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

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

doi:10.1055/s-0039-3400284

European Journal of Pediatric Surgery, Inhaltsverzeichnis

Eur J Pediatr Surg 2020; 30(06): 475-482
DOI: 10.1055/s-0039-3400284

Review Article

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

Carmen Dingemann

¹Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany

,

Simon Eaton

²Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health, University College London Institute of Child Health, London, United Kingdom

,

Gunnar Aksnes

³Department of Pediatric Surgery, Oslo University Hospital, Oslo, Norway

,

Pietro Bagolan

⁴Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital-Research Institute, Rome, Italy

,

Kate M. Cross

⁵Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

,

Paolo De Coppi

⁵Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom

⁶Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health Library, London, United Kingdom

,

JoAnne Fruithof

⁷Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - VOKS, Lichtenvoorde, The Netherlands

,

Piergiorgio Gamba

⁸Department of Pediatric Surgery, University of Padua, Padua, Italy

,

Steffen Husby

⁹Department of Pediatric Gastroenterology, Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark

,

Antti Koivusalo

¹⁰Department of Pediatric Surgery, University of Helsinki, Helsinki, Finland

,

Lars Rasmussen

¹¹Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark

,

Rony Sfeir

¹²Department of Pediatric Surgery CRACMO, Centre Hospitalier Régional Universitaire de Lille, Lille, France

,

Graham Slater

¹³Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - TOFS, Nottingham, United Kingdom

,

Jan F. Svensson

¹⁴Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

,

David C. Van der Zee

¹⁵Department of Pediatric Surgery, University Medical Center Utrecht, Utrecht, The Netherlands

,

Lucas M. Wessel

¹⁶Department of Pediatric Surgery, University of Mannheim, Medical Faculty of Heidelberg, Mannheim, Germany

,

Anke Widenmann-Grolig

¹⁷Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - KEKS, Stuttgart, Germany

,

Rene Wijnen

¹⁸Department of Pediatric Surgery, Erasmus MC Rotterdam, Rotterdam, The Netherlands

,

Benno M. Ure

¹Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany

› Institutsangaben

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.

Keywords

esophageal atresia with tracheoesophageal fistula - pediatric surgery - follow-up - management - consensus conference

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