Eur J Pediatr Surg 2021; 31(03): 214-225
DOI: 10.1055/s-0040-1713932
Original Article

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

Carmen Dingemann
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Simon Eaton
2   NIHR Biomedical Research Centre at UCLH, Developmental Biology and Cancer Programme, 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, Research Institute, Bambino Gesù Children's Hospital, Rome, Italy
,
Kate M. Cross
5   Department of Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
,
Paolo De Coppi
2   NIHR Biomedical Research Centre at UCLH, Developmental Biology and Cancer Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom
5   Department of 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
,
Imeke Goldschmidt
8   Department of Pediatric Gastroenterology and Hepatology, Hannover Medical School, Hannover, Germany
,
9   Department of Pediatric Gastroenterology, Hepatology and Nutrition, Reference Center for Rare Esophageal Diseases, CHU Lille, University of Lille, Lille, France
,
Sabine Pirr
10   Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany
,
Lars Rasmussen
11   Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark
,
Rony Sfeir
12   Department of Pediatric Surgery, Reference Center for Rare Esophageal Diseases, CHU Lille, University of Lille, Lille, France
,
Graham Slater
13   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and TOFS, Nottingham, United Kingdom
,
Janne Suominen
14   Department of Pediatric Surgery, University of Helsinki, Helsinki, Finland
,
Jan F. Svensson
15   Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
,
Joergen M. Thorup
16   Department of Pediatric Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
,
Stefaan H. A. J. Tytgat
17   Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
,
David C van der Zee
17   Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
,
Lucas Wessel
18   Department of Pediatirc Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany
,
Anke Widenmann-Grolig
19   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and KEKS, Stuttgart, Germany
,
René Wijnen
20   Department of Pediatric Surgery, Erasmus MC, Rotterdam, The Netherlands
,
Wilhelm Zetterquist
21   Department of Woman and Child Health, Karolinska University Hospital, Stockholm, Sweden
,
Benno M. Ure
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
› Author Affiliations
Funding ERNICA provided financial support for the conference.

Abstract

Introduction Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect.

Materials and Methods Twenty-four ERNICA representatives from nine European countries participated. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing items on perioperative, surgical, and long-term management, and literature review. The 2-day conference was held in Berlin in November 2019. Anonymous voting was conducted via an internet-based system using a 1 to 9 scale. Consensus was defined as ≥75% of those voting scoring 6 to 9.

Results Ninety-seven items were generated. Complete consensus (100%) was achieved on 56 items (58%), e.g., avoidance of a cervical esophagostomy, promotion of sham feeding, details of delayed anastomosis, thoracoscopic pouch mobilization and placement of traction sutures as novel technique, replacement techniques, and follow-up. Consensus ≥75% was achieved on 90 items (93%), e.g., definition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougienage to enable delayed anastomosis. Nineteen items (20%), e.g., methods of gap measurement were discussed controversially (range 1–9).

Conclusion This is the first consensus conference on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to improve patient care.



Publication History

Received: 09 April 2020

Accepted: 25 May 2020

Publication Date:
15 July 2020 (online)

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