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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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© 2022. Crown.)

Background: Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications., Aims: We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility., Methods: We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought., Results: A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms., Conclusions: This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial., Type of Study: Prognosis study., Level of Evidence: Level 3., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years., Methods: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes., Results: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%., Conclusions: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable., Level of Evidence: IV., Competing Interests: Declaration of competing interest None., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

There have been major advances in the surgery for oesophageal atresia (OA) and tracheo-oesophageal fistula(TOF) with survival now exceeding 90%. The standard open approach to OA and distal TOF has been well described and essentially unchanged for the last 60 years. Improved survival in recent decades is most attributable to advances in neonatal anaesthesia and perioperative care. Recent surgical advances include the use of thoracoscopic surgery for the repair of OA/TOF and in some centres isolated OA, thereby minimising the long term musculo-skeletal morbidity associated with open surgery. The introduction of growth induction by external traction (Foker procedure) for the treatment of long-gap OA has provided an important tool enabling increased preservation of the native oesophagus. Despite this, long-gap OA still poses a number of challenges, and oesophageal replacement still may be required in some cases., (Copyright © 2016 Elsevier Ltd. All rights reserved.)