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.

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.

Introduction of pediatric thoracoscopic lung resections in a low-volume center - feasibility, outcome and cost analysis.

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.

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.

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

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

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.

Congenital diaphragmatic hernia-does the presence of a hernia sac improve outcome? A systematic review of published studies.

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.

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.

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.

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.

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.

Current treatment and outcomes of pediatric gastrointestinal stromal tumors (GIST): a systematic review of published studies.

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.

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

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

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.

Treatment and outcomes in pediatric inflammatory myofibroblastic tumors - A systematic review of published studies.

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.