Early treatment of type II SMA slows rate of progression of scoliosis.

BACKGROUND: Type II spinal muscular atrophy (SMA) often leads to scoliosis in up to 90% of cases. While pharmacological treatments have shown improvements in motor function, their impact on scoliosis progression remains unclear. This study aims to evaluate potential differences in scoliosis progression between treated and untreated SMA II patients. METHODS: Treatment effect on Cobb's angle annual changes and on reaching a 50° Cobb angle was analysed in treated and untreated type II SMA patients with a minimum 1.5-year follow-up. A sliding cut-off approach identified the optimal treatment subpopulation based on age, Cobb angle and Hammersmith Functional Motor Scale Expanded at the initial visit. Mann-Whitney U-test assessed statistical significance. RESULTS: There were no significant differences in baseline characteristics between the untreated (n=46) and treated (n=39) populations. The mean Cobb angle variation did not significantly differ between the two groups (p=0.4). Optimal cut-off values for a better outcome were found to be having a Cobb angle <26° or an age <4.5 years. When using optimal cut-off, the treated group showed a lower mean Cobb variation compared with the untreated group (5.61 (SD 4.72) degrees/year vs 10.05 (SD 6.38) degrees/year; p=0.01). Cox-regression analysis indicated a protective treatment effect in reaching a 50° Cobb angle, significant in patients <4.5 years old (p=0.016). CONCLUSION: This study highlights that pharmacological treatment, if initiated early, may slow down the progression of scoliosis in type II SMA patients. Larger studies are warranted to further investigate the effectiveness of individual pharmacological treatment on scoliosis progression in this patient population.

Vertebral Fractures in Pediatric Suicidal Jumpers: A Retrospective Study with Epidemiological and Clinical Analysis before and after the COVID-19 Pandemic.

BACKGROUND: From the beginning of the COVID-19 pandemic, reports in the literature confirm a significant increase in suicide attempts in children and adolescents. At the Bambino Gesù Pediatric Hospital Emergency Department (Rome, Italy), there was a dramatic increase in suicidal jumpers. Many of these presented vertebral fractures. METHODS: This retrospective study includes all suicidal jumpers with vertebral fractures treated from April 2017 to March 2023. We collected and compared data from three years before to three years after the pandemic, analyzing vertebral fractures. RESULTS: From April 2019 to March 2020, 141 cases of suicide attempt arrived at the emergency department. Five of these were suicidal jumpers without vertebral fractures. From April 2020 to March 2023, 362 cases of suicide were hospitalized and 19 were suicidal jumpers; 12 reported vertebral fractures (mean age 14 years). Seven patients were treated by percutaneous pedicle fixation. Three patients needed an open spinal surgery by posterior approach. One case with cervical fractures was treated by Halo-Vest. CONCLUSIONS: This is the first report that shows a sharp increase in vertebral body fractures due to suicide jumping attempts in children and adolescents. This could be a new epidemiological phenomenon persisting or even increasing over time in the pediatric population as a consequence of the COVID-19 pandemic.

Delayed postoperative cervical spinal cord ischemic lesion after a thoracolumbar fusion for syndromic scoliosis: a case report and systematic review of the literature.

INTRODUCTION: We report a case and a literature review of delayed postoperative cervical spinal cord injury after thoraco-lumbar spine surgery. CASE REPORT: A 13-year-old Prader-Willi Syndrome female was treated by a T3-L5 posterior spine fusion for progressive scoliosis. Intraoperative neuromonitoring and immediate postoperative neurological examination were normal. Sixty hours after surgery, she developed a tetraplegia. The immediate MRI and CT scan of the spine were negative. Two days after, a new MRI revealed an ischemic cervical lesion at the level C5-C6. After 1 week, she gradually improved breathing and motility/sensibility at the extremities. After 4 months of intensive neurologic rehabilitation, the patient improved to ASIA grade D and was discharged. At 1-year follow, the neurologic recovery was nearly completed. METHODS: We performed a systematic review of the literature through PubMed and Embase database focused on delayed postoperative cervical spinal cord lesion after a thoraco-lumbar fusion for spinal deformity. RESULTS: Only 14 cases of neurological injuries at levels above the site of surgery have been previously reported and never in Prader Willy Syndrome. All patients were adolescent and 86,7% were females but no specific risk factors were found. CONCLUSIONS: Delayed postoperative neurological deficit far from the surgical site can be considered a specific subgroup of these rare complication that can occur several hours after spine surgery, regardless of intraoperative complication. Despite the rarity of this complication, clinicians should be aware of it. Perioperative optimization of spinal cord perfusion and close neurological examination in first postoperative days may be helpful to quickly recognize and treat this complication.

How SARS-CoV-2 Pandemic Changed Traumatology and Hospital Setting: An Analysis of 498 Fractured Patients.

BACKGROUND: SARS-CoV-2 pandemic is one of the biggest challenges for many health systems in the world, making lots of them overwhelmed by the enormous pressure to manage patients. We reported our Institutional Experience, with specific aims to describe the distribution and type of treated injuries, and the organizational setup of our hospital. METHODS: Data of fractured patients admitted for surgical treatment in the time frames 9 March 2020-4 May 2020 and 1 March 2019-31 May 2019 were collected and compared. Furthermore, surgery duration and some parameters of effectiveness in health management were compared. RESULTS: A total of 498 patients were included. Mean age significantly lower age in 2019 and femoral fractures were significantly more frequent 2020. Mean surgery time was significantly longer in 2020. Mortality rate difference between the two years was found to be statistically significant. Time interval between diagnosis and surgery and between diagnosis and discharge/decease was significantly lower in 2020. In 2020, no patient admitted with a negative swab turned positive in any of the following tests for SARS-CoV-2. CONCLUSIONS: The COVID-19 pandemic has modified the epidemiology of hospitalized patients for traumatic reasons, leading to an increased admission of older patients with femoral fractures. Nevertheless, our institutional experience showed that an efficient change in the hospital organization, with an improvement of several parameters of effectiveness in health management, led to a null infection rate between patients.

Long-term follow-up of Bologna-Oxford (BOX) total ankle arthroplasty.

PURPOSE: The purpose of this retrospective study was to evaluate the long-term results with a minimum of ten years follow-up of primary Bologna-Oxford (BOX) TAA. METHODS: Between December 2004 and December 2009, 80 patients (82 ankles) underwent a primary BOX TAA performed by a single senior surgeon, expert in foot and ankle surgery. Pain and functional outcomes were analysed using Visual Analogue Scale (VAS) for pain, American Orthopaedic Foot & Ankle Society (AOFAS) scoring system, Foot Functional Index Disability and Pain (FFI-Disability, FFI-Pain) score for comparative analysis. RESULTS: A total of 52 patients (54 implants) in a cohort of 80 (82 implants) were examined at a minimum ten years follow-up. Twenty implants out of 54 underwent implant failure (37 %) and 34 patients were enrolled in the present study. The mean VAS for pain decreased significantly from 8.5 ± 1.2 to 2.9 ± 2.2 (p<0.01) and the mean AFOAS score changed from 28.6 ± 11.8 pre-operatively to 72.7 ± 16.9 (p<0.01) at last follow-up. Ninety-seven percent was satisfied with a mean FFI-Disability score that improved from 77.6 ± 19.3 to 26.7 ± 25.4 (p<0.01) and FFI-Pain score that decreased from 76.2 ± 14.2 to 31.4 ± 25.6 (p<0.01). We calculated post-operative alignment using alpha, beta and gamma angles with no difference at long-term follow-up. The survival rate of the implant was 66% at ten years of follow-up. CONCLUSIONS: Our data suggest that BOX TAA is an implant with a good patient satisfaction rate at long-term follow-up; therefore, it is a valid option to increase the quality of life in subjects with end-stage osteoarthritis; however, long-term survivorship is unsatisfactory when compared to modern knee and hip implant.