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Aim of the Study: Continuous surgical developments, growing awareness of patient representatives and limited health-care resources are pushing for innovative approaches to ensure equal high-quality pediatric surgical care. We aimed to describe early experiences and assess surgical safety of a novel nationwide pediatric surgery collaborative initiative., Methods: In 2021, general pediatric surgeons representing all five independent university hospitals performing neonatal surgery in Finland initiated national collaboration, the Finnish pediatric surgery hub (FPSH), for sharing of surgical expertise and collective learning. For each case addressed by FPSH, place of care and surgical team were decided individually, and when deemed necessary, operations were performed in cooperation. Operations performed during 2021-2023 and associated early (<30 days) postoperative complications were analyzed according to Clavien-Madadi classification., Results: Of the total 40 surgeries managed co-operatively by FPSH, 30 (75%) took place in local university hospitals and 10 in Helsinki University Hospital. There were 34 (85%) elective and 6 urgent cases, which were operated within median 1 (range, 1-3) days. Most frequent underlying diagnoses included anorectal malformations, esophageal atresia and Hirschsprung disease. Overall, 12 (30%) had any early postoperative complications, all Clavien-Madadi grade IIIB or lower, and five patients (13%) were reoperated. Rate or grade of complications was not associated with place of care. In addition to regular virtual case meetings, national care protocols and research projects were introduced., Conclusion: These preliminary findings suggest that our national collaborative initiative, FPSH, not only provided practical and safe framework for sharing of surgical expertise but also for collective learning., Level of Evidence: III., Competing Interests: Conflict of interest Authors have no competing interests to declare., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Case: A 1-year old boy was presented with cauda equina syndrome and progressive loss of motor function in lower limbs. MRI and CT scans revealed a sacrococcygeal teratoma with metastases para-aortically and in L3 producing compression into the epidural space. Despite metastases and a progressive cauda equina, neoadjuvant treatment was given to achieve cytoreduction for neurological recovery and facilitate curative treatment., Conclusion: Malignant sacrococcygeal teratoma with intraspinal infiltration or metastasis requires surgical and adjuvant treatment. If primary neurologic function-sparing surgery makes curative treatment impossible, adequate and quick decompression of neural structures with similar results can be achieved by preoperative chemotherapy, avoiding the local spill of malignant cells., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/C434)., (Copyright © 2024 by The Journal of Bone and Joint Surgery, Incorporated.)

Introduction:  The aim of the study was to investigate the prevalence of esophageal atresia (EA), frequency of associated anomalies, and mortality from 2004 to 2017 in the Finnish population. We hypothesized the Spitz classification and the presence of other congenital malformations would predict mortality in patients with EA as well as assumed the survival to be high among patients with EA., Materials and Methods:  This retrospective, population-based study was based on the registries maintained by THL Finnish Institute for Health and Welfare and Statistics Finland. The cases were identified and classified according to the World Health Organization's International Classification of Diseases and Health Related Problems (ICD) revisions 9 and 10 (ICD-9 and ICD-10) codes and accompanying written diagnoses. Associated anomalies were classified based on the EUROCAT criteria, and minor anomalies were excluded. All statistical tests were performed as a two-sided significance level set at p  < 0.05. The chi-square or Fisher's exact test was utilized for categorical variables. The change in prevalence rates during the study period was evaluated with linear regression., Results:  In total, 337 cases with EA were identified including 295 (87.5%) live births, 17 (5.0%) stillbirths, and 25 (7.4%) terminations of pregnancy. The total prevalence for EA in Finland was 4.17/10,000 births with no significant change during the study period, p  = 0.35. Neonatal mortality was 5% ( n  = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases ( p  = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone ( p  < 0.001 and p  = 0.6, respectively). Type C was the most common atresia type (65.9%) followed by type A (14.8%) and B (6.8%). The most common group of associated malformations were heart defects (35.0%) followed by other gastrointestinal tract malformations (15.3%) and limb anomalies (12.2%). Syndromic cases (12.2%) were associated with type A and B atresias ( p  = 0.001). VACTERL association was observed in 16.6% of the cases., Conclusion:  The overall prevalence of EA remains stable and relatively high in Finland. Despite the high prevalence of co-occurring malformations, the overall survival rate is high. Spitz classification predicted neonatal survival well., Competing Interests: None declared., (Thieme. All rights reserved.)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Chyle leakage/ascites after surgical resection of neuroblastic tumors may delay the start of chemotherapy and worsen prognosis. Previous studies have reported a highly variable incidence and risk factors remain largely unknown. This study aims to analyze the true incidence of chyle leaks and ascites and seeks to identify risk factors and optimal treatment strategies., Methods: Medline/Embase databases were searched according to PRISMA guidelines. Literature reviews, case reports, and non-English papers were excluded. Data were extracted independently following paper selection by 2 authors., Results: The final analysis yielded 15 studies with N = 1468 patients. Chylous ascites was recorded postoperatively in 171 patients (12%). Most patients experiencing chyle leaks were successfully treated conservatively with drainage, bowel rest, parenteral nutrition and octreotide with variable combinations of these treatment options. 7/171 (4%) patients required operative exploration to control troublesome persistent chyle leaks. In risk factor analysis, higher tumor stage was significantly associated with the risk of chyle leak (P < 0.0001) whereas no correlation was observed with adrenal vs non-adrenal tumor location, INRG risk groups and tumor laterality., Conclusion: Chyle leakage after surgery for neuroblastic tumors is a common morbid complication occurring in some 12% of patients. Higher INSS tumor stage portends greater risk(s). Conservative therapy strategies appear successful in the majority of cases. To avert this complication meticulous mesenteric lymphatic ligation is recommended especially for those patients with higher tumor stage(s) requiring extensive radical surgery including retroperitoneal lymph node resection., Level of Evidence: III., Type of Study: Systematic review., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality., Competing Interests: Declaration of competing interest Dr Raitio reports research grants from Päivikki and Sakari Sohlberg Foundation. Funder had no role in the current study., (© 2024 Elsevier Ltd, BASO ∼ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

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., (© 2024. The Author(s).)

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., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Background: Biomaterials are routinely used in orthopedic surgery to fill bone defects, improve bone healing, and as degradable fixation material. A wide range of materials are currently in use, and the materials are chosen according to their bioactive properties. Osteoinductive materials stimulate bone healing by promoting osteogenesis. Osteoconductive materials facilitate bone growth on the surface of the material. Despite the many materials in use and an increasing number of published studies, randomized controlled trials on the subject are scarce., Methods: This review aims to summarize the history of biodegradable biomaterials and also the published level I evidence currently available on orthopedic biomaterials., Results: Most of the studies have been superiority trials with non-significant differences compared to conventional treatment options, confirming that several biomaterials are suitable treatment options for multiple indications including bone and/or tendon fixation, filling bone defects, and spinal fusion. Biomaterials help to avoid donor site complications associated with autogenous bone grafts and often eliminate the need for implant removal. However, the surgical technique may in some cases be more demanding than with conventional methods. Careful consideration of the pros and cons is therefore recommended in clinical practice., Conclusion: Biodegradable biomaterials complement the range of available treatment options in several fields of orthopedic surgery. However, some biomaterials performed worse than expected and were not recommended for clinical use, emphasizing the need for high-quality randomized trials. It is also noteworthy that several trials included only a limited number of patients, rendering the interpretation of the results of these underpowered studies challenging., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Scientific funding has been received from Medtronic, Stryker, Cerapedics, Nuvasive, and Bonalive to institution. I.H. works as a consultant for Medtronic, Bonalive, and Globus. I.H. and J.-J.S. are the section editors of the Scandinavian Journal of Surgery.

Normal childhood growth is an indicator of good health, but data addressing the growth of children born with abdominal wall defects (AWDs) are limited. The detailed growth phenotypes of children born with gastroschisis or omphalocele are described and compared to peers without AWDs from birth to adolescence. Data from 183 gastroschisis and 144 omphalocele patients born between 1993 and 2017 were gathered from Finnish nationwide registers and electronic health records. Weight (n = 3033), length/height (n = 2034), weight-for-length (0-24 months, n = 909), and body mass index measures (2-15 years, n = 423) were converted into sex- and age-specific Z-scores. Linear mixed models were used for comparisons. Intrauterine growth failure was common in infants with gastroschisis. Birth weight Z-scores in girls and boys were - 1.2 (0.2) and - 1.3 (0.2) and length Z-scores - 0.7 (0.2) and - 1.0 (0.2), respectively (p < 0.001 for all comparisons to infants without AWDs). During early infancy, growth failure increased in infants with gastroschisis, and thereafter, catch-up growth was prominent and faster in girls than in boys. Gastroschisis children gained weight and reached their peers' weights permanently at 5 to 10 years. By 15 years or older, 30% of gastroschisis patients were overweight. Infants with omphalocele were born with a normal birth size but grew shorter and weighing less than the reference population until the teen-age years., Conclusion: Children with gastroschisis and omphalocele have distinct growth patterns from fetal life onwards. These growth trajectories may also provide some opportunities to modulate adult health., What Is Known: • Intrauterine and postnatal growth failure can be seen frequently in gastroschisis and they often show significant catch-up growth later in infancy. It is assumed that part of the children with gastroschisis will become overweight during later childhood., What Is New:  • The longitudinal growth of girls and boys with gastroschisis or omphalocele is described separately until the teenage years. The risk of gaining excessive weight in puberty was confirmed in girls with gastroschisis., (© 2023. The Author(s).)

Background: Early series of pediatric thoracoscopic surgery have reported high conversion rates and significant complications. This study investigated the introduction of pediatric thoracoscopic lung resections in a low-volume center with reference to corresponding open thoracotomy procedures with regards to operative times, length of stay, cost of admission, and outcomes., Methods: A single surgeon series. Data from the first 10 consecutive thoracoscopic lung resections were compared to a cohort of 10 consecutive open lung resections performed before the introduction of the thoracoscopic technique. All operations were performed between December 2015 and October 2021. The median follow-up was 34 months (range 4-65)., Results: The cohort included 14 lobectomies (8 thoracoscopic and 6 open) for congenital pulmonary airway malformation (CPAM), and 6 resections (mainly non-anatomic) of pulmonary sequestration (2 thoracoscopic and 4 open). One lobectomy required conversion to thoracotomy, and one patient required reinsertion of a chest drain after open lobectomy due to persistent air leak. No other complications were recorded. All patients were asymptomatic at their follow-up. There was no significant difference in the mean age, mean weight, operative times, and intraoperative blood loss between open and minimally invasive procedures. Thoracoscopic technique was associated with significantly shorter stay at pediatric intensive care unit and shorter overall inpatients stay., Conclusion: Thoracoscopic lung resections can be safely introduced in a low-volume center with comparable cost, operative time, and results and significantly shorter inpatient stay.

Neuroblastoma is a malignant tumour affecting 10.5/1 million children annually. It arises from sympathetic nervous system precursor cells and is most frequently found in the adrenal gland and abdominal paravertebral ganglia. Diarrhoea as a presenting symptom of neuroblastoma is uncommon and usually linked to vasoactive intestinal peptide (VIP) tumour secretion. Even more rarely, postoperative diarrhoea may follow neuroblastoma tumour resection. Published studies generally associate postoperative diarrhoea with subadventitial tumour resection. These findings are however based on a handful of reports. This systematic review therefore aims to analyse the true incidence of postoperative diarrhoea and its morbid correlation with the extent/type of surgical resection. Pubmed/Embase databases were searched according to PRISMA guidelines. Final analysis consisted of 16 studies: N = 779 patients. Postoperative diarrhoea was significantly more common in all patients who underwent subadventitial resection compared to non subadventitial resection, p < 0.001 (OR 25.9, 95% CI 9.3-72.4). 5-year survival rates were equivalent in both groups. Preoperative diarrhoea was rarely reported in studies and always strongly linked to elevated VIP secretion. In the majority of neuroblastoma patients, preoperative diarrhoea ameliorated after gross tumour resection with elevated VIP normalized. The operative technique of subadventitial neuroblastoma resection portends significant risk(s) of post operative diarrhoea not seen in those patients undergoing other classical methods of tumour resection with 5-year survival rates strikingly similar. These findings affirm that subadventitial tumour resection should be avoided when undertaking surgery for neuroblastoma to minimize the risk(s) of persistent postoperative diarrhoea., Competing Interests: Declaration of competing interest Dr Raitio reports research grants from Päivikki and Sakari Sohlberg Foundation. Funder had no role in the current study., (Copyright © 2023 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

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., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H541 )., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

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., Competing Interests: A.R. has received research grants Päivikki and Sakari Sohlberg Foundation, and I.H. scientific funding to Institution from The Finnish Pediatric Research Foundation, Nuvasive, and Medtronic. The remaining authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H409 )., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

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.

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.

Sacrococcygeal teratoma (SCT) is a rare neoplasm affecting 1:35,000 newborns. Long-term follow-up from small observational studies report impaired bladder/bowel function. This current study comprehensively analyses all published studies to better define true long-term functional sequelae. Medline/Embase databases were searched with PRISMA guidelines. Final analysis yielded 37 studies involving 1116 patients (854 female; 77%). Individual datasets were available in 14 studies (222 patients). According to Altman classification - 298/845 (35%) were Type I, 252/845 (30%) Type II, 133/845 (16%) Type III, and 128/845 (15%) Type IV tumours. Most neoplasms were benign (640/858; 75%), 77/858 (9%) immature and 141/858 (16%) malignant. Abnormal bladder function was reported in 7/39 (18%) Altman Type I, 23/61 (37.7%) Type II, 11/34 (32.4%) Type III, and 15/25 (60%) Type IV cases (p = 0.007). Adverse urological outcomes were notably common in immature/malignant neoplasms vs benign tumours and in patients requiring reoperation(s); p = 0.002 and p = 0.01. Bowel dysfunction was evident in 19% index cases and constipation in 26% with no significant association(s) with tumour characteristics. Higher Altman stage, unfavourable tumour biology and reoperation are associated with poor functional outcome(s). Multidisciplinary management from primary diagnosis of SCT is crucially important for all patients to best optimise functional outcomes across surgical specialities., Competing Interests: Declaration of competing interest Dr Raitio reports research grants from Paulo Foundation. Funder had no role in the current study., (Copyright © 2022 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

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., Competing Interests: One or more of the authors declare financial or professional relationships on ICMJE-NASSJ disclosure forms., (© 2022 The Authors. Published by Elsevier Ltd on behalf of North American Spine Society.)

Purpose: The aim of the study was to compare and evaluate the utility of three different risk stratification scores for gastroschisis neonates; simple/complex gastroschisis, gastroschisis prognostic score and risk stratification index., Methods: Data of neonates born with gastroschisis between the years 1993 and 2015 were collected. The national registers and patient records of four Finnish University Hospitals were retrospectively reviewed. Logistic and linear regression analysis were performed to identify independent predictors for adverse outcomes. The efficacy of these prognostic methods was further assessed using ROC-curves and DeLong (1988) test., Results: Gastroschisis risk stratification index was an acceptable predictor of in-hospital mortality, AUC 0.70, 95% CI 0.48-0.91, p = 0.049. Complex gastroschisis and gastroschisis prognostic score were able to predict short bowel syndrome, AUC 0.80, 95% CI 0.58-1.00, p = 0.012 and AUC 0.80, 95% CI 0.59-1.00, p = 0.012, respectively., Conclusion: There are three easily obtainable risk stratification scores for outcome prediction in gastroschisis patients, however, their predictive ability did not have a statistical difference in the present study. The Gastroschisis risk stratification index seemed to perform moderately well in mortality prediction., (© 2022. The Author(s).)

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., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Orthopaedic Surgeons.)

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.

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., (© 2021. The Author(s).)

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.

Background: Congenital vertebral anomalies are a heterogeneous group of diagnoses, and studies on their epidemiology are sparse. Our aim was to investigate the national prevalence and mortality of these anomalies, and to identify associated anomalies., Methods: We conducted a population-based nationwide register study and identified all cases with congenital vertebral anomalies in the Finnish Register of Congenital Malformations from 1997 to 2016 including live births, stillbirths, and elective terminations of pregnancy because of major fetal anomalies. Cases were categorized based on the recorded diagnoses, associated major anomalies were analyzed, and prevalence and infant mortality were calculated., Results: We identified 255 cases of congenital vertebral anomalies. Of these, 92 (36%) were diagnosed with formation defects, 18 (7.1%) with segmentation defects, and 145 (57%) had mixed vertebral anomalies. Live birth prevalence was 1.89 per 10,000, and total prevalence was 2.20/10,000, with a significantly increasing trend over time (P<0.001). Overall infant mortality was 8.2% (18/219); 3.5% (3/86) in patients with formation defects, 5.6% (1/18) in segmentation defects, and 12.2% (14/115) in mixed vertebral anomalies (P=0.06). Co-occurring anomalies and syndromes were associated with increased mortality, P=0.006. Majority of the cases (82%) were associated with other major anomalies affecting most often the heart, limbs, and digestive system., Conclusions: In conclusion, the prevalence of congenital vertebral anomalies is increasing significantly in Finnish registers. Detailed and systematic examination is warranted in this patient population to identify underlying comorbidities as the majority of cases are associated with congenital major anomalies., Level of Evidence: Level III., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

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.

Purpose: Optimal treatment of gastroschisis is not determined. The aim of the present study was to investigate treatment methods of gastroschisis in four university hospitals in Finland., Methods: The data of neonates with gastroschisis born between 1993 and 2015 were collected. The primary outcomes were short and long-term mortality and the length of stay (LOS). Statistical analyses consisted of uni- and multivariate models., Results: Total of 154 patients were included (range from 31 to 52 in each hospital). There were no statistically significant differences in mortality or LOS between centers. Significant differences were observed between the hospitals in the duration of mechanical ventilation (p = 0.046), time to full enteral nutrition (p = 0.043), delay until full defect closure (p = 0.003), central line sepsis (p = 0.025), abdominal compartment syndrome (p = 0.018), number of abdominal operations during initial hospitalization (p = 0.000) and follow-up (p = 0.000), and ventral hernia at follow-up (p = 0.000). In a Cox multivariate analysis, the treating hospital was not associated with mortality., Conclusion: There were no differences in short or long-term mortality between four university hospitals in Finland. However, some inter-hospital variation in postoperative outcomes was present., Level of Evidence: Level III., (© 2021. The Author(s).)

Introduction:  The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma., Materials and Methods:  Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author., Results:  The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fatality rate. Management included observation only (201 patients, 8.5% mortality), surgical resection of primary tumor only (153 patients, 6.5% mortality), chemotherapy only (186 patients, 21% mortality), radiotherapy (5 deaths, 33% mortality), chemotherapy with surgery (160 patients, 10% mortality), surgery with radiotherapy (21 patients, 19% mortality), radiotherapy with chemotherapy (42 patients, 29% mortality), and surgery with chemotherapy and radiotherapy (27 patients, 33% mortality)., Conclusion:  There is a significant mortality observed in stage 4S/MS neuroblastoma infants with a dismal outcome observed in those patients with MYCN amplification and 1p/11q deletion. Those patients suitably amenable for conservative management or surgery to excise the primary tumor carry the best prognosis., Competing Interests: Dr. Raitio reports grants from Emil Aaltonen Foundation and Turku University Foundation, outside the submitted work., (Thieme. All rights reserved.)

Background: Prune belly syndrome (PBS) is a multisystem disease characterized by absent or deficient abdominal musculature with accompanying lax skin, urinary tract abnormalities, and cryptorchidism. Previous studies have estimated a birth prevalence of 1 in 35,000-50,000 live births., Objective: We set out to clarify the epidemiology and early hospital admissions of PBS in Finland through a population-based register study. Further, possible maternal risk factors for PBS were analyzed in a case-control setting., Study Design: The Finnish Register of Congenital Malformations was linked to the Care Register for Health Care, a population-based hospital admission data for PBS patients. Additionally, five matched controls were identified in the Birth Register and maternal risk factors of PBS were studied utilizing data from the Drugs and Pregnancy database., Results: We identified 31 cases of PBS during 1993-2015, 15 of which were live born and 16 elective terminations. The total prevalence was 1 in 44,000 births. Three patients (20%) died during infancy. On average, PBS-patients had 3.2 admissions and 10.6 hospital days per year in Finland during the study period years 1998-2015, 35- and 27-fold compared to children in Finland in general. Multiple miscarriages were significantly associated to PBS in maternal risk factor analyses., Discussion: The burden of disease is significant in PBS, demonstrated as a high infant mortality rate (20%), multiple hospital admissions, and inpatient care in days. The available variables are limited as a register-based study., Conclusion: We present data on contemporary epidemiology in a population-based study and show that the total prevalence of PBS is 1 in 44,000 in Finland. PBS entails a significant disease burden with admissions and hospital days over 35- and 27-fold compared to the general pediatric population, further aggravated by an infant mortality rate of 20%., Competing Interests: Conflicts of interest Dr Raitio, Dr Helenius and Dr Syvänen report grants from Clinical Research Institute HUCH, and Dr Raitio reports grants from the Finnish Paediatric Research Foundation., (Copyright © 2021 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Competing Interests: Competing interests: None declared.

Gastrointestinal stromal tumor (GIST) is a rare cancer of mesenchymal origin mostly seen in adult and elderly populations. Therefore, the prognostic and therapeutic features of pediatric GIST are not clearly defined. Clinical knowledge has been largely extrapolated from case series and adult studies. In this systematic review, we aimed to analyze the health outcome metrics of pediatric GIST. Medline and Embase databases were searched using relevant key terms. The original search retrieved 1,892 titles; 27 studies with 184 patients (68% female) were included for final review. The primary tumors were located in the stomach (165/184, 90%), small bowel (12/184, 7%), and elsewhere (7/184, 4%). Individual patient data were available in 125 cases with a median follow-up of 6.7 years. All patients underwent surgical resection, which varied from wide local excision to total gastrectomy. There were 12 deaths (10%), 65 (52%) patients were alive with no evidence of disease, and 31 cases (25%) were alive with disease. Tumor size > 5 cm, high mitotic index, and spindle morphology were predictive of mortality. Pediatric GIST has a more favorable prognosis and different characteristics versus adult tumors. There is a crucial need for international consensus and specific pediatric guidelines for the treatment of this rare tumor., (© 2021. The Author(s).)

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., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

The aim of this nationwide population-based case-control study was to assess the incidence of inguinal hernia (IH) among patients with congenital abdominal wall defects. All infants born with congenital abdominal wall defects between Jan 1, 1998, and Dec 31, 2014, were identified in the Finnish Register of Congenital Malformations. Six controls matched for gestational age, sex, and year of birth were selected for each case in the Medical Birth Register. The Finnish Hospital Discharge Register was searched for relevant diagnosis codes for IH, and hernia incidence was compared between cases and controls. We identified 178 infants with gastroschisis and 150 with omphalocele and selected randomly 1968 matched, healthy controls for comparison. Incidence of IH was significantly higher in gastroschisis girls than in matched controls, relative risk (RR) 7.20 (95% confidence interval [CI] 2.25-23.07). In boys with gastroschisis, no statistically significant difference was observed, RR 1.60 (95% CI 0.75-3.38). Omphalocele was associated with higher risk of IH compared to matched controls, RR 6.46 (95% CI 3.90-10.71), and the risk was equally elevated in male and female patients.Conclusion: Risk of IH is significantly higher among patients with congenital abdominal wall defects than in healthy controls supporting hypothesis that elevated intra-abdominal pressure could prevent natural closure of processus vaginalis. Parents should be informed of this elevated hernia risk to avoid delays in seeking care. We also recommend careful follow-up during the first months of life as most of these hernias are diagnosed early in life. What is Known: • Inguinal hernia is one of the most common disorders encountered by a pediatric surgeon. • Prematurity increases the risk of inguinal hernia. What is New: • Children with congenital abdominal wall defects have a significantly higher risk of inguinal hernia than general population. • Families should be informed of this elevated hernia risk to avoid delays in seeking care., (© 2021. The Author(s).)

Competing Interests: Competing interests: None declared.

Case: A 10-year-old boy presented with pain in the lateral hip and buttock area and fever. Magnetic resonance imaging revealed osteomyelitis of the pelvis with extensive Brodie's abscess (2.6 × 4.5 × 10.0 cm) continuing into the pelvic cavity. Surgical evacuation through the ilioinguinal approach was performed. In prolonged culture and in polymerase chain reaction of pus, the Fusobacterium nucleatum was found., Conclusion: Pelvic osteomyelitis caused by Fusobacterium is very rare. In this case, surgical treatment was required after conservative treatment with antibiotics was ineffective., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B623)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

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.

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.

Background: Risk factors for congenital limb deficiencies are poorly understood., Objective: To investigate risk factors for congenital limb deficiencies., Methods: We conducted a nationwide population-based case-control (1:5) study in Finland, using national registers on congenital anomalies, births, and induced abortions, cross-linked with data on maternal prescription medicine use obtained from the registers on Reimbursed Drug Purchases and Medical Special Reimbursements. Five hundred and four children with limb deficiencies (241 isolated, 181 syndromic, and 82 other associated anomalies) were identified, and 2,520 controls were matched to cases on residence and year of pregnancy. Non-syndromic cases (n = 323) were subdivided into longitudinal (n = 120), transverse (n = 123), intercalary (n = 24), mixed (n = 18), and unknown (n = 38) deficiencies., Results: Pregestational diabetes was associated with all limb deficiencies (adjusted odds ratio [OR] 12.71, 95% confidence interval [CI] 2.37, 68.25) and with isolated (OR 11.42, 95% CI 2.00, 64.60) deficiencies. Primiparity was associated with increased risk of congenital limb deficiencies among all cases (OR 1.49, 95% CI 1.15, 1.93), isolated cases (OR 1.46, 95% CI 1.09, 1.96), and among cases with longitudinal (OR 1.90, 95% CI 1.24, 2.90) and transverse deficiencies (OR 1.75, 95% CI 1.13, 2.70). Young maternal age (<25 years) was associated with all congenital limb deficiencies (OR 1.40, 95% CI 1.02, 1.90) and transverse deficiencies (OR 1.76, 95% CI 1.05, 2.96). Advanced maternal age (≥35 years) was associated with syndromic (OR 1.82, 95% CI 1.19, 2.78) and transverse deficiencies (OR 1.94, 95% CI 1.06, 3.57). Maternal antiepileptic medication was associated with all (OR 5.77, 95% CI 1.75, 19.04) and with isolated cases (OR 3.83, 95% CI 1.02, 14.34)., Conclusions: It is important that pregnant women taking medications, especially antiepileptics, or women with pregestational diabetes are carefully monitored with regard to the occurrence and risk of limb deficiencies in the fetus., (© 2021 The Authors. Paediatric and Perinatal Epidemiology published by John Wiley & Sons Ltd.)

The aim of the present study was to assess the prognostic factors for the outcome of gastroschisis in Finland. A retrospective multicenter study of gastroschisis patients born between 1993 and 2015 in four Finnish university hospitals was undertaken, collecting perinatal, surgical, and clinical data of neonates for uni- and multifactorial modeling analysis. The aim of the present study was to identify risk factors for mortality and the composite adverse outcome (death and/or short bowel syndrome or hospital stay > 60 days). Of the 154 infants with gastroschisis, the overall survival rate was 90.9%. In Cox regression analysis, independent risk factors for mortality included liver herniation, pulmonary hypoplasia, relaparotomy for perforation or necrosis, abdominal compartment syndrome, and central line sepsis. Furthermore, a logistic regression analysis identified central line sepsis, abdominal compartment syndrome, complex gastroschisis, and a younger gestational age as independent predictors of the composite adverse outcome.Conclusion: The risk of death is increased in newborns with gastroschisis who have liver herniation, pulmonary hypoplasia, abdominal compartment syndrome, relaparotomy for perforation or necrosis, or central line-associated sepsis. Special care should be taken to minimize the risk of central line sepsis in the clinical setting. What is known: • Gastroschisis is a relatively rare congenital anomaly of the abdominal wall and its incidence is increasing. • Complex gastroschisis has been reported to increase risk of mortality and complications. What is new: • Central line sepsis was found to be independently associated with mortality in gastroschisis patients. • Liver herniation was also significantly associated with mortality.

Background: Numerous factors have been identified as carrying prognostic value in neuroblastoma (NB) and therefore incorporated in risk stratification of disease. Here, we investigate the association of anatomical site of NB with molecular biology and clinical outcomes., Methods: A total of 117 patients with NB were studied over a 30-year period. Tumour location was confirmed with computed tomography/magnetic resonance imaging. Data on molecular biology were obtained as testing became available. Chi-squared, Fisher's exact test and Kaplan-Meier log-rank tests were used for statistical analysis., Results: Tumour originated in the thoracic region (thoracic NB, TNB) in 15 patients (13%), adrenal gland (adrenal NB, ANB) in 88 patients (75%) and abdominal/paravertebral chain (paravertebral NB, PVNB) in 14 patients (12%). Overall survival (OS) for ANB was significantly lower (38%; P = 0.015). ANB cases were more frequently diagnosed at stage IV (69%; P = 0.001). MYCN amplification was noted in 33% of ANB cases and associated with lower OS (17% versus 62% MYCN non-amplified ANB; P = 0.01). The vast majority of TNB and PVNB were non-MYCN amplified (100% and 86%, respectively) and carried better prognosis (OS 86% and 83%, respectively). Forty-two percent of ANB cases were diploid and had lower OS (20% versus 71% hyperdiploid ANB; P = 0.079). TNB and PVNB were found to be mostly hyperdiploid (86% and 100%, respectively) with better OS (83% and 33%, respectively). Segmental chromosomal alterations had prognostic significance in those with PVNB (P = 0.03)., Conclusion: TNB tumours have better outcomes than adrenal tumours. This may be due to varied factors reported here including non-metastatic disease at presentation, non-amplification of the MYCN oncogene and overall favourable molecular biology characteristics., (© 2021 The Authors. ANZ Journal of Surgery published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Surgeons.)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

Radial ray deficiency is the most common congenital deficiency of the upper limb. The aim of our study was to investigate maternal risk factors for radial ray deficiencies. We conducted a nationwide population-based case-control study using national registers. All cases with a radial ray deficiency born between 1996 and 2008 were included in the study and compared with five controls without limb deficiency. In total, 115 (10 isolated, 18 with multiple congenital anomalies, and 87 syndromic) cases with radial ray deficiencies were identified and compared with 575 matched controls. The total prevalence in Finland was 1.22 per 10,000 births. No significant risk factors were observed for nonsyndromic cases. In the syndromic group, advanced maternal age (≥35 years) increased the risk of radial aplasia (aOR 2.45, 95% CI 1.37-4.36), and a similar association was observed with multiple pregnancy (aOR 2.97, 1.16-7.62) and male sex (aOR 1.96, 1.18-3.25). Valproic acid was also a risk factor (p = .002). In conclusion, novel associations in the syndromic group of advanced maternal age and multiple pregnancy and increased risk of radial ray deficiencies were observed. Also, early reports on increased risk of RRD associated with valproate and male sex were supported by our results., (© 2020 Wiley Periodicals LLC.)

Early reports have suggested survival benefits associated with a hernia sac in congenital diaphragmatic hernia (CDH). However, these studies have included only small subsets of patients. This systematic review aimed to evaluate differences in outcomes of CDH newborns with and without a hernia sac. PubMed and Embase databases were searched using relevant key terms. Papers were independently reviewed by two authors with final selection approved by the senior author. Original search retrieved 537 papers; the final review included 8 studies (n = 837 patients). There were 168 CDH patients (20%) with a hernia sac with an overall survival of 93% vs 73% for CDH newborns without a sac (p < 0.001). Twenty-three percent of patients with a CDH sac required diaphragm patch repair vs 44% patients without a sac (p < 0.001). Pulmonary hypertension was manifested in 44% of CDH babies with a hernia sac vs 64% without a sac (p < 0.001). Three studies compared ECMO requirement: 15% with a hernia sac and 34% without sac, p < 0.001.Conclusion: This study shows significant survival benefits in newborns associated with presence of a CDH sac. This may be likely related to these infants having more favourable physiology with less severe pulmonary hypertension and/or smaller anatomical defects requiring primary closure only. What is Known: • Early reports have suggested survival benefits associated with a hernia sac in CDH. • Previous studies have included only a small number of patients. What is New: • A systematic review of published studies clearly shows that CDH newborns with a hernia sac have better overall survival outcomes and less severe pulmonary hypertension. • ECMO utilization and patch repair were also less often required in newborns with a hernia sac.

Background: Limb deficiencies associated with amniotic bands comprise a wide range of congenital anomalies. The association of maternal medication and the risk of amniotic band sequence (ABS) has not yet been addressed., Methods: This nationwide population-based case-control study used national registers on congenital anomalies, births and induced abortions, cross-linked with information on maternal prescription medicine use obtained from the registers on Reimbursed Drug Purchases and Medical Special Reimbursements. All cases with congenital limb deficiency associated with amniotic bands born between 1996 and 2008 were included in the study. Five controls without limb deficiency matched for residency and time of conception were randomly selected from the Medical Birth Register., Results: In total, 106 children with limb deficiency associated with ABS were identified and compared with 530 matched controls. Young maternal age (less than 25 y) increased the risk of limb deficiencies [odds ratio=1.72; 95% confidence interval (CI): 1.06, 2.80]. Primiparity was also associated with increased risk [adjusted odds ratio (aOR)=2.42; 95% CI: 1.52, 3.88]. After adjusting for maternal age, pregestational diabetes, and parity, maternal use of beta-blockers (adjusted OR=24.2; 95% CI: 2.57, 228) and progestogens (adjusted OR=3.79; 95% CI: 1.38, 10.4) during the first trimester of pregnancy significantly increased the risk of limb deficiencies associated with amniotic bands., Conclusions: Primiparity significantly increased the risk of limb defects associated with amniotic bands. Also, a novel association on increased risk of ABS with maternal use of progestogens or beta-blockers during the first trimester of pregnancy was observed., Level of Evidence: Level III.

Introduction:  The study aims to assess the changes in prevalence and mortality of gastroschisis, and to identify associated anomalies., Materials and Methods:  It is a population-based nationwide study. All gastroschisis cases 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 prevalence and infant mortality were calculated., Results:  There were 320 cases of gastroschisis; 235 (73%) live births, 16 (5%) stillbirths, and 69 (22%) terminations of pregnancy. Live birth prevalence of gastroschisis in Finland was lower than generally reported (1.73 in 10,000). However, due to relatively high rates of abortion, our total prevalence of 2.57/10,000 was similar with other reports. The most common risk factor was young maternal age. Babies with gastroschisis were born prematurely, on average on the 36th week and most are delivered by caesarean section. There was a significant increasing trend in live birth prevalence ( p  = 0.0018). Overall infant mortality was 7.7% (18/235), 7.2% (16/222) in simple gastroschisis and 15% (2/13) in complex gastroschisis. Associated anomalies were rare both in aborted fetuses and neonates, and there was only one case with a chromosomal abnormality., Conclusion:  Gastroschisis is usually an isolated anomaly with increasing birth prevalence and excellent survival rates. Regardless of the good prognosis, the abortion rates in Finland are higher than previously reported, and we hypothesize this to be due to lack of appropriate antenatal counselling., Competing Interests: A.R. reports grants from Clinical Research Institute HUCH, during the conduct of the study. All the other authors report no conflict of interest., (Thieme. All rights reserved.)

Background: Gastroschisis is an open abdominal wall defect with low mortality but significant morbidity. The prevalence has been increasing worldwide for the past decades. Several risk factors for gastroschisis have been identified, but no clear reason for increasing prevalence has been found. In our study, we aimed to assess and identify maternal risk factors for gastroschisis., Methods: In our nationwide register-based case-control study, we identified all gastroschisis cases in the Finnish Register of Congenital Malformations from 2004 to 2014. Information on drug prescriptions and purchases was received from Drugs and Pregnancy database. Five healthy age-matched controls from the same geographical region were randomly selected for each case. Conditional logistic regression was used to evaluate different risk factors., Results: One-hundred-eighty-eight cases of gastroschisis were identified and compared with 910 matched controls. Nulliparity was a significant risk factor for gastroschisis, aOR 2.00 (95% CI 1.29-3.11) whereas obesity was protective, aOR 0.35 (95% CI 0.15-0.83). Smoking appeared to increase the risk for gastroschisis, aOR 1.32 (95% CI 0.88-1.97). The mean maternal age of newborns with gastroschisis was significantly lower than average (p <.001)., Conclusion: As in previous studies, nulliparity and young maternal age were significant risk factors for gastroschisis. Maternal obesity significantly reduced the risk of gastroschisis regardless of maternal age and gestational diabetes., (© 2020 The Authors. Birth Defects Research published by Wiley Periodicals, LLC.)

Aim of the Study: To assess the safety and effectiveness of fluoroscopic balloon dilatation (FBD) in children with esophageal anastomotic stricture after surgical repair of esophageal atresia., Methods: All patients undergoing surgery for esophageal atresia and requiring dilatation(s) during a consecutive 15-year period [April 2000-September 2014] were analyzed. Dilatations were performed as day case procedures under general anesthesia using a radial force generating balloon device (Boston Scientific Corporation) by surgeons. Outcomes assessed included - (1) the number of dilatations/patient, (2) effectiveness and (3) need for surgery and (4) complications., Results: One hundred thirty seven patients underwent 625 FBD sessions (median 3 dilations per patient; range 1-24 dilatations). Median age at 1st FBD was 0.74years (range 0.05-16.1years). Balloon catheter sizes ranged from 6mm to 20mm. FBD yielded excellent results in 99 patients (74%), while 17 cases (13%) had mild ongoing dysphagia/dysmotility. Ten patients (7%) required further dilatation(s) to control symptoms. No patient(s) required esophageal stenting. Five cases required G-tube feeds as a result of oral aversion behavior - all of these cases were complex/VACTERL patients. Only 1 minor radiological leak occurred after a dilatation session and this did not require surgical intervention. A single patient (long gap EA TEF) with severe neurological impairment having multiple dilatations and stricture resection ultimately required esophageal replacement. Anti-reflux surgery was performed in 36 patients (26%) for medical therapy resistant GER., Conclusion: FBD for anastomotic stricture(s) following esophageal atresia repair achieved very good outcomes for the majority of EA TEF patients. The procedure can be accomplished safely as indicated by the low complication rate herein reported. Although some children may require more than one dilatation session prompt relief of symptoms can be achieved with a vigilant care program co-ordinated by a multidisciplinary specialist EA TEF team., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Intestinal malrotation classically presents in the neonatal period with bilious vomiting. However, population studies suggest that up to two-thirds of these patients are diagnosed later in childhood or in adulthood. Increased morbidity in the adult population has been reported. Local experience suggested that surgery was technically more difficult in older children and led to the hypothesis that it would be associated with increased morbidity., Methods: A retrospective case note analysis was performed on all children presenting with intestinal malrotation to a tertiary referral center between January 2002 and November 2014. Case notes and operation records were reviewed and those who underwent laparotomy for confirmed malrotation were included. Children were grouped as infants (< 1 year) and older (> 1 year). The primary outcome was total emergency reoperation rate. Secondary outcomes were requirement for a bypass at reoperation and mortality., Results: A total of 131 children with malrotation were identified (104 infants, 27 older children; 78 males; age range, 0-16 years). Overall, 13 patients had emergency reoperation following initial Ladd procedure (6 infants and 7 older children). Risk for reoperation was significantly higher in older children (p = 0.005) and additionally a bypass procedure was more often required in older children than infants (4 children, 2 infants, p = 0.016). Adhesiolysis was required on four occasions and redo Ladd procedure in two; these were evenly distributed between both groups. One child was found to have distal bowel obstruction at reoperation. There were three deaths (2.3%), all in the infant group. One was directly associated with malrotation with extensive bowel necrosis. The other two died of unrelated sepsis several months later., Conclusions: Malrotation surgery in older children is associated with a significantly higher emergency reoperation rate. The primary duodenal bypass procedure should always be considered with longstanding chronic intermittent obstruction associated with malrotation if the simple Ladd procedure is deemed inadequate., (Georg Thieme Verlag KG Stuttgart · New York.)