Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Paediatric surgical care in Sweden. Studies on incidence, outcome and access

Abstract Background: Advanced paediatric surgery is increasingly centralised in Sweden in order to improve treatment outcomes and reduce surgical risks. However, the overall need for surgery in the paediatric population has not been estimated, and the association between geographic access to surgical facilities and timely treatment for common surgical conditions in children is largely unknown. Aims: To estimate the overall incidence of surgery for Swedish children (I); to describe disease-specific incidences, treatment characteristics and surgical risks for one paediatric non-emergent surgical condition (cryptorchidism) (II) and one acute surgical condition (appendicitis) (III); and to assess for associations between distance to surgical facility and timely treatment with adjustment for medical and socioeconomic risk (IV-V). Methods: National healthcare and population registers of the total Swedish paediatric population, or subsets of it, for the years 2001-2014, were analysed in five observational cohort studies. The overall incidence of surgery was estimated in the Swedish paediatric population, and the disease-specific incidences were analysed for cryptorchidism and appendicitis. Treatment characteristics and surgical risks were presented, and the associations between travel time to hospital and treatment delay were analysed for these two conditions. Regression methods and survival analysis have been applied to estimate associations and calculate cumulative incidences. Results: On average, every year of childhood, 1 in 20 children had some kind of surgical procedures (6784 operations per 100,000 person-years), and 27% of all paediatric hospital admissions involved surgical procedures. >67% of all operations were performed as day surgery. 1.4% of Swedish boys were treated for cryptorchidism by age 14, and 1.8% were treated by 18 years of age, with prematurity and low birth weight being strongly associated with this risk. Only a minority (<6%) were treated during their first year of life according to the recommendations, with considerable regional variations. There was no postoperative mortality detected, and the risk of postoperative infection was low (1.4%), with no geographic variations. There was an association between travel time to hospital and timely treatment at 3 years of age, adjusted for birth-related risk factors and socioeconomic determinants (per 30-minute increase in travel time: aHR 0.91 [95% CI 0.88-0.95], p<0.001). 1 in 40 Swedish children (2.5%) had appendicitis during childhood. One in 6 (17%) had a more severe type of appendicitis, with increased postoperative infection rate (5.9% versus 2.3%, aOR 2.6 [2.2-3.2]) and readmission rate (5.5% versus 1.2%, aOR 4.8 [95% CI 4.1-5.5]), and higher risk for small bowel obstruction later on (0.2% versus 0.7%, aHR 3.9 [95% CI 2.6-5.8]). These risks were also associated with treatment modality; postoperative infection rates (aOR 0.6 [95% CI 0.5-0.8]) and small bowel obstruction rates (aHR 0.3 [95% CI 0.1-0.6], p=0.002) were lower after laparoscopic surgery, as compared to open appendectomy. There was no observed association between travel time to treatment and the risk for more severe type of paediatric appendicitis in Sweden. Significance: In this thesis, the overall incidence of surgery in Swedish children has been estimated. One elective condition and one emergency surgical condition have been anlysed for outcomes in terms of timing of treatment and surgery-related risks. Access to timely surgical treatment have been estimated in the elective and the emergancy care settings. Findings from these studies may be relevant to consider on a regional and national level in future planning of paediatric surgery

Resolution of hydronephrosis after pyeloplasty in children

Background: There is still a lack of knowledge regarding the natural course of resolution of hydronephrosis after pyeloplasty, and no consensus exists on how resolution of hydronephrosis is defined or when resolution is expected to occur. Objective: To determine when resolution of hydronephrosis occurs following pyeloplasty, by type of obstruction and by surgical approach. Methods: This retrospective study included 125 children age 50% reduction of APD) and 2-year success rate. Exposure was surgical approach and type of obstruction (intrinsic/extrinsic). Survival analysis was performed, adjusting for age, gender, year, laterality, preoperative renal function on MAG3, calyces dilatation and APD in the multivariable analysis. Results: At 12 months and 24 months follow-up, 90% and 93% had reached resolution, respectively. All children with persistent dilatation had improved drainage and stable or improved function on MAG3. There was no difference in time to resolution of hydronephrosis between open versus robotic-assisted laparoscopic surgery (adjusted HR 0.90, [0.54–1.52], p = 0.70), nor between different types of obstruction (aHR 0.84 [0.53–1.34], p = 0.47). Eight children had re-intervention, all identified within 3 months after primary surgery, and four had a postoperative drop on MAG3, giving a total success rate of 91% (121/135). Discussion: The vast majority of cases resolve and do so within 12 months from surgery. Since the improvement of hydronephrosis seems small between the first and second year after surgery, the value of follow-up beyond 12 months could be questioned. Based on the present study and previous literature we would recommend that children with persisting dilatation should continue their follow-up with ultrasound beyond 12 months. Children with complete resolution of their hydronephrosis at 12 months do not likely benefit from further follow-up. The same follow-up protocol should be applied, regardless of whether the obstruction is intrinsic or extrinsic in nature, or the surgery is performed with open or robotic-assisted approach. Overall, the definition of resolution of hydronephrosis varies in the literature and have impact on the results and may compromise comparisons. Conclusion: Surgical approach or type of obstruction does not seem to affect time to resolution of hydronephrosis after pyeloplasty in children. Follow-up with ultrasound beyond 12 months does not seem to benefit children with complete resolution.[Formula presented

Cryptorchidism in Sweden : A Nationwide Study of Prevalence, Operative Management, and Complications

Objectives: To review the cumulative prevalence, operative management, and complications of treatment for cryptorchidism in Sweden. Study design: A nationwide observational study from longitudinal register data of all Swedish-born boys 0-18 years of age, diagnosed with cryptorchidism from 2001 to 2014. Primary outcomes were occurrence and age at primary surgery. Secondary outcomes included type of procedure and surgical site infection. Results: Of 20 375 boys diagnosed with cryptorchidism in 2001-2014, 12 766 were surgically treated. The cumulative childhood prevalence was 1.8% (95% CI, 1.5-2.0), with a higher prevalence in boys born prematurely, small for gestational age, or with low birth weight. The median age at treatment decreased from 6.2 years in 2001 to 3.4 years in 2014 (P < .001). Still, 94.1% (95% CI, 92.7-95.6) had surgery after the recommended 1 year of age in 2014. Variations in age at surgery between Swedish counties were great (range, 2.9-5.9 years of age). There were no deaths within 30 days after surgery and the frequency of surgical site infection was low (1.4%; 95% CI, 1.1-1.6). Conclusions: The cumulative childhood prevalence of cryptorchidism was high, and complications were rare. Few boys underwent surgery in a timely manner according to clinical guidelines, and standards of care varied considerably across the country. Further research and collective actions are needed to improve the detection and management of congenital cryptorchidism

Association of IgE-Mediated Allergy with Risk of Complicated Appendicitis in a Pediatric Population

Importance: Childhood appendicitis is commonly complicated by gangrene and perforation, yet the causes of complicated appendicitis and how to avoid it remain unknown. Objective: To investigate whether children with IgE-mediated allergy have a lower risk of complicated appendicitis. Design, Setting, and Participants: This retrospective cohort study included all consecutive patients younger than 15 years (hereinafter referred to as children) who underwent appendectomy for acute appendicitis at a tertiary pediatric surgery center in Sweden between January 1, 2007, through July 31, 2017. Children were stratified between those with and without IgE-mediated allergies. Main Outcome and Measures: Risk of complicated appendicitis with gangrene or perforation, with occurrence of IgE-mediated allergy as an independent variable and adjusted for age, sex, primary health care contacts, seasonal antigenic exposure, allergy medications, appendicolith, and duration of symptoms. Results: Of 605 included children (63.0% boys; median age, 10 years; interquartile range, 7-12 years), 102 (16.9%) had IgE-mediated allergy and 503 (83.1%) had no allergy. Complicated appendicitis occurred in 20 children with IgE-mediated allergy (19.6%) compared with 236 with no allergy (46.9%; adjusted odds ratio, 0.33; 95% CI, 0.18-0.59). No significant allergy effect modification by sex, seasonal antigenic exposure, or allergy medication was found. Children with IgE-mediated allergy had a shorter hospital stay (median, 2 days for both groups; interquartile range, 1-2 days vs 1-5 days; P =.004). Conclusions and Relevance: In this study, children with IgE-mediated allergy had a lower risk of complicated appendicitis. The findings suggest that immunologic disposition modifies the clinical pattern of appendiceal disease. This theory introduces novel opportunities for understanding of the pathogenesis and clinical decision making for one of childhood's most common surgical emergencies

How boys and testicles wander to surgery: a nationwide cohort study of surgical delay in Sweden

BackgroundEarly orchidopexy is recommended for cryptorchidism and the surgery is increasingly centralised. The objectives were to determine the incidence, risk factors and if distance to treating hospital impacted on timely treatment of cryptorchidism.MethodsIn this observational study, all boys born in Sweden from 2001 to 2014 were followed in national registers to determine the incidence of cryptorchidism by levels of birth-related risk factors and social determinants. Travel time to hospital was used as the primary exposure in multivariable survival analysis, with age at surgery as main outcome.ResultsOf 748 678 boys at risk for cryptorchidism, 7351 were treated and evaluated for timing of surgery (cumulative childhood incidence 1.4%, 95% CI 1.3% to 1.5%). The incidence was clearly associated with prematurity and overdue pregnancy (HR for &lt;32 weeks 2.77 (95% CI 2.39 to 3.21); 32–36 weeks HR 1.36 (95% CI 1.24 to 1.49); &gt;41 weeks HR 1.19 (95% CI 1.10 to 1.29)), low birth weight (&lt;1000 g HR 3.94 (95% CI 3.15 to 4.92); 1000–1499 g HR 3.70 (95% CI 3.07 to 4.46); 1500–2500 g HR 1.69 (95% CI 1.52 to 1.88)) and intrauterine growth restriction (small for gestational age HR 2.38 (95% CI 2.14 to 2.65); large for gestational age HR 1.26 (95% CI 1.13 to 1.42)), but not with smoking or maternal age. Each 30 min increase in travel time was associated with a reduced probability of timely treatment (HR for being treated by age 3 adjusted for risk factors and socioeconomic determinants: 0.91 (95% CI 0.88 to 0.95)). Lower income and financial support were also associated with treatment delays (adjusted HR for lowest income quintile 0.82 (95% CI 0.72 to 0.93) and for families with financial support 0.85 (95% CI 0.73 to 0.97)).ConclusionsTravel distance to treating hospital was associated with delayed treatment. ‘Not all those who wander are lost’, but these findings suggest a trade-off between centralisation benefits and barriers of geography also in elective paediatric surgery.</jats:sec

A Nationwide Cohort Study of Outcome after Pediatric Appendicitis

Abstract Introduction Children with appendicitis often present with complicated disease. The aim of this study was to describe the clinical management of pediatric appendicitis, and to report how disease severity and operative modality are associated with short- and long-term risks of adverse outcome. Materials and Methods A nationwide retrospective cohort study of all Swedish children (&lt;18 years) diagnosed with appendicitis, 2001 to 2014 (n = 38,939). Primary and secondary outcomes were length of stay, surgical site infections, readmissions, 30-day mortality, and long-term risk of surgery for small bowel obstruction (SBO). Implications of complicated disease and operative modality were assessed with adjustment for age, gender, and trends over time. Results Complicated appendicitis was associated with longer hospital stay (4 vs. 2 days, p &lt; 0.001), increased risk of surgical site infection (5.9 vs. 2.3%, adjusted odds ratio [aOR]: 2.64 [95% confidence interval, CI: 2.18–3.18], p &lt; 0.001), readmission (5.5 vs. 1.2, aOR: 4.74 [95% CI: 4.08–5.53], p &lt; 0.001), as well as long-term risk of surgery for SBO (0.7 vs. 0.2%, adjusted hazard ratio [aHR]: 3.89 [95% CI: 2.61–5.78], p &lt; 0.001). Intended laparoscopic approach was associated with reduced risk of surgical site infections (2.3 vs. 3.1%, aOR: 0.74 [95% CI: 0.62–0.89], p = 0.001), but no overall reduction in risk for SBO; however, successful laparoscopic appendectomy was associated with less SBO during follow-up compared with open appendectomy (aHR: 0.27 [95% CI: 0.11–0.63], p = 0.002). Conclusion Children treated for complicated appendicitis are at risk of substantial short- and long-term morbidities. Fewer surgical site infections were seen after intended laparoscopic appendectomy, compared with open appendectomy, also when converted procedures were accounted for.</jats:p