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Eur J Pediatr Surg 2020; 30(06): 475-482
DOI: 10.1055/s-0039-3400284
Review Article

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

Carmen Dingemann
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Simon Eaton
2   Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health, University College London 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, Bambino Gesù Children's Hospital-Research Institute, Rome, Italy
,
Kate M. Cross
5   Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
,
Paolo De Coppi
5   Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
6   Department of Paediatric Surgery & Metabolic Biochemistry, UCL Great Ormond Street Institute of Child Health Library, London, United Kingdom
,
JoAnne Fruithof
7   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - VOKS, Lichtenvoorde, The Netherlands
,
Piergiorgio Gamba
8   Department of Pediatric Surgery, University of Padua, Padua, Italy
,
Steffen Husby
9   Department of Pediatric Gastroenterology, Hans Christian Andersen Children's Hospital, Odense University Hospital, Odense, Denmark
,
Antti Koivusalo
10   Department of Pediatric Surgery, University of Helsinki, Helsinki, Finland
,
Lars Rasmussen
11   Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark
,
Rony Sfeir
12   Department of Pediatric Surgery CRACMO, Centre Hospitalier Régional Universitaire de Lille, Lille, France
,
Graham Slater
13   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - TOFS, Nottingham, United Kingdom
,
Jan F. Svensson
14   Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
,
David C. Van der Zee
15   Department of Pediatric Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
,
Lucas M. Wessel
16   Department of Pediatric Surgery, University of Mannheim, Medical Faculty of Heidelberg, Mannheim, Germany
,
Anke Widenmann-Grolig
17   Esophageal Atresia and Tracheo-Esophageal Fistula Support Federation & - KEKS, Stuttgart, Germany
,
Rene Wijnen
18   Department of Pediatric Surgery, Erasmus MC Rotterdam, Rotterdam, The Netherlands
,
Benno M. Ure
1   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
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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

Supplementary Material



Publication History

Received: 05 September 2019

Accepted: 04 October 2019

Article published online:
27 November 2019

© 2020. Thieme. All rights reserved.

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  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 5 Rintala RJ, Pakarinen MP. Long-term outcome of esophageal anastomosis. Eur J Pediatr Surg 2013; 23 (03) 219-225
    Article in Thieme ConnectPubMedGoogle Scholar
  • 6 Wijnen RM, Ure B. Bridging the gap—more than surgery only. Eur J Pediatr Surg 2015; 25 (04) 311
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 12 European Reference Networks. Accessed February 25, 2019 at: https://ern-ernica.eu/about/european-reference-networks/
    Google Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 14 Rolle U. Centralization of pediatric surgery: European perspective. Eur J Pediatr Surg 2017; 27 (05) 387
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 16 Héon-Klin V. European Reference networks for rare diseases: what is the conceptual framework?. Orphanet J Rare Dis 2017; 12 (01) 137
    CrossrefPubMedGoogle Scholar
  • 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
    Google Scholar
  • 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; DOI: 10.1055/s-0039-1693116.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Google Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 28 Lévesque D, Baird R, Laberge JM. Refractory strictures post-esophageal atresia repair: what are the alternatives?. Dis Esophagus 2013; 26 (04) 382-387
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 31 Gandhi RP, Cooper A, Barlow BA. Successful management of esophageal strictures without resection or replacement. J Pediatr Surg 1989; 24 (08) 745-749
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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; DOI: 10.1016/j.jpedsurg.2019.05.013.
    CrossrefPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 35 Wijnen MH. Centralization of pediatric surgery in The Netherlands. Eur J Pediatr Surg 2017; 27 (05) 407-409
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 37 Pintér A, Vajda P. Centralization of pediatric surgery in Hungary. Eur J Pediatr Surg 2017; 27 (05) 429-430
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    Article in Thieme ConnectPubMedGoogle Scholar
  • 39 Durkin N, Davenport M. Centralization of pediatric surgical procedures in the United Kingdom. Eur J Pediatr Surg 2017; 27 (05) 416-421
    Article in Thieme ConnectPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    PubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar
  • 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
    CrossrefPubMedGoogle Scholar