Carmen Dingemann1, Simon Eaton2, Gunnar Aksnes3, Pietro Bagolan4, Kate M Cross5, Paolo De Coppi2,5, JoAnne Fruithof6, Piergiorgio Gamba7, Imeke Goldschmidt8, Frederic Gottrand9, Sabine Pirr10, Lars Rasmussen11, Rony Sfeir12, Graham Slater13, Janne Suominen14, Jan F Svensson15, Joergen M Thorup16, Stefaan H A J Tytgat17, David C van der Zee17, Lucas Wessel18, Anke Widenmann-Grolig19, René Wijnen20, Wilhelm Zetterquist21, Benno M Ure1. 1. Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany. 2. NIHR Biomedical Research Centre at UCLH, Developmental Biology and Cancer Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom. 3. Department of Pediatric Surgery, Oslo University Hospital, Oslo, Norway. 4. Department of Medical and Surgical Neonatology, Research Institute, Bambino Gesù Children's Hospital, Rome, Italy. 5. Department of Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom. 6. Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and VOKS, Lichtenvoorde, The Netherlands. 7. Department of Pediatric Surgery, University of Padua, Padua, Italy. 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. 10. Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany. 11. Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark. 12. Department of Pediatric Surgery, Reference Center for Rare Esophageal Diseases, CHU Lille, University of Lille, Lille, France. 13. Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and TOFS, Nottingham, United Kingdom. 14. Department of Pediatric Surgery, University of Helsinki, Helsinki, Finland. 15. Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden. 16. Department of Pediatric Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. 17. Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands. 18. Department of Pediatirc Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany. 19. Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation and KEKS, Stuttgart, Germany. 20. Department of Pediatric Surgery, Erasmus MC, Rotterdam, The Netherlands. 21. Department of Woman and Child Health, Karolinska University Hospital, Stockholm, Sweden.
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. Thieme. All rights reserved.
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. Thieme. All rights reserved.