Why all newborn hip screening programs have same results-a mini review.

All newborns are screened for developmental dysplasia of the hip (DDH), but countries have varying screening practices. The aim of this narrative mini review is to discuss the controversies of the screening and why it seems that all screening programs are likely to have same outcome. Different screening strategies are discussed alongside with other factors influencing DDH in this review. Universal ultrasound (US) has been praised as it finds more immature hips than clinical examination, but it has not been proven to reduce the rates of late-detected DDH or surgical management. Universal US screening increases initial treatment rates, while selective US and clinical screening have similar outcomes regarding late detection rates than universal US. This can be explained by the extrinsic factor affecting the development of the hip joint after birth and thus initial screening during the early weeks cannot find these cases.  Conclusion: It seems that DDH screening strategies have strengths and limitations without notable differences in the most severe outcomes (late-detected cases requiring operative treatment). Thus, it is important to acknowledge that the used screening policy is a combination of values and available resources rather than a decision based on clear evidence.

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.

Bado type III Monteggia fractures have a high injury- and treatment-related complication rate: a single center study of 73 fractures.

BACKGROUND AND PURPOSE:  Monteggia fractures can be problematic injuries. The aim of this population-based study is to evaluate the risk of complications according to the Bado types, clinical outcome, and incidence. METHODS:  72 children (median age 6, range 2-11 years) with 73 Monteggia fractures treated during 2014-2022 were identified from the institutional fracture register. Timing of diagnosis, complications, and method of treatment were registered. Outcomes were assessed at mean 4 years (1-9) follow-up in 68 (94%) children. The census population (< 16 years old) in Helsinki metropolitan area during the study period was assessed. RESULTS: Bado types I (n = 43) and III (n = 27) comprised all but 3 of the fractures. Diagnosis was made on admission in 57, and with a 1-8-day delay in 16 children. 8 children had sustained an associated nerve injury. 35 children were treated operatively, 7 after failed closed treatment. 4 reoperations were performed, including 3 ulnar osteotomies. The risk of complications (odds ratio [OR] 4.9, 95% confidence interval [CI] 1.7-14) and closed treatment failures (OR 12.3, CI 1.3-118) was higher in Bado type III than in type I injuries. 60 children attended for clinical follow-up, all had congruent radio-humeral joints and full range of elbow and forearm motion. Mean PedsQL was 94 (72-100) and QuickDash 3 (0-13). 8 additional children reported normal elbow functions by phone. The calculated mean annual incidence of Monteggia injuries was 2.9/100,000 children. CONCLUSION:  Monteggia fractures are rare (2.9/100,000 yearly). Bado type III injuries are associated with a high risk of complications.

Activity-restriction only as treatment yields positive outcomes in pediatric spinal compression fractures: a prospective study of 47 patients at medium-term follow-up.

BACKGROUND AND PURPOSE: We aimed to evaluate the clinical outcomes, radiographic results, and health-related quality of life in pediatric AO type A1 spinal compression fractures treated with activity-restriction only. PATIENTS AND METHODS: All children between 2014 and 2020 with an AO type A1 spinal compression fracture treated with activity-restriction only identified from an institutional fracture registry were invited to a prospective outcomes study. Clinical findings and spinal radiographs were assessed at median 3 years, interquartile range (IQR) 1-4 follow-up from injury. Oswestry Disability Index, Pediatric Quality of Life Inventory Generic Core Scale (PedsQL), and PedsQL Pediatric Pain Questionnaire were compared with reference values. 63 children were identified, of whom 47 agreed to participate. 8 were polytrauma patients. RESULTS: Age at injury was median 11 (IQR 9-14) years. The number of injured vertebrae was median 2 (IQR 1-3). 82% (94 of 115) were thoracic vertebrae fractures and 70% (33 of 47) of the patients had thoracic vertebrae fractures only. At follow-up all but 2 fractures showed radiographic remodeling. There was no difference from the published reference values in the patient-reported outcome measures. A lower PedsQL physical functioning score was associated with higher patient-reported pain (P = 0.03). At follow-up 12 patients had hyperkyphosis (median difference from the reference values 4°, IQR 3-6, 95% confidence interval [CI] 3-6) and 5 hypolordosis (median difference from reference 8°, IQR 4-11, CI 4-14). None of the patients had surgery for deformity during follow-up. CONCLUSION: Clinical, radiographic, and health-related quality of life outcomes were good after activity-restriction treatment in pediatric A1 spinal compression fractures.

Longitudinal growth of children born with gastroschisis or omphalocele.

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:  â€¢ 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.

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.

Epidemiology and risk factors for premature physeal closure in distal femur fractures.

BACKGROUND AND PURPOSE: Premature physeal closure (PPC) is a common and concerning complication to distal femoral fractures as the distal growth plate accounts for 70% of the growth of the femur. The literature is not unanimous in determining the risk factors of PPC, and the epidemiological characterization of these fractures is limited. Our aim was to calculate the population-based incidence and investigate risk factors for PPC in these fractures. PATIENTS AND METHODS: In this register-based study, between 2014 and 2021, 70 children with distal femoral physeal fractures presented to our hospital. Demographic data, and fracture- and treatment-related details were collected using the Kids' Fracture Tool. A directed acyclic graph (DAG) was constructed to determine confounding factors used in the risk analysis. RESULTS: Physeal fractures of the distal femur occurred with an annual incidence of 6/105 children, and a resulting PPC occurred in 16/70 (23%) with an annual incidence of 1.3/105 children. In multivariable analysis, dislocation exceeding 10 mm was a risk factor for PPC (OR 6.3, CI 1.4-22). CONCLUSION: One-fourth of distal femoral physeal fractures developed PPC. Greater dislocation and higher injury energy were significant risk factors, whereas choice of fracture treatment was not an independent risk factor. All patients with PPC belonged in the age group 11-16 years.

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

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

Increasing Prevalence and High Risk of Associated Anomalies in Congenital Vertebral Defects: A Population-based Study.

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.

Comparison of three risk stratification scores in gastroschisis neonates: gastroschisis prognostic score, gastroschisis risk stratification index and complex gastroschisis.

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.

Epidemiology and management of proximal tibia fractures in children and adolescents: a population-based study based on the Kids' Fracture Tool.

BACKGROUND AND PURPOSE: Proximal tibial fractures are infrequent injuries in children, and the literature on epidemiology, associated injuries, and management is limited. We calculated a population-based incidence and described the characteristics of proximal tibial fractures in children in terms of complications and management. PATIENTS AND METHODS: This is a retrospective study over a 6-year-period during including 241 children with proximal tibial fractures who presented to our university hospital. Demographic and fracture-related data was collected from the Kids' Fracture Tool. The number of children during the study period was collected from statistical yearbooks of the City of Helsinki to estimate annual incidence. RESULTS: Extra-articular fractures (129/241) peaked at the age of 3 and tibial tubercle (42/241) and intra-articular fractures (70/241) peaked at the age of 15. Annual incidences were estimated to be 3.4/100,000 children and 22/100,000 children in the age group of 13-16 years for ACL avulsions, and 3.8/100,000 children and 21/100,000 children in the age group of 13-16 years for tibial tubercle fractures. The incidence of vascular compromise (0%) and compartment syndrome was low (0.4 %, 1/241). CONCLUSION: Proximal tibial fractures present with a bimodal distribution, with extra-articular fractures peaking at the age of 3 years and fractures of the tibial tuberosity and intra-articular fractures peaking at the age of 15 years. Additionally, associated compartment syndrome and vascular compromise was not as common as previously reported.

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.

Prevalence and risk factors of radial ray deficiencies: A population-based case-control study.

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.

Maternal risk factors for congenital limb deficiencies: A population-based case-control study.

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.

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.

The association of perinatal and clinical factors with outcomes in infants with gastroschisis-a retrospective multicenter study in Finland.

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.

High incidence of inguinal hernias among patients with congenital abdominal wall defects: a population-based case-control study.

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.

Predictors of postoperative urinary retention after posterior spinal fusion for adolescent idiopathic scoliosis.

PURPOSE: To determine predictors for postoperative urinary retention in adolescents undergoing posterior spinal fusion for idiopathic scoliosis. Postoperative urinary retention affects almost every third adolescent after spinal fusion for idiopathic scoliosis. There are limited data regarding the risk factors of postoperative urinary retention in this patient group. METHODS: A retrospective study with prospectively collected urinary retention data from paediatric spine register with 159 consecutive patients (114 females, mean age 15.6 years, range 10-21 years) undergoing pedicle screw instrumentation for adolescent idiopathic scoliosis at a university hospital between May 2010 and April 2020. Postoperative urinary retention was defined as an inability to void after catheter removal and documented residual over 300 mL as confirmed using an ultrasound or by catheterization. RESULTS: Postoperative urinary retention was diagnosed in 33% (53 of 159) of the patients during hospital stay. Opioid amount on the day of catheter removal (OR 6.74 [95% CI: 2.47, 18.36], p < 0.001), male gender (OR 2.26 [95% CI: 1.01, 5.05], p = 0.048), and increasing weight (OR 1.04 [95% CI: 1.01, 1.07], p = 0.014) were associated with postoperative urinary retention. Mean opioid consumption on the day of catheter removal was 0.81 mg/kg (95% CI: 0.66, 0.96) in the retention group vs 0.57 mg/kg (95% CI: 0.51, 0.64) in the non-retention group, p < 0.001. CONCLUSIONS: Higher total opioid consumption, opioid amount on the day of catheter removal, higher weight, and male gender increases the risk of postoperative urinary retention in adolescents with idiopathic scoliosis undergoing posterior spinal fusion.

Risk Factors and Prevalence of Limb Deficiencies Associated With Amniotic Band Sequence: A Population-based Case-control Study.

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.

Different strategies, equivalent treatment approaches in terms of mortality in four university hospitals: a retrospective multicenter study of gastroschisis in Finland.

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.