Esophago-gastric junction findings on high resolution impedance manometry in children with esophageal atresia.

OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

Developing a core outcome set for the health outcomes for children and adults with congenital oesophageal atresia and/or tracheo-oesophageal fistula: OCELOT task group study protocol.

INTRODUCTION: Heterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions to standardise outcome reporting, facilitate meta-analysis and improve the relevance of research for patients and families. Our aim is to develop an internationally-agreed, comprehensive COS for OA-TOF, relevant from birth through to transition and adulthood. METHODS AND ANALYSIS: A long list of outcomes will be generated using (1) a systematic review of existing studies on OA-TOF and (2) qualitative research with children (patients), adults (patients) and families involving focus groups, semistructured interviews and self-reported outcome activity packs. A two-phase Delphi survey will then be completed by four key stakeholder groups: (1) patients (paediatric and adult); (2) families; (3) healthcare professionals; and (4) researchers. Phase I will include stakeholders individually rating the importance and relevance of each long-listed outcome using a 9-point Likert scale, with the option to suggest additional outcomes not already included. During phase II, stakeholders will review summarised results from phase I relative to their own initial score and then will be asked to rescore the outcome based on this information. Responses from phase II will be summarised using descriptive statistics and a predefined definition of consensus for inclusion or exclusion of outcomes. Following the Delphi process, stakeholder experts will be invited to review data at a consensus meeting and agree on a COS for OA-TOF. ETHICS AND DISSEMINATION: Ethical approval was sought through the Health Research Authority via the Integrated Research Application System, registration no. 297026. However, approval was deemed not to be required, so study sponsorship and oversight were provided by Alder Hey Children's NHS Foundation Trust. The study has been prospectively registered with the COMET Initiative. The study will be published in an open access forum.

Trampoline Park Injury Trends.

OBJECTIVES: Trampolines are an important cause of childhood injury and focus of injury prevention. Understanding and prevention of trampoline park injury is constrained by inadequate exposure data to estimate the at-risk population. This study aimed to measure trampoline park injury incidence and time trends using industry data. METHODS: Cross-sectional study to retrospectively analyze reported injuries and exposure in 18 trampoline parks operating in Australia and the Middle East, from 2017 to 2019. Exposure was derived from ticket sales and expressed as jumper hours. Exposure-adjusted incidence was measured using marginalized 0-inflated Poisson modeling and time trends using Joinpoint regression. RESULTS: There were 13 256 injured trampoline park users reported from 8 387 178 jumper hours; 11% sustained significant injury. Overall, trampoline park injuries occurred at a rate of 1.14 injuries per 1000 jumper hours (95% confidence intervals 1.00 to 1.28), with rates highest for high-performance (2.11/1000 jumper hours, 1.66 to 2.56) and inflatable bag or foam pit (1.91/1000 jumper hours, 1.35 to 2.50) jumping. Significant injuries occurred at a rate of 0.11 injuries per 1000 jumper hours (0.10 to 0.13), with rates highest for high-performance (0.29/1000 jumper hours, 0.23 to 0.36), and parkour (0.22/1000 jumper hours, 0.15 to 0.28) jumping. Overall, injury rates decreased by 0.72%/month (-1.05 to -0.40) over the study period. CONCLUSIONS: Trampoline park injuries occur in important numbers with sometimes serious consequences. However, within these safety standard-compliant parks, exposure-adjusted estimates show injuries to be uncommon and injury rates to be declining. Further reductions are required, especially severe injuries, and this study can enhance injury prevention initiatives.

Craniofacial anomalies in a murine model of heterozygous fibroblast growth factor 10 gene mutation.

OBJECTIVE: Dysregulation of Fibroblast Growth Factor 10 (FGF10), a member of the family of Fibroblast Growth Factor (FGF) proteins, has been implicated in craniofacial and dental anomalies, including craniosynostosis, cleft palate, and Lacrimo-Auriculo-Dento-Digital Syndrome. The aim of this murine study was to assess the craniofacial and dental phenotypes associated with a heterozygous FGF10 gene (FGF10+/- ) mutation at skeletal maturity. METHODS: Skulls of 40 skeletally mature mice, comprising two genotypes (heterozygous FGF10+/- mutation, n = 22; wildtype, n = 18) and two sexes (male, n = 23; female, n = 17), were subjected to micro-computed tomography. Landmark-based linear dimensions were measured for the cranial vault, maxilla, mandible, and first molar teeth. Multivariate analysis of variance was performed to assess whether there were significant differences in the craniofacial and dental structures between genotypes and sexes. RESULTS: The craniomaxillary skeleton and the first molar teeth were smaller in the FGF10+/- mice (P < .05), but the mandible was unaffected. Sex did not have a significant effect on these structures (P > .05). Cranial sutural defects were noted in 5/22 (22.7%) mutant versus 2/18 (11.1%) wildtype mice, and cleft palate in only one (4.5%) mutant mouse. None of the mice displayed craniosynostosis, expansive bony lesions, bifid condyles, or impacted teeth. CONCLUSION: The FGF10+/- mutation was associated with craniomaxillary skeletal hypoplasia that probably arose from deficient (delayed) intramembranous ossification of the sutured bones. Overall, the skeletal and dental data suggest that the FGF10 gene plays an important role in the aetiology of craniofacial dysmorphology and malocclusion.

Workflow Improvement of Electronic Health Record Usage in a Tertiary Pediatric Burns Clinic.

BACKGROUND: As a high patient-throughput clinic, the Royal Children's Hospital's multidisciplinary burns clinic's efficiency of clinic workflow and streamlined patient assessment is crucial. The clinic has been using a customized "burns assessment tool" (BAT) as part of its integrated electronic health record (EHR) since 2016. OBJECTIVES: The aim was to assess the usage patterns of the BAT at baseline, followed by re-evaluation following interventions to improve efficiency and utilization of the BAT. METHODS: This study was a prospective observational time-motion quality improvement study. Observations of 19 clinicians in the pediatric burns clinic by five trained observers using a validated time-motion capture tool (TimeCaT 3.9) to map clinician workflow, with specific reference to time spent on a list of predetermined tasks, were conducted. Baseline data were collected for 7 weeks followed by three cycles of interventions and observations over 5 months. RESULTS: At baseline, the median time for a patient visit was 24.56 minutes (range: 2.78-73.72 minutes, interquartile range: 14.17-27 minutes), with most of the time spent on documentation (34.6%) and patient contact tasks (26.0%). In each of the study cycles, the median time spent on documentation within the EHR was significantly reduced compared with baseline (cycle 1 29.8%, p = 0.08; cycle 2 20.4%, p ≤ 0.01; cycle 3 27.32%, p = 0.04). The time spent on patient contact increased when comparing baseline to data of cycles 1, 2, and 3 (25.96 vs. 33.27% of visit, p = 0.04). There was no significant change in absolute time spent on the BAT during the study. CONCLUSION: The study findings of clear, significant, and sustained improvement in documentation efficiency and the corresponding increase in patient contact time after interventions were introduced reinforce the importance of integration of an EHR with clinical workflow.

Playground injury prevention: the need for consistent and national implementation of Australian safety standards.

OBJECTIVES: Hospitalisation rates for injury, including at playgrounds, have not changed in the past decade. There are nine Australian Standards specific to playgrounds. The impact (if any) of these standards on playground injury resulting in hospitalisation is unknown. METHODS: Retrospective data for patients under 18 years presenting to emergency departments and/or admitted between October 2015 and December 2019 due to an injury documented as occurring at a playground were retrieved by the Illawarra Shoalhaven Local Health District Planning, Information and Performance Department. Maintenance and Australian Standard (AS) compliance data for the 401 local playgrounds were requested from the four Local Governments in Illawarra Shoalhaven Local Health District. Descriptive statistics were used. RESULTS: A total of 548 children were treated in emergency departments and/or admitted following playground injury. There was an overall increase of 39.3% in playground injury across the study period, and expenditure rose from $43,478 in 2011 to $367,259 in 2019 (a 744.7% increase). CONCLUSIONS: Playground injury has not decreased in the Illawarra Shoalhaven. Data regarding maintenance and AS compliance are lacking. This is not unique to our region. IMPLICATIONS FOR PUBLIC HEALTH: Without a national approach to adequately resource and monitor playground injury, it is not possible to assess the impact of Australian Standards or any injury prevention program.

Screening for VACTERL Anomalies in Children with Anorectal Malformations: Outcomes of a Standardized Approach.

PURPOSE: The majority of patients with an anorectal malformation (ARM) have associated congenital anomalies. It is well established that all patients diagnosed with an ARM should undergo systematic screening, including renal, spinal, and cardiac imaging. This study aimed to evaluate the findings and completeness of screening, following local implementation of standardized protocols. METHODS: A retrospective cohort study was performed assessing all patients with an ARM managed at our tertiary pediatric surgical center, following a standardized protocol implementation for VACTERL screening (January 2016-December 2021). Cohort demographics, medical characteristics, and screening investigations were analyzed. Findings were compared with our previously published data (2000-2015), conducted prior to protocol implementation. RESULTS: One hundred twenty-seven (64 male, 50.4%) children were eligible for inclusion. Complete screening was performed in 107/127 (84.3%) children. Of these, one or more associated anomalies were diagnosed in 85/107 (79.4%), whilst the VACTERL association was demonstrated in 57/107 (53.3%). The proportion of children that underwent complete screening increased significantly in comparison with those assessed prior to protocol implementation (RR 0.43 [CI 0.27-0.66]; p < 0.001). Children with less complex ARM types were significantly less likely to receive complete screening (p = 0.028). Neither presence of an associated anomaly, nor prevalence of the VACTERL association, differed significantly by ARM type complexity. CONCLUSION: Screening for associated VACTERL anomalies in children with ARM was significantly improved following standardized protocol implementation. The prevalence of associated anomalies in our cohort supports the value of routine VACTERL screening in all children with ARM, regardless of malformation type. LEVEL OF EVIDENCE: II.

Mid-Arm Point in PAEDiatrics (MAPPAED): An effective procedural aid for safe pleural decompression in trauma.

OBJECTIVE: Life-threatening thoracic trauma requires emergency pleural decompression and thoracostomy and chest drain insertion are core trauma procedures. Reliably determining a safe site for pleural decompression in children can be challenging. We assessed whether the Mid-Arm Point (MAP) technique, a procedural aid proposed for use with injured adults, would also identify a safe site for pleural decompression in children. METHODS: Children (0-18 years) attending four EDs were prospectively recruited. The MAP technique was performed, and chest wall skin marked bilaterally at the level of the MAP; no pleural decompression was performed. Radio-opaque markers were placed over the MAP-determined skin marks and corresponding intercostal space (ICS) reported using chest X-ray. RESULTS: A total of 392 children participated, and 712 markers sited using the MAP technique were analysed. Eighty-three percentage of markers were sited within the 'safe zone' for pleural decompression (4th to 6th ICSs). When sited outside the 'safe zone', MAP-determined markers were typically too caudal. However, if the site for pleural decompression was transposed one ICS cranially in children ≥4 years, the MAP technique performance improved significantly with 91% within the 'safe zone'. CONCLUSIONS: The MAP technique reliably determines a safe site for pleural decompression in children, albeit with an age-based adjustment, the Mid-Arm Point in PAEDiatrics (MAPPAED) rule: 'in children aged ≥4 years, use the MAP and go up one ICS to hit the safe zone. In children <4 years, use the MAP.' When together with this rule, the MAP technique will identify a site within the 'safe zone' in 9 out of 10 children.

Preexisting neural factors that contribute to dysmotility in esophageal atresia: a systematic review.

Esophageal dysmotility in esophageal atresia (EA) relates to abnormal development of esophageal innervation and musculature and to the esophageal repair. Few studies have investigated the preexisting dysmotility in EA, present prior to surgery. This systematic review aims to summarize the literature on neuronal studies in EA, to understand the causative factors for esophageal dysmotility. We performed a systematic review (PubMed, EMBASE, EBM, CINAHL databases; January 1947-February 2021) in accordance with PRISMA (PROSPERO number CRD42020171014). Fourteen studies were identified (eleven human, 187 EA patients; three animal, 64 EA rat specimens). Neural factors affecting esophageal dysmotility in human and animal studies included proteins, enzymes, growth factors, and genes, which play a role in the nervous system or neuroendocrine system, some of which have functions as neuromodulators or neurotransmitters. This systematic review has identified neural factors that affect esophageal dysmotility and contributes toward our understanding of the underlying dysmotility in patients with EA. The studies identified are important and essential for successful translation of basic science knowledge to impact clinical practice and understanding. Level of evidence: III.

Patient journey mapping to investigate quality and cultural safety in burn care for Aboriginal and Torres Strait Islander children and families - development, application and implications.

BACKGROUND: Quality and safety in Australian healthcare is inequitably distributed, highlighted by gaps in the provision of quality care for Aboriginal and Torres Strait Islander children. Burns have potential for long-term adverse outcomes, and quality care, including culturally safe care, is critical to recovery. This study aimed to develop and apply an Aboriginal Patient Journey Mapping (APJM) tool to investigate the quality of healthcare systems for burn care with Aboriginal and Torres Strait Islander children. STUDY DESIGN: Interface research methodology, using biomedical and cultural evidence, informed the modification of an existing APJM tool. The tool was then applied to the journey of one family accessing a paediatric tertiary burn care site. Data were collected through yarning with the family, case note review and clinician interviews. Data were analysed using Emden's core story and thematic analysis methods. Reflexivity informed consideration of the implications of the APJM tool, including its effectiveness and efficiency in eliciting information about quality and cultural safety. RESULTS: Through application of a modified APJM tool, gaps in quality care for Aboriginal and Torres Strait Islander children and families were identified at the individual, service and system levels. Engagement in innovative methodology incorporating more than biomedical standards of care, uncovered critical information about the experiences of culturally safe care in complex patient journeys. CONCLUSION: Based on our application of the tool, APJM can identify and evaluate specific aspects of culturally safe care as experienced by Aboriginal and Torres Strait Islander peoples and be used for quality improvement.

Characteristics, management and changing incidence of children with empyema in a paediatric intensive care unit.

AIM: Paediatric intensive care unit (PICU) admissions for empyema increased following the 13-valent pneumococcal conjugate vaccine (PCV13). We describe the clinical characteristics, management and outcomes for children with empyema and compare incidence before and after PCV13. METHODS: Retrospective study of patients <18 years admitted to The Royal Children's Hospital Melbourne PICU with empyema between January 2016 and July 2019. We investigated the incidence of empyema during two time periods: 2007-2010 (pre-PCV13) and 2016-2019 (post-PCV13). RESULTS: Seventy-one children (1.9% of all PICU admissions) were admitted to PICU with empyema between 2016 and 2019. Sixty-one (86%) had unilateral disease, 11 (16%) presented with shock and 44 (62%) were ventilated. Streptococcus pneumoniae and group A Streptococcus were the most commonly identified pathogens. Forty-five (63%) were managed with video-assisted thoracoscopic surgery (VATS). There was a 31% reduction in empyema hospitalisations as a proportion of all hospitalisations (IRR 0.69, 95% CI 0.59-0.8), but a 2.8-fold increase in empyema PICU admissions as a proportion of all PICU admissions (95% CI 2.2-3.5, P < 0.001). For the PICU cohort, this was accompanied by reduction in PIM2 probability of death (median 1% vs. 1.9%, P = 0.02) and duration of intubation (median 69 h vs. 126.5 h, P = 0.045). CONCLUSIONS: In children with empyema in PICU 62% required ventilation, 16% had features of shock and 63% received VATS. Empyema admissions, as a proportion of all PICU admissions, increased in the era post-PCV13 compared to pre-PCV13 despite no increase in illness severity at admission.

Variation in burn wound management approaches for paediatric burn patients in Australia and New Zealand.

BACKGROUND: To date, no large-scale exploration of the profile of, and variance among paediatric patients who underwent a burn wound management procedure in theatre exists in an Australian and New Zealand context. This study aims to provide a profile of paediatric burn patients who underwent a burn wound management procedure in theatre during an acute admission and highlight specific areas of practice where there is variation between burn services that may affect treatment efficacy and efficiency. METHODS: We performed a retrospective review of all paediatric patients (ages <16 years) who sustained a burn injury between July 2016 and June 2019 and underwent a burn wound management procedure in theatre, using data from the Burns Registry Australia New Zealand. RESULTS: The number of patients across burn services decreased as TBSA increased. Deep dermal burns represented the majority of cases across services. There was significant variation in time from injury to admission and the proportion of patients who received skin grafts across services. CONCLUSIONS: Significant differences in the patient profile and clinical practices were observed among burn services. A greater understanding of the factors underlying the variations at each particular service will also be helpful.

Review of the requirements for effective mass casualty preparedness for trauma systems. A disaster waiting to happen?

Mass casualty incidents (MCIs) are diverse, unpredictable, and increasing in frequency, but preparation is possible and necessary. The nature of MCIs requires a trauma response but also requires effective and tested disaster preparedness planning. From an international perspective, the aims of this narrative review are to describe the key components necessary for optimisation of trauma system preparedness for MCIs, whether trauma systems and centres meet these components and areas for improvement of trauma system response. Many of the principles necessary for response to MCIs are embedded in trauma system design and trauma centre function. These include robust communication networks, established triage systems, and capacity to secure centres from threats to safety and quality of care. However, evidence from the current literature indicates the need to strengthen trauma system preparedness for MCIs through greater trauma leader representation at all levels of disaster preparedness planning, enhanced training of staff and simulated disaster training, expanded surge capacity planning, improved staff management and support during the MCI and in the post-disaster recovery phase, clear provision for the treatment of paediatric patients in disaster plans, and diversified and pre-agreed systems for essential supplies and services continuity. Mass casualty preparedness is a complex, iterative process that requires an integrated, multidisciplinary, and tiered approach. Through effective preparedness planning, trauma systems should be well-placed to deliver an optimal response when faced with MCIs.

Post-operative colonic manometry in children with Hirschsprung disease: A systematic review.

BACKGROUND: A significant proportion of children experience bowel dysfunction (including constipation and fecal incontinence) following surgical repair of Hirschsprung disease (HD). Persistent symptoms are thought to relate to underlying colonic and/or anorectal dysmotility. Manometry may be used to investigate the gastrointestinal motility patterns of this population. PURPOSE: To (1) evaluate the colonic manometry equipment and protocols used in the assessment of the post-operative HD population and (2) summarize the available evidence regarding colonic motility patterns in children with HD following surgical repair. DATA SOURCES: We performed a systematic review of the Cochrane Library, Embase, MEDLINE, and PubMed databases (January 1, 1980 and March 9, 2020). Data were extracted independently by two authors. STUDY SELECTION: This systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Studies reporting the post-operative assessment of children with HD using colonic manometry were considered for inclusion. RESULTS: Five studies satisfied selection criteria, providing a combined total of 496 children. Of these, 184 children with repaired HD underwent colonic manometry. Studies assessed heterogeneous populations, utilized variable manometry equipment and protocols, and reported limited baseline symptom characteristics, thus restricting comparability. All studies used low-resolution colonic manometry. CONCLUSIONS: This systematic review highlighted the paucity of evidence informing the understanding of colonic dysmotility in the post-operative HD cohort. Current literature is limited by variable methodologies, heterogeneous cohorts, and the lack of high-resolution manometry.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Considering difference: clinician insights into providing equal and equitable burns care for Aboriginal and Torres Strait Islander children.

OBJECTIVE: To better understand issues driving quality in burn care related to equity of outcomes and equality of provision for Aboriginal and Torres Strait Islander children. METHODS: Seventy-six interviews with team members who provide care for Aboriginal and Torres Strait Islander children in six paediatric burn units across five Australian jurisdictions were completed. Interface research methodology within a qualitative design guided data collection and analysis. RESULTS: Three themes were identified: i) Burn team members who identify the requirement to meet the specific needs of Aboriginal and Torres Strait Islander children and deliver differential care; ii) Burn team members who believe in equal care, but deliver differential care based on the specific needs of Aboriginal and Torres Strait Islander children; and iii) Burn team members who see little need for provision of differential care for Aboriginal and Torres Strait Islander children and rather, value the provision of equal care for all. CONCLUSION: Burn team members conflate equitable and equal care, which has implications for the delivery of care for Aboriginal and Torres Strait Islander children. Equitable care is needed to address disparities in post-burn outcomes, and this requires clinicians, healthcare services and relevant system structures to work coherently and intentionally to reflect these needs. Implications for public health: Changes in health policy, the embedding of Aboriginal and Torres Strait Islander liaison officers in burn care teams and systems that prioritise critical reflexive practice are fundamental to improving care.

Survival Trends and Syndromic Esophageal Atresia.

BACKGROUND AND OBJECTIVES: Presence of a syndrome (or association) is predictive of poor survival in esophageal atresia (EA). However, most reports rely on historical patient outcomes, limiting their usefulness when estimating risk for neonates born today. We hypothesized improved syndromic EA survival due to advances in neonatal care. METHODS: A retrospective single-center review of survival in 626 consecutive patients with EA from 1980 to 2017 was performed. Data were collected for recognized risk factors: preterm delivery; birth weight <1500 g; major cardiac disease; vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL); and non-VACTERL syndromes. Cox proportional hazards regression models were used to evaluate temporal trends in survival with respect to year of birth and syndromic EA. RESULTS: Overall, 87% of 626 patients with EA survived, ranging from 82% in the 1980s to 91% in the 2010s. After adjusting for confounders, syndromic EA survival did not improve during the study, with no association found between year of birth and survival (hazard ratio [HR] 0.98, 95% confidence interval [CI]: 0.95-1.01). Aside from lethal non-VACTERL syndromes, patients with nonlethal non-VACTERL syndromes (HR 6.85, 95% CI: 3.50-13.41) and VACTERL syndrome (HR 3.02, 95% CI: 1.66-5.49) had a higher risk of death than those with nonsyndromic EA. CONCLUSIONS: Survival of patients with syndromic EA has not improved, and patients with non-VACTERL syndromes have the highest risk of death. Importantly, this is independent of syndrome lethality, birth weight, and cardiac disease. This contemporary survival assessment will enable more accurate perinatal counseling of parents of patients with syndromic EA.

Changes in emergency department blood product use for major paediatric trauma following the implementation of a major haemorrhage protocol.

OBJECTIVE: Fixed ratio blood product administration may improve outcomes in trauma patients with massive blood loss. The present study aimed to describe the impact of a major haemorrhage protocol (MHP) on the ratio of blood products administered for paediatric major trauma. METHODS: Retrospective observational study in a state-designated paediatric major trauma centre in Melbourne, Australia. Children with major trauma who received blood products in the ED were identified from a hospital trauma registry. Blood product ratios before, during and after implementation of a hospital MHP were compared in consecutive 2 year blocks. RESULTS: Over a 6 year period, 767 major trauma patients were identified, of whom 47 received blood products in the ED and were included in the analysis; 14 pre-MHP implementation, 24 during-MHP implementation and nine post-MHP implementation. No patients received blood products at a ratio of 1:1:1 for red blood cells:fresh frozen plasma:platelets, respectively, during any time period. In this cohort of predominantly blunt trauma, blood products were infrequently administered in the ED because of the low prevalence of massive blood loss. Coagulopathy and hypofibrinogenaemia were commonly observed, nearly half of included patients were managed operatively and one quarter did not survive their injuries. CONCLUSION: The implementation of a MHP did not change the ratio of blood product administration in this cohort of patients because of the infrequency of massive blood loss. Future studies may focus on the impact of treating coagulopathy and hypofibrinogenaemia on patient-centred outcomes.