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

Complexity of gastroschisis predicts outcome: epidemiology and experience in an Australian tertiary centre

Abstract Background Gastroschisis is a congenital anomaly of the fetal abdominal wall, usually to the right side of umbilical insertion. It is often detected by routine antenatal ultrasound. Significant maternal and pediatric resources are utilised in the care of women and infants with gastroschisis. Increasing rates of gastroschisis worldwide have led institutions to review local data and investigate outcomes. A collaborative project was developed to review local epidemiology and investigate antenatal and neonatal factors influencing hospital length of stay (LOS) and total parental nutrition (TPN) in infants born with gastroschisis. Methods We performed a five-year review of infants born with gastroschisis (2011–2015) at a major Australian centre. Complex gastroschisis was defined as involvement of stenosis, atresia, ischemia, volvulus or perforation and closed or vanishing gastroschisis. We extracted data from files and databases at the two participating hospitals, a major maternal fetal medicine centre and the affiliated children’s hospital. Results There were 56 infants antenatally diagnosed with gastroschisis with no terminations, one stillbirth (2%) and one infant with ‘vanishing’ gastroschisis. The mean maternal age was 23.9 years (range, 15–39 years). The mean gestation at delivery was 36 weeks (range, 25–39+ 3 weeks). Of the 55 neonates who received surgical management, 62% had primary closure. The median LOS was 33 (IQR, 23–45) days and the median duration of TPN was 26 (IQR, 17–36) days. Longer days on TPN (median 35 vs 16 days, P = 0.03) was associated with antenatal finding of multiple dilated bowel loops. Postnatal diagnosis of complex gastroschisis was made in 16% of cases and was associated with both longer LOS (median 89 vs 30 days, P = 0.003) and days on TPN (median 46 vs 21 days, P = 0.009). Conclusion Complex gastroschisis was associated with greater days on TPN and LOS. We found no late-gestation stillbirths and a low overall rate of 1.8%, suggesting the risk for stillbirth associated with gastroschisis is lower than previously documented. This information may assist counselling families. Improved data collection worldwide may reveal causative factors and enable antenatal outcome predictors

Successful treatment of recurrent epididymo-orchitis: Laparoscopic excision of the prostatic utricle

Prostatic utricle presenting with recurrent epididymo-orchitis is not uncommon. Excision of prostatic utricle is the treatment of choice. The various techniques described in literature suffer from the disadvantages of incomplete excision due to poor view. We report the successful laparoscopic excision of prostatic utricle in childhood

Features and outcomes of neonatal neuroblastoma

Purpose: Neonatal neuroblastoma (NNBL) is a rare tumour with few reported cases in the literature. The prognosis of NNBL is unclear with reported survival between 76 and 91%. The growing use of ante-natal ultrasound (USS) in recent years has resulted in an increasing incidence of NNBL. The purpose of this study is to review our experience with incidence, clinical features and outcome of NNBL in those children diagnosed antenatally compared to those diagnosed post-natally. Methods: Twelve cases of NNBL were detected antenatally or in the neonatal period (0–28 days) from a cohort of 120 children diagnosed with neuroblastoma (10%) over a 10-year period at the study institutions. Review of these 12 children forms the basis of this report. Results: Ante-natal diagnosis (ADNB) was made in six children (50%) and post-natal diagnosis (PDNB) in six (50%). Tumour site in both cohorts were predominantly adrenal and tumour staging was similar in both groups. There was no difference in outcome in ADNB compared to PDNB with overall 100% survival for the entire group. Conclusions: NNBL is a subset of neuroblastoma with apparent excellent outcome irrespective of the time of diagnosis. Clinical features and outcomes of ADNB are no different to PDNB

Comparative analysis of trends in paediatric trauma outcomes in New South Wales, Australia

Paediatric trauma centres seek to optimise the care of injured children. Trends in state-wide paediatric care and outcomes have not been examined in detail in Australia. This study examines temporal trends in paediatric trauma outcomes and factors influencing survival and length of stay. A retrospective review was conducted using data from the NSW Trauma Registry during 2003-2008 for children aged 15 years and younger who were severely injured (injury severity score >15). To examine trauma outcomes descriptive statistics and multivariable logistic and linear regression were conducted. There were 1138 children severely injured. Two-thirds were male. Road trauma and falls were the most common injury mechanisms and over one-third of incidents occurred in the home. Forty-eight percent of violence-related injuries were experienced by infants aged less than 1 year. For the majority of children definitive care was provided at a paediatric trauma centre, but less than one-third of children were taken directly to a paediatric trauma centre post-injury. Children who received definitive treatment at a paediatric trauma centre had between 3 and 6 times higher odds of having a survival advantage than if treated at an adult trauma centre. The number of severe injury presentations to the 14 major trauma centres in NSW remains constant. It is possible that injury prevention measures are having a limited effect on severe injury in NSW. This research provides stimulus for change in the provision and co-ordination in the delivery of trauma care for injured children. An erratum exists for this article and can be found in: Injury 2014 Volume 44(10) p. 1375 at doi: 10.1016/j.injury.2013.07.0037 page(s

Bystander basic life support: An important link in the chain of survival for children suffering a drowning or near-drowning episode

Eight children suffered drowning or near-drowning in Sydney pools over an 11-day period in January 2007. Four received basic life support (BLS) within 5 minutes of immersion and survived with good functional neurological outcomes. The other four were not discovered for ≥ 5 minutes and all died. This cluster serves as a reminder that timely effective bystander BLS is crucial to survival and good clinical outcomes in near-drowning episodes