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.

ObjectiveThe 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.DesignA 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.SettingThe study was conducted remotely using online surveys and a virtual consensus meeting.Participants120 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 measuresConsensus 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.ResultsTen 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.ConclusionsThis 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.

Background and AimsWe aimed to provide a contemporaneous assessment of outcomes at one-year post oesophageal atresia/tracheoesophageal fistula (OA-TOF) repair, focussing particularly on post-operative complications. It is generally accepted that oesophageal stricture is the most common complication and causes significant morbidity. We also aimed to assess the efficacy of prophylactic anti-reflux medication (PARM) in reducing stricture formation.MethodA prospective, multi-centre cohort study of all infants live-born with oesophageal atresia in the United Kingdom and Ireland in 2008/9 was performed, recording clinical management and outcomes at one year. The effect of PARM on stricture formation in infants with the type-c anomaly was assessed using logistic regression analysis.Results151 infants were live-born with oesophageal atresia in the defined reporting period, 126 of whom had the type-c anomaly. One-year follow-up information was returned for 105 infants (70%); the mortality rate was 8.6% (95% CI 4.7–14.3%). Post-operative complications included anastomotic leak (5.4%), recurrent fistula (3.3%) and oesophageal stricture (39%). Seventy-six (60%) of those with type-c anomaly were alive at one-year with returned follow-up, 57(75%) of whom had received PARM. Of these, 24 (42%) developed a stricture, compared to 4 (21%) of those who had not received PARM (adjusted odds ratio 2.60, 95% CI 0.71–9.46, p = 0.147).ConclusionsThis study provides a benchmark for current outcomes and complication rates following OA-TOF repair, with oesophageal stricture causing significant morbidity. The use of PARM appeared ineffective in preventing strictures. This study creates enough doubt about the efficacy of PARM in preventing stricture formation to warrant further investigation of its use with a randomised controlled trial.

ObjectiveThe objective of this study was to develop a Hirschsprung’s disease (HD) core outcome set (COS).MethodsCandidate 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).ResultsEighty-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.ConclusionsThis 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.

ObjectiveTo compare outcomes following totally transanal endorectal pull-through (TTERPT) versus pull-through with any form of laparoscopic assistance (LAPT) for infants with uncomplicated Hirschsprung's disease.DesignSystematic review and meta-analysis.SettingFive hospitals with a paediatric surgical service.Participants405 infants with uncomplicated Hirschsprung's disease.InterventionsTTERPT versus LAPT.Primary and secondary outcome measuresPrimary outcomes: mortality, postoperative enterocolitis, faecal incontinence, constipation, unplanned laparotomy or stoma formation, and injury to abdominal viscera.Secondary outcomesHaemorrhage requiring transfusion of blood products, abscess formation, intestinal obstruction, intestinal ischaemia, enteric fistula formation, urinary incontinence or retention, impotency and duration of procedure.ResultsFive eligible studies comprising 405 patients were identified from 2107 studies. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. Operative duration was 50.29 min shorter with TTERPT (95% CI 39.83 to 60.74, p<0.00001). There were no significant differences identified between TTERPT and LAPT for incidence of postoperative enterocolitis (OR=0.78, 95% CI 0.44 to 1.38, p=0.39), faecal incontinence (OR=0.44, 95% CI 0.09 to 2.20, p=0.32) or constipation (OR=0.84, 95% CI 0.32 to 2.17, p=0.71).ConclusionsThis meta-analysis did not find any evidence to suggest a higher rate of enterocolitis, incontinence or constipation following TTERPT compared with LAPT. Further long-term comparative studies and multicentre data pooling are needed to determine whether a purely transanal approach offers any advantages over a laparoscopically assisted approach to rectosigmoid Hirschsprung's disease.Trial registration numberPROSPERO registry- CRD42013005698.

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.

Background Research studies to inform clinical practice and policy in children and young people with appendicitis are hampered by inconsistent selection and reporting of outcomes. The aim of this study was to develop a core outcome set for reporting all studies of uncomplicated acute appendicitis in children and young people. Methods Systematic literature reviews, qualitative interviews with parents and patients treated for uncomplicated acute appendicitis, and a Study‐Specific Advisory Group informed a long list of outcomes. Outcomes were then prioritized by stakeholders based in the UK (patients, parents, and paediatric and general surgeons) in an online three‐round Delphi consensus process, followed by face‐to‐face consensus meetings. Results A long list of 40 items was scored by 147 key stakeholders in the first Delphi round, of whom 90 completed the two subsequent Delphi rounds. The final core outcome set comprises 14 outcomes: intra‐abdominal abscess, reoperation (including interventional radiology procedure), readmission to hospital, bowel obstruction, wound infection, antibiotic failure, wound complication, negative appendicectomy, recurrent appendicitis, death, patient stress/psychological distress, length of hospital stay, time away from full activity and child's quality of life. Conclusion A core outcome set comprising 14 outcomes across five key domains has been developed for reporting studies in children and young people with uncomplicated acute appendicitis. Further work is required to determine how and when to measure these outcomes.

Objective: To develop a consensus management pathway for children with Paediatric Inflammatory Multisystem Syndrome - Temporally associated with SARS-CoV-2 (PIMS-TS). Design: A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management and research priorities from 98 multidisciplinary participants caring for children with PIMS-TS. 46 participants (47%) completed all three phases. Participants were grouped into three panels and scored each statement from 1 (disagree) to 9 (strongly agree). In phase two participants were shown their panels scores, and in phase three all panels scores. Consensus agreement was defined as ≥70% of participants in each panel scoring the statement 7-9, and <15% scoring 1-3, and consensus disagreement was the opposite of this. Statements which achieved consensus in 2/3 panels were discussed at the consensus meeting, and when ≥70% participants agreed with the statement it achieved consensus. Results: 255 statements were assessed, with consensus agreement achieved for 111 (44%), consensus disagreement for 29 (11%), and no consensus for 115 (45%). The 140 consensus statements were used to derive the consensus management pathway. Conclusions: A national consensus pathway has been developed for children suspected of having the novel syndrome PIMS-TS in a timely, cost-efficient manner, in the midst of a global pandemic. Use of a rapid online Delphi process has made this consensus process possible. 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.