Maternal risk factors for congenital vertebral formation and mixed defects: A population-based case-control study.

Background: The etiology and risk factors of congenital vertebral anomalies are mainly unclear in isolated cases. Also, there are no reports on the risk factors for different subgroups of vertebral anomalies. Therefore, we assessed and identified potential maternal risk factors for these anomalies and hypothesized that diabetes, other chronic diseases, smoking, obesity, and medication in early pregnancy would increase the risk of congenital vertebral anomalies. Methods: All cases with congenital vertebral anomalies were identified in the Finnish Register of Congenital Malformations from 1997 to 2016 for this nationwide register-based case-control study. Five matched controls without vertebral malformations were randomly selected. Analyzed maternal risk factors included maternal age, body mass index, parity, smoking, history of miscarriages, chronic diseases, and prescription drug purchases in early pregnancy. Results: The register search identified 256 cases with congenital vertebral malformations. After excluding 66 syndromic cases, 190 non-syndromic malformations (74 formation defects, 4 segmentation defects, and 112 mixed anomalies) were included in the study. Maternal smoking was a significant risk factor for formation defects (adjusted odds ratio 2.33, 95% confidence interval 1.21-4.47). Also, pregestational diabetes (adjusted odds ratio 8.53, 95% confidence interval 2.33-31.20) and rheumatoid arthritis (adjusted odds ratio 13.19, 95% confidence interval 1.31-132.95) were associated with mixed vertebral anomalies. Conclusion: Maternal pregestational diabetes and rheumatoid arthritis were associated with an increased risk of mixed vertebral anomalies. Maternal smoking increases the risk of formation defects and represents an avoidable risk factor for congenital scoliosis. Level of evidence: III.

DUODENAL ATRESIA IN FINLAND FROM 2004 to 2017: PREVALENCE, MORTALITY AND ASSOCIATED ANOMALIES A population-based study.

Introduction Duodenal atresia (DA) is the most common atresia of the small bowel. This study aims to assess the prevalence, mortality, and associated anomalies related to DA in Finland during 2004-2017. Material and Methods A Nationwide study based on registers maintained by the Finnish Institute for Health and Welfare and Statistics Finland containing data on all live births and stillbirths and terminations of pregnancy. The cases were identified based on the ICD-9 and 10 codes. Associated anomalies were classified based on the EUROCAT criteria; minor anomalies were excluded. Results There were 249 DA cases including 222 (89.2%) live births, 16 (6.4%) stillbirths, and 11 (4.4%) terminations. There was no significant change in the prevalence rates between 2004 and 2017. Live birth prevalence was 2.75/10000 and total prevalence 3.08/10000 births. One hundred (40.2%) cases were isolated, 67 (26.9%) had other major congenital anomalies and 83 (33.3%) were syndromic. There were no terminations in isolated DA. Most associated anomalies were cardiac (36.1%), followed by other gastrointestinal tract anomalies (23.7%) and limb deformities/defects (7.2%). Trisomy 21 was observed in 63 cases (25.3%). Neonatal mortality was 3.6% (n=8) and at one year 95.0% were alive. Both neonatal and infant mortality were associated with cardiac anomalies (p<0.001 and p=0.001 respectively). All neonatal deaths had associated cardiac defect(s). Conclusions The prevalence of DA in Finland remains stable and among the highest reported. DA is often associated with cardiac anomalies which portend high risk for mortality. Despite the burden of associated anomalies, overall survival is high.

Pulmonary Function at Minimum 10 Years After Segmental Pedicle Screw Instrumentation for Thoracic Adolescent Idiopathic Scoliosis.

STUDY DESIGN: A prospective multi-center cohort study. OBJECTIVE: This study evaluates the 10-year pulmonary function outcomes for patients with structural thoracic adolescent idiopathic scoliosis (Lenke curve types 1-4, 6) after segmental pedicle screw instrumentation. SUMMARY OF BACKGROUND DATA: Adolescent idiopathic scoliosis with thoracic curves is associated with reduced pulmonary function preoperatively. It remains unclear how much pulmonary improvement can be obtained using pedicle screw instrumentation at long-term follow-up. METHODS: Out of 64 consecutively surgically treated patients with thoracic AIS (Lenke 1-4, 6) using pedicle screw instrumentation, 50 (mean age at final FU 14.7 ± 1.9, 44 females) participated in a prospective 10-year follow-up study. They were evaluated using clinical examination, spinal radiographs, and spirometry preoperatively and at 10-year follow-up. The preoperative percentage predicted values were adjusted for the height loss caused by the scoliosis according to curve size. RESULTS: Preoperatively 49% (20/41) had forced vital capacity (FVC) or forced expiratory volume in one second (FEV1) below 80% of the predicted normal values representing pulmonary function impairment. Major curve correction averaged 76% (SD 9%). FVC improved from preoperative 3.29 L (SD 0.78L) to 3.87 L (0.79L) at 10-year follow-up (P<0.001). This improvement averaged 510 mL (SD 560 mL) in patients having both preoperative and 10-year follow-up measurements available. The percentage predicted values for FVC showed an improvement from 83% preoperatively to 86% at 10-year follow-up (P=0.048). At 10-year follow-up 38% (19/50) of the patients had FVC or FEV1 below 80% of the predicted values. CONCLUSIONS: FVC improved by a mean of 510 ml from preoperative to 10-year follow-up in patients undergoing pedicle screw instrumentation for thoracic AIS. Despite 76% scoliosis correction and significant improvement of absolute lung volume values, more than one-third of these surgically treated otherwise healthy young adults fulfilled the criteria for pulmonary function impairment at 10-year follow-up. LEVEL OF EVIDENCE: II.

Reliability and validity of the adapted Finnish version of the early onset scoliosis questionnaire (EOSQ-24).

BACKGROUND: EOSQ-24 is a disease specific patient-reported outcome score used to assess the quality of life in patients with early-onset scoliosis. The aim of this study was to translate and cross-culturally adapt the English version of the EOSQ-24 to Finnish language and to assess the reliability and validity of the translation. METHODS: Cross-cultural adaptation and cross-cultural validation were performed to the Finnish translation of the EOSQ-24. Patients and/or their caretakers were then recruited to assess the psychometric properties of the translation. We assessed the internal consistency, test-retest reliability, floor and ceiling effects, and discriminative abilities. One-hundred-and-three patients filled the questionnaire. RESULTS: EOSQ-24 was successfully translated into Finnish. The translation showed excellent internal consistency (Cronbach alpha 0.94), satisfactory item-total correlations ranging from 0.6 to 0.9, and moderate to strong inter item correlations. Test-retest reliability ranged from 0.7 to 0.96 indicating good to excellent agreement. Patients with neuromuscular and syndromic scoliosis reported lower EOSQ-24 scores when compared to patients' idiopathic and congenital scoliosis. There was a significant negative correlation between major curve and EOSQ-24 scores in patients with idiopathic early onset scoliosis. CONCLUSION: The internal consistency and test-retest reliability of the measure were found to be satisfactory. A marked ceiling effect was observed, indicating a potential source of error.

Utility of brain imaging in pediatric patients with a suspected accidental spinal injury but no brain injury-related symptoms.

PURPOSE: Imaging is the gold standard in diagnosing traumatic brain injury, but unnecessary scans should be avoided, especially in children and adolescents. Clinical decision-making rules often help to distinguish the patients who need imaging, but if spinal trauma is suspected, concomitant brain imaging is often conducted. Whether the co-occurrence of brain and spine injuries is high enough to justify head imaging in patients without symptoms suggesting brain injury is unknown. OBJECTIVE: This study aims to assess the diagnostic yield of brain MRI in pediatric patients with suspected or confirmed accidental spinal trauma but no potential brain injury symptoms. METHODS: We retrospectively reviewed the medical and imaging data of pediatric patients (under 18 years old) who have undergone concomitant MRI of the brain and spine because of acute spinal trauma in our emergency radiology department over a period of 8 years. We compared the brain MRI findings in patients with and without symptoms suggesting brain injury and contrasted spine and brain MRI findings. RESULTS: Of 179 patients (mean age 11.7 years, range 0-17), 137 had symptoms or clinical findings suggesting brain injury, and 42 did not. None of the patients without potential brain injury symptoms had traumatic findings in brain MRI. This finding also applied to patients with high-energy trauma (n = 47) and was unrelated to spinal MRI findings. CONCLUSION: Pediatric accidental trauma patients with suspected or confirmed spine trauma but no symptoms or clinical findings suggesting brain injury seem not to benefit from brain imaging.

Outcomes of Follow-up Imaging After Pediatric Spinal Trauma Confirmed With Magnetic Resonance Imaging.

BACKGROUND: Imaging plays a crucial role in the diagnostic workup of pediatric spinal trauma. Computed tomography and conventional radiographs are widely used as the primary imaging methods. Magnetic resonance imaging (MRI) is a radiation-free alternative with high sensitivity for bony and soft tissue injuries. There is no consensus on the optimal use of follow-up imaging in pediatric spinal trauma without immediate surgical treatment, especially if the injury is primarily confirmed with MRI. This study aimed to assess the diagnostic value of follow-up imaging after MRI-confirmed spinal trauma in children. METHODS: The medical records and the imaging data of children and adolescents with emergency spinal MRI and follow-up imaging over 8 years were retrospectively reviewed. The primary study outcome was the outcome of follow-up imaging and its effect on management. RESULTS: The study population consisted of 127 patients. The follow-up imaging did not alter the management in any patient with presumably stable injury in emergency MRI. Short-term follow-up imaging showed no clinically significant progression in thoracolumbar compression fractures. Flexion-extension radiographs had no additional value in cases with stable cervical spinal injury on emergency MRI. CONCLUSIONS: The clinical utility of short-term follow-up imaging is low in children with stable spinal injury on emergency MRI. LEVEL OF EVIDENCE: Level III-retrospective observational study.

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.

Emergency MRI in Spine Trauma of Children and Adolescents-A Pictorial Review.

Severe spinal trauma is uncommon in the pediatric population, but due to the potentially devastating consequences of missed injury, it poses a diagnostic challenge in emergency departments. Diagnostic imaging is often needed to exclude or confirm the injury and to assess its extent. Magnetic resonance imaging (MRI) offers an excellent view of both bony and soft tissue structures and their traumatic findings without exposing children to ionizing radiation. Our pictorial review aims to demonstrate the typical traumatic findings, physiological phenomena, and potential pitfalls of emergency MRI in the trauma of the growing spine.

Clinical outcome following magnetic resonance imaging as first-line imaging in low-impact pediatric spine trauma: a single-center retrospective observational study.

BACKGROUND: Pediatric spinal trauma is rare, but the consequences of a missed injury can be devastating. Medical imaging is often needed in addition to physical examination. Conventional radiographs are widely recommended, but their negative predictive value is limited. Computed tomography (CT) is more sensitive but has a higher radiation dose. Magnetic resonance imaging (MRI) has superior soft tissue contrast and lacks ionizing radiation, but it is more expensive and time-consuming. Thus, the debate regarding the most suitable imaging method is still ongoing. OBJECTIVE: This study examined the ability of MRI to exclude injuries requiring surgical treatment as a first-line imaging method in low-impact pediatric spine trauma. MATERIALS AND METHODS: We retrospectively reviewed the medical records and imaging data of children (under 18 years old) who had suspected spinal trauma and were scanned using MRI in our emergency radiology department over a period of 8 years. We assessed the ability of MRI to detect unstable injuries by searching for later occurrences of primarily missed injuries requiring surgery. RESULTS: Of 396 patients (median age 12 years, range 0-17), 114 (29%) had MRI findings suggesting an acute injury. Bony injuries were detected in 78 patients (20%) and ligamentous or other soft tissue injuries in 82 patients (21%). In the subgroup of 376 patients (median age 12 years, range 0-17) with at least 6 months of clinical follow-up, no missed injuries demanding surgical intervention or immobilization occurred after spinal MRI as  first-line imaging. No adverse events related to MRI or anesthesia occurred. CONCLUSION: MRI can detect injuries requiring surgical treatment as a first-line imaging method in suspected low-impact pediatric spinal trauma and is safe to use in this selected population.

Association Between Lenke Classification, The Extent of Lumbar Spinal Fusion, and Health-Related Quality of Life After Instrumented Spinal Fusion for Adolescent Idiopathic Scoliosis.

STUDY DESIGN: Prospective cohort study. SUMMARY OF BACKGROUND DATA: Lenke classification is used to define the curve type in adolescent idiopathic scoliosis (AIS). The association of Lenke classification and long-term postoperative health-related quality of life (HRQoL) remains unclear. OBJECTIVE: The purpose of this study was to assess the association between Lenke classification and HRQoL in patients who underwent spinal fusion for AIS. MATERIALS AND METHODS: In all, 146 consecutive patients (mean age 15.1 yr) operated for AIS between 2007 and 2019 with a minimum 2-year follow-up were included. Fifty-three (36%) patients reached the 10-year follow-up. Their HRQoL was assessed with the SRS-24 questionnaire preoperatively, at six months, two years, and 10 years after surgery. RESULTS: The preoperative major curve was the largest in Lenke 3 (mean 63 ° ) and 4 (mean 62 ° ) groups and the lowest in Lenke 5 groups (mean 48 ° , P <0.05). These curves were corrected to a mean of 15 ° with no differences between groups. We found no evidence of differences between the preoperative HRQoL scores between the Lenke groups. The self-image domain of SRS-24 was lower in patients with isolated major thoracolumbar scoliosis (Lenke 5) when compared with double-thoracic (Lenke 2) group at the two-year follow-up (mean [95% CI] 3.6 [3.3-3.9] vs. 4.3 [4.1-4.6]). The postoperative satisfaction domain was lower in Lenke 5 group when compared with main thoracic (Lenke 1) group (mean [95% CI] 3.8 [3.5-4.0] vs. 4.3 [4.2-4.5]) and Lenke 2 group (mean 4.4, 95% CI 4.2-4.6) at the two-year follow-up. The mean total score of SRS-24 at the 10-year follow-up was highest in Lenke 1 group (mean 4.06, 95% CI 3.79-4.33) and lowest in Lenke 6 group (mean 2.92, 95% CI 2.22-3.61). CONCLUSIONS: Lenke classification and especially its curve type (major thoracic vs. major thoracolumbar scoliosis) was associated with long-term health-related quality of life after instrumented spinal fusion for AIS.

Maternal Risk Factors for Congenital Vertebral Anomalies: A Population-Based Study.

BACKGROUND: The spectrum of congenital vertebral defects varies from benign lesions to severe, life-threatening conditions. The etiology and maternal risk factors remain mainly unclear in isolated cases. Hence, we aimed to assess and identify potential maternal risk factors for these anomalies. Based on previous studies, we hypothesized that maternal diabetes, smoking, advanced maternal age, obesity, chronic diseases, and medication used during the first trimester of pregnancy might increase the risk of congenital vertebral malformations. METHODS: We performed a nationwide register-based case-control study. All cases with vertebral anomalies (including live births, stillbirths, and terminations for fetal anomaly) were identified in the Finnish Register of Congenital Malformations from 1997 to 2016. Five matched controls from the same geographic region were randomly selected for each case. Analyzed maternal risk factors included age, body mass index (BMI), parity, smoking, history of miscarriages, chronic diseases, and prescription drugs dispensed during the first trimester of pregnancy. RESULTS: In total, 256 cases with diagnosed congenital vertebral anomalies were identified. After excluding 66 malformations associated with known syndromes, 190 nonsyndromic malformation cases were included. These were compared with 950 matched controls. Maternal pregestational diabetes was a significant risk factor for congenital vertebral anomalies (adjusted odds ratio [OR], 7.30 [95% confidence interval (CI), 2.53 to 21.09). Also, rheumatoid arthritis (adjusted OR, 22.91 [95% CI, 2.67 to 196.40]), estrogens (adjusted OR, 5.30 [95% CI, 1.57 to 17.8]), and heparins (adjusted OR, 8.94 [95% CI, 1.38 to 57.9]) were associated with elevated risk. In a sensitivity analysis using imputation, maternal smoking was also significantly associated with an elevated risk (adjusted OR, 1.57 [95% CI, 1.05 to 2.34]). CONCLUSIONS: Maternal pregestational diabetes and rheumatoid arthritis increased the risk of congenital vertebral anomalies. Also, estrogens and heparins, both of which are frequently used in assisted reproductive technologies, were associated with an increased risk. Sensitivity analysis suggested an increased risk of vertebral anomalies with maternal smoking, warranting further studies. LEVEL OF EVIDENCE: Prognostic Level III . See Instructions for Authors for a complete description of levels of evidence.

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.

Surgical and Health-related Quality of life Outcomes in Children With Congenital Scoliosis During 5-year Follow-up. Comparison to Age and Sex-matched Healthy Controls.

BACKGROUND: Congenital spinal anomalies represent a heterogeneous group of spinal deformities, of which only progressive or severe curves warrant surgical management. Only a limited number of studies have investigated the impact of surgery on the health-related quality of life and very limited data exists comparing these outcomes to healthy controls. METHODS: A single surgeon series of 67 consecutive children with congenital scoliosis (mean age at surgery 8.0 y, range: 1.0 to 18.3 y, 28 girls) undergoing hemivertebrectomy (n = 34), instrumented spinal fusion (n = 20), or vertical expandable prosthetic titanium rib procedure (n = 13) with a mean follow-up of 5.8 years (range: 2 to 13 y). The comparison was made to age and sex-matched healthy controls. Outcome measures included the Scoliosis Research Society questionnaire both pre and postoperatively, radiographic outcomes, and complications. RESULTS: The average major curve correction was significantly better in the hemivertebrectomy (60%) and instrumented spinal fusion (51%) than in the vertical expandable prosthetic titanium rib group (24%), respectively ( P < 0.001). Complications were noted in 8 of 67 (12%) children, but all patients recovered fully during follow-up. Pain, self-image, and function domains improved numerically from preoperative to final follow-up, but the pain score was the only one with a statistically significant change ( P = 0.033). The Scoliosis Research Society pain, self-image, and function domain scores remained at a significantly lower level at the final follow-up than in the healthy controls ( P ≤ 0.05), while activity scores improved to a similar level. CONCLUSIONS: Surgery for congenital scoliosis improved angular spinal deformities with a reasonable risk of complications. Health-related quality of life outcomes improved from preoperative to final follow-up, but especially pain and function domains remained at a significantly lower level than in the age and sex-matched healthy controls. LEVEL OF EVIDENCE: Level III, therapeutic.

Back Pain and Quality of Life 10 Years After Segmental Pedicle Screw Instrumentation for Adolescent Idiopathic Scoliosis.

STUDY DESIGN: Comparative cohort study. OBJECTIVE: The aim of the present study was to evaluate pain and health-related quality of life (HRQoL) in surgically managed patients with a minimum follow-up of 10 years compared with patients with untreated adolescent idiopathic scoliosis (AIS) and a healthy control group. SUMMARY OF BACKGROUND DATA: Posterior spinal fusion with pedicle screws is the standard treatment for AIS, although it remains unclear whether this procedure results in improved long-term HRQoL compared with untreated patients with AIS. PATIENTS AND METHODS: Sixty-four consecutive patients at a minimum follow-up of 10 years, who underwent posterior pedicle screw instrumentation for AIS were prospectively enrolled. Fifty-three (83%) of these patients completed Scoliosis Research Society (SRS) 24 questionnaires, clinical examination, and standing spinal radiographs. Pain and HRQoL were compared with age and sex-matched patients with untreated AIS and healthy individuals. RESULTS: The mean major curve was 57° preoperatively and 15° at the 10-year follow-up. SRS-24 self-image domain score showed a significant improvement from preoperative to 2 years and remained significantly better at the 10-year follow-up ( P < 0.001). Patients fused to L3 or below had lower pain, satisfaction, and total score than patients fused to L2 or above ( P < 0.05), but self-image, function, and activity scores did not differ between groups at 10-year follow-up. Pain, self-image, general activity, and total SRS domains were significantly better at 10-year follow-up in the surgically treated patients as compared with untreated patients (all P < 0.05). Healthy controls had significantly higher total score s than those surgically treated at 10-year follow-ups ( P < 0.001). CONCLUSION: Patients undergoing segmental pedicle screw instrumentation for AIS maintain high-level HRQoL during a 10-year follow-up. Their HRQoL was significantly better than in the untreated patients with AIS, except for the function domain. However, HRQoL remained at a lower level than in healthy controls.

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.

Allograft Versus Bioactive Glass (BG-S53P4) in Pediatric Benign Bone Lesions: A Randomized Clinical Trial.

BACKGROUND: Benign bone cysts in children have a high risk of recurrence after bone grafting. The optimal treatment and filling material for these lesions are currently unknown. METHODS: We compared cyst recurrence after intralesional curettage and filling with allograft versus bioactive glass (BG-S53P4; Bonalive) in a randomized clinical trial. The volume of recurrent cyst at 2-year follow-up was the primary outcome. RESULTS: Of 64 eligible children, 51 (mean age, 11.1 years) were randomized to undergo filling of the cyst using morselized allograft (26) or bioactive glass (25). Twelve (46%) of the children in the allograft group and 10 (40%) in the bioactive glass group developed a recurrence (odds ratio [OR] for bioactive glass = 0.79, 95% confidence interval [CI] = 0.25 to 2.56, p = 0.77). The size of the recurrent cyst did not differ between the allograft group (mean, 3.3 mL; range, 0 to 13.2 mL) and the bioactive glass group (mean, 2.2 mL; range, 0 to 16.6 mL, p = 0.43). After adjusting for the type of lesion (aneurysmal bone cyst versus other), bioactive glass also did not prevent larger (>1 mL) recurrent cysts (adjusted OR = 0.42, 95% CI = 0.13 to 1.40, p = 0.16). The Musculoskeletal Tumor Society score improved significantly (p ≤ 0.013) from preoperatively to the 2-year follow-up in both groups (to 28.7 for bioactive glass and 29.1 for bone graft). Four (15%) of the children in the allograft group and 6 (24%) in the bioactive glass group required a reoperation during the follow-up (OR for bioactive glass = 1.74, 95% CI = 0.43 to 7.09, p = 0.50). CONCLUSIONS: Filling with bioactive glass and with allograft in the treatment of benign bone lesions provided comparable results in terms of recurrence and complications. LEVEL OF EVIDENCE: Therapeutic Level I . See Instructions for Authors for a complete description of levels of evidence.

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.

Amelia and phocomelia in Finland: Characteristics and prevalences in a nationwide population-based study.

BACKGROUND: Amelia and phocomelia represent severe limb reduction defects. Specific epidemiologic data on these defects are scarce. We conducted a descriptive analysis of prevalence data in Finland during 1993-2008 to clarify the epidemiology nationwide in a population-based register study. We hypothesized that increasing maternal age would affect the total prevalence of each disorder. MATERIALS AND METHODS: We collected information on all fetuses and infants affected by amelia and phocomelia during 1993-2008 from the National Register of Congenital Malformations in Finland. The clinical, laboratory, autopsy, and imaging data were re-evaluated where available for all cases found. RESULTS: A total of 23 amelia and 7 phocomelia patients were identified. Thalidomide was not an etiological factor in any of the cases. The total prevalence of amelia was 2.43 per 100,000 births. The live birth prevalence was 0.63 per 100,000 live births. The total prevalence of phocomelia was 0.74 per 100,000 births, and the live birth prevalence was 0.53 per 100,000 live births. Infant mortality in amelia and phocomelia was 67% and 60%, respectively. CONCLUSIONS: Infant mortality is high among amelia and phocomelia. Most cases had other major associated anomalies, but syndromic amelia cases were rare. Total prevalences were higher than previously reported and showed an increase in prevalence toward the end of the study period. The percentage of elective terminations of pregnancy for these disorders is high. While isolated cases are rare, they most likely present a better prognosis. Thus, correct diagnosis is essential in counseling for possible elective termination.

Beam-Like rods do not Provide Additional Improvement to Thoracic Kyphosis Restoration when Compared to Sagittal Reinforced rods in Adolescents Undergoing Spinal Fusion with Pedicle Screw Instrumentation for Idiopathic Scoliosis.

OBJECTIVE: Operative treatment of adolescent idiopathic scoliosis (AIS) with posterior spinal fusion aims for three-dimensional correction of coronal curve and thoracic kyphosis. Our aim was to compare two different designs of asymmetrical rods in adolescents who underwent a posterior spinal fusion with pedicle screw instrumentation for AIS with an emphasis on thoracic kyphosis restoration. METHODS: This study was made with 76 consecutive adolescents (mean age 15.6 years, SD 2.0). Thirty-nine patients were operated with sagittal reinforced rods and 37 patients were operated with beam-like rods. The clinical and radiological results were assessed preoperatively, postoperatively, and during the follow-up visits at the outpatient clinic 6 months and 2 years after the surgery. RESULTS: At the last follow-up, the mean (SD) major thoracic curves were 13° (6.2°) and 13° (6.0°) (P = 0.717). Correction percentages were 75% in the sagittal reinforced group and 73% in the beam-like rod group (P = 0.517). The mean (SD) thoracic kyphosis was 24° (11°) and 22° (7.8°) at the two year follow-up in the sagittal reinforced rod group and beamlike rod group (P = 0.517). There was a slight negative correlation between the major curve correction and thoracic kyphosis change in both groups, although this was not statistically significant (R = -0.19, P = 0.094 in the sagittal reinforced rod group, R=-0.16, P = 0.180 in the beam like rod group). CONCLUSIONS: There are no significant differences in the coronal or sagittal deformity restoration in adolescent patients who underwent a posterior spinal fusion with sagittal reinforced rods and beam-like rods for adolescent idiopathic scoliosis.

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.