Behandlung der Ösophagusatresie mit unterer
                    tracheoösophagealer Fistel – Zusammenfassung der aktuellen
                    S2K-Leitlinie der DGKCH

Steffi Mayer; Heidrun Gitter; Peter Göbel; Franz Wolfgang Hirsch; Claudia Höhne; Stuart Hosie; Jochen Hubertus; Andreas Leutner; Oliver Muensterer; Peter Schmittenbecher; Elias Seidl; Holger Stepan; Ulrich Thome; Holger Till; Anke Widenmann-Grolig; Martin Lacher

doi:10.1055/a-1149-9483

Klin Padiatr 2020; 232(04): 178-186
DOI: 10.1055/a-1149-9483

Diagnostic and Treatment Recommendation

Behandlung der Ösophagusatresie mit unterer tracheoösophagealer Fistel – Zusammenfassung der aktuellen S2K-Leitlinie der DGKCH

Current Treatment of Esophageal Atresia with Tracheoesophageal Fistula – Updated Guidelines of the German Society of Pediatric Surgery

Steffi Mayer

¹Department of Pediatric Surgery, University Hospital Leipzig, Leipzig

,

Heidrun Gitter

²Department of Pediatric Surgery and Urology, Klinikum Bremen-Mitte, Children's Hospital, Bremen

,

Peter Göbel

³Department of Pediatric Surgery and Pediatric Urology, Elisatbeth Hospital, Halle (Saale)

,

Franz Wolfgang Hirsch

⁴Department of Pediatric Radiology, University Hospital Leipzig, Leipzig

,

Claudia Höhne

⁵Department of Anesthesiology, Intensive Medicine and Pain Medicine, DRK Hospitals Berlin Koepenick, Berlin

,

Stuart Hosie

⁶Department of Pediatric Surgery, München Klinik gGmbH, Munich

,

Jochen Hubertus

⁷Department of Pediatric Surgery, Munich University Hospital Dr von Hauner Children’s Hospital, Munich

,

Andreas Leutner

⁸Department of Pediatric Surgery, Medical Center Dortmund, Dortmund

,

Oliver Muensterer

⁹Department of Pediatric Surgery, Johannes Gutenberg University Hospital Mainz, Mainz

,

Peter Schmittenbecher

¹⁰Department of Pediatric Surgery, Klinikum Karlsruhe, Karlsruhe

,

Elias Seidl

¹¹Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig Maximilians University Munich, Munich

,

Holger Stepan

¹²Department of Obstetrics, University Hospital Leipzig, Leipzig

,

Ulrich Thome

¹³Divsion of Neonatology, University Hospital Leipzig, Leipzig

,

Holger Till

¹⁴Department of Pediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria

,

Anke Widenmann-Grolig

¹⁵The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula Support Groups, KEKS e.V., Stuttgart

,

Martin Lacher

¹Department of Pediatric Surgery, University Hospital Leipzig, Leipzig

› Author Affiliations

Abstract

Zusammenfassung

Die Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

Abstract

Esophageal atresia (EA) is a congenital anomaly that entails an interrupted esophagus with or without tracheoesophageal fistula (TEF). Depending on the distance of the two esophageal pouches a “short-gap” is distinguished from a „long-gap“ variant. Up to 50% of newborns have additional anomalies. EA is prenatally diagnosed in 32–63% of cases. Recently, the interdisciplinary care in these children underwent substantial changes. Therefore, we summarize the current guideline of the German society of pediatric surgery for the treatment of patients with EA and distal TEF (Gross Type C). Controversies regarding the perioperative management include surgical-technical aspects, such as the thoracoscopic approach to EA, as well as general anesthesia (preoperative tracheobronchoscopy, intraoperative hypercapnia and acidosis). Moreover, postoperative complications and their management like anastomotic stricture are outlined. Despite significant improvements in the treatment of EA, there is still a relevant amount of long-term morbidity after surgical correction. This includes dysmotility of the esophagus, gastroesophageal reflux disease, recurrent respiratory infections, tracheomalacia, failure to thrive, and orthopedic complications following thoracotomy in the neonatal age. Therefore, close follow-up is mandatory to attain optimal quality of life.

Schlüsselwörter

Ösophagusatresie - tracheoösophageale Fistel - Therapie - Komplikationen - Prognose - Leitlinie

Key words

esophageal atresia - tracheoesophageal fistula - therapy - complications - outcome - guideline

Full Text

References

Literatur
1 Baird R. et al. Management of long gap esophageal atresia: A systematic review and evidence-based guidelines from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg 2019; 54: 675-687
2 Bairdain S. et al. Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients. J Pediatr Surg 2015; 50: 972-977
3 Bastard F. et al. Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort. J Pediatr Surg 2018; 53: 605-609
4 van der Zee DC. et al. A novel technique for risk calculation of anastomotic leakage after thoracoscopic repair for esophageal atresia with distal fistula. World J Surg 2008; 32: 1396-1399
5 Berger M, Ure B, Lacher M. Mitomycin C in the therapy of recurrent esophageal strictures: Hype or hope?. Eur J Pediatr Surg 2012; 22: 109-116
6 Bishay M. et al. Hypercapnia and Acidosis During Open and Thoracoscopic Repair of Congenital Diaphragmatic Hernia and Esophageal Atresia Results of a Pilot Randomized Controlled Trial. Ann Surg 2013; 258: 895-900
7 Bradshaw CJ. et al. Accuracy of prenatal detection of tracheoesophageal fistula and oesophageal atresia. J Pediatr Surg 2016; 51: 1268-1272
8 Burjonrappa SC, Youssef S, St-Vil D. What is the incidence of Barrett's and gastric metaplasia in esophageal atresia/tracheoesophageal fistula (EA/TEF) patients?. Eur J Pediatr Surg 2011; 21: 25-29
9 Chapuy L, Pomerleau M, Faure C, Burjonrappa SC. Topical mitomycin-C application in recurrent esophageal strictures after surgical repair of esophageal atresia. J Pediatr Gastroenterol Nutr 2014; 59: 608-611
10 Craig WR, Hanlon-Dearman A, Sinclair C, Taback SP, Moffatt M. Metoclopramide, thickened feedings, and positioning for gastro-oesophageal reflux in children under two years. Cochrane Database of Systematic Reviews. 2004 Issue 3. Art. No.. CD003502
11 Dal'Astra APL. et al. Tracheostomy in childhood: Review of the literature on complications and mortality over the last three decades. Braz J Otorhinolaryngol 2017; 83: 207-214
12 Deurloo JA. et al. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg 2001; 36: 629-630
13 Dingemann C. 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: 631-638
14 Dingemann C. et al. Early complications after esophageal atresia repair: Analysis of a German health insurance database covering a population of 8 million. Dis Esophagus 2016; 29: 780-786
15 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: 50-55
16 Duvoisin G, Krishnan U. Gastric Function in Children with Oesophageal Atresia and Tracheoesophageal Fistula. Front Pediatr 2017; 5: 76
17 El-Asmar KM. et al. Topical mitomycin C application is effective in management of localized caustic esophageal stricture: A double-blinded, randomized, placebo-controlled trial. J Pediatr Surg 2013; 48: 1621-1627
18 Encinas JL. et al. Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia. Pediatr Surg Int 2006; 22: 150-153
19 Fallon SC. et al. Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease. J Pediatr Surg 2017; 52: 1711-1714
20 Faure C, Righini Grunder F. Dysmotility in Esophageal Atresia: Pathophysiology, Characterization, and Treatment. Front Pediatr 2017; 5: 130
21 Felix JF, Tibboel D, Klein de A. Chromosomal anomalies in the aetiology of oesophageal atresia and tracheo-oesophageal fistula. Eur J Med Genet 2007; 50: 163-175
22 Fraga JC, Jennings RW, Kim PCW. Pediatric tracheomalacia. Semin Pediatr Surg 2016; 25: 156-164
23 Gallo G. et al. Long-gap esophageal atresia: A meta-analysis of jejunal interposition, colon interposition, and gastric pull-up. Eur J Pediatr Surg 2012; 22: 420-425
24 Garabedian C. et al. Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia?. Am J Obstet Gynecol 2015; 212: 340.e1-7
25 Garg MK. Case report: Upper neck pouch sign in the antenatal diagnosis of esophageal atresia. Indian J Radiol Imaging 2009; 19: 252-254
26 Gawad N. et al. A chest tube may not be needed after surgical repair of esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int 2018; 34: 967-970
27 Geneviève D. et al. An overview of isolated and syndromic oesophageal atresia. Clin Genet 2007; 71: 392-399
28 Golden J. et al. Routine post-operative esophagram Is not necessary after repair of esophageal atresia. Am J Surg 2017; 213: 640-644
29 Goyal V, Masters IB, Chang AB. Interventions for primary (intrinsic) tracheomalacia in children. Cochrane Database Syst Rev 2012; 10: CD005304
30 Gregory S. et al. Endoscopic esophageal and tracheal cauterization for closure of recurrent tracheoesophageal fistula: A case report and review of the literature. Int J Pediatr Otorhinolaryngol 2017; 98: 158-161
31 Gross ER. et al. The role of fiberoptic endoscopy in the evaluation and management of long gap isolated esophageal atresia. Pediatr Surg Int 2010; 26: 1223-1227
32 Gross RE. Atresia of the esophagus. Am J Dis Child 1947; 74: 369
33 Heran MKS. et al. Topical mitomycin-C for recalcitrant esophageal strictures: A novel endoscopic/fluoroscopic technique for safe endoluminal delivery. J Pediatr Surg 2008; 43: 815-818
34 Hochart V. et al. The contribution of fetal MR imaging to the assessment of oesophageal atresia. Eur Radiol 2015; 25: 306-314
35 Hölscher AC. et al. Quality of Life after Surgical Treatment for Esophageal Atresia: Long-Term Outcome of 154 Patients. Eur J Pediatr Surg 2017; 27: 443-448
36 Horvath A, Dziechciarz P, Szajewska H. The effect of thickened-feed interventions on gastroesophageal reflux in infants: Systematic review and meta-analysis of randomized, controlled trials. Pediatrics 2008; 122: e1268-e1277
37 Jancelewicz T. et al. Surgical management of gastroesophageal reflux disease (GERD) in children: A systematic review. J Pediatr Surg 2017; 52: 1228-1238
38 Jennings RW. et al. Surgical approaches to aortopexy for severe tracheomalacia. J Pediatr Surg 2014; 49: 66-70 discussion 70-1
39 Jong EMde. et al. Pre- and postnatal diagnosis and outcome of fetuses and neonates with esophageal atresia and tracheoesophageal fistula. Prenat Diagn 2010; 30: 274-279
40 Kikuchi S. et al. Successful treatment of tracheomalacia associated with esophageal atresia without a tracheoesophageal fistula by aortopexy: Report of a case. Surg Today 1999; 29: 344-346
41 Koivusalo A, Pakarinen MP, Rintala RJ. Anastomotic dilatation after repair of esophageal atresia with distal fistula. Comparison of results after routine versus selective dilatation. Dis Esophagus 2009; 22: 190-194
42 Koivusalo AI. et al. Revisional surgery for recurrent tracheoesophageal fistula and anastomotic complications after repair of esophageal atresia in 258 infants. J Pediatr Surg 2015; 50: 250-254
43 Krishnan U. 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: 550-570
44 Lacher M. et al. Early and long term outcome in children with esophageal atresia treated over the last 22 years. Klin Padiatr 2010; 222: 296-301
45 Lacher M. et al. Minimal invasive surgery in the newborn: Current status and evidence. Semin Pediatr Surg 2014; 23: 249-256
46 Lal DR. et al. Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2017; 52: 1245-1251
47 Lal DR. et al. Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium. J Pediatr Surg 2018; 53: 1267-1272
48 Lange B. et al. Use of fully covered self-expandable metal stents for benign esophageal disorders in children. J Laparoendosc Adv Surg Tech A 2015; 25: 335-341
49 Lange B. et al. Experience with fully covered self-expandable metal stents for anastomotic stricture following esophageal atresia repair Diseases of the Esophagus. 2018 31. doy061
50 Lawal TA. et al. Thoracoscopy versus thoracotomy improves midterm musculoskeletal status and cosmesis in infants and children. Ann Thorac Surg 2009; 87: 224-228
51 Lee S. Basic Knowledge of Tracheoesophageal Fistula and Esophageal Atresia. Adv Neonatal Care 2018; 18: 14-21
52 Lévesque D, Baird R, Laberge J-M. Refractory strictures post-esophageal atresia repair: What are the alternatives?. Dis Esophagus 2013; 26: 382-387
53 Levin DN, Diamond IR, Langer JC. Complete vs partial fundoplication in children with esophageal atresia. J Pediatr Surg 2011; 46: 854-858
54 Lo A. et al. Arterioesophageal fistula after stenting for esophageal atresia. J Pediatr Gastroenterol Nutr 2013; 56: e30-e31
55 Madadi-Sanjani O. et al. Topical Mitomycin C Application in Pediatric Patients with Recurrent Esophageal Strictures-Report on Unfavorable Results. Eur J Pediatr Surg 2018; 28: 539-546
56 Malmström K. et al. Longitudinal follow-up of bronchial inflammation, respiratory symptoms, and pulmonary function in adolescents after repair of esophageal atresia with tracheoesophageal fistula. J Pediatr 2008; 153: 396-401
57 Manfredi MA. et al. Externally removable stents in the treatment of benign recalcitrant strictures and esophageal perforations in pediatric patients with esophageal atresia. Gastrointest Endosc 2014; 80: 246-252
58 Michaud L. et al. Dumping syndrome after esophageal atresia repair without antireflux surgery. J Pediatr Surg 2010; 45: E13-E15
59 Mirra V. et al. Longitudinal Follow-up of Chronic Pulmonary Manifestations in Esophageal Atresia: A Clinical Algorithm and Review of the Literature. Pediatr Neonatol 2017; 58: 8-15
60 Morini F, Conforti A, Bagolan P. Perioperative Complications of Esophageal Atresia. Eur J Pediatr Surg 2018; 28: 133-140
61 Mortellaro VE. et al. The Use of High-Frequency Oscillating Ventilation to Facilitate Stability During Neonatal Thoracoscopic Operations. Laparoendosc Adv Surg Tech A 2011; 21: 877-879
62 Mutalib M. et al. BSPGHAN Motility Working Group position statement: Paediatric multichannel intraluminal pH impedance monitoring-indications, methods and interpretation. Frontline Gastroenterol 2017; 8: 156-162
63 Oddsberg J, Lu Y, Lagergren J. Aspects of esophageal atresia in a population-based setting: Incidence, mortality, and cancer risk. Pediatr Surg Int 2012; 28: 249-257
64 Ohkawa H. et al. Clinical experience with a sucking sump catheter in the treatment of esophageal atresia. J Pediatr Surg 1989; 24: 333-335
65 Petrosyan M. et al. Esophageal atresia/tracheoesophageal fistula in very low-birth-weight neonates: Improved outcomes with staged repair. J Pediatr Surg 2009; 44: 2278-2281
66 Raicevic M, Saxena AK. Barrett’s esophagus in children: What is the evidence?. World J Pediatr 2018; 14: 330-334
67 Rosen R. et al. Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2018; 66: 516-554
68 Rothenberg SS. Thoracoscopic repair of esophageal atresia and tracheo-esophageal fistula. Semin Pediatr Surg 2005; 14: 2-7
69 Rothenberg SS, Pokorny WJ. Experience with a total muscle-sparing approach for thoracotomies in neonates, infants, and children. J Pediatr Surg 1992; 27: 1157-1159, discussion 1159-60
70 Shah PS. et al. Does continuous positive airway pressure for extubation in congenital tracheoesophageal fistula increase the risk of anastomotic leak? A retrospective cohort study. J Paediatr Child Health 2016; 52: 710-714
71 Sharma S. et al. Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula. Pediatr Surg Int 2007; 23: 1215-1218
72 Spaggiari E. et al. Performance of prenatal diagnosis in esophageal atresia. Prenat Diagn 2015; 35: 888-893
73 Spitz L. et al. Oesophageal atresia: At-risk groups for the 1990s. J Pediatr Surg 1994; 29: 723-725
74 Sri Paran T. et al. Long-term results of delayed primary anastomosis for pure oesophageal atresia: A 27-year follow up. Pediatr Surg Int 2007; 23: 647-651
75 Stenström P. et al. Prolonged Use of Proton Pump Inhibitors as Stricture Prophylaxis in Infants with Reconstructed Esophageal Atresia. Eur J Pediatr Surg 2017; 27: 192-195
76 Tighe M. et al. Pharmacological treatment of children with gastro-oesophageal reflux. Cochrane Database Syst Rev. 2014 11. CD008550
77 Tracy S. et al. The Distended Fetal Hypopharynx: A Sensitive and Novel Sign for the Prenatal Diagnosis of Esophageal Atresia. J Pediatr Surg 2018; 53: 1137-1141
78 Tytgat SHAJ. et al. Neonatal brain oxygenation during thoracoscopic correction of esophageal atresia. Surg Endosc 2016; 30: 2811-2817
79 Vaghela MM. et al. Role of glycopyrrolate in healing of anastomotic dehiscence after primary repair of esophageal atresia in a low resource setting-A randomized controlled study. J Pediatr Surg 2017; 52: 420-423
80 Valerie EP. et al. A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: Is it better than aortopexy?. J Pediatr Surg 2005; 40: 904-907 discussion 907
81 Valusek PA. et al. Does an upper gastrointestinal study change operative management for gastroesophageal reflux?. J Pediatr Surg 2010; 45: 1169-1172
82 van der Zee D, Hulsker C. Indwelling esophageal balloon catheter for benign esophageal stenosis in infants and children. Surg Endosc 2014; 28: 1126-1130
83 van Wijk M. et al. Evaluation of gastroesophageal function and mechanisms underlying gastroesophageal reflux in infants and adults born with esophageal atresia. J Pediatr Surg 2013; 48: 2496-2505
84 Vogt EC. Congenital esophageal atresia. AJR Am J Roentgenol 1929; 463-465
85 Witt S. et al. Quality of Life in Parents of Children Born with Esophageal Atresia. Eur J Pediatr Surg 2019; 29: 371-377
86 Wu Y. et al. Comparison of clinical outcomes between open and thoracoscopic repair for esophageal atresia with tracheoesophageal fistula: A systematic review and meta-analysis. Pediatr Surg Int 2017; 33: 1147-1157
87 Yalçin Ş. et al. Management of acquired tracheoesophageal fistula with various clinical presentations. J Pediatr Surg 2011; 46: 1887-1892
88 Zani A. et al. International survey on the management of esophageal atresia. Eur J Pediatr Surg 2014; 24: 3-8
89 Zani A. et al. Intraoperative acidosis and hypercapnia during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula. Paediatr Anaesth 2017; 27: 841-848