Since the first successful surgery in 1941 (1), the survival of patients with esophageal atresia (EA) has improved greatly. Consequently, the follow-up of these patients has revealed gastrointestinal (GI) complications unrecognized 60 years ago, such as gastroesophageal reflux and esophagitis with their related consequences. Among these complications, 1 of the major concerns in long-term GI follow-up is the increased incidence of gastric metaplasia of the esophagus (2,3), intestinal metaplasia (4,5), and esophageal adenocarcinoma (6–8), all of which have been reported in young adults with EA (5–7). However, the exact incidence and natural history of these complications are unknown in patients with EA, and whether systematic upper GI endoscopic screening and surveillance in the follow-up of patients with EA is recommended is not yet established.
In this article, we review the upper GI endoscopy findings in patients with EA, the specificities of upper GI endoscopy in patients with EA (How to scope them? What about landmarks in EA? Do we need to scope the patients? When? How often?), and present a proposed algorithm for the surveillance of patients, children, and adults with EA. We do not address the problem of congenital stenosis or anastomotic and peptic strictures.
WHAT IS ACTUALLY OBSERVED IN PATIENTS WITH EA?
Esophagitis and Long-Term Consequences of Esophagitis
Peptic esophagitis (2,9) and Barrett esophagus (2,9) have been reported in children (2,3,9,10) and more recently in adults with repaired EA (Fig. 1) (4,5,11,12). We recently reported on a systematic cross-sectional endoscopic evaluation in 45 children with EA (median age 7.3 years, range 0.4–17.9). Twenty-six patients (58%) had normal endoscopy, 14 (31%) had esophagitis, and 16 (36%) had gastric metaplasia (10). No intestinal metaplasia or adenocarcinoma was detected, similar to other reports. Data in adults are sparse and have been published recently (4,5,11,12). Maynard et al reported preliminary results of a systematic endoscopic screening in 21 adults ages 27 ± 7 years born with EA (personal communication). Esophagitis was found in 29% of patients (n = 6) and Barrett endoscopically suspected in 24% of patients (n = 5) in whom intestinal metaplasia was documented in 3 cases. These data demonstrate that EA, even in childhood, is a significant risk factor for the development of esophagitis and Barrett esophagus.
Various anecdotal features can also be observed in patients with EA. Some of them may have clinical consequences (eg, diverticulum) and may be related to symptoms. Others such as a high incidence of heterotopic pancreas (13) could lead to a new pathophysiological hypothesis whether or not this association between EA and heterotopic pancreas is related to the same developmental mechanisms. Eosinophilic esophagitis should be ruled out in patients with EA for whom dysphagia worsen without stricture (14).
SPECIFICITIES OF UPPER GI ENDOSCOPY IN EA
Do the Patients Need Endoscopy? When? How Often?
Performing a systematic endoscopic assessment in patients with EA is largely debated in the pediatric (9,15) and, more recently, adult literature (11). The predictive value of an early (<3 years old) normal upper gastrointestinal endoscopy is unknown regarding development of peptic esophagitis and Barrett esophagus later in life. In a study designed to determine whether clinical symptoms may predict endoscopic lesions (10), 63 patients with EA were recruited. Eighteen had dysphagia related to an esophageal stricture needing dilatation and were not included in the analysis. Forty-five patients (26 girls) with a median age of 7.3 years (range 0.4–17.9) were evaluated. Mucosal abnormalities at endoscopy were observed in 19 of 45 patients (42%). Symptoms were reported by 62%, but none could be identified as statistically associated with an abnormal endoscopy. Furthermore, 6 asymptomatic patients had abnormal endoscopies, reflecting the difficulty in adequately deciding which patients need to be investigated (10). Similarly, in adults, the symptoms are frequent but underreported by the patients who do not seek medical attention.
Pitfalls: Of the Importance of Correct Landmarks in Patients With EA
Hiatal hernia or traction on the stomach during surgery can complicate landmark recognition and lead to erroneous diagnosis of gastric metaplasia, mostly in patients with a long-gap atresia. In this regard, the esophageal mucosa should be determined by carefully delimiting the gastroesophageal junction, identified as the proximal margin of the gastric mucosal folds, as defined by Prague C & M criteria (16).
CONCLUSIONs AND PERSPECTIVES
The present data demonstrate the high incidence of esophageal mucosal lesions in pediatric and adult patients with EA without any predictive clinical symptoms. This argues for a systematic screening of endoscopic lesions in all patients with EA even in the absence of upper GI symptoms; a proposed algorithm for the evaluation and surveillance of esophageal mucosa by upper endoscopy in patients with EA is provided (Fig. 2). However, the evaluation of systematic endoscopic follow-up with esophageal biopsies in patients with EA to evaluate accurately the complications, the response to treatment, and the outcome are urgently warranted. The predictive value of a normal endoscopy should be assessed as well as the value of a new technology such as narrow band imaging in the characterization of the gastroesophageal junction of these patients. Finally, a close collaboration should be established with pathologists, the lesions should be described according to standardized landmarks, and biopsy samples should be taken with special care.
1. Haight C, Towslet H. Congenital atresia of osephagus with tracheo-esophageal fistula. Extrapleural ligation of fistula and end-to-end anastomosis of esophageal segments. Surg Gynecol Obstet 1943; 76:672–688.
2. Lindahl H, Rintala R, Sariola H. Chronic esophagitis and gastric metaplasia are frequent late complications of esophageal atresia. J Pediatr Surg 1993; 28:1178–1180.
3. Somppi E, Tammela O, Ruuska T, et al
. Outcome of patients operated on for esophageal atresia: 30 years’ experience. J Pediatr Surg 1998; 33:1341–1346.
4. 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; 238:686–689.
5. Krug E, Bergmeijer JH, Dees J, et al
. Gastroesophageal reflux and Barrett's esophagus in adults born with esophageal atresia. Am J Gastroenterol 1999; 94:2825–2828.
6. Adzick NS, Fisher JH, Winter HS, et al
. Esophageal adenocarcinoma 20 years after esophageal atresia repair. J Pediatr Surg 1989; 24:741–744.
7. Pultrum BB, Bijleveld CM, de Langen ZJ, et al
. Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia. J Pediatr Surg 2005; 40:e1–e4.
8. Alfaro L, Bermas H, Fenoglio M, et al
. Are patients who have had a tracheoesophageal fistula repair during infancy at risk for esophageal adenocarcinoma during adulthood? J Pediatr Surg 2005; 40:719–720.
9. Deurloo JA, Ekkelkamp S, Taminiau JA, et al
. Esophagitis and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg 2005; 40:1227–1231.
10. 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; 24:312–316.
11. 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; 5:702–706.
12. 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; 251:1167–1173.
13. Moreau B, Levesque D, Faure C. Association of gastric heterotopic pancreas and esophageal atresia in children. J Pediatr Gastroenterol Nutr
14. Batres LA, Liacouras C, Schnaufer L, et al
. Eosinophilic esophagitis associated with anastomotic strictures after esophageal atresia repair. J Pediatr Gastroenterol Nutr 2002; 35:224–226.
15. Schalamon J, Lindahl H, Saarikoski H, et al
. Endoscopic follow-up in esophageal atresia-for how long is it necessary? J Pediatr Surg 2003; 38:702–704.
16. Sharma P, Dent J, Armstrong D, et al. The development and validation of an endoscopic grading system for Barrett's esophagus: the Prague C & M Criteria. Gastroenterology
. 2006; 131:1392–1399.