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

Antti Koivusalo, Benno M Ure, Rene Wijnen, Anke Widenmann-Grolig, Lucas M Wessel, David C Van der Zee, Jan F Svensson, Graham Slater, Rony Sfeir, Lars Rasmussen, Carmen Dingemann, Steffen Husby, Piergiorgio Gamba, JoAnne Fruithof, Paolo De Coppi, Kate M Cross, Pietro Bagolan, Gunnar Aksnes, Simon Eaton

Journal: European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie 2021;30(6):475-482

PMID: 31777030

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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.

Address: Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.; Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health, University College London Institute of Child Health, London, United Kingdom.; Department of Pediatric Surgery, Oslo University Hospital, Oslo, Norway.; Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital-Research Institute, Rome, Italy.; Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom.; Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health Library, London, United Kingdom.; Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - VOKS, Lichtenvoorde, The Netherlands.; Department of Pediatric Surgery, University of Padua, Padua, Italy.; Department of Pediatric Gastroenterology, Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark.; Department of Pediatric Surgery, University of Helsinki, Helsinki, Finland.; Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark.; Department of Pediatric Surgery CRACMO, Centre Hospitalier Régional Universitaire de Lille, Lille, France.; Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - TOFS, Nottingham, United Kingdom.; Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden.; Department of Pediatric Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.; Department of Pediatric Surgery, University of Mannheim, Medical Faculty of Heidelberg, Mannheim, Germany.; Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - KEKS, Stuttgart, Germany.; Department of Pediatric Surgery, Erasmus MC Rotterdam, Rotterdam, The Netherlands.

DOI: 10.1055/s-0039-3400284

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MeSH Terms: Anastomosis, Surgical, Child, Consensus Development Conferences as Topic, Esophageal Atresia, Europe, Humans, Tracheoesophageal Fistula