Screening and Surveillance in Esophageal Atresia Patients: Current Knowledge and Future Perspectives

 

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Abstract

Esophageal atresia (EA) is a rare congenital anomaly. Enhanced operative techniques and intensive care treatment have improved survival among children with repaired EA (range, 93–95%). Many (up to 67%) suffer from gastroesophageal reflux (GER). The high incidence of GER and improved survival among EA patients raises concerns about an increased risk of developing Barrett esophagus (BE) and esophageal cancer. This review provides an overview of the prevalence of esophagitis, BE, and esophageal cancer in EA patients and outlines suggestions for future research. A literature search indeed revealed a higher prevalence of BE in EA patients than in the generalized population and that this condition occurs at a much younger age. It should be noted that in some studies gastric metaplasia without intestinal metaplasia is defined as BE. Gastric-type mucosa in columnar-lined esophagus is probably of less clinical importance in terms of the likelihood of malignant transformation. Its inclusion therefore confounds the risk of esophageal adenocarcinoma. A total of eight cases of esophageal carcinoma at a young age, either squamous cell carcinoma or adenocarcinoma, have been reported. These observations bear important implications prompting for early onset lifelong BE/esophageal cancer surveillance to facilitate the diagnosis of (pre)neoplastic changes and early treatment.

• References

• 1 EUROCAT. EUROCAT Website Database. Available at: http://www.eurocat-network.eu/ACCESSPREVALENCEDATA/PrevalenceTables . Accessed May 11, 2015
  PubMedGoogle Scholar
• 2 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 (2) 604-612
  CrossrefPubMedGoogle Scholar
• 3 Sulkowski JP, Cooper JN, Lopez JJ , et al. Morbidity and mortality in patients with esophageal atresia. Surgery 2014; 156 (2) 483-491
  CrossrefPubMedGoogle Scholar
• 4 Deurloo JA, Ekkelkamp S, Schoorl M, Heij HA, Aronson DC. Esophageal atresia: historical evolution of management and results in 371 patients. Ann Thorac Surg 2002; 73 (1) 267-272
  CrossrefPubMedGoogle Scholar
• 5 Rintala RJ, Pakarinen MP. Long-term outcome of esophageal anastomosis. Eur J Pediatr Surg 2013; 23 (3) 219-225
  Article in Thieme ConnectPubMedGoogle Scholar
• 6 Sistonen SJ, Koivusalo A, Nieminen U , et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann Surg 2010; 251 (6) 1167-1173
  CrossrefPubMedGoogle Scholar
• 7 Taylor AC, Breen KJ, Auldist A , et al. Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long-term follow-up study. Clin Gastroenterol Hepatol 2007; 5 (6) 702-706
  CrossrefPubMedGoogle Scholar
• 8 Biller JA, Allen JL, Schuster SR, Treves ST, Winter HS. Long-term evaluation of esophageal and pulmonary function in patients with repaired esophageal atresia and tracheoesophageal fistula. Dig Dis Sci 1987; 32 (9) 985-990
  CrossrefPubMedGoogle Scholar
• 9 Schneider A, Michaud L, Gottrand F. Esophageal atresia: metaplasia, Barrett. Dis Esophagus 2013; 26 (4) 425-427
  CrossrefPubMedGoogle Scholar
• 10 Spechler SJ, Sharma P, Souza RF, Inadomi JM, Shaheen NJ ; American Gastroenterological Association. American Gastroenterological Association medical position statement on the management of Barrett's esophagus. Gastroenterology 2011; 140 (3) 1084-1091
  CrossrefPubMedGoogle Scholar
• 11 Ronkainen J, Aro P, Storskrubb T , et al. Prevalence of Barrett's esophagus in the general population: an endoscopic study. Gastroenterology 2005; 129 (6) 1825-1831
  CrossrefPubMedGoogle Scholar
• 12 Deurloo JA, van Lanschot JJ, Drillenburg P, Aronson DC. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg 2001; 36 (4) 629-630
  CrossrefPubMedGoogle Scholar
• 13 Jayasekera CS, Desmond PV, Holmes JA, Kitson M, Taylor AC. Cluster of 4 cases of esophageal squamous cell cancer developing in adults with surgically corrected esophageal atresia—time for screening to start. J Pediatr Surg 2012; 47 (4) 646-651
  CrossrefPubMedGoogle Scholar
• 14 Adzick NS, Fisher JH, Winter HS, Sandler RH, Hendren WH. Esophageal adenocarcinoma 20 years after esophageal atresia repair. J Pediatr Surg 1989; 24 (8) 741-744
  CrossrefPubMedGoogle Scholar
• 15 Alfaro L, Bermas H, Fenoglio M, Parker R, Janik JS. Are patients who have had a tracheoesophageal fistula repair during infancy at risk for esophageal adenocarcinoma during adulthood?. J Pediatr Surg 2005; 40 (4) 719-720
  CrossrefPubMedGoogle Scholar
• 16 Pultrum BB, Bijleveld CM, de Langen ZJ, Plukker JT. Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia. J Pediatr Surg 2005; 40 (12) e1-e4
  PubMedGoogle Scholar
• 17 Esquibies AE, Zambrano E, Ziai J , et al. Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia. Pediatr Pulmonol 2010; 45 (2) 202-204
  CrossrefPubMedGoogle Scholar
• 18 LaQuaglia MP, Gray M, Schuster SR. Esophageal atresia and ante-thoracic skin tube esophageal conduits: squamous cell carcinoma in the conduit 44 years following surgery. J Pediatr Surg 1987; 22 (1) 44-47
  CrossrefPubMedGoogle Scholar
• 19 van Wijk M, Knüppe F, Omari T, de Jong J, Benninga M. Evaluation of gastroesophageal function and mechanisms underlying gastroesophageal reflux in infants and adults born with esophageal atresia. J Pediatr Surg 2013; 48 (12) 2496-2505
  CrossrefPubMedGoogle Scholar
• 20 Tovar JA, Fragoso AC. Gastroesophageal reflux after repair of esophageal atresia. Eur J Pediatr Surg 2013; 23 (3) 175-181
  Article in Thieme ConnectPubMedGoogle Scholar
• 21 Wheatley MJ, Coran AG, Wesley JR. Efficacy of the Nissen fundoplication in the management of gastroesophageal reflux following esophageal atresia repair. J Pediatr Surg 1993; 28 (1) 53-55
  CrossrefPubMedGoogle Scholar
• 22 Pedersen RN, Markøw S, Kruse-Andersen S , et al. Esophageal atresia: gastroesophageal functional follow-up in 5-15 year old children. J Pediatr Surg 2013; 48 (12) 2487-2495
  CrossrefPubMedGoogle Scholar
• 23 Catalano P, Di Pace MR, Caruso AM, Casuccio A, De Grazia E. Gastroesophageal reflux in young children treated for esophageal atresia: evaluation with pH-multichannel intraluminal impedance. J Pediatr Gastroenterol Nutr 2011; 52 (6) 686-690
  CrossrefPubMedGoogle Scholar
• 24 Castilloux J, Bouron-Dal Soglio D, Faure C. Endoscopic assessment of children with esophageal atresia: Lack of relationship of esophagitis and esophageal metaplasia to symptomatology. Can J Gastroenterol 2010; 24 (5) 312-316
  PubMedGoogle Scholar
• 25 Kawahara H, Okuyama H, Mitani Y , et al. Influence of thoracoscopic esophageal atresia repair on esophageal motor function and gastroesophageal reflux. J Pediatr Surg 2009; 44 (12) 2282-2286
  CrossrefPubMedGoogle Scholar
• 26 Koivusalo A, Pakarinen MP, Rintala RJ. The cumulative incidence of significant gastrooesophageal reflux in patients with oesophageal atresia with a distal fistula—a systematic clinical, pH-metric, and endoscopic follow-up study. J Pediatr Surg 2007; 42 (2) 370-374
  CrossrefPubMedGoogle Scholar
• 27 Lindahl H, Rintala R, Sariola H. Chronic esophagitis and gastric metaplasia are frequent late complications of esophageal atresia. J Pediatr Surg 1993; 28 (9) 1178-1180
  CrossrefPubMedGoogle Scholar
• 28 Gatzinsky V, Andersson O, Eriksson A, Jönsson L, Abrahamsson K, Sillén U. Added Value of pH Multichannel Intraluminal Impedance in Adults Operated for Esophageal Atresia. Eur J Pediatr Surg 2015;
  PubMedGoogle Scholar
• 29 Huynh Trudeau V, Maynard S, Terzic T, Soucy G, Bouin M. Dysphagia among adult patients who underwent surgery for esophageal atresia. Chin J Gastroenterol Hepatol 2015; 29 (2) 91-94
  PubMedGoogle Scholar
• 30 Deurloo JA, Klinkenberg EC, Ekkelkamp S, Heij HA, Aronson DC. Adults with corrected oesophageal atresia: is oesophageal function associated with complaints and/or quality of life?. Pediatr Surg Int 2008; 24 (5) 537-541
  CrossrefPubMedGoogle Scholar
• 31 Krug E, Bergmeijer JH, Dees J, de Krijger R, Mooi WJ, Hazebroek FW. Gastroesophageal reflux and Barrett's esophagus in adults born with esophageal atresia. Am J Gastroenterol 1999; 94 (10) 2825-2828
  CrossrefPubMedGoogle Scholar
• 32 Deurloo JA, Ekkelkamp S, Bartelsman JF , et al. Gastroesophageal reflux: prevalence in adults older than 28 years after correction of esophageal atresia. Ann Surg 2003; 238 (5) 686-689
  CrossrefPubMedGoogle Scholar
• 33 Tovar JA, Diez Pardo JA, Murcia J, Prieto G, Molina M, Polanco I. Ambulatory 24-hour manometric and pH metric evidence of permanent impairment of clearance capacity in patients with esophageal atresia. J Pediatr Surg 1995; 30 (8) 1224-1231
  CrossrefPubMedGoogle Scholar
• 34 Koivusalo AI, Pakarinen MP, Rintala RJ. Modern outcomes of oesophageal atresia: single centre experience over the last twenty years. J Pediatr Surg 2013; 48 (2) 297-303
  CrossrefPubMedGoogle Scholar
• 35 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 (1) 25-29
  Article in Thieme ConnectPubMedGoogle Scholar
• 36 Deurloo JA, Ekkelkamp S, Taminiau JA , et al. Esophagitis and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg 2005; 40 (8) 1227-1231
  CrossrefPubMedGoogle Scholar
• 37 Schalamon J, Lindahl H, Saarikoski H, Rintala RJ. Endoscopic follow-up in esophageal atresia-for how long is it necessary?. J Pediatr Surg 2003; 38 (5) 702-704
  CrossrefPubMedGoogle Scholar
• 38 Tomaselli V, Volpi ML, Dell'Agnola CA, Bini M, Rossi A, Indriolo A. Long-term evaluation of esophageal function in patients treated at birth for esophageal atresia. Pediatr Surg Int 2003; 19 (1-2) 40-43
  PubMedGoogle Scholar
• 39 Somppi E, Tammela O, Ruuska T , et al. Outcome of patients operated on for esophageal atresia: 30 years' experience. J Pediatr Surg 1998; 33 (9) 1341-1346
  CrossrefPubMedGoogle Scholar
• 40 Connor MJ, Springford LR, Kapetanakis VV, Giuliani S. Esophageal atresia and transitional care—step 1: a systematic review and meta-analysis of the literature to define the prevalence of chronic long-term problems. Am J Surg 2015; 209 (4) 747-759
  CrossrefPubMedGoogle Scholar
• 41 Dent J, El-Serag HB, Wallander MA, Johansson S. Epidemiology of gastro-oesophageal reflux disease: a systematic review. Gut 2005; 54 (5) 710-717
  CrossrefPubMedGoogle Scholar
• 42 Hershcovici T, Fass R. Nonerosive Reflux Disease (NERD) - An Update. J Neurogastroenterol Motil 2010; 16 (1) 8-21
  CrossrefPubMedGoogle Scholar
• 43 Katz PO, Gerson LB, Vela MF. Guidelines for the diagnosis and management of gastroesophageal reflux disease. Am J Gastroenterol 2013; 108 (3) 308-328 , quiz 329
  CrossrefPubMedGoogle Scholar
• 44 Liacouras CA, Furuta GT, Hirano I, Atkins D, Attwood SE, Bonis PA , et al. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol 2011; 128 (1) 3-20.e6 , quiz 21–22
  CrossrefPubMedGoogle Scholar
• 45 Ismail-Beigi F, Horton PF, Pope II CE. Histological consequences of gastroesophageal reflux in man. Gastroenterology 1970; 58 (2) 163-174
  CrossrefPubMedGoogle Scholar
• 46 Schindlbeck NE, Wiebecke B, Klauser AG, Voderholzer WA, Müller-Lissner SA. Diagnostic value of histology in non-erosive gastro-oesophageal reflux disease. Gut 1996; 39 (2) 151-154
  CrossrefPubMedGoogle Scholar
• 47 Shawyer AC, D'Souza J, Pemberton J, Flageole H. The management of postoperative reflux in congenital esophageal atresia-tracheoesophageal fistula: a systematic review. Pediatr Surg Int 2014; 30 (10) 987-996
  CrossrefPubMedGoogle Scholar
• 48 Bergmeijer JH, Tibboel D, Hazebroek FW. Nissen fundoplication in the management of gastroesophageal reflux occurring after repair of esophageal atresia. J Pediatr Surg 2000; 35 (4) 573-576
  CrossrefPubMedGoogle Scholar
• 49 Godoy J, Tovar JA, Vicente Y, Olivares P, Molina M, Prieto G. Esophageal motor dysfunction persists in children after surgical cure of reflux: an ambulatory manometric study. J Pediatr Surg 2001; 36 (9) 1405-1411
  CrossrefPubMedGoogle Scholar
• 50 Lundell LR, Dent J, Bennett JR , et al. Endoscopic assessment of oesophagitis: clinical and functional correlates and further validation of the Los Angeles classification. Gut 1999; 45 (2) 172-180
  CrossrefPubMedGoogle Scholar
• 51 Zagari RM, Fuccio L, Wallander MA , et al. Gastro-oesophageal reflux symptoms, oesophagitis and Barrett's oesophagus in the general population: the Loiano-Monghidoro study. Gut 2008; 57 (10) 1354-1359
  CrossrefPubMedGoogle Scholar
• 52 Ronkainen J, Aro P, Storskrubb T , et al. High prevalence of gastroesophageal reflux symptoms and esophagitis with or without symptoms in the general adult Swedish population: a Kalixanda study report. Scand J Gastroenterol 2005; 40 (3) 275-285
  CrossrefPubMedGoogle Scholar
• 53 Wang KK, Sampliner RE ; Practice Parameters Committee of the American College of Gastroenterology. Updated guidelines 2008 for the diagnosis, surveillance and therapy of Barrett's esophagus. Am J Gastroenterol 2008; 103 (3) 788-797
  CrossrefPubMedGoogle Scholar
• 54 Fitzgerald RC, di Pietro M, Ragunath K , et al; British Society of Gastroenterology. British Society of Gastroenterology guidelines on the diagnosis and management of Barrett's oesophagus. Gut 2014; 63 (1) 7-42
  CrossrefPubMedGoogle Scholar
• 55 Paull A, Trier JS, Dalton MD, Camp RC, Loeb P, Goyal RK. The histologic spectrum of Barrett's esophagus. N Engl J Med 1976; 295 (9) 476-480
  CrossrefPubMedGoogle Scholar
• 56 Riddell RH, Odze RD. Definition of Barrett's esophagus: time for a rethink—is intestinal metaplasia dead?. Am J Gastroenterol 2009; 104 (10) 2588-2594
  CrossrefPubMedGoogle Scholar
• 57 Allum WH, Blazeby JM, Griffin SM, Cunningham D, Jankowski JA, Wong R ; Association of Upper Gastrointestinal Surgeons of Great Britain and Ireland, the British Society of Gastroenterology and the British Association of Surgical Oncology. Guidelines for the management of oesophageal and gastric cancer. Gut 2011; 60 (11) 1449-1472
  CrossrefPubMedGoogle Scholar
• 58 Lightdale CJ ; American College of Gastroenterology. Esophageal cancer. Am J Gastroenterol 1999; 94 (1) 20-29
  CrossrefPubMedGoogle Scholar
• 59 Didden P, Koch AD. Endoscopic assessment of early neoplasia in the gastrointestinal tract. EMJ Gastroenterol 2012; 1: 45-52
  PubMedGoogle Scholar
• 60 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 (3) 249-257
  CrossrefPubMedGoogle Scholar
• 61 Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, Pakarinen MP. Cancer after repair of esophageal atresia: population-based long-term follow-up. J Pediatr Surg 2008; 43 (4) 602-605
  CrossrefPubMedGoogle Scholar