ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework

Abstract

Introduction Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF.

Materials and Methods The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9.

Results Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items.

Conclusion Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

Keywords

esophageal atresia with tracheoesophageal fistula - pediatric surgery - follow-up - management - consensus conference

Publication History

Received: 05 September 2019

Accepted: 04 October 2019

Article published online:
27 November 2019

• References

• 1 Krishnan U, Mousa H, Dall'Oglio L. et al. ESPGHAN-NASPGHAN guidelines for the evaluation and treatment of gastrointestinal and nutritional complications in children with esophageal atresia-tracheoesophageal fistula. J Pediatr Gastroenterol Nutr 2016; 63 (05) 550-570
• 2 Zimmer J, Eaton S, Murchison LE, De Coppi P, Ure BM, Dingemann C. State of play: eight decades of surgery for esophageal atresia. Eur J Pediatr Surg 2019; 29 (01) 39-48
• 3 Wang B, Tashiro J, Allan BJ. et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res 2014; 190 (02) 604-612
• 4 Sfeir R, Bonnard A, Khen-Dunlop N. et al. Esophageal atresia: data from a national cohort. J Pediatr Surg 2013; 48 (08) 1664-1669
• 5 Rintala RJ, Pakarinen MP. Long-term outcome of esophageal anastomosis. Eur J Pediatr Surg 2013; 23 (03) 219-225
• 6 Wijnen RM, Ure B. Bridging the gap—more than surgery only. Eur J Pediatr Surg 2015; 25 (04) 311
• 7 Acher CW, Ostlie DJ, Leys CM, Struckmeyer S, Parker M, Nichol PF. Long-term outcomes of patients with tracheoesophageal fistula/esophageal atresia: survey results from tracheoesophageal fistula/esophageal atresia online communities. Eur J Pediatr Surg 2016; 26 (06) 476-480
• 8 Witt S, Dellenmark-Blom M, Flieder S. et al. Health-related quality of life experiences in children and adolescents born with esophageal atresia: a Swedish-German focus group study. Child Care Health Dev 2019; 45 (01) 79-88
• 9 Flieder S, Dellenmark-Blom M, Witt S. et al. Generic health-related quality of life after repair of esophageal atresia and its determinants within a German-Swedish Cohort. Eur J Pediatr Surg 2019; 29 (01) 75-84
• 10 Vergouwe FW, IJsselstijn H, Wijnen RM, Bruno MJ, Spaander MC. Screening and surveillance in esophageal atresia patients: current knowledge and future perspectives. Eur J Pediatr Surg 2015; 25 (04) 345-352
• 11 Zani A, Eaton S, Hoellwarth ME. et al. International survey on the management of esophageal atresia. Eur J Pediatr Surg 2014; 24 (01) 3-8
• 12 European Reference Networks. Accessed February 25, 2019 at: https://ern-ernica.eu/about/european-reference-networks/
• 13 Wijnen R, Anzelewicz SM, Petersen C, Czauderna P. European reference networks: share, care, and cure-future or dream?. Eur J Pediatr Surg 2017; 27 (05) 388-394
• 14 Rolle U. Centralization of pediatric surgery: European perspective. Eur J Pediatr Surg 2017; 27 (05) 387
• 15 Pakarinen M, Bjørland K, Qvist N, Wester T. Centralized pediatric surgery in the Nordic countries: a role model for Europe?. Eur J Pediatr Surg 2017; 27 (05) 395-398
• 16 Héon-Klin V. European Reference networks for rare diseases: what is the conceptual framework?. Orphanet J Rare Dis 2017; 12 (01) 137
• 17 WHO. Building European Reference Networks in health care-exploring concepts and national practices in the European Union. In: Palm W, Glinos IA, Rechel B, Garel P, Busse R, Figueras J. eds. European Observatory on Health Systems and Policies. Copenhagen, Denmark: World Health Organization; 2013
• 18 Dingemann C, Eaton S, Aksnes G. et al. ERNICA consensus conference on the management of patients with esophageal atresia and tracheoesophageal fistula: diagnostics, preoperative, operative, and postoperative management. Eur J Pediatr Surg 2019;
• 19 The Federation Of Esophageal Atresia And Tracheo-Esophageal Fistula Support Groups E.V . Accessed February 25, 2019 at: http://www.we-are-eat.org/
• 20 Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009; 42 (02) 377-381
• 21 Voxvote. Accessed February 25, 2019 at: www.voxvote.com
• 22 Oxford Centre for Evidence-based Medicine - Levels of Evidence. March 2009. Accessed June 23, 2019 at: https://www.cebm.net/2009/06/oxford-centre-evidence-based-medicine-levels-evidence-march-2009/
• 23 Cloud DT. Anastomotic technic in esophageal atresia. J Pediatr Surg 1968; 3 (05) 561-564
• 24 Stenström P, Anderberg M, Börjesson A, Arnbjornsson E. Prolonged use of proton pump inhibitors as stricture prophylaxis in infants with reconstructed esophageal atresia. Eur J Pediatr Surg 2017; 27 (02) 192-195
• 25 Donoso F, Lilja HE. Risk factors for anastomotic strictures after esophageal atresia repair: prophylactic proton pump inhibitors do not reduce the incidence of strictures. Eur J Pediatr Surg 2017; 27 (01) 50-55
• 26 Berger M, Ure B, Lacher M. Mitomycin C in the therapy of recurrent esophageal strictures: hype or hope?. Eur J Pediatr Surg 2012; 22 (02) 109-116
• 27 Sweed AS, Fawaz SA, Ezzat WF, Sabri SM. A prospective controlled study to assess the use of mitomycin C in improving the results of esophageal dilatation in post corrosive esophageal stricture in children. Int J Pediatr Otorhinolaryngol 2015; 79 (01) 23-25
• 28 Lévesque D, Baird R, Laberge JM. Refractory strictures post-esophageal atresia repair: what are the alternatives?. Dis Esophagus 2013; 26 (04) 382-387
• 29 Chapuy L, Pomerleau M, Faure C. Topical mitomycin-C application in recurrent esophageal strictures after surgical repair of esophageal atresia. J Pediatr Gastroenterol Nutr 2014; 59 (05) 608-611
• 30 Madadi-Sanjani O, Zimmer J, Gosemann JH, Ure BM, Lacher M, Boehm R. Topical mitomycin C application in pediatric patients with recurrent esophageal strictures-report on unfavorable results. Eur J Pediatr Surg 2018; 28 (06) 539-546
• 31 Gandhi RP, Cooper A, Barlow BA. Successful management of esophageal strictures without resection or replacement. J Pediatr Surg 1989; 24 (08) 745-749
• 32 Zamiara P, Thomas KE, Connolly BL, Lane H, Marcon MA, Chiu PP. Long-term burden of care and radiation exposure in survivors of esophageal atresia. J Pediatr Surg 2015; 50 (10) 1686-1690
• 33 Ten Kate CA, Vlot J, Sloots CEJ, van den Akker ELT, Wijnen RMH. The effect of intralesional steroid injections on esophageal strictures and the child as whole: a case series. J Pediatr Surg 2019;
• 34 Peycelon M, Faraj S, Leclair MD, Bonnard A. French connection between specialized and routine pediatric surgical care. Eur J Pediatr Surg 2017; 27 (05) 410-415
• 35 Wijnen MH. Centralization of pediatric surgery in The Netherlands. Eur J Pediatr Surg 2017; 27 (05) 407-409
• 36 Lampela H, Ritvanen A, Kosola S. et al. National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes. Scand J Gastroenterol 2012; 47 (01) 99-107
• 37 Pintér A, Vajda P. Centralization of pediatric surgery in Hungary. Eur J Pediatr Surg 2017; 27 (05) 429-430
• 38 Riccipetitoni G, Gamba P, Lima M. et al. Bella Italia: specialized pediatric surgical care in Italy. Eur J Pediatr Surg 2017; 27 (05) 422-428
• 39 Durkin N, Davenport M. Centralization of pediatric surgical procedures in the United Kingdom. Eur J Pediatr Surg 2017; 27 (05) 416-421
• 40 The European Parliament and of the Council. Commission Delegated Decision 2014/286/EU. I. 2014
• 41 Delisle VC, Gumuchian ST, Rice DB. et al. Perceived benefits and factors that influence the ability to establish and maintain patient support groups in rare diseases: a scoping review. Patient 2017; 10 (03) 283-293
• 42 European Commission Directorate-General for Health and Food Safety. Rare Disease European Reference Networks: Addendum to Eucerd recommendations of January 2013: the decision of the commission expert group on rare diseases. Off J Eur Union 2013/c 219/04: 4-7
• 43 Taruscio D, Gentile AE, Evangelista T, Frazzica RG, Bushby K, Montserrat AM. Centres of Expertise and European Reference Networks: key issues in the field of rare diseases. The EUCERD recommendations. Blood Transfus 2014; 12 (Suppl. 03) s621-s625
• 44 Svoboda E, Fruithof J, Widenmann-Grolig A. et al. A patient led, international study of long term outcomes of esophageal atresia: EAT 1. J Pediatr Surg 2018; 53 (04) 610-615
• 45 Anzelewicz S, Garnier H, Rangaswami A, Czauderna P. Cultural, geographical and ethical questions when looking to enroll pediatric patients in rare disease clinical trials. Expert Opin Orphan Drugs 2017; 5 (08) 613-621
• 46 Dingemann C, Meyer A, Kircher G. et al. Long-term health-related quality of life after complex and/or complicated esophageal atresia in adults and children registered in a German patient support group. J Pediatr Surg 2014; 49 (04) 631-638