein, we reviewed literature on the EF and its derivatives to elucidate the dynamic change and complex function of EF in the local milieu, especially at the pathophysiological conditions. We start with an introduction to EF and the current pathogenic landscape, emphasizing the interlink between the EF and adjacent structures. We generally categorize the major pathological changes of the EF into hypertrophy, atrophy, and inflammation. RESULTS AND CONCLUSIONS: It is acknowledged that not only the EF (or its cellular components) may be influenced by various endogenic/exogenic and focal/systematic stimuli, but the adjacent structures can also in turn be affected by the EF, which may be a hidden pathogenic clue for specific spinal disease. Meanwhile, the unrevealed sections, which are also the directions the future research, are proposed according to the objective result and rational inference. Further effort should be taken to reveal the underlying mechanism and develop novel therapeutic pathways for the relevant diseases.

Effect of Narrow Chest on Minimally Invasive Mitral Valve Surgery via Right Minithoracotomy.

BACKGROUND: The effect of a narrow chest on minimally invasive mitral valve surgery (MIMVS) is unclear.Methods and Results: We enrolled 206 MIMVS patients and measured anteroposterior diameter (APD) between the sternum and vertebra, transverse thoracic diameter (TD), right and left APD of the hemithorax (RD and LD, respectively), and the Haller index (HI; TD/APD ratio) on computed tomography. Preoperative characteristics and operative outcomes were compared between patients with a narrow chest (Group N; HI >2.5; n=53) and those with a normal chest (control [C]; HI ≤2.5; n=153), and the correlations of these measurements with operation time were evaluated in 133 patients undergoing an isolated mitral procedure. Groups N and C differed significantly in APD (89.4 vs. 114.3 mm, respectively; P<0.001), TD (251.5 vs. 240.3 mm, respectively; P=0.002), RD (152.5 vs. 172.5 mm, respectively; P<0.001), LD (155.0 vs. 172.4 mm, respectively; P<0.001), and HI (2.84 vs. 2.12, respectively; P<0.001). Procedural characteristics were comparable, except for a longer aortic cross-clamp time (ACCT) in Group N (118.7 vs. 105.8 min; P=0.047). Rates of surgical death, re-exploration, cerebral infarction, and prolonged ventilation were comparable between the 2 groups. TD was significantly correlated with ACCT (R2=0.037, P=0.028) in patients undergoing an isolated mitral procedure. CONCLUSIONS: Early MIMVS outcomes in patients with narrow chests are satisfactory. TD prolongs ACCT during MIMVS.

Prevalence and natural history of gibbus deformity in patients with Hurler syndrome.

INTRODUCTION: Gibbus deformity has been documented as a common musculoskeletal abnormality in mucopolysaccharidosis type I (Hurler syndrome, MPS IH), and its recognition often leads to the diagnosis of MPS IH. While the incidence has been described, the progression of gibbus deformities is not well known. Here we describe the natural history of gibbus deformity in a single center patient population using serial spinal MRI scans. METHODS: All spinal MRI scans in MPS IH patients were retrospectively reviewed. The presence, spinal location, and angulation of the gibbus deformities were collected. The angles between the superior endplate of the superior normal vertebral body and the inferior endplate of the inferior normal vertebral body were measured. RESULTS: 24 of 47 patients (51%) were found to have cervico-thoracic deformity on their cervical MRI scans, and 19 of those 24 (79%) patients were found to have progressive cervico-thoracic deformity with average change of angle of 17.1 degrees [range 3.9, 62.8] over 5.3 years. 7 of 8 patients who had thoraco-lumbar MRI were found to have thoraco-lumbar deformity, and 4 of those 7 patients (57%) were found to have progressive thoraco-lumbar deformity with the average increase angle of 16.7 degrees [range 3.3, 47.1] over an average of 4.1 years. CONCLUSION: We found out that baseline spinal measurement cannot reliably predict the progression as multiple patients with normal alignment eventually developed severe deformity, whereases patients with severe deformity did not progress to require surgical intervention.

Correlation of Posterior Tibial Tendon Ultrasound with Calcaneal Inclination Angle in Indonesian Professional Athletes with Medial Ankle Pain.

Background: Adult-acquired flatfoot deformity (AAFD) is characterized by partial or complete flattening of the longitudinal medial arch, which develops after maturity. AAFD secondary to posterior tibialis tendon dysfunction (PTTD) is one of professional athletes' most common foot and ankle pathologies. Different modalities and procedures can be used to establish the diagnosis of AAFD and PTTD. However, imaging measurements such as the calcaneal inclination index and ultrasonography (US) of the posterior tibialis tendon (PTT) in professional athletes with medial ankle and focal pain along the PTT have yet to be widely studied. This study investigates the correlation of PTT ultrasound for evaluating PTTD with calcaneal inclination angle (CIA) for evaluating AAFD in professional athletes with medial ankle and focal pain along the PTT. Through this study, clinicians and radiologists may benefit from considering AAFD in athletes with PTTD. Methods: 112 Indonesian professional athletes with medial ankle or foot pain and focal pain along the direction of the PTT underwent foot radiography using the CIA and ankle ultrasound to observe PTT abnormalities. Results: A negative correlation between fluid thickness surrounding the PTT and the CIA (p<0.001; 95% CI - 0.945, - 0.885), as well as a negative correlation between PTT thickness and CIA (p<0.001, 95% CI - 0.926, - 0.845), with a correlation coefficient (r) of - 0.921 and - 0.892, respectively. No significant correlation was found between PTT tear and CIA (p = 0.728; 95% CI -0.223, - 0.159; r - 0.033). Conclusion: This study showed a negative correlation between PTTD and AAFD via ultrasound and CIA in professional athletes with medial ankle and focal pain along the PTT. A better understanding of PTTD and AAFD imaging will lead to more effective management and prompt treatment.

Supraperiosteal fat repositioning with midface lift in lower eyelid blepharoplasty: an 18-year experience.

PURPOSE: To assess the clinical outcomes of fat repositioning via supraperiosteal dissection with midface lift for correction of tear trough deformity in a large Asian patient population. METHODS: Retrospectively review 1152 Asian patients who underwent fat repositioning to the supraperiosteal plane with a midface lift between 2005 and 2022. Surgical technique, postoperative course, and complications were recorded. At the 6-month postoperative follow-up, the degree of patient satisfaction was assessed. RESULTS: A total of 2304 eyes from 1152 patients with an average follow-up of 10 months. These procedures were performed using a transforniceal approach in 185 patients (16%) or a transcutaneous skin excision approach in 967 patients (84%). Among the patients who underwent the transcutaneous technique, seven individuals (0.6%) experienced effective treatment of lower lid ectropion through lateral tarsal strip procedures. Nine patients (0.7%) required revision surgery to address the remaining lateral fat pad due to inadequate lateral orbital fat excision during the initial procedure. At the 6-month follow-up, most patients reported a high level of satisfaction, with 800 patients (78%) expressing extreme satisfaction and 196 patients (19.1%) reporting satisfaction with the improvement in their appearance. No one reported facial numbness, lower eyelid or cheek paralysis, newly developed diplopia or granuloma formation. CONCLUSION: The procedure of fat repositioning involving supraperiosteal dissection and a midface lift, whether performed using a transforniceal approach or a transcutaneous skin excision approach, in lower eyelid blepharoplasty proves to be a secure and auspicious surgical technique for rectifying tear trough deformity and attaining a pleasing aesthetic outcome.

Morphological changes in flatfoot: a 3D analysis using weight-bearing CT scans.

BACKGROUND: Flatfoot is a condition resulting from complex three-dimensional (3D) morphological changes. Most Previous studies have been constrained by using two-dimensional radiographs and non-weight-bearing conditions. The deformity in flatfoot is associated with the 3D morphology of the bone. These morphological changes affect the force line conduction of the hindfoot/midfoot/forefoot, leading to further morphological alterations. Given that a two-dimensional plane axis overlooks the 3D structural information, it is essential to measure the 3D model of the entire foot in conjunction with the definition under the standing position. This study aims to analyze the morphological changes in flatfoot using 3D measurements from weight-bearing CT (WBCT). METHOD: In this retrospective comparative our CT database was searched between 4-2021 and 3-2022. Following inclusion criteria were used: Patients were required to exhibit clinical symptoms suggestive of flatfoot, including painful swelling of the medial plantar area or abnormal gait, corroborated by clinical examination and confirmatory radiological findings on CT or MRI. Healthy participants were required to be free of any foot diseases or conditions affecting lower limb movement. After applying the exclusion criteria (Flatfoot with other foot diseases), CT scans (mean age = 20.9375, SD = 16.1) confirmed eligible for further analysis. The distance, angle in sagittal/transverse/coronal planes, and volume of the two groups were compared on reconstructed 3D models using the t-test. Logistic regression was used to identify flatfoot risk factors, which were then analyzed using receiver operating characteristic curves and nomogram. RESULT: The flatfoot group exhibited significantly lower values for calcaneofibular distance (p = 0.001), sagittal and transverse calcaneal inclination angle (p < 0.001), medial column height (p < 0.001), sagittal talonavicular coverage angle (p < 0.001), and sagittal (p < 0.001) and transverse (p = 0.015) Hibb angle. In contrast, the sagittal lateral talocalcaneal angle (p = 0.013), sagittal (p < 0.001) and transverse (p = 0.004) talocalcaneal angle, transverse talonavicular coverage angle (p < 0.001), coronal Hibb angle (p < 0.001), and sagittal (p < 0.001) and transverse (p = 0.001) Meary's angle were significantly higher in the flatfoot group. The sagittal Hibb angle (B = - 0.379, OR = 0.684) and medial column height (B = - 0.990, OR = 0.372) were identified as significant risk factors for acquiring a flatfoot. CONCLUSION: The findings validate the 3D spatial position alterations in flatfoot. These include the abduction of the forefoot and prolapse of the first metatarsal proximal, the arch collapsed, subluxation of the talonavicular joint in the midfoot, adduction and valgus of the calcaneus, adduction and plantar ward movement of the talus in the hindfoot, along with the first metatarsal's abduction and dorsiflexion in the forefoot.

Thoracic and vertebral deformities in lung transplantation: perioperative complications and long-term prognoses.

BACKGROUND: Lung transplantation (LTx) is a crucial therapeutic strategy for patients suffering from end-stage respiratory diseases, necessitating precise donor-recipient size matching to ensure optimal graft function. While standard allocation protocols rely on predicted lung capacity based on factors such as sex, age, and height, a subset of patients with respiratory diseases presents an additional challenge - thoracic or vertebral deformities. These deformities can complicate accurate volume predictions and may impact the success of lung transplantation. METHODS: In this retrospective cohort study of patients who underwent LTx at Tohoku University Hospital between January 2007 and April 2022, with follow-up until October 2022, the primary objective was to assess the influence of thoracic and vertebral deformities on perioperative complications, emphasizing interventions, such as volume reduction surgery. The secondary objective aimed to identify any noticeable impact on long-term prognoses in recipients with these deformities. RESULTS: Of 129 LTx recipients analyzed, 17.8% exhibited thoracic deformities, characterized by pectus excavatum, while 16.3% had vertebral deformities. Perioperative complications, requiring delayed chest closure, tracheostomy, and volume reduction surgery, were more prevalent in the deformity group. Thoracic deformities were notably associated with the need for volume reduction surgery. However, long-term prognoses did not differ significantly between patients with deformities and those without. Vertebral deformities did not appear to significantly impact perioperative or long-term outcomes. CONCLUSIONS: This study highlights the prevalence of thoracic deformities in LTx recipients, correlating with increased perioperative complications, particularly the potential need for volume reduction surgery. Importantly, these deformities do not exert a significant impact on long-term prognoses. Additionally, patients with vertebral deformities, such as scoliosis and kyphosis, appear to be manageable in the context of LTx.

Multiple spinal extradural arachnoid cysts presenting as compressive myelopathy in a teenager: case report and literature review with special emphasis on postoperative spine deformity in the current minimally invasive era.

INTRODUCTION: Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.). MATERIAL AND METHODS: We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease. RESULTS: A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique "not reported" in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up. CONCLUSIONS: Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary.

Surgical limits, pitfalls, and potential solutions in kyphectomy in myelomeningocele: three cases and systematic review of the literature.

OBJECTIVES: To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic. METHODS: Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients' information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies. RESULTS: Three cases underwent posterior vertebral column resection (pVCR) of 2-4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other. CONCLUSION: Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.

Evaluation of the use of tranexamic acid in the postoperative period in patients with scoliosis undergoing posterior fusion.

Scoliosis is the most prevalent type of spinal deformity, with a 2-3% prevalence in the general population. Moreover, surgery for scoliotic deformity may result in severe blood loss and, consequently, the need for blood transfusions, thereby increasing surgical morbidity and the rate of complications. Several antifibrinolytic drugs, such as tranexamic acid, have been regarded as safe and effective options for reducing blood loss. Therefore, the present study aimed to analyse the effectiveness of this drug for controlling bleeding when used intraoperatively and in the first 48 h after surgery. A prospective randomized study of a cohort of patients included in a mass event for scoliosis treatment using PSF was performed. Twenty-eight patients were analysed and divided into two groups: 14 patients were selected for intraoperative and postoperative use of tranexamic acid (TXA), and the other 14 were selected only during the intraoperative period. The drainage bleeding rate, length of hospital stay, number of transfused blood units, and rate of adverse clinical effects were compared. All the patients involved had similar numbers of fusion levels addressed and similar scoliosis profiles. The postoperative bleeding rate through the drain did not significantly differ between the two groups (p > 0.05). There was no significant difference in the number of transfused blood units between the groups (p = 0.473); however, in absolute numbers, patients in the control group received more transfusions. The length of hospital stay was fairly similar between the groups, with no statistically significant difference. Furthermore, the groups had similar adverse effects (p = 0.440), with the exception of nausea and vomiting, which were twice as common in the TXA group postoperatively than in the control group. No significant differences were found in the use of TXA during the first 48 postoperative hours or in postoperative outcomes.

The more the better? Integration of vertebral pelvic angles (VPA) PJK thresholds to existing alignment schemas for prevention of mechanical complications after adult spinal deformity surgery.

PURPOSE: While existing adult spinal deformity (ASD) alignment schemas acknowledge the dynamic relationship between the pelvis and spine, consideration of vertebral pelvic angles (VPA) thresholds for PJK may provide further insight into the relationship of each individual vertebra to the pelvis, which may allow for greater individualization of operative targets. Herein, we examine VPA's utility in preventing mechanical complications and its possible unification with prevalent scoring systems. METHODS: In a retrospective cohort study of a prospectively collected database, operative ASD patients ≥ 18 years with complete baseline (BL) and two-year (Y) operative, radiographic, and health-related quality of life data were included. Descriptive analyses, means comparison, and logistic regression tests were applied to explore demographic and surgical differences, as well as the impact of alignment goals on outcomes. Cohorts were grouped as patients who met VPA non-PJK thresholds, as defined by Duvvuri et al. 2023 alone versus traditional GAP/SAAS alignment matching versus combined VPA + SAAS + GAP. The Non-PJK VPA validated mean for L1PA was 10.4 ± 7.0 and T9PA 8.9 ± 7.5. RESULTS: 398 patients met inclusion criteria (mean age 61 ± 14 years, 78% female, BL BMI 27 ± 6, BL CCI 2 ± 2). At baseline, mean vertebral pelvic angles were as follows: T1PA: 24 ± 14; T4PA 20 ± 13, T9PA 15 ± 12, L1PA 11 ± 10, L4PA 11 ± 6. Mean vertebral pelvic angles at 6 W postoperatively: T1PA 16 ± 10, T4PA 12 ± 10, T9PA 8 ± 9, L1PA 9 ± 8, L4PA 11 ± 5. 240 (60%) patients attained optimal L1PA, while 104 patients (26.1%) reached non-PJK mean for T9PA. 89 patients (22%) were optimal by both VPA standards. VPA-Optimal group demonstrated significantly lower rates of 1Y PJK (17% v 83%, p = 0.042) and PJF by 2Y (7% v. 93%, p = 0.038). When patients attained VPA goals in addition to GAP/SAAS goals at 6 W, they demonstrated significantly lower rates of Y1 PJK (p = 0.026) and Y1 and Y2 PJF. Those with optimal VPA registered greater SRS-22 scores across multiple domains (p < 0.02) as well as a greater rate of normal neurological examination at 6 W (p = 0.048). CONCLUSIONS: Vertebral pelvic angles are a reliable measure of global alignment, and respecting certain targets may help prevent development of PJK/PJF. The value of VPA can be augmented through integration with GAP/SAAS frameworks to prevent complications and improve quality of life.

Growing rods in meningomyelocele lead to increased risk for complications in comparison with fusion; a retrospective study of 30 patients treated for at the University Hospital of Uppsala.

PURPOSE: To compare the complication rates of two different types of posterior instrumentation in patients with MMC, namely, definitive fusion and fusionless surgery (growing rods). METHODS: Single-center retrospective study of 30 MMC patients that underwent posterior instrumentation for deformity (scoliosis and/or kyphosis) treatment from 2008 until 2020. The patients were grouped based on whether they received definitive fusion or a growth-accommodating system, whether they had a complication that led to early surgery, osteotomy or non-osteotomy. Number of major operations, Cobb angle correction and perioperative blood loss were the outcomes. RESULTS: 18 patients received a growing system and 12 were fused at index surgery. The growing system group underwent a mean of 2.38 (± 1.03) surgeries versus 1.91 (± 2.27) in the fusion group, p = 0.01. If an early revision was necessitated due to a complication, then the number of major surgeries per patient was 3.37 (± 2.44) versus 1.77 (± 0.97) in the group that did not undergo an early revision, p = 0.01. Four patients developed a superficial and six a deep wound infection, while loosening/breakage occurred in 10 patients. The Cobb angle was improved from a mean of 69 to 22 degrees postoperatively. Osteotomy did not lead to an increase in perioperative blood loss or number of major operations. CONCLUSION: Growing systems had more major operations in comparison with fusion surgery and early revision surgery led to higher numbers of major operations per patient; these differences were statistically significant. Definitive fusion at index surgery might be the better option in some MMC patients with a high-risk profile.

Scoliosis and rare diseases: our experience with the Prader-Willi syndrome.

INTRODUCTION: Prader-Willi syndrome (PWS) represents a difficult challenge for spine surgeons, due to the association of a structural scoliosis, with a prevalence between 15 and 86%. Conservative therapy is a viable option, but surgery is increasingly becoming the treatment of choice. METHODS: The authors reviewed a series of 15 patients affected by PWS treated at their institution between 2008 and 2023. The mean age at index treatment was 9 years and 3 months (range 1-15 years) with a prevalence of female subjects. Primary scoliotic curve ranged from 14 to 102°, and mean thoracic kyphosis was 56° (range 20-75°). Eleven patients underwent conservative treatment, while four were treated surgically. RESULTS: Mean follow-up was 5 years and 3 months (range 2-12 years). Among the 11 patients treated conservatively, only two showed improvements of the coronal curve, while the remaining nine displayed a worsening of the deformity during follow-up. Complication rate after surgery was 75%. One patient developed paraplegia after pedicle screw positioning. One patient displayed rod breakage and PJK that required revision surgery proximally. Hardware deep infection was seen in one case where it was necessary to proceed with instrumentation removal after 10 years. DISCUSSION AND CONCLUSIONS: Spine surgery represents a convincing option in patients affected by PWS, but the risks of complications are high. Correct patient selection must be the main objective, and multilevel pedicle screw fixation should be the procedure of choice. Traditional growing rod should be prudently evaluated in every single case.

Restoration of the sagittal profile according to the Roussouly classification reduces mechanical complications and revision surgery in older patients undergoing surgery for adult spinal deformity (ASD).

PURPOSE: The mechanical complications related to ASD remain a concern due to their morbidity and associated revision surgery. Restoration of each patient's Roussouly profile may reduce these. Our aim was to examine if the restoration of the Roussouly profile reduced these complications and revision rates in older patients operated for degenerative ASD. METHODS: Retrospective analysis of a single-centre, 2-year minimum follow-up patient database. All patients undergoing corrective surgery (≥ 4 levels) for ASD were included with analysis of demographic data, operative records, restoration of Roussouly sagittal profile, mechanical complications and revision rates. Univariate and multivariate analysis was conducted. RESULTS: Fifty-two patients were included (mean age was 72.3 years, average follow-up 56.3 months). Twenty-six patients had a "restored" profile (50%) and 26 an "unrestored" profile (50%). The incidence of mechanical complications was 7 (27%) and 23 (88%) for the restored and unrestored groups, respectively (p < 0.001). Revision rates were 4 (15.4%) and 18 (69.2%), respectively (p < 0.000), in the restored and unrestored profiles. Univariate analysis determined that profile restoration and BMI were associated with mechanical complications and revision surgery, whilst only the profile restoration status maintained its statistical power in multivariate analysis (p = 0.002 and p = 0.002, respectively). Age was not a significant factor in univariate analysis. The relative risk for mechanical failure and revision surgery was 5.6 times (CI 1.929-16.39) and 3.08 times (CI 1.642-5.734) greater if the profile was not restored. CONCLUSIONS: Achieving each patient's ideal Roussouly profile is associated with a reduced incidence of mechanical complications and revision rates in the older population after surgery for degenerative ASD.

Assessing the predictive power of the GAP score on mechanical complications: a comprehensive systematic review and meta-analysis.

PURPOSE: The prevention of mechanical complications (MC) is a major concern in adult spinal deformity (ASD) correction surgery; thus, the global alignment and proportion (GAP) score was developed to assess MC risk. Numerous studies have clarified the validity of the GAP score, but their contradictory results have prevented researchers from reaching compelling conclusions. This study aimed to analyze the predictive power of the GAP score on MC via a meta-analysis. METHODS: A total of 1,617 patients were included in the meta-analysis. Studies relevant to the GAP score and MC were identified in PubMed, EMBASE, and Cochrane CENTRAL and screened according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The GAP score categories of the patients and their MC/revision surgery status were collected. The data collected for the meta-analysis of odds ratios (OR) included the number of patients in the GAP score subgroups and their MC/revision surgery status. To calculate the OR, three GAP score subgroups were combined into two groups; hence, the analysis was conducted twice (gap proportioned [GAP-P] and higher groups, and gap severely disproportioned [GAP-SD] and lower groups). RESULTS: Eleven studies were collected; of them, revision surgery data were available for seven. The proportion of MC in the studies was 27.7-60.6%, while that of revision surgery was 11.7-34.9%. In the meta-analysis of the GAP-P and higher score groups, the difference in MC ratio was significant (OR = 2.83; 95% confidence interval [CI] = 1.20-6.67; P = 0.02), whereas that for revision surgery was not. For the GAP-SD and lower score groups, the GAP-SD group had significantly higher proportions of both MC (OR = 2.65; 95% CI = 1.57-4.45; P < 0.001) and revision surgery (OR = 2.27; 95% CI = 1.33-3.88; P = 0.003). Publication bias was significant only in the latter MC analysis. CONCLUSION: The GAP score offers predictive value for the risk of mechanical complications.

Should the pelvis be included? An analysis of distal junctional failure risk factors in correcting adult spinal deformity in women with fusion to L5.

PURPOSE: To identify the risk factors for distal junctional failure (DJF) in women treated for adult spine deformity with fusion to L5 and to highlight the significance of preoperative assessment, surgical decision-making, and postoperative care. METHODS: This is a retrospective study of data collected prospectively on the local institutional spine surgery registry (2016-2021). All patients, women older than 18 years, with a diagnosis of adult spine deformity who underwent long posterior instrumentation to L5 and had a minimum of 2-years follow-up were included in the study (two groups: with or without DJF). Demographic and radiographic data, corrective strategy, preoperative level of degeneration at L5/S1 and GAP score were compared between the two groups. RESULTS: Forty-eight patients (n = 48) satisfied eligibility criteria. At two-years follow-up, nine patients (18,7%) developed a DJF that required surgical revision. Thirty-nine patients did not present distal junctional complications. Patients with or without DJF showed significant differences in terms of preoperative spinopelvic parameters (PT: 28°± 6° vs. 23°± 9°, p-value 0.05; DJF group vs. not DJF) and degeneration of L5-S1 (Pfirmann grade L5-S1 disc 3.7 ± 1.0 vs. 2.6 ± 0.8, p-value 0.001; DJF group vs. not DJF) (L5-S1 Facet joint Osteoarthritis 3.1 ± 0.8 vs.2.4 ± 0.8, p-value 0.023; DJF group vs. not DJF). CONCLUSION: DJF following spinal deformity correction surgery is influenced by a combination of patient-related, surgical and implant-related factors. Fusion construct length, preoperative and postoperative sagittal alignment and the grade of degeneration of the distal disc have been identified as significant risk factors. Surgeons should carefully evaluate these factors and employ appropriate strategies.