Place of birth and postnatal transfers in infants with congenital diaphragmatic hernia in England and Wales: a descriptive observational cohort study.

OBJECTIVE: To describe clinical pathways for infants with congenital diaphragmatic hernia (CDH) and short-term outcomes. DESIGN: Retrospective observational cohort study using the UK National Neonatal Research Database (NNRD). PATIENTS: Babies with a diagnosis of CDH admitted to a neonatal unit in England and Wales between 2012 and 2020. MAIN OUTCOME MEASURES: Clinical pathways defined by place of birth (with or without colocated neonatal and surgical facilities), transfers, clinical interventions, length of hospital stay and discharge outcome. RESULTS: There were 1319 babies with a diagnosis of CDH cared for in four clinical pathways: born in maternity units with (1) colocated tertiary neonatal and surgical units ('neonatal surgical units'), 50% (660/1319); (2) designated tertiary neonatal unit and transfer to stand-alone surgical centre ('tertiary designated'), 25% (337/1319); (3) non-designated tertiary neonatal unit ('tertiary non-designated'), 7% (89/1319); or (4) non-tertiary unit ('non-tertiary'), 18% (233/1319)-the latter three needing postnatal transfers. Infant characteristics were similar for infants born in neonatal surgical and tertiary designated units. Excluding 149 infants with minimal data due to early transfer (median (IQR) 2.2 (0.4-4.5) days) to other settings, survival to neonatal discharge was 73% (851/1170), with a median (IQR) stay of 26 (16-44) days. CONCLUSIONS: We found that half of the babies with CDH were born in hospitals that did not have on-site surgical services and required postnatal transfer. Similar characteristics between infants born in neonatal surgical units and tertiary designated units suggest that organisation rather than infant factors influence place of birth. Future work linking the NNRD to other datasets will enable comparisons between care pathways.

Development Methodology, Availability, and Implementation of Core Outcome Sets in Pediatric Surgery.

Core outcome sets (COSs) provide a mechanism to guide researchers and clinicians when deciding which outcomes to report in research related to a specific clinical condition or intervention. The intention behind creating a COS for a specific condition is to improve the reporting of important and meaningful outcomes, thus enhancing the relevance of research. Additionally, a COS helps facilitate comparison of outcomes between different clinical studies and reduces research waste. In this paper, we discuss the availability of COSs in the field of pediatric general surgery. We provide an overview of the methodologies used to develop a COS, including common pitfalls, and finally, we discuss COS uptake and implementation. An understanding of all these aspects is important for researchers considering developing a new COS and for those reading research where a COS has been developed or used within a study. Failure to adequately appreciate the nuances of COS development, in particular, risks fundamental flaws that may jeopardize COS validity and subsequently hinder COS uptake and implementation.

Defining treatment success in children with surgical conditions.

OBJECTIVES: Develop a score summarising how successfully a child with any surgical condition has been treated, and test the clinical validity of the score. DESIGN: Discrete choice experiment (DCE), and secondary analysis of data from six UK-wide prospective cohort studies. PARTICIPANTS: 253 people with lived experience of childhood surgical conditions, 114 health professionals caring for children with surgical conditions and 753 members of the general population completed the DCE. Data from 1383 children with surgical conditions were used in the secondary analysis. MAIN OUTCOME MEASURES: Normalised importance value of attribute (NIVA) for number/type of operations, hospital-treated infections, quality of life and duration of survival (reference attribute). RESULTS: Quality of life and duration of survival were the most important attributes in deciding whether a child had been successfully treated. Parents, carers and previously treated adults placed equal weight on both attributes (NIVA=0.996; 0.798 to 1.194). Healthcare professionals placed more weight on quality of life (NIVA=1.469; 0.950 to 1.987). The general population placed more weight on survival (NIVA=0.823; 95% CI 0.708 to 0.938). The resulting score (the Children's Surgery Outcome Reporting (CSOR) Treatment Success Score (TSS)) has the best possible value of 1, a value of 0 describes palliation and values less than 0 describe outcomes worse than palliation. CSOR TSSs varied clinically appropriately for infants whose data were included in the UK-wide cohort studies. CONCLUSIONS: The CSOR TSS summarises how successfully children with surgical conditions have been treated, and can therefore be used to compare hospitals' observed and expected outcomes.

How to reach agreement: the impact of different analytical approaches to Delphi process results in core outcomes set development.

BACKGROUND: Core outcomes sets are increasingly used to define research outcomes that are most important for a condition. Different consensus methods are used in the development of core outcomes sets; the most common is the Delphi process. Delphi methodology is increasingly standardised for core outcomes set development, but uncertainties remain. We aimed to empirically test how the use of different summary statistics and consensus criteria impact Delphi process results. METHODS: Results from two unrelated child health Delphi processes were analysed. Outcomes were ranked by mean, median, or rate of exceedance, and then pairwise comparisons were undertaken to analyse whether the rankings were similar. The correlation coefficient for each comparison was calculated, and Bland-Altman plots produced. Youden's index was used to assess how well the outcomes ranked highest by each summary statistic matched the final core outcomes sets. Consensus criteria identified in a review of published Delphi processes were applied to the results of the two child-health Delphi processes. The size of the consensus sets produced by different criteria was compared, and Youden's index was used to assess how well the outcomes that met different criteria matched the final core outcomes sets. RESULTS: Pairwise comparisons of different summary statistics produced similar correlation coefficients. Bland-Altman plots showed that comparisons involving ranked medians had wider variation in the ranking. No difference in Youden's index for the summary statistics was found. Different consensus criteria produced widely different sets of consensus outcomes (range: 5-44 included outcomes). They also showed differing abilities to identify core outcomes (Youden's index range: 0.32-0.92). The choice of consensus criteria had a large impact on Delphi results. DISCUSSION: The use of different summary statistics is unlikely to affect how outcomes are ranked during a Delphi process: mean, median, and rates of exceedance produce similar results. Different consensus criteria have a large impact on resultant consensus outcomes and potentially on subsequent core outcomes sets: our results confirm the importance of adhering to pre-specified consensus criteria.

Impact of rectal dissection technique on primary-school-age outcomes for a British and Irish cohort of children with Hirschsprung disease.

BACKGROUND: This prospective cohort study compared primary-school-aged outcomes between children with Hirschsprung disease (HD) following Soave, Duhamel or Swenson procedures. METHODS: Children with histologically proven HD were identified in British/Irish paediatric surgical centers (01/10/2010-30/09/2012). Parent/clinician outcomes were collected when children were 5-8 years old and combined with management/early outcomes data. Propensity score/covariate adjusted multiple-event-Cox and multivariable logistic regression analyses were used. RESULTS: 277 (91%) of 305 children underwent a pull-through (53% Soave, 37% Duhamel, 9% Swenson). Based upon 259 children (94%) with complete operative data, unplanned reoperation rates (95% CI) per-person year of follow-up were 0.11 (0.08-0.13), 0.34 (0.29-0.40) and 1.06 (0.86-1.31) in the Soave/Duhamel/Swenson groups respectively. Adjusted Hazard Ratios for unplanned reoperation compared with the Soave were 1.50 (95% CI 0.66-3.44, p = 0.335) and 7.57 (95% CI 3.39-16.93, p < 0.001) for the Duhamel/Swenson respectively. Of 217 post-pull-through children with 5-8 year follow-up, 62%, 55%, and 62% in Soave/Duhamel/Swenson groups reported faecal incontinence. In comparison to Soave, Duhamel was associated with lower risk of faecal incontinence (aOR 0.34,95%CI 0.13-0.89,p = 0.028). Of 191 children without a stoma, 42%, 59% and 30% in Soave/Duhamel/Swenson groups required assistance to maintain bowel movements; compared to Soave, the Duhamel group were more likely to require assistance (aOR 2.61,95% CI 1.03-6.60,p = 0.043). CONCLUSIONS: Compared with Soave, Swenson was associated with increased risk of unplanned reoperation, whilst Duhamel was associated with reduced risk of faecal incontinence, but increased risk of constipation at 5-8 years of age. The risk profiles described can be used to inform consent discussions between surgeons and parents. LEVEL OF EVIDENCE: Level II.

Using stated-preferences methods to develop a summary metric to determine successful treatment of children with a surgical condition: a study protocol.

INTRODUCTION: Wide variation in the management of key paediatric surgical conditions in the UK has likely resulted in outcomes for some children being worse than they could be. Consequently, it is important to reduce unwarranted variation. However, major barriers to this are the inability to detect differences between observed and expected hospital outcomes based on the casemix of the children they have treated, and the inability to detect variation in significant outcomes between hospitals. A stated-preference study has been designed to estimate the value key stakeholders place on different elements of the outcomes for a child with a surgical condition. This study proposes to develop a summary metric to determine what represents successful treatment of children with surgical conditions. METHODS AND ANALYSIS: Preferences from parents, individuals treated for surgical conditions as infants/children, healthcare professionals and members of the public will be elicited using paired comparisons and kaizen tasks. A descriptive framework consisting of seven attributes representing types of operations, infections treated in hospital, quality of life and survival was identified. An experimental design has been completed using a D-efficient design with overlap in three attributes and excluding implausible combinations. All participants will be presented with an additional choice task including a palliative scenario that will be used as an anchor. The survey will be administered online. Primary analysis will estimate a mixed multinomial logit model. A traffic light system to determine what combination of attributes and levels represent successful treatment will be created. ETHICS AND DISSEMINATION: Ethics approval to conduct this study has been obtained from the Medical Sciences Inter-Divisional Research Ethics Committee (IDREC) at the University of Oxford (R59631/RE001-05). We will disseminate all of our results in peer-review publications and scientific presentations. Findings will be additionally disseminated through relevant charities and support groups and professional organisations.

Long COVID (post-COVID-19 condition) in children: a modified Delphi process.

OBJECTIVE: The aim of this study was to derive a research definition for 'Long COVID (post-COVID-19 condition)' in children and young people (CYP) to allow comparisons between research studies. DESIGN: A three-phase online Delphi process was used, followed by a consensus meeting. Participants were presented with 49 statements in each phase and scored them from 1 to 9 based on how important they were for inclusion in the research definition of Long COVID in CYP. The consensus meeting was held to achieve representation across the stakeholder groups. Statements agreed at the consensus meeting were reviewed by participants in the Patient and Public Involvement (PPI) Research Advisory Group. SETTING: The study was conducted remotely using online surveys and a virtual consensus meeting. PARTICIPANTS: 120 people with relevant expertise were divided into three panels according to their area of expertise: Service Delivery, Research (or combination of research and service delivery) and Lived Experience. The PPI Research Advisory group consisted of CYP aged 11-17 years. MAIN OUTCOME MEASURES: Consensus was defined using existing guidelines. If consensus was achieved in two or more panels or was on the border between one and two panels, those statements were discussed and voted on at the consensus meeting. RESULTS: Ten statements were taken forward for discussion in the consensus meeting and five statements met threshold to be included in the research definition of Long COVID among CYP. The research definition, aligned to the clinical case definition of the WHO, is proposed as follows: Post-COVID-19 condition occurs in young people with a history of confirmed SARS-CoV-2 infection, with at least one persisting physical symptom for a minimum duration of 12 weeks after initial testing that cannot be explained by an alternative diagnosis. The symptoms have an impact on everyday functioning, may continue or develop after COVID infection, and may fluctuate or relapse over time. The positive COVID-19 test referred to in this definition can be a lateral flow antigen test, a PCR test or an antibody test. CONCLUSIONS: This is the first research definition of Long COVID (post-COVID-19 condition) in CYP and complements the clinical case definition in adults proposed by the WHO.

Association between timing of re-introduction of enteral feeding and short-term outcomes following laparotomy for necrotising enterocolitis.

PURPOSE: To investigate the relationship between timing of re-introduction of feeds following surgery for Necrotising Enterocolitis (NEC) and important early outcomes. METHODS: Secondary analysis of prospectively collected data from paediatric surgical units in UK/Ireland of infants who underwent laparotomy for NEC between 01/03/2013 and 28/02/2014. Multivariable logistic regression analysis was used to compare the relationship of early (≤ 7 days) and later (8-27 days) re-introduction of feeding after surgery on death or need for PN at 28 days, correcting for known cofounders. RESULTS: 41/143 infants (29%) received early and 102/143 infants (71%) had delayed reintroduction of feeding. Infants in the early feeding group had a higher gestational age at birth, higher proportion of growth restriction, lower inotrope requirement, and weremore likely to have undergone primary anastomosis. Following adjustment there was no statistically significant difference detected in the rate of death or need for PN at 28 days, adjusted OR 0.4 (95% CI 0.2-1.1), noting the limited statistical power of this comparison. CONCLUSIONS: There is no evidence from this study to support a minimum period of 7 days nil by mouth post laparotomy for infants with NEC. Early feed reintroduction following laparotomy for NEC is safe in appropriate cases. LEVEL OF EVIDENCE: Level II - Treatment Study Group; Prospective comparative study.

A national consensus management pathway for paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS): results of a national Delphi process.

Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.

Management of Gastroschisis: Results From the NETS2G Study, a Joint British, Irish, and Canadian Prospective Cohort Study of 1268 Infants.

OBJECTIVE: In infants with gastroschisis, outcomes were compared between those where operative reduction and fascial closure were attempted ≤24 hours of age (PC), and those who underwent planned closure of their defect >24 hours of age following reduction with a pre-formed silo (SR). SUMMARY OF BACKGROUND DATA: Inadequate evidence exists to determine how best to treat infants with gastroschisis. METHODS: A secondary analysis was conducted of data collected 2006-2008 using the British Association of Pediatric Surgeons Congenital Anomalies Surveillance System, and 2005-2016 using the Canadian Pediatric Surgery Network.28-day outcomes were compared between infants undergoing PC and SR. Primary outcome was number of gastrointestinal complications. Interactions were investigated between infant characteristics and treatment to determine whether intervention effect varied in sub-groups of infants. RESULTS: Data from 341 British and Irish infants (27%) and 927 Canadian infants (73%) were used. 671 infants (42%) underwent PC and 597 (37%) underwent SR. The effect of SR on outcome varied according to the presence/absence of intestinal perforation, intestinal matting and intestinal necrosis. In infants without these features, SR was associated with fewer gastrointestinal complications [aIRR 0.25 (95% CI 0.09-0.67, P = 0.006)], more operations [aIRR 1.40 (95% CI 1.22-1.60, P < 0.001)], more days PN [aIRR 1.08 (95% CI 1.03-1.13, P < 0.001)], and a higher infection risk [aOR 2.06 (95% CI 1.10-3.87, P = 0.025)]. In infants with these features, SR was associated with a greater number of operations [aIRR 1.30 (95% CI 1.17-1.45, P < 0.001)], and more days PN [aIRR 1.06 (95% CI 1.02-1.10, P = 0.003)]. CONCLUSIONS: In infants without intestinal perforation, matting, or necrosis, the benefits of SR outweigh its drawbacks. In infants with these features, the opposite is true. Treatment choice should be based upon these features.

Outcomes at five to eight years of age for children with Hirschsprung's disease.

OBJECTIVE: This study describes core outcomes of Hirschsprung's disease (HD) in a UK-wide cohort of primary school-aged children. DESIGN: A prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5-8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis. SETTING: All 28 UK and Irish paediatric surgical centres. PARTICIPANTS: Children with histologically proven HD diagnosed at <6 months of age. MAIN OUTCOME MEASURES: NETS1HD core outcomes. RESULTS: Data were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age.Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex.Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD. CONCLUSION: This study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority.

Development of a gastroschisis core outcome set.

OBJECTIVE: Outcome reporting heterogeneity impedes identification of gold standard treatments for children born with gastroschisis. Use of core outcome sets (COSs) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS. DESIGN AND SETTING: Systematic reviews and stakeholder nomination were used to identify candidate outcomes that were subsequently prioritised by key stakeholders in a three-phase online Delphi process and face-to-face consensus meeting using a 9-point Likert scale. In phases two and three of the Delphi process, participants were shown graphical and numerical representations of their own, and all panels scores for each outcome respectively and asked to review their previous score in light of this information. Outcomes were carried forward to the consensus meeting if prioritised by two or three stakeholder panels in the third phase of the Delphi process. The COS was formed from outcomes where ≥70% of consensus meeting participants scored the outcome 7-9 and <15% of participants scored it 1-3. RESULTS: 71 participants (84%) completed all phases of the Delphi process, during which 87 outcomes were assessed. Eight outcomes, mortality, sepsis, growth, number of operations, severe gastrointestinal complication, time on parenteral nutrition, liver disease and quality of life for the child, met criteria for inclusion in the COS. CONCLUSIONS: Eight outcomes have been included in the gastroschisis COS as a result of their importance to key stakeholders. Implementing use of the COS will increase the potential for identification of gold standard treatments for the management of children born with gastroschisis.

One-year outcomes following surgery for necrotising enterocolitis: a UK-wide cohort study.

OBJECTIVE: The objective was to describe outcomes and investigate factors affecting prognosis at 1 year post intervention for infants with surgical necrotising enterocolitis (NEC). DESIGN: Using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System, we conducted a prospective, multicentre cohort study of every infant reported to require surgical intervention for NEC in the UK and Ireland between 1 March 2013 and 28 February 2014. Association of independent variables with 1-year mortality was investigated using multivariable logistic regression analysis. SETTING: All 28 paediatric surgical centres in the UK and Ireland. PATIENTS: Infants were eligible for inclusion if they were diagnosed with NEC and deemed to require surgical intervention, regardless of whether that intervention was delivered. OUTCOMES: Primary outcome was mortality within 1 year of the decision to intervene surgically. RESULTS: 236 infants were included in the study. 208 (88%) infants had 1-year follow-up. 59 of the 203 infants with known survival status (29%, 95% CI 23% to 36%) died within 1 year of the decision to intervene surgically. Following adjustment, key factors associated with reduced 1-year mortality included older gestational age at birth (adjusted OR (aOR) 0.87, 95% CI 0.78 to 0.96). Being small for gestational age (SGA) (aOR 3.6, 95% CI 1.4 to 9.5) and requiring parenteral nutrition at 28 days post-decision to intervene surgically (aOR 3.5, 95% CI 1.1 to 11.03) were associated with increased 1-year mortality. CONCLUSIONS: Parents of infants undergoing surgery for NEC should be counselled that there is approximately a 1:3 risk of death in the first post-operative year but that the risk is lower for infants who are of greater gestational age at birth, who are not SGA and who do not require parenteral nutrition at 28 days post-intervention.

NETS1HD study: development of a Hirschsprung's disease core outcome set.

OBJECTIVE: The objective of this study was to develop a Hirschsprung's disease (HD) core outcome set (COS). METHODS: Candidate outcomes were identified from a systematic review and stakeholder nomination. A three-phase Delphi process and consensus meeting were used to prioritise candidate outcomes based on scores assigned by stakeholder participants using a nine-point scale. In phases two and three, participants were shown graphical representations of their panel's scores and all panels' scores respectively for each outcome from the previous phase. After the third phase, outcomes prioritised by two or three panels were taken forward to the consensus meeting. The COS was formed from the 10 highest scoring outcomes meeting the threshold for inclusion (≥70% 7-9 and <15% 1-3). RESULTS: Eighty-nine stakeholders (82%) completed all three phases of the Delphi process. Seventy-four outcomes were assessed in phase one of the Delphi process, the following 10 of which met criteria for inclusion in the COS: (1) death with cause specified, (2) long-term faecal incontinence, (3) long-term voluntary bowel movements without need for enemas, or rectal or colonic irrigation, (4) long-term psychological stress for the individual with Hirschsprung's disease, (5) long-term urinary incontinence, (6) objective score of quality of life, (7) objective score of bowel function, (8) unplanned reoperation, (9) >need for a permanent stoma, (10) enterocolitis. CONCLUSIONS: This HD COS is formed of 10 outcomes deemed important by key stakeholders. Use of this COS in research will reduce outcome reporting heterogeneity and increase our ability to identify gold standard treatments for HD.

Corrigendum: A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

This corrects the article DOI: 10.1038/srep41149.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anomaly (aOR 5.17, 95% CI 1.9-14.1), abdominal wall erythema or discolouration at presentation (aOR 2.51, 95% CI 1.23-5.1), diagnosis of single intestinal perforation at laparotomy (aOR 3.1 95% CI 1.05-9.3), and necessity to perform a clip and drop procedure (aOR 30, 95% CI 3.9-237) were associated with increased 28-day mortality. These results can be used for national benchmarking and counselling of parents.

Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort.

PURPOSE: To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort. METHODS: A subgroup analysis of a prospective multicentre population cohort study was undertaken describing the outcomes of infants with OA and no tracheoesophageal fistula, (type A) and those with only an upper pouch fistula, (type B). MAIN RESULTS: Twenty-one of 151 infants in the whole cohort were diagnosed with type A or B oesophageal atresia (14%). Fifteen were type A (71%) and six type B (29%). Infants with type B had a shorter gap length than those with type A: 2.5 vertebral bodies (2-3) vs. 5 (4-6) (p=0.008). All infants with type B OA underwent oesophageal anastomosis, 83% (n=5) as the primary procedure. All infants with type A, underwent staged management. Six (40%) had delayed primary anastomosis and eight required oesophageal replacement (53%). One infant died prior to reconstruction. The median time to delayed primary anastomosis in infants with type A or B OA was 82days (75-89days) (n=7). The median time to oesophageal replacement was 94days (89-147days) (n=8). Median length of stay for infants with type A or B OA from first operation to first discharge was 101days (31-123days). CONCLUSIONS: Infants with type B OA had a shorter gap length and all were managed with oesophageal anastomosis. OA with no distal tracheoesophageal fistula is uncommon at a population level and frequently has a complex course. LEVEL OF EVIDENCE: Rating: II.

Variability of outcome reporting in Hirschsprung's Disease and gastroschisis: a systematic review.

Heterogeneity in outcome reporting limits identification of gold-standard treatments for Hirschsprung's Disease(HD) and gastroschisis. This review aimed to identify which outcomes are currently investigated in HD and gastroschisis research so as to counter this heterogeneity through informing development of a core outcome set(COS). Two systematic reviews were conducted. Studies were eligible for inclusion if they compared surgical interventions for primary treatment of HD in review one, and gastroschisis in review two. Studies available only as abstracts were excluded from analysis of reporting transparency. Thirty-five HD studies were eligible for inclusion in the review, and 74 unique outcomes were investigated. The most commonly investigated was faecal incontinence (32 studies, 91%). Seven of the 28 assessed studies (25%) met all criteria for transparent outcome reporting. Thirty gastroschisis studies were eligible for inclusion in the review, and 62 unique outcomes were investigated. The most commonly investigated was length of stay (24 studies, 80%). None of the assessed studies met all criteria for transparent outcome reporting. This review demonstrates that heterogeneity in outcome reporting and a significant risk of reporting bias exist in HD and gastroschisis research. Development of a COS could counter these problems, and the outcome lists developed from this review could be used in that process.