Eur J Pediatr Surg 2020; 30(04): 326-336
DOI: 10.1055/s-0039-1693116
Original Article
Georg Thieme Verlag KG Stuttgart · New York

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management

Carmen Dingemann
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Lower Saxony, Germany
,
Simon Eaton
2   Department of Paediatric Surgery and Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health, London, United Kingdom
,
Gunnar Aksnes
3   Department of Pediatric Surgery, Oslo University Hospital, Oslo, Norway
,
Pietro Bagolan
4   Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Kate M. Cross
5   Department of Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
,
Paolo De Coppi
2   Department of Paediatric Surgery and Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health, London, United Kingdom
5   Department of Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
,
JoAnne Fruithof
6   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and—VOKS, Lichtenvoorde, The Netherlands
,
Piergiorgio Gamba
7   Department of Pediatric Surgery, University of Padua, Padua, Italy
,
Steffen Husby
8   Department of Pediatric Gastroenterology, Hans Christian Andersen Children’s Hospital, Odense University Hospital, Odense, Denmark
,
Antti Koivusalo
9   Department of Pediatric Surgery, University of Helsinki, Children's Hospital, Helsinki, Finland
,
Lars Rasmussen
10   Department of Pediatric Surgery, Odense University hospital, Odense, Denmark
,
Rony Sfeir
11   Department of Pediatric Surgery, Centre Hospitalier Régional Universitaire de Lille, Lille, France
,
Graham Slater
12   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and—TOFS, Nottingham, United Kingdom
,
Jan F. Svensson
13   Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
,
David C. Van der Zee
14   Department of Pediatric Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
,
Lucas M. Wessel
15   Department of Pediatric Surgery, University of Mannheim, Medical Faculty of Heidelberg, Mannheim, Germany
,
Anke Widenmann-Grolig
16   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and KEKS, Stuttgart, Germany
,
Rene Wijnen
17   Department of Pediatric Surgery, Erasmus MC Sophia Ringgold standard institution, Rotterdam, The Netherlands
,
Benno M. Ure
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Lower Saxony, Germany
› Author Affiliations
Funding ERNICA provided financial support for the conference. S.E., K.M.C., and P.D.C. gratefully acknowledge support by the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR, or the UK Department of Health.
Further Information

Publication History

29 March 2019

21 May 2019

Publication Date:
02 July 2019 (online)

Abstract

Introduction Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature.

Materials and Methods Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9.

Results Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1–9). Eight of these (62%) did not reach consensus.

Conclusion Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

Supplementary Material