Hidden blood loss and bleeding characteristics in children with congenital scoliosis undergoing spinal osteotomies.

PURPOSE: Spinal osteotomies are often essential in the treatment of congenital scoliosis. Risk factors for bleeding in these patients needing extracavitatory approaches, especially hidden blood loss, are sparsely investigated. We aimed to investigate the bleeding characteristics and hidden blood loss in paediatric patients undergoing spinal osteotomies for congenital scoliosis. METHODS: A retrospective analysis identified all patients with congenital scoliosis were retrospectively identified from the prospectively collected spine register from 2010 to 2022. Operative technique, perioperative laboratory results and imaging studies were extracted. The primary outcome was total blood loss including intraoperative, drain output and hidden blood loss. RESULTS: Fifty-seven children (32 boys) with a mean age of 8.3 years underwent spinal osteotomy for congenital scoliosis. Posterolateral hemivertebrectomy was sufficient in 34 (59%) patients, while vertebral column resection (VCR) was required in 23 patients. Total bleeding averaged 792 (523) ml accounting for 42% of the estimated blood volume. Hidden blood loss accounted for 40% of total bleeding and 21% of estimated blood volume with a mean of 317 (256) ml. VCR was associated with greater intraoperative and total bleeding than hemivertebrectomies (p = 0.001 and 0.007, respectively). After adjusting for patient weight and fusion levels, hidden blood loss was larger in hemivertebrectomies (4.18 vs. 1.77 ml/kg/fused level, p = 0.049). In multivariable analysis, intraoperative blood loss was inversely correlated with preoperative erythrocyte levels. Younger age was associated with significantly greater drain, hidden and total blood loss. CONCLUSION: Hidden blood loss constitutes a significant portion (40%) of total bleeding in congenital scoliosis surgery. Younger age is a risk factor for bleeding and the hidden blood loss should be taken into consideration in their perioperative management.

Early Development of Spinal Deformities in Children Severely Affected with Spinal Muscular Atrophy after Gene Therapy with Onasemnogene Abeparvovec-Preliminary Results.

Spinal muscular atrophy (SMA) is a rare genetic disorder, with the most common form being 5q SMA. Survival of children with severe SMA is poor, yet major advances have been made in recent years in pharmaceutical treatment, such as gene-therapy, which has improved patient survival. Therefore, clinical problems, such as the development of spinal deformities in these genetically treated SMA children represent an unknown challenge in clinical work. In a retrospective case series, the development of spinal deformities was analyzed in 16 SMA children (9 male, 7 female) treated with onasemnogene abeparvovec in two institutions during the years 2020 to 2022. Ten out of sixteen patients had a significant kyphosis, and nine out of sixteen patients had significant scoliosis, with the mean curvature angles of 24 ± 27° for scoliosis, and 69 ± 15° for kyphosis. Based on these preliminary data, it can be assumed that early-onset kyphosis presents a clinical challenge in gene-therapy-treated SMA children. Larger datasets with longer follow-up times need to be collected in order to verify these preliminary observations.

Health-related quality of life after segmental pedicle screw instrumentation: a matched comparison of patients with neuromuscular and adolescent idiopathic scoliosis.

BACKGROUND AND PURPOSE: Progressive neuromuscular scoliosis (NMS) often requires a long instrumented spinal fusion to improve health-related quality of life (HRQoL) and sitting balance. Segmental pedicle screw instrumentation improves HRQoL in patients with adolescent idiopathic scoliosis (AIS), but data on NMS is limited. We aimed to assess the impact of spinal fusion on HRQoL in NMS patients. PATIENTS AND METHODS: We conducted a retrospective case-control study with prospective data collection of NMS patients undergoing posterior spinal fusion at a tertiary level hospital in 2009-2021. 2 controls with AIS matched for sex and age were selected for each NMS patient. The Scoliosis Research Society-24 (SRS-24) questionnaire was utilized for pre- and postoperative HRQoL assessment. Follow-up time was a minimum of 2 years. RESULTS: 60 NMS and 120 AIS patients were included in the analysis, and the mean age (SD) at operation was 14.6 (2.7) in NMS and 15.7 (2.5) in AIS groups. Total SRS score and all domains showed a significant improvement in NMS patients (p < 0.05). Total SRS score improved more (p < 0.001), while pain score improved less (p = 0.04) in NMS (change [95% CI], 0.31 [0.05-0.58] and 0.55 [0.27-0.81]) compared with AIS (0.01 [-0.10 to 0.12] and 0.88 [0.74-1.03]). Postoperative self-image was significantly better in NMS than in AIS at 2-year follow up (p = 0.01). Pelvic instrumentation reduced improvements in the SRS domains. CONCLUSION: HRQoL in NMS patients improved significantly after spinal fusion, and these benefits are comparable to those of AIS patients.

Scoliosis Treatment With Growth-Friendly Spinal Implants (GFSI) Relates to Low Bone Mineral Mass in Children With Spinal Muscular Atrophy.

BACKGROUND: Children with spinal muscular atrophy (SMA) frequently develop neuromuscular scoliosis at an early age, requiring surgical treatment with growth-friendly spinal implants (GFSI), such as magnetically controlled growing rods. This study investigated the effect of GFSI on the volumetric bone mineral density (vBMD) of the spine in SMA children. METHODS: Seventeen children (age 13.2±1.2 y) with SMA and GFSI-treated spinal deformity were compared with 25 scoliotic SMA children (age 12.9±1.7 y) without prior surgical treatment as well as age-matched healthy controls (n=29; age 13.3±2.0). Clinical, radiologic, and demographic data were analyzed. For the calculation of the vBMD Z-scores of the thoracic and lumbar vertebrae, phantom precalibrated spinal computed tomography scans were analyzed using quantitative computed tomography (QCT). RESULTS: Average vBMD was lower in SMA patients with GFSI (82.1±8.4 mg/cm 3) compared with those without prior treatment (108.0±6.8 mg/cm 3 ). The difference was more prominent in and around the thoracolumbar region. The vBMD of all SMA patients was significantly lower in comparison with healthy controls, especially in SMA patients with previous fragility fractures. CONCLUSIONS: The results of this study support the hypothesis of reduced vertebral bone mineral mass in SMA children with scoliosis at the end of GFSI treatment in comparison with SMA patients undergoing primary spinal fusion. Improving vBMD through pharmaceutical therapy in SMA patients could have a beneficial effect on the surgical outcome of scoliosis correction while reducing complications. LEVEL OF EVIDENCE: Therapeutic Level III.

Perioperative Risk Factors for Bleeding in Adolescents Undergoing Pedicle Screw Instrumentation for Scoliosis.

Progressive scoliosis eventually leads to extensive spinal fusion surgery, which carries a risk for significant bleeding. Neuromuscular scoliosis (NMS) patients have an additional inherent risk of major perioperative bleeding. The purpose of our research was to investigate the risk factors for measured (intraoperative, drain output) and hidden blood loss related to pedicle screw instrumentation in adolescents, divided into adolescent idiopathic scoliosis (AIS) and NMS patient groups. A retrospective cohort study with prospectively collected data of consecutive AIS and NMS patients undergoing segmental pedicle screw instrumentation at a tertiary level hospital between 2009 and 2021 was conducted. In total, 199 AIS (mean age 15.8 years, 143 females) and 81 NMS patients (mean age 15.2 years, 37 females) were included in the analysis. In both groups, levels fused, increased operative time, and smaller or larger size of erythrocytes were associated with perioperative blood loss (p < 0.05 for all correlations). In AIS, male sex (p < 0.001) and the number of osteotomies correlated with more drain output. In NMS, levels fused correlated with drain output, p = 0.00180. In AIS, patients' lower preoperative MCV levels (p = 0.0391) and longer operation times, p = 0.0038, resulted into more hidden blood loss, but we did not find any significant risk factors for hidden blood loss in NMS patients.

A retrospective cohort study of bleeding characteristics and hidden blood loss after segmental pedicle screw instrumentation in neuromuscular scoliosis as compared with adolescent idiopathic scoliosis.

Background: Progressive scoliosis in neuromuscular patients often requires a long instrumented spinal fusion. Previous studies have shown larger intraoperative blood loss in these patients than those with adolescent idiopathic scoliosis (AIS), but the total blood loss composed of visible and hidden blood loss has not been described in this patient population. The aim of our study was to investigate the bleeding characteristics and hidden blood loss related to spinal fusion in neuromuscular scoliosis (NMS) as compared to AIS patients. Methods: A retrospective cohort study with prospective data collection of NMS patients undergoing segmental pedicle screw instrumentation at a university hospital between 2009 and 2021. A comprehensive statistical analysis was performed regarding intra- and postoperative blood loss compared to patient characteristics such as age and diagnosis. Hidden blood loss was estimated and compared to the total blood loss. We standardized amount of bleeding with patient weight and fusion level for further analysis. Consecutive AIS patients served as a control population. Eighty-one consecutive patients with NMS (mean age 15.2 years, 37 females) were included and their bleeding characters were compared with 199 AIS patients (mean age 15.8 years, 143 females). The primary outcome was total blood loss including intraoperative, 24-hour drain output and hidden blood loss. Secondary outcome measures included requirement for blood transfusion. Results: Neuromuscular patients had a significantly larger total blood loss with mean values of 1914 mL in the NMS and 1357 mL in the AIS groups, respectively (p<0.001). The hidden blood loss was also significantly larger in NMS than in AIS group (566 ± 533 mL vs. 398 ±411 mL, p=0.0332). Neuromuscular scoliosis was also associated with significantly greater weight and levels of fused adjusted intraoperative bleeding (1.79 ml/kg/fused level in NMS vs. 0.87 ml/kg/fused level in AIS vs. p< 0.0001) and hidden blood loss (1.00 ml/kg/levels fused vs. 0.65 ml/kg/fused levels, p=0.053). NMS diagnosis was also a risk factor for greater hidden blood loss in multivariable analysis (p=0.0011). 24-hour drain output was similar in the NMS and AIS groups. Male gender was a risk factor for greater hidden blood loss in the NMS group (p=0.0429). Fifty-nine (73%) NMS and 27 (14%) AIS patients received allogenic red blood cell infusions perioperatively (p<0.001). Conclusions: Hidden blood loss constitutes one-third of total blood loss in children undergoing segmental pedicle screw instrumentation for neuromuscular scoliosis. Hidden blood loss is significantly greater in the neuromuscular as compared with idiopathic scoliosis. Hidden blood loss should be considered in the perioperative management of NMS.

Distal Femoral Focal Deficiency.

Distal femoral focal deficiency is an extremely rare type of congenital femoral deficiency that comprises hypoplasia of the distal femur, with a normally developed hip. We represent a unique case of distal femoral hypoplasia and deficiency of knee extensors, childhood follow-up and final treatment with exarticulation, and a comparison with previous literature.

Treatment of congenital bipartite patella in pediatric population - a systematic review of the published studies.

Bipartite patella is an abnormality where patella is incorrectly ossified resulting in fibrocartilaginous synchondrosis. In most cases bipartite patella is asymptomatic and incidental finding but can also be painful especially in adolescents. The aim of our analysis was to determine the preferable treatment for pediatric bipartite patella. We conducted a systematic review based on PubMed and Embase searches. All original articles with reference to treatment in the population of ≤ 20 years of age, were included. Case reports, and articles without individualized data were excluded. In total, the individual data of 126 knees in 116 patients was analyzed. In 77.8% (98/126) of the analyzed knees the result was excellent, and the majority of the knees, 89.7% (105/126) received surgical intervention, and seven different techniques were used. None of operative methods were superior to others. Excellent treatment outcomes were achieved more often in younger patients (p=0.004), and the median duration of symptoms in operatively treated patients was shorter (p=0.018). If operative treatment is used after unsuccessful conservative treatment, it should be considered early in adolescence and also within a relatively short period after the onset of symptoms. The best operative method could not be found. More research and RCTs are needed concerning the conservative methods and different operative techniques.

S-ketamine in patient-controlled analgesia reduces opioid consumption in a dose-dependent manner after major lumbar fusion surgery: A randomized, double-blind, placebo-controlled clinical trial.

BACKGROUND: Spinal fusion surgery causes severe pain. Strong opioids, commonly used as postoperative analgesics, may have unwanted side effects. S-ketamine may be an effective analgesic adjuvant in opioid patient-controlled analgesia (PCA). However, the optimal adjunct S-ketamine dose to reduce postoperative opioid consumption is still unknown. METHODS: We randomized 107 patients at two tertiary hospitals in a double-blinded, placebo-controlled clinical trial of adults undergoing major lumbar spinal fusion surgery. Patients were randomly allocated to four groups in order to compare the effects of three different doses of adjunct S-ketamine (0.25, 0.5, and 0.75 mg ml-1) or placebo on postoperative analgesia in oxycodone PCA. Study drugs were administered for 24 hours postoperative after which oxycodone-PCA was continued for further 48 hours. Our primary outcome was cumulative oxycodone consumption at 24 hours after surgery. RESULTS: Of the 100 patients analyzed, patients receiving 0.75 mg ml-1 S-ketamine in oxycodone PCA needed 25% less oxycodone at 24 h postoperatively (61.2 mg) compared with patients receiving 0.5 mg ml-1 (74.7 mg) or 0.25 mg ml-1 (74.1 mg) S-ketamine in oxycodone or oxycodone alone (81.9 mg) (mean difference: -20.6 mg; 95% confidence interval [CI]: -41 to -0.20; P = 0.048). A beneficial effect in mean change of pain intensity at rest was seen in the group receiving 0.75 mg ml-1 S-ketamine in oxycodone PCA compared with patients receiving lower ketamine doses or oxycodone alone (standardized effect size: 0.17, 95% CI: 0.013-0.32, P = 0.033). The occurrence of adverse events was similar among the groups. CONCLUSIONS: Oxycodone PCA containing S-ketamine as an adjunct at a ratio of 1: 0.75 decreased cumulative oxycodone consumption at 24 h after major lumbar spinal fusion surgery without additional adverse effects.