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 from 2004 to 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 (International Classification of Diseases revisions 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 2.75/10,000 and total prevalence was 3.08/10,000 births. A total of 100 (40.2%) cases were isolated, 67 (26.9%) had other multiple 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 1 year 95.0% were alive. Both neonatal and infant mortalities 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 a 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.

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.

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.

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.

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.

Intraoperative 3D Imaging Reduces Pedicle Screw Related Complications and Reoperations in Adolescents Undergoing Posterior Spinal Fusion for Idiopathic Scoliosis: A Retrospective Study.

Widely used surgical treatment for adolescent idiopathic scoliosis (AIS) is posterior spinal fusion using pedicle screw instrumentation (PSI). Two-dimensional (2D) or three-dimensional (3D) navigation is used to track the screw positioning during surgery. In this study, we evaluated the screw misplacement, complications, and need for reoperations of intraoperative 3D as compared to 2D imaging in AIS patients. There were 198 adolescents, of which 101 (51%) were evaluated with 2D imaging and 97 (49%) with 3D imaging. Outcome parameters included radiographic correction, health-related quality of life (HRQOL), complications, and reoperations. The mean age was 15.5 (SD 2.1) years at the time of the surgery. Forty-four (45%) patients in the 3D group and 13 (13%) patients in the 2D group had at least one pedicle screw repositioned in the index operation (p < 0.001). Six (6%) patients in the 2D group, and none in the 3D group had a neurological complication (p = 0.015). Five (5%) patients in the 2D group and none in the 3D group required reoperation (p = 0.009). There were no significant differences in HRQOL score at two-year follow-up between the groups. In conclusion, intraoperative 3D imaging reduced pedicle screw-related complications and reoperations in AIS patients undergoing PSI as compared with 2D imaging.

Vertebral Body Tethering: Indications, Surgical Technique, and a Systematic Review of Published Results.

Vertebral body tethering (VBT) represents a new surgical technique to correct idiopathic scoliosis using an anterior approach, spinal instrumentation with vertebral body screws, and a cable compressing the convexity of the curve. According to the Hueter-Volkmann principle, compression reduces and distraction increases growth on the growth plates. VBT was designed to modulate spinal growth of vertebral bodies and hence, the term 'growth modulation' has also been used. This review describes the indications and surgical technique of VBT. Further, a systematic review of published studies was conducted to critically evaluate the results and complications of this technique. In a total of 23 included studies on 843 patients, the preoperative main thoracic curve corrected from 49 to 23 degrees in a minimum 2 year follow-up. The complication rate of VBT was 18%. The results showed that 15% of VBT patients required reoperations for pulmonary or tether-related issues (10%) and less than 5% required conversion to spinal fusion. While the reported median-term results of VBT appear promising, long-term results of this technique are currently lacking.

Health-related quality of life after posterior vertebral column resection in children: comparison with healthy controls.

PURPOSE: Vertebral column resection (VCR) is a technique performed for short, angular spinal deformities. Several studies have reported good radiographic results with VCR regarding curve correction. However, only a few studies have reported the impact of this technique on the health-related quality-of-life measures (HRQoL). METHODS: A single surgeon series of 27 consecutive children (mean age at surgery 12.3 years, range 1.1-20.7 years) undergoing posterior VCR with a minimum of 2-year follow-up. The comparison was made to age- and gender-matched healthy controls. Outcome measures included Scoliosis Research Society (SRS) questionnaire both pre- and postoperatively, radiographic outcomes, and complications. RESULTS: The average major curve correction was 60.3% in the VCR patients. Complications were noted in 12 out of 27 (44%) of the VCR patients but all patients recovered fully during follow-up. The SRS pain domain scores improved significantly after VCR (p = 0.0002). The SRS total and domain scores were significantly lower than in the healthy controls especially in the self-image and function domains, but the pain and activity domains improved from preoperative to similar level than in the control group. CONCLUSIONS: HRQoL showed significant improvement in pain scores despite 44% risk of transient complications after VCR in pediatric patients. This health-related quality-of-life improvement remained at a significantly lower level than in the healthy control group. LEVEL OF EVIDENCE: Therapeutic Level III.

Casting versus flexible intramedullary nailing in displaced forearm shaft fractures in children aged 7-12 years: a study protocol for a randomised controlled trial.

INTRODUCTION: The forearm is the most common fracture location in children, with an increasing incidence. Displaced forearm shaft fractures have traditionally been treated with closed reduction and cast immobilisation. Diaphyseal fractures in children have poor remodelling capacity. Malunion can cause permanent cosmetic and functional disability. Internal fixation with flexible intramedullary nails has gained increasing popularity, without evidence of a better outcome compared with closed reduction and cast immobilisation. METHOD AND ANALYSIS: This is a multicentre, randomised superiority trial comparing closed reduction and cast immobilisation to flexible intramedullary nails in children aged 7-12 years with >10° of angulation and/or >10 mm of shortening in displaced both bone forearm shaft fractures (AO-paediatric classification: 22D/2.1-5.2). A total of 78 patients with minimum 2 years of expected growth left are randomised in 1:1 ratio to either treatment group. The study has a parallel non-randomised patient preference arm. Both treatments are performed under general anaesthesia. In the cast group a long arm cast is applied for 6 weeks. The flexible intramedullary nail group is immobilised in a collar and cuff sling for 4 weeks. Data are collected at baseline and at each follow-up until 1 year.Primary outcome is (1) PROMIS paediatric upper extremity and (2) forearm pronation-supination range of motion at 1-year follow-up. Secondary outcomes are Quick DASH, Paediatric Pain Questionnaire, Cosmetic Visual Analogue Scale, wrist and elbow range of motion as well as any complications and costs of treatment.We hypothesise that flexible intramedullary nailing results in a superior outcome. ETHICS AND DISSEMINATION: We have received ethical board approval (number: 78/1801/2020) and permissions to conduct the study from all five participating university hospitals. Informed consent is obtained from the parent(s). Results will be disseminated in peer-reviewed publications. TRIAL REGISTRATION NUMBER: NCT04664517.

Long-term hospital admissions and surgical treatment of children with congenital abdominal wall defects: a population-based study.

Congenital abdominal wall defects, namely, gastroschisis and omphalocele, are rare congenital malformations with significant morbidity. The long-term burden of these anomalies to families and health care providers has not previously been assessed. We aimed to determine the need for hospital admissions and the requirement for surgery after initial admission at birth. For our analyses, we identified all infants with either gastroschisis (n=178) or omphalocele (n=150) born between Jan 1, 1998, and Dec 31, 2014, in the Register of Congenital Malformations. The data on all hospital admissions and operations performed were acquired from the Finnish Hospital Discharge Register between Jan 1, 1998, and Dec 31, 2015, and compared to data on the whole Finnish pediatric population (0.9 million) live born 1993-2008. Patients with gastroschisis and particularly those with omphalocele required hospital admissions 1.8 to 5.7 times more than the general pediatric population (p<0.0001). Surgical interventions were more common among omphalocele than gastroschisis patients (p=0.013). At the mean follow-up of 8.9 (range 1.0-18.0) years, 29% (51/178) of gastroschisis and 30% (45/150) of omphalocele patients required further abdominal surgery after discharge from the neonatal admission.Conclusion: Patients with gastroschisis and especially those with omphalocele, are significantly more likely than the general pediatric population to require hospital care. Nevertheless, almost half of the patients can be treated without further surgery, and redo abdominal surgery is only required in a third of these children. What is Known: • Gastroschisis and omphalocele are congenital malformations with significant morbidity • There are no reports on the long-term need for hospital admissions and surgery in these children What is New: • Patients with abdominal wall defects are significantly more likely than the general pediatric population to require hospital care • Almost half of the patients can be treated without further surgery, and abdominal redo operations are only required in a third of these children.

Congenital abdominal wall defects and cryptorchidism: a population-based study.

PURPOSE: Several studies have reported high prevalence of undescended testis (UDT) among boys with congenital abdominal wall defects (AWD). Due to rarity of AWDs, however, true prevalence of testicular maldescent among these boys is not known. We conducted a national register study to determine the prevalence of UDT among Finnish males with an AWD. METHODS: All male infants with either gastroschisis or omphalocele born between Jan 1, 1998 and Dec 31, 2015 were identified in the Register of Congenital Malformations. The data on all performed operations were acquired from the Care Register for Health Care. The register data were examined for relevant UDT diagnosis and operation codes. RESULTS: We identified 99 males with gastroschisis and 89 with omphalocele. UDT was diagnosed in 10 (10.1%) infants with gastroschisis and 22 (24.7%) with omphalocele. Majority of these required an operation; 8/99 (8.1%) gastroschisis and 19/89 (21.3%) omphalocele patients. UDT is more common among AWD patients than general population with the highest prevalence in omphalocele. CONCLUSIONS: Cryptorchidism is more common among boys with an AWD than general population. Furthermore, omphalocele carries significantly higher risk of UDT and need for orchidopexy than gastroschisis. Due to high prevalence testicular maldescent, careful follow-up for UDT is recommended.

Omphalocele in Finland from 1993 to 2014: Trends, Prevalence, Mortality, and Associated Malformations-A Population-Based Study.

INRODUCTION: The aim of this study is to assess the changes in prevalence, mortality and termination pregnancy of omphalocele, and to identify associated anomalies. MATERIALS AND METHODS: A population-based nationwide register study. All cases with omphalocele were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded and analyzed, and perinatal and infant mortality and prevalence were calculated. RESULTS: There were 600 cases with omphalocele including 229 live births, 39 stillbirths, and 332 (55%) abortions. Birth prevalence in Finland was 1.96 per 10,000 births with no consistent trend over time. However, total prevalence was much higher (4.71/10,000) because more than half of these families chose option for the termination of pregnancy. Omphalocele is often complicated with other anomalies; most commonly chromosomal abnormalities (9.3%), heart defects (6.3%), central nervous system anomalies (3.0%), gastrointestinal, and urogenital malformations (both 2.0%). Proportion of chromosomal and central nervous system abnormalities were even higher in terminated pregnancies. Overall infant mortality was 22%. Total 1-year survival rates for isolated omphalocele, cases with multiple anomalies and neonates with chromosomal defects were 80, 88, and 17%, respectively. CONCLUSION: Omphalocele is a rare congenital anomaly, often associated with other malformations. Our data suggest that isolated cases may be more common than previously thought. In the absence of chromosomal defects, survival is reasonably good. Regardless, more than half of these pregnancies are often terminated.

Extended spectrum penicillins reduce the risk of omphalocele: A population-based case-control study.

BACKGROUND: Omphalocele is a major congenital anomaly associated with significant morbidity and mortality. Regardless, the influence of maternal use of prescription drugs on the risk of omphalocele has only been addressed in a handful of studies. The aim of this study was to assess the influence of maternal risk factors and prescription drugs in early pregnancy on the risk of omphalocele. METHODS: We performed a nationwide register-based case-control study in Finland. The analysis is based on the Finnish Register of Congenital Malformations and Drugs and Pregnancy databases, both upheld by the Finnish Institute for Health and Welfare. All omphalocele cases were identified between Jan 1, 2004, and Dec 31, 2014. Five age-matched controls from the same geographical region were randomly selected for each case. The main outcome measures were maternal risk factors for omphalocele. Our analysis compared the maternal characteristics and the use of prescription drugs during the first trimester of pregnancy between case and control mothers. RESULTS: Mothers of 359 omphalocele cases were compared with 1738 randomly selected age and area-matched mothers of healthy infants between 1 January 2014 and 31 December 2014. Both maternal obesity (BMI ≥30) and diabetes increased the risk for omphalocele, and their co-occurrence accumulated this risk (aOR 5.06, 95% Cl 1.19-21.4). Similarly, history of multiple miscarriages was an independent risk factor (2.51, 1.16-5.43). The oral use of extended spectrum penicillins during the first trimester of pregnancy had a significant, protective influence (0.17, 0.04-0.71). These analyses were adjusted for sex, parity, and risk factors reported above. No significant changes in risk were observed with any other medication used during the first trimester. CONCLUSION: In conclusion, these findings may suggest that extended spectrum penicillins in the first trimester reduces the risk of omphalocle formation. Additionally, consistent with earlier studies, previous repeated miscarriages, maternal obesity, and diabetes were significant risk factors for omphalocele.