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

Dingemann, Carmen; Eaton, Simon; Aksnes, Gunnar; Bagolan, Pietro; Cross, Kate M; De Coppi, Paolo; Fruithof, JoAnne; Gamba, Piergiorgio; Goldschmidt, Imeke; Gottrand, Frederic; Pirr, Sabine; Rasmussen, Lars; Sfeir, Rony; Slater, Graham; Suominen, Janne; Svensson, Jan F; Thorup, Joergen M; Tytgat, Stefaan HAJ; van der Zee, David C; Wessel, Lucas; Widenmann-Grolig, Anke; Wijnen, Rene; Zetterquist, Wilhelm; Ure, Benno M

doi:10.1055/s-0040-1713932

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

Author:

Dingemann, Carmen

Eaton, Simon ; Aksnes, Gunnar ; Bagolan, Pietro ; Cross, Kate M ; De Coppi, Paolo ; Fruithof, JoAnne ; Gamba, Piergiorgio ; Goldschmidt, Imeke ; Gottrand, Frederic ; Pirr, Sabine ; Rasmussen, Lars ; Sfeir, Rony ; Slater, Graham ; Suominen, Janne ; Svensson, Jan F ; Thorup, Joergen M ; Tytgat, Stefaan HAJ ; van der Zee, David C ; Wessel, Lucas ; Widenmann-Grolig, Anke ; Wijnen, Rene ; Zetterquist, Wilhelm ; Ure, Benno M

Keywords:

CHILDREN, COLONIC INTERPOSITION, COMPLICATIONS, consensus conference, EUROPEAN REFERENCE NETWORKS, follow-up, FOLLOW-UP, Life Sciences & Biomedicine, long-gap esophageal atresia, management, OUTCOMES, pediatric surgery, Pediatrics, PEDIATRIC-SURGERY, REPAIR, REPLACEMENT, Science & Technology, Surgery, TRACHEOESOPHAGEAL FISTULA, Aftercare, Esophageal Atresia, Esophagoplasty, Humans, Infant, Newborn, Perioperative Care, Treatment Outcome, 1114 Paediatrics and Reproductive Medicine, 3202 Clinical sciences, 3213 Paediatrics

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.