Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management - ERNICA Consensus Conference

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management Carmen Dingemann 1 Simon Eaton 2 Gunnar Aksnes 3 Pietro Bagolan 4 Kate M. Cross 5 Paolo De Coppi 2,5 JoAnne Fruithof 6 Piergiorgio Gamba 7 Imeke Goldschmidt 8 Frederic Gottrand 9 Sabine Pirr 10 Lars Rasmussen 11 Rony Sfeir 12 Graham Slater 13 Janne Suominen 14 Jan F. Svensson 15 Joergen M. Thorup 16 Stefaan H. A. J. Tytgat 17 David C van der Zee 17 Lucas Wessel 18 Anke Widenmann-Grolig 19 René Wijnen 20 Wilhelm Zetterquist 21 Benno M. Ure 1 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, Lichte nvoorde, 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 Eur J Pediatr Surg 2021;31:214 –225. Address for correspondence Carmen Dingemann, MD, Department of Pediatric Surgery, Hannover Medical School, Carl-Neuberg-Str. 1, Hannover 30625, Lower Saxony, Germany (e-mail: dingemann.ca rmen@mh-hannover.de; carmendingemann@gmail.com). 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 Keywords ► long-gap esophageal atresia ► pediatric surgery ► management ► follow-up ► consensus conference 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 conferenc e 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 s urvey, formulation of a final list containing items on perioper- ative, surgical, and long-term management, and literature review. The 2-day confer- ence was held in Berlin in November 2019. Anonymous voting was conducted via an received April 9, 2020 accepted May 25, 2020 published online July 15, 2020 © 2020. Thieme. All rights reserved. Georg Thieme Verlag KG, Rüdigerstraße 14, 70469 Stuttgart, Germany DOI https://doi.org/ 10.1055/s-0040-1713932. ISSN 0939-7248. Original Article214 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Article published online: 2020-07-15 Introduction In 1 out of 10 patients with esophageal atresia, primary anasto- mosis of the two esophageal ends is not feasible1,2 which poses an additional major challenge to restore continuity. 1–7 The rarity of the condition, the variability in case de finition, multiple approaches to management and follow-up, and the heterogeneity of the reported outcomes contribute to this challenge. 3 In addition, evidence supporting best practice for long-gap esophageal atresia is limited, mostly consisting of single-center retrospective reviews with low numbers of patients, and low quality of data to date. 1–5 As a result, there is a variety of coexisting protocols on the perioperative, surgi- cal, and long-term management of patients with long-gap esophageal atresia based on opinion rather than on evidence. This was con firmed in a recent survey of pediatric surgeons demonstrating the variability in opinion, in terms of both case definition and preferred operative approaches.8 The European Reference Network for Rare Inherited Con- genital Anomalies (ERNICA) has been established in response to the European Commission ’s call for the setup of European Reference Networks for rare diseases in 2017. 9 The network seeks to deliver high standards of care, to build capacity where there is lack of knowledge and infrastructure, and to promote optimal patient care for rare inherited and congenital digestive tract-related disorders from pediatric age to adulthood. 10–13 ERNICA organized a first consensus conference on the pre- , peri-, and postoperative management as well as on the follow-up of patients with esophageal atresia with trache- oesophageal fistula in October 2018. 14,15 ERNICA has now conducted a second consensus conference which focused on the management of patients with long-gap esophageal atre- sia based on expert opinions referring to the latest literature. The aim of this conference was to develop clear and uniform statements in this respect. Materials and Methods The general methodological approach has been recently published when presenting the results of the first ERNICA consensus conference. 14,15 It consisted of two parts: (1) diagnostics, preoperative, operative, and postoperative man- agement 14 and (2) follow-up and framework. 15 The conference dealt exclusively with the management of patients with long-gap esophageal atresia, and took place in Berlin on November 13 –14, 2019. In total, 24 ERNICA representatives from nine European countries participated: 16 pediatric surgeons, 2 pediatric gastroenterologists, 1 neonatologist, 1 pediatric pulmonologist, 3 representatives of patient support groups acting under the umbrella of the Federation of Esophageal Atresia and Tracheo-Esophageal Fistula Support Groups (EAT). 16 One nonsurgeon method- ologist (S.E.) took part in all steps of the preparation and the conference itself. With regard to literature selection, publications with the highest grade of evidence according to the Centre for Evi- dence-Based Medicine (CEBM) classi fication were suggested to be preferred 17 as previously reported. 14,15 Literature was distributed and made available to all participants via a Dropbox (Dropbox Inc., San Francisco, California, United States, 2007) link prior to the conference. The preparation and implementation of the conference included the following steps 14,15: (1) generation of a list of items; (2) prioritization of the items using the online REDCap electronic data capture tools 18; (3) literature-based discus- sion of all items on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia during the conference, formulation of statements; (4) anon- ymous voting via the internet-based system VoxVote (Vox- Vote, Breda, The Netherlands) using a 1 to 9 scale. 19 It was suggested to the participants that they abstained from voting on any individual item when they felt that they had no expertise or an opinion on that item. Therefore, participants were allowed to vote online “no relevant expertise on this statement.” As a result, the number of scoring participants varied for individual statements. Consensus was de fined as /C21 75% of those voting scored 6, 7, 8, or 9, excluding those who declared no relevant expertise on that statement. 14,15 The wordings of the statements on items were updated during the discussion by the participants of the conference internet-based system using a 1 to 9 scale. Consensus was de fined as /C21 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 /C21 75% was achieved on 90 items (93%), e.g., de finition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougien- age 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. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al. 215 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. and prepared for voting by the nonsurgical methodologist (S. E.) who did not himself vote. The final scores and the consensus results were shown to all participants after all votes were obtained, but individual scores remained anonymous. Details of the discussions, in particular the controversial aspects, were documented throughout the conference by C.D. Results and Consensus Statements Item Generation and Prioritization The systematic literature search and the discussion of the members of the ERNICA Workstream Congenital Malforma- tions and Diseases of the Esophagus during the third ERNICA Annual Meeting in Padua, Italy on April 11–12, 2019, resulted in a total of 87 items. After the online prioritization phase, two items were ex- cluded. Following the participants’ suggestions, one new item was added. Consequently, the list included 86 items prior to the conference, for which literature was obtained and circulated. As a result of the presentations by the domain leaders and active discussion during the conference, 14 items were excluded, and 25 were added as some items were split into several separate questions. Finally, 97 items were reworded and confirmed for voting. This included 8 items in the domain Diagnostics, 4 items in the domain Definitions, 43 items in the domain Esophageal Reconstruction , 9 items in the domain Esophageal Replace- ment, 6 items in the domain Postoperative Management ,2 3 items in the domain Follow-up, and 4 items in the domain Framework ( ►Tables 1 –7). Several items of the domain Follow-up have been adopted from the guidelines released by theEuropean Society for Pediat- ric Gastroenterology Hepatology and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hep- atology and Nutrition20 (NASPGHAN) and were discussed from a surgical perspective. Some items had already been discussed in a different context during thefirst ERNICA consensus conference on the management of patients with esophageal atresia and tracheoesophagealfistula. Therefore, the wording might be the same or modified to some extent, but was considered essential to be rediscussed to determine whether the statement was also relevant to the group of patients with long-gap esophageal atresia. Consensus Detailed results on the consensus among participants of the conference are summarized in ►Tables 1 to 7. For 19 items (20%), the results ranged from 1 to 9 re flecting controversial opinions in these cases. In six of these (32%), no consensus was reached. ►Table 8 depicts the general distribution of voting results. There was a deviation from the established voting process using the 1 to 9 scale for one statement voting on the preferred option for esophageal replacement. In this case, participants were asked to vote on only one single option. Nine (39%) participants preferred gastric transposition, 4 (17%) participants preferred jejunal interposition, and 3 (13%) participants preferred colonic interposition for esoph- ageal replacement ( ►Fig. 1 ). For this vote, seven (30%) participants abstained from voting. Controversial Items Discussed without Voting Several items were discussed controversially, and the par- ticipants agreed verbally that more data from future research should be available before a meaningful question and vote Table 1 Diagnostics Statement Consensus % Votes Median (range) 1a A nasogastric tube 10 Fr or larger (modi fied for preterm infants) should be routinely inserted as a diagnostic procedure in cases with suspected esophageal atresia þ 100 21/21 9 (8 –9) 2a A thoracoabdominal X-ray should be routinely performed þ 100 21/21 9 (9 –9) 3b An ultrasound of the abdomen (including kidney/urinary tract) should be routinely performed within the first week of life þ 100 20/20 9 (7 –9) 4a Echocardiography should be routinely performed, especially to exclude a right descending aorta þ 100 21/21 9 (9 –9) 5a A contrast study of a potential upper esophageal pouch should be routinely performed as a preoperative diagnostic procedure /C0 16.7 3/18 2 (1 –9) 6 Gap measurement by bougies via the upper and lower bougies/gastroscope via the lower pouch is a viable option þ 94.7 18/19 9 [5 –9] 7 Gap measurement by contrast study (upper and lower pouches) is a viable option /C0 40 8/20 3 (1 –9) 8 Ab o u g i ei nt h eu p p e rp o u c ha tt h et i m eo ft r a c h e o s c o p yf o rd i s t a l fistula is viable option for gap measurement þ 83.3 15/18 8 (2 –9) Abbreviation: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies. aThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula. bThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula, but in another context. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al.216 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Table 2 Definitions Statement Consensus % Votes Median (range) 1 Any esophageal atresia without air in the abdomen should be assumed to be a long gap þ 100 22/22 9 (7 –9) 2 Only patients with esophageal atresia Gross types A and B should be considered as long gap /C0 42.9 9/21 5 (1 –9) 3 Patients with a distal tracheoesophageal fistula at the carina or below should be considered as long gap /C0 54.5 12/22 6.5 (1 –9) 4 Any esophageal atresia with a gap of three vertebral bodies or more should be considered as long-gap þ 76.2 16/21 8 (1 –9) Table 3 Esophageal reconstruction Statement C o n s e n s u s% V o t e sM e d i a n (range) a. Initial management before reconstruction 1a A replogle tube should be routinely placed into the upper esophageal pouch to allow continuous low pressure suction þ 100 22/22 9 (6 –9) 2 Gastrostomy should usually be performed initially to allow enteral feeding and to stimulate growing of the stomach þ 100 22/22 9 (6 –9) 3 Formation of a cervical esophagostomy should be avoided þ 100 21/21 9 (8 –9) 4 Bougienage of the proximal and/or distal stump to enable delayed primary anastomosis should be avoided þ 78.9 15/19 9 (2 –9) 5 Tracheobronchoscopy under spontaneous breathing should be performed in all patients þ 100 22/22 9 (8 –9) 6 Tracheobronchoscopy should evaluate the presence of vocal cords, airway anomalies (e.g., cleft), proximal/distal fistula location, and tracheobronchomalacia þ 100 23/23 9 (9 –9) 7 Parents should be routinely informed during counseling about all different surgical options (replacement strategies, lengthening procedure, timing, minimal invasive, and conventional techniques) þ 81.8 18/22 9 (1 –9) 8 Parents should be able to request a second opinion and made aware of patient support organizations þ 100 23/23 9 (6 –9) 9 Pain assessment and management protocols should be applied þ 100 23/23 9 (7 –9) 10 Parental involvement and training are an essential integral part of care þ 100 23/23 9 (8 –9) 11 Early oral stimulation, including sensory stimulation and sham feeding, is required to prevent abnormal oral feeding behavior, especially in the case of delayed anastomosis þ 100 23/23 9 (7 –9) 12 Sham feeding should be performed as soon as possible, including when a Replogle tube is in place þ 100 23/23 9 (6 –9) 13 Professional nutritional assessment and support are mandatory to prevent undernutrition þ 100 23/23 9 (6 –9) b. Principle statements on esophageal reconstruction techniques 14 Preserving the native esophagus shou ld be preferred as initial management þ 100 22/22 9 (9 –9) 15 Delayed primary anastomosis should be preferred þ 100 21/21 9 (6 –9) 16 Axial lengthening procedure is a viable option þ 83.3 15/18 7 (3 –9) 17 The Kimura advancement method of lengthening the upper pouch by extra- thoracic resiting a spit fistula is not recommended þ 100 17/17 9 (6 –9) 18 Other esophageal lengthening techniques ( flap, Livaditis circular myotomy, gastric division) are not recommended þ 88.2 15/17 9 (2 –9) 19 Jejunal interposition is a viable option for esophageal replacement þ 87.5 14/16 8.5 (2 –9) 20 Colonic interposition is a viable option for esophageal replacement /C0 47.1 8/17 5 (1 –9) 21 Gastric transposition is a viable option for esophageal replacement þ 100 17/17 9 (6 –9) 22 þ 100 22/22 9 (6 –9) (Continued ) European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al. 217 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. could be conducted. The discussion included in particular the following aspects: Definitions The participants agreed to define long-gap esophageal atresia as “any esophageal atresia without air in the abdomen” or “any esophageal atresia with a gap of three vertebral bodies or more” as the lowest common denominator. However, there was a debate on whether only patients with esophageal atresia Gross types A and B or also patients with a distal tracheoeso- phageal fistula at the carina or below should be considered as long-gap esophageal atresia. The participants voted on the two Table 3 (Continued ) Statement C o n s e n s u s% V o t e sM e d i a n (range) Reconstructive surgery should only be performed in centers with recognized expertise c. Timing of esophageal reconstruction 23 Gap assessment should be performed at 4 –6w e e k s þ 100 19/19 9 (6 –9) 24 Delayed primary anastomosis should be performed at the age of around 2 –3 months also depending on the gap assessment þ 100 18/18 9 (7 –9) 25 Esophageal replacement should be performed at the age of 2 –3m o n t h sa l s o depending on the gap assessment þ 82.4 14/17 8 (1 –9) 26 Reconstruction at a very early age is a vi able option when esophageal lengthening technique is used þ 93.8 15/16 8 (5 –9) d. General aspects of operative management 27a Antibiotics should be routinely administered perioperatively þ 100 20/20 9 (6 –9) 28a A central venous line should be placed before the operation þ 100 20/20 9 (6 –9) 29a An arterial line should be placed before the operation þ 94.4 17/18 9 (1 –9) e. Delayed primary anastomosis 30a Horizontal or vertical or U-shaped (Bianchi) approaches (skin incision) are viable approaches for conventional thoracotomy þ 100 15/15 9 (7 –9) 31a Muscle-sparing approach is the recommended approach for conventional thoracotomy þ 100 17/17 9 (7 –9) 32a Entry through the fourth intercostal space is the recommended approach for conventional thoracotomy depending on assessment of gap length þ 100 16/16 9 (7 –9) 33a The extrapleural approach is the preferred approach for thoracotomy þ 86.7 13/15 9 (1 –9) 34a In cases with suspected right descending a orta, a right-sided thoracic approach is the first option þ 93.8 15/16 8 (1 –9) 35b The azygos vein should be preserved whenever possible þ 88.2 15/17 7 (4 –9) 36a The esophageal anastomosis should be preferably performed with absorbable sutures þ 88.2 15/17 9 (1 –9) 37a The esophageal anastomosis should be preferably performed with interrupted sutures þ 100 16/16 9 (7 –9) 38a A transanastomotic tube should be routinely inserted. þ 94.7 18/19 9 (5 –9) 39a A chest drain should be routinely placed /C0 64.7 11/17 7 (2 –9) 40a The thoracoscopic approach is a viable option þ 100 16/16 9 (6 –9) 41a The thoracoscopic approach should be only performed if suitable expertise is available þ 95.2 20/21 9 (1 –9) f. Lengthening techniques 42 Thoracoscopic pouch mobilization and p lacement of traction sutures are a novel technique that shows promise, but should only be performed in specialized centers with prospective review and reporting of outcomes þ 100 23/23 9 (6 –9) 43 Open pouch mobilization and placement of traction sutures are a viable technique that should only be performed in specialized centers with prospective review and reporting of outcomes þ 86.4 19/22 9 (2 –9) Abbreviation: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies. aThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula. bThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula, but consensus could not be reached on this item. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al.218 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Table 4 Esophageal replacement Statement Consensus % Votes Median (range) Gastric transposition 1 The anastomosis for gastric transposition should be routinely performed on the patient ’sr i g h ts i d e þ 90.9 10/11 8 (5 –9) 2 Thoracotomy for gastric transposition should be avoided whenever possible þ 91.7 11/12 8.5 (4 –9) 3 Partial gastric transposition with intr athoracic anastomosis should be avoided þ 100 13/13 8 (6 –9) 4 A pyloroplasty (Mikulicz) should be routinely performed þ 91.7 11/12 8 (1 –9) 5 Laparoscopically assisted gastric transposition is a viable option þ 100 17/17 9 (6 –9) 6 A jejunostomy should be routinely performed to allow postoperative feeding unless sham feeding is well established þ 95 19/20 8 (1 –9) Jejunal interposition 7 A cervical esophagostomy is a contra indication for jejunal interposition þ 100 12/12 7 (6 –) Colonic interposition 8 The right hemicolon should be routinely used in an isoperistaltic manner þ 100 10/10 8 (6 –9) 9 The preferred position is the posterior mediastinum þ 100 12/12 8.5 (6 –9) Table 5 Postoperative management Statement C o n s e n s u s% V o t e sM e d i a n (range) 1 Postoperative ventilation and relaxation should be performed for up to 5 d in anastomoses under tension þ 100 17/17 8 (6 –9) 2 Routine postoperative antibiotic prophylaxis beyond 48 h is not recommended þ 95 19/20 9 (5 –9) 3a A postoperative contrast study of the esophagus should be routinely performed before the initiation of oral feeding /C0 40 8/20 5 (1 –9) 4 Enteral feeding should be routinely init iated on the second postoperative day via a gastric or jejunal route þ 85 17/20 8.5 (3 –9) 5a A clinical checklist should be made avai lable including items which should be performed before first discharge (e.g., abdominal and renal ultrasound, resusci- tation training for parents/caregivers) þ 95.5 21/22 9 (5 –9) 6 Resuscitation training for parents and caregivers is mandatory before discharge þ 91.3 21/23 9 (1 –9) Abbreviation: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies. aThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula. Table 6 Follow-up Statement Consensus % Votes Median (range) 1a There should be a structured schedule for lifelong follow-up þ 100 23/23 9 (8 –9) 2a There should be an interdisciplinary follow-up program including surgeons, gastroenterologists, pulmonologists, otolaryngologists, nutrition counseling and others, with one specialist leading þ 100 23/23 9 (8 –9) 3a Proton pump inhibitors should be used for antacid prophylaxis þ 90 18/20 9 (3 –9) 4b Antacid medication should be routinely ad ministered to at least until the age of 12 months þ 90.5 19/21 9 (2 –9) 5a Antacid therapy should be tapered at the end of prophylaxis þ 100 22/22 9 (6 –9) 6a In patients with symptoms, anastomotic strictures should be diagnosed by contrast and/or endoscopy þ 100 21/21 9 (8 –9) 7a Anastomotic stricture should be mana ged by balloon or semirigid dilatation þ 100 19/19 9 (8 –9) 8a The de finition of recurrent anastomotic stricture is three anastomotic stricture relapses requiring dilatation þ 100 23/23 9 (7 –9) (Continued ) European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al. 219 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Table 6 (Continued ) Statement Consensus % Votes Median (range) 9b Topical application of mitomycin C is a viable option in patients with recurrent strictures þ 77.8 14/18 7.5 (1 –9) 10a Intralesional steroids are a viable optio ni np a t i e n t sw i t hr e c u r r e n ts t r i c t u r e sþ 94.4 17/18 7.5 (5 –9) 11 Stents are a viable option in patients with recurrent strictures, but should only be used with caution þ 94.7 18/19 9 (4 –9) 12 Outpatient clinical and n utritional assessment shoul d be performed every 3 month during the first year after reconstruction þ 100 23/23 9 (6 –9) 13 Upper GI endoscopy and/or pH –impedance-metry should be performed 1 year after reconstruction after tapering proton pump inhibitors þ 100 19/19 9 (7 –9) 14 Outpatient clinical and nutritional assessment is recommended at least every second year until transition þ 100 23/23 9 (7 –9) 15a At least two additional endoscopies of the upper gastrointestinal tract should be performed until transition þ 100 23/23 9 (6 –9) 16 Respiratory review by pulmonologists should be routinely performed for children and adolescents according to a speci fics c h e d u l e þ 100 23/23 9 (7 –9) 17 Contrast study of the upper gastrointestinal tract should not be routinely used for monitoring children and adolescents according to a speci fics c h e d u l e þ 100 23/23 9 (7 –9) 18 Bronchoscopy is recommended for symptomatic children þ 100 23/23 9 (6 –9) 19a Adult patients need surveillance as per ESPGHAN guidelines: (1) routine endoscopy every 5 –10 y, (2) endoscopy if new or worsening symptoms occur, and (3) in presence of Barrett as per consensus recommendations þ 100 23/23 9 (6 –9) 20 A speci fic transition program for adolescents with long-gap esophageal atresia should be organized þ 100 23/23 9 (6 –9) 21a Quality of life assessment using a validated instrument should be offered during follow-up in children, adolescents, and adult patients þ 100 23/23 9 (6 –9) 22 Screening for dumping syndrome in children is required especially in children with microgastria, or when pyloroplasty or antire flux surgery has been performed þ 95.7 22/23 9 (5 –9) 23 When endoscopy is performed, there should be awareness of eosinophilic esophagitis, and biopsies should be taken according to ESPGHAN guidelines þ 100 22/22 9 (6 –9) Abbreviations: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies; ESPGHAN, European Society for Pediatric Gastroenterology Hepatology and Nutrition; GI, gastrointestinal. aThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula. bThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula, but consensus could not be reached on this item. Table 7 Framework Statement C o n s e n s u s% V o t e sM e d i a n (range) 1 When the diagnosis of long-gap esophageal atresia is con firmed, the patient should be referred to a center of expertise in esophageal reconstructive surgery þ 100 23/23 9 (7 –9) 2 Long-gap esophageal atresia should be managed in centers with expertise in esophageal reconstructive surgery, preferably with more than two cases per year þ 91.3 21/23 9 (1 –9) 3 When long-gap esophageal atresia is suspected, referral to antenatal multidisci- plinary counseling in a center of expertise should be made þ 95.7 22/23 9 (4 –9) 4a Parents of esophageal atresia patients sh ould be informed about, and encouraged to contact parent and patient support groups as early as possible þ 100 23/23 9 (6 –9) Abbreviation: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies. aThis item has already been discussed during the first ERNICA consensus conference on the management of patient with esophageal atresia with tracheoesophageal fistula. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al.220 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. latter statements, though they did not reach consensus (►Table 2 ). In addition, there was agreement that “long gap” is not an appropriate term to define the condition of long-gap esophageal atresia in its entirety. The term“wide-gap” esoph- ageal atresia, which is used in some countries to identify cases where the anastomosis cannot be achieved despite the pres- ence of afistula, was proposed to be introduced as an adequate definition. However, participants decided not to vote on this new definition to avoid more confusion as long-gap esophageal atresia is a well-established and accepted term. Esophageal Reconstruction It had been suggested to vote on the surgical option of a gastric tube for esophageal reconstruction. After extensive discussion, it was decided not to vote on this item due to limited evidence in the literature 21,22 and lack of personal experience within the group. Postoperative Management An attempt was made to formulate a statement on the commencement of oral feeding. However, most participants felt that a de finition of a speci fic time point would not be appropriate as individual parameters, such as duration of ventilator dependency, have a decisive impact on the post- operative time management. Therefore, no statement was formulated on the commencement of oral feeding. Follow-up Complete consensus was reached on the management of anastomotic strictures with balloon or semirigid dilatation ( ►Table 6 ). In this context, participants discussed the indi- cation of peri-interventional antibiotic prophylaxis. Even if most of the participants supported peri-interventional anti- biotic prophylaxis, the evidence in the available literature was considered to be too low allowing a meaningful vote. Participants voted on treatment options for recurrent anastomotic strictures, such as topical application of mito- mycin C, intralesional steroids, and stents ( ►Table 6 ). Par- ticipants also considered whether to vote on the application of indwelling balloon dilatation or endoscopic knife for recurrent anastomotic strictures. Moreover, surgical resec - tion and esophageal reanastomosis was proposed as viable option in cases of failed treatment. Nonetheless, evidence for each of these options is scarce, 23–25 and therefore, it was decided to abstain from formulating items for voting. Complete consensus was reached on the statement that antacid therapy should be tapered at the end of prophylaxis (►Table 6 ). However, it was not possible to formulate a concrete instruction of how to taper the antacid medication due to a lack of evidence in literature and multiple sugges- tions by the participants. Need for Further Research The items on which either no consensus was reached or it was felt that there was insuf ficient evidence were suggested to be priorities for future research. ►Table 9 summarizes relevant topics which urgently need further studies. Discussion The management of long-gap esophageal atresia remains challenging with limited evidence and consensus on the definition, evaluation, and surgical approach. 2–4,9,26–28 The variety of management strategies that have been employed testify to the challenge it presents. 8 In 2017, the International Network of Esophageal Atresia (INoEA) presented a position paper on the de finition of long- gap esophageal atresia and the best diagnostic and treatment strategies also highlighting the necessity of experience and communication in the management of these challenging patients.5 Recently, the American Pediatric Surgery Associa- tion (APSA) released a systematic review and 18 evidence- based guidelines, primarily based on levels 4 and 5 evidence, on the management of long-gap esophageal atresia. 3 We hereby present the results of the second ERNICA consensus conference focusing on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. In line with the first ERNICA consensus conference,14,15 this conference was based on two keystones: (1) on evidence from literature and (2) on expert opinion. Participants of this conference achieved general consen- sus (defined by /C21 75% of votes scoring 6–9) in 93% of all items (n ¼ 97) which indicates a considerable level of agreement and suggests predominantly homogeneous approaches in ERNICA institutions. This is supported by the high rate of total agreement (de fined as 100% consensus among voters) of 58% of all items. A maximum range of voting from 1 to 9, indicating widely diverse opinions, was evident for only 20% of items which is low considering the huge variability of current treatment strategies. Consensus was not reached in only 7/97 votes (7%), and all of these statements were discussed controversially (range 1–9; except one with a range 2 –9): Table 8 Consensus amongst participants of the second ERNICA consensus conference Consensus No. of items (%) 100% 56 (58) >75%a 90 (93) <75% 7 (7) Abbreviation: ERNICA, European Reference Network for Rare Inherited Congenital Anomalies. aV o t e st h a ts c o r e d6 ,7 ,8 ,o r9a l s oi n c l u d i n gi t e m st h a tr e a c h e d1 0 0 % . Fig. 1 Preferred option of esophageal replacement. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al. 221 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Gap Measurement Accurate measurement of gap length is critical for operative planning for long-gap esophageal atresia.29,30 It is still a matter of debate whether preoperative contrast study to evaluate the upper esophageal pouch is needed. McDuffie et al stressed the high risk of aspiration31 which can be disastrous, as exempli- fied in a case-reported death of an infant. 32 Moreover, Gross et al postulated that gap measurement with contrast media is not reliable as the lower pouch might be underestimated in the absence of re flux. 29 Based on these arguments, the need for contrast studies for diagnostics has been essentially eliminat- ed,33 and hence, gap measurement by contrast study of the upper and lower pouches was not considered to be a viable option by the participants of the conference. Definition of “Long-Gap ” Esophageal Atresia Efforts have been made to define long-gap esophageal atresia as precisely as possible and not to accept subjective terms such as “inability to achieve primary end-to-end anastomo- sis.” 3,4,6,9,34–36 However, there is no universally accepted methodology for determining either the gap length or what constitutes long-gap esophageal atresia. 3 The APSA Commit- tee—based on its members ’ opinion—stated that the nomen- clature “Long Gap Esophageal Atresia ” should not be reserved for Gross type A atresia exclusively, which is in line with the results of the participants ’ votes. The INoEA working group recently reaf firmed the unclear de finition and suggested to define any esophageal atresia that has no intra-abdominal air should be considered a long gap. 5 This statement reached complete consensus by all partic - ipants. However, it was additionally stated that this definition should not confine long-gap esophageal atresia to pure atresia (Gross type A or B), but should allow inclusion of esophageal atresia with tracheoesophageal fistula with a wide gap, re- spectively, with different anatomic configurations. 1,36–39 Par- ticipants decided not to vote on this proposed term of “wide gap” to avoid more confusion. As a result of a highly contro- versial discussion on the de finition of long-gap esophageal atresia, consensus was reached on important statements. It must be emphasized, however, that the condition of long-gap esophageal atresia is not fully depicted yet by these definitions. Esophageal Reconstruction There is no consensus on the preferred method of esophageal reconstruction to date.2,4,8,26,40–42 No randomized controlled trials comparing different approaches of surgical repair or comparing the various techniques used to manage long-gap esophageal atresia are available. 8 Fundamentally, it must be Table 9 Priorities for further research Domain Topic 1 Diagnostics Optimal approach for gap measurement 2D e finitions Comprehensive de finition of “long-gap esophageal atresia ” 3 Esophageal reconstruction - Initial management before reconstruction Counseling of parents (ideally including t he involvement of patient support groups) 4 Esophageal reconstruction - Delayed primary anastomosis Evidence for routine insertion of a transanastomotic tube 5 Esophageal reconstruction - Delayed primary anastomosis Evidence for routine placement of a chest drain 6 Esophageal reconstruction - Lengthening techniques Early and long-term outcome of different esophageal lengthening techniques 7 Esophageal replacement Evidence for optimal surgical technique for esophageal replacement 8 Esophageal replacement - Gastric transposition Early and long-term outcome after gastric tube formation as an option for esophageal replacement 9 Esophageal replacement - Gastric transposition Evidence for insertion of a transanastomotic tube during gastric transposition 10 Esophageal replacement - Gastric transposition Relevance of pyloroplasty (Mikulicz) during gastric transposition 11 Postoperative management Evidence for routine postoperative contrast study of the esophagus before initiation of oral feeding 12 Postoperative management Timing of the initiation of oral feeding 13 Follow-up Duration of postoperative antacid therapy 14 Follow-up Mode of tapering the postoperative antacid therapy 15 Follow-up Evidence for peri-interventional antibiotic prophylaxis in balloon or semirigid dilatation for anastomotic stricture 16 Follow-up Application of indwelling balloon dilatation, endoscopic knife, and surgical resection and reanastomosis in cases of recurrent anastomotic stricture European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al.222 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. stated that preservation of the native esophagus should be aimed before considering any replacement technique9,28,43 as “no other conduit can replace its function in transporting food from the oral cavity to the stomach satisfactorily”.44 In this light, it is interesting to mention that thoracoscopic pouch mobili- zation and placement of traction sutures are considered a novel technique that shows promise, but should only be performed in specialized centers with prospective review and reporting of outcomes ( ►Table 3 ,f ) . Various different techniques deal with esophageal replace- ment, reflecting that none has been suggested to be ideal, and the patients are left with many challenges to over- come.2,9,43,45,46 In this context, participants voted on the preferred option for esophageal replacement ( ►Fig. 1 ). The majority of participants voted for gastric transposition (56%), followed by jejunal interposition (25%) andfinally followed by colonic interposition (19%). The voting result corresponds to the current literature as multiple studies con firmed that the stomach is the preferred organ for esophageal replace- ment. 8,47–50 In addition, the high rate of abstention from voting (30%) indicates the disagreement of preferred surgical options. The latest systematic review of the surgical treatment of long-gap esophageal atresia by Stadil et al aimed to compare the postoperative complications related to the different meth- ods within the first postoperative year. 40 Fifty-seven articles were included involving 326 patients with Gross types A and B long-gap esophageal atresia. 40 Delayed primary anastomosis was the most applied surgical method (68.4%) in both types, followed by gastric transposition (8.3%) which is in line with the statements achieved during the consensus conference. Literature provides some evidence for colonic interposi- tion to be a surgical option comparable to other replacement techniques. 2,51,52 Nonetheless, the INoEA working group stated that colonic interposition is mainly reserved as a last option, when all other techniques have failed or are considered unfeasible. 5 After extensive discussion, partici- pants of this conference did not reach consensus on colonic interposition as a viable option for esophageal replacement mainly driven by evidence from literature 5,53–55 and indi- vidual experience. Placement of Chest Drain Routine placement of a chest drain in delayed primary anastomosis was also controversially discussed. Several studies postulated that a chest drain does not alter early postoperative complications after repair of esophageal atre- sia with tracheoesophageal fistula. 56–58 Participants gener- ally agreed that the placement of a chest drain for delayed primary anastomosis has to be valued differently compared with primary repair due to higher risk of anastomotic leakage based on an anastomosis under tension. Even though a clear majority of voters supported the routine placement of a chest drain, consensus was not reached on this item. Postoperative Contrast Study Evidence on “routine postoperative contrast study of the esophagus before the initiation of oral feeding ” is scarce.59,60 Yanchar et al presented a study including 90 patients who underwent esophageal atresia repair and postoperative up- per gastrointestinal contrast study before consideration of oral feeding. 60 They concluded that the use of early routine contrast studies, with no suspicion of a problem, has little value in terms of predicting complications or future clinical course. 60 These findings support the participants ’ votes not advocating for a routine postoperative contrast study in the absence of any clinical findings. Given the complexity of this patient population with significant morbidity and associated anomalies, treatment and long-term follow-up should be managed by specialized and multidisciplinary teams. 9,11,28,61 There are growing demands for referral of esophageal atresia patients to desig- nated centers of expertise just as it is common practice in the Netherlands or in France, and these demands are even stronger for patients with long gap. 3,4,8,9,14,15,62,63 These repeatedly expressed requirements to ful fill the criteria of optimal patient care are re flected in the consensus state- ments in the domain Framework. Although the consensus meeting was focused entirely on long-gap congenital esophageal atresia, esophageal recon- struction is also often necessary for children with caustic injury, button battery ingestion, or other acquired/iatrogenic esophageal damage. Although the statements generated are not directly applicable to such children, some of the state- ments might be considered as relevant and useful in the absence of any specific consensus guidelines on treatment of children with acquired esophageal damage. As stated previously, the strength of this conference is the pool of participating specialists with extensive expertise in this field. 14,15 The multidisciplinary approach was highly valued in the first ERNICA consensus conference allowing discussion from various perspectives. Considering this ben- eficial methodological approach, the group of participants had been extended to representatives of the specialty neo- natology and pediatric pulmonology. Further advantages, such as the methodology (characterized by meticulous item generation and prioritization, systematic literature search, and anonymous voting), ability to modify wording of statements via the online voting system, abstention from voting in case of lacking expertise, and involvement of representatives of the patient support groups ensure the high quality of results and indicate the great validity of votes. Nonetheless, it has to be emphasized that the results of the conference were mainly based on expert opinion, and not on evidence. 14,15 Conclusion Evidence supporting best practices for long-gap esophageal atresia is weak. We hereby present consensus statements on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia based on a critical evaluation of the current literature. Areas of controversy were identi fied for future research. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to opti- mized and uniform patient care. European Journal of Pediatric Surgery Vol. 3 1 No. 3/2021 © 2020. Thieme. All rights reserved. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia Dingemann et al. 223 Downloaded by: Erasmus Universiteit Rotterdam. Copyrighted material. Funding ERNICA provided financial support for the conference. Conflict of Interest None declared. Acknowledgments S.E., K.M.C., and P.D.C. gratefully acknowledge the support of the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR, or the UK Department of Health. References 1 Bagolan P, Valfrè L, Morini F, Conforti A. Long-gap esophageal atresia: traction-growth and anastomosis - before and beyond. 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