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

Carmen Dingemann*, Simon Eaton, Gunnar Aksnes, Pietro Bagolan, Kate M. Cross, Paolo De Coppi, Joanne Fruithof, Piergiorgio Gamba, Imeke Goldschmidt, Frederic Gottrand, Sabine Pirr, Lars Rasmussen, Rony Sfeir, Graham Slater, Janne Suominen, Jan F. Svensson, Joergen M. Thorup, Stefaan H.A.J. Tytgat, David C. Van Der Zee, Lucas WesselAnke Widenmann-Grolig, René Wijnen, Wilhelm Zetterquist, Benno M. Ure

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

35 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)214-225
Number of pages12
JournalEuropean Journal of Pediatric Surgery
Issue number3
Publication statusPublished - 1 Jun 2021

Bibliographical note

Funding Information:
ERNICA provided financial support for the conference.

Publisher Copyright:
© 2021 Georg Thieme Verlag. All rights reserved.


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