ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework

Abstract

Introduction Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. Materials and Methods The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. Results Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. Conclusion Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management

“…The general methodological approach has been recently published when presenting the results of the first ERNICA consensus conference. 14,15 It consisted of two parts: (1) diagnostics, preoperative, operative, and postoperative management 14 and (2) follow-up and framework. 15 The conference dealt exclusively with the management of patients with long-gap esophageal atresia, and took place in Berlin on November 13-14, 2019.…”

“…14,15 It consisted of two parts: (1) diagnostics, preoperative, operative, and postoperative management 14 and (2) follow-up and framework. 15 The conference dealt exclusively with the management of patients with long-gap esophageal atresia, and took place in Berlin on November 13-14, 2019. In total, 24 ERNICA representatives from nine European countries participated: 16 pediatric surgeons, 2 pediatric gastroenterologists, 1 neonatologist, 1 pediatric pulmonologist, 3 representatives of patient support groups acting under the umbrella of the Federation of Esophageal Atresia and Tracheo-Esophageal Fistula Support Groups (EAT).…”

“…With regard to literature selection, publications with the highest grade of evidence according to the Centre for Evidence-Based Medicine (CEBM) classification were suggested to be preferred 17 as previously reported. 14,15 Literature was distributed and made available to all participants via a Dropbox (Dropbox Inc., San Francisco, California, United States, 2007) link prior to the conference.…”

“…[10][11][12][13] ERNICA organized a first consensus conference on the pre-, peri-, and postoperative management as well as on the follow-up of patients with esophageal atresia with tracheoesophageal fistula in October 2018. 14,15 ERNICA has now conducted a second consensus conference which focused on the management of patients with long-gap esophageal atresia based on expert opinions referring to the latest literature. The aim of this conference was to develop clear and uniform statements in this respect.…”

Health-Related Quality of Life in Patients after Repair of Esophageal Atresia: A Review of Current Literature

“…17 The risk of developing scoliosis and psychological impairments due to surgical scar(s) 18,19 further illustrates the complexity of problems that this patient group may experience. Multidisciplinary and standardized follow-up care has been recommended in patients with EA, including guidelines published by the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) 20 and The European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)/The North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN). 13 Moreover, there is also a focus on the patients' subjective experiences of how the disease and/or treatment influences their physical, psychological, and social well-being, which makes up their health-related quality of life (HRQoL).…”