Narrative Review
Management of Adults With Esophageal Atresia

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Esophageal atresia (EA) with or without trachea-esophageal fistula is relatively common congenital malformation with most patients living into adulthood. As a result, care of the adult patient with EA is becoming more common. Although surgical repair has changed EA from a fatal to a livable condition, the residual effects of the anomaly may lead to a lifetime of complications. These include effects related to the underlying deformity such as atonicity of the esophageal segment, fistula recurrence, and esophageal cancer to complications of the surgery including anastomotic stricture, gastroesophageal reflux, and coping with an organ transposition. This review discusses the occurrence and management of these conditions in adulthood and the role of an effective transition from pediatric to adult care to optimize adult care treatment.

Section snippets

Description of the Disease

EA is the most common congenital anomaly of the esophagus, occurring in approximately 1 in 3000 live births3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 (Figure 1). Type C type distal TEF occurring in 85% of cases is the most common type of EA. The embryologic origin of EA is thought to be an incomplete evagination of the tracheal diverticulum to form the trachea, failure to form septal tissue dividing the esophagus and trachea, or regression of initially successful esophageal tube formation. Several

Gastroesophageal Reflux Disease

The main complications that follow EA surgery persisting into adulthood are gastroesophageal reflux disease (GERD) and dysphagia. GERD is reported to occur in 27% to 75% of adult patients with EA depending on GERD definition.27, 21, 22, 28, 29, 30 In one study, erosive esophagitis found in 19% of patients.31 When GERD is defined by symptoms, there is a marked variation in the reported prevalence of GERD symptoms in the literature ranging between 17% and 75%.28,32,33 This is potentially

Barrett’s Esophagus and Esophageal Cancer

One of the concerns in adult patients with EA is the development of Barrett’s metaplasia owing to chronic gastroesophageal reflux.27,29,30,69 Patients as young as 20 years old with Barrett’s have been described.70,71 An interesting finding from multiple studies is that grossly visible metaplastic epithelium seen on endoscopy does not consistently indicate the presence of intestinal metaplasia. For example, in one study of 60 patients with EA followed for 30 years, Barrett’s esophagus (BE) was

Conclusion

Previously a congenital anomaly that uniformly led to death in infancy, patients with EA can expect to live long healthy lives. Nevertheless, the surgical magnitude of initial therapy, the chronic complications that may ensue from this disorder and its correction, and the potential for health-impairing additional congenital anomalies mandate a lifetime of medical care and supervision. This supervision needs to attend to multiple potential issues including dysphagia from anastomotic strictures

CRediT Authorship Contributions

Julie Khlevner (Conceptualization; Writing Review and Editing; Supervision)

Daniela Jodorkovsky (Conceptualization; Writing Review and Editing; Supervision)

Dominique D. Bailey (Conceptualization; Writing Review and Editing; Supervision)

William Middlesworth (Conceptualization; Writing Review and Editing; Supervision)

Amrita Sethi (Conceptualization; Writing Review and Editing; Supervision)

Julian Abrams (Conceptualization; Writing Review and Editing; Supervision)

David A. Katzka (Conceptualization;

References (111)

  • J.A. Deurloo et al.

    Esophagitis and Barrett esophagus after correction of esophageal atresia

    J Pediatr Surg

    (2005)
  • H. Kawahara et al.

    Lack of distal esophageal contractions is a key determinant of gastroesophageal reflux disease after repair of esophageal atresia

    J Pediatr Surg

    (2007)
  • E. Somppi et al.

    Outcome of patients operated on for esophageal atresia: 30 years' experience

    J Pediatr Surg

    (1998)
  • E. Hassall et al.

    Omeprazole for treatment of chronic erosive esophagitis in children: a multicenter study of efficacy, safety, tolerability and dose requirements. International Pediatric Omeprazole Study Group

    J Pediatr

    (2000)
  • S. Van Biervliet et al.

    High-dose omeprazole in esophagitis with stenosis after surgical treatment of esophageal atresia

    J Pediatr Surg

    (2001)
  • F.W.T. Vergouwe et al.

    High prevalence of Barrett’s esophagus and esophageal squamous cell carcinoma after repair of esophageal atresia

    Clin Gastroenterol Hepatol

    (2018)
  • T.M. Holder et al.

    The treatment of patients with esophageal strictures by local steroid injections

    J Pediatr Surg

    (1969)
  • N.N. Zein et al.

    Endoscopic intralesional steroid injections in the management of refractory esophageal strictures

    Gastrointest Endosc

    (1995)
  • M.J. Connor et al.

    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)
  • M.R. Di Pace et al.

    Evaluation of esophageal motility and reflux in children treated for congenital diaphragmatic hernia with the use of combined multichannel intraluminal impedance and pH monitoring

    J Pediatr Surg

    (2011)
  • F. Pederiva et al.

    Tracheal innervation is abnormal in rats with experimental congenital diaphragmatic hernia

    J Pediatr Surg

    (2009)
  • K. Li et al.

    The structural characteristics and expression of neuropeptides in the esophagus of patients with congenital esophageal atresia and tracheoesophageal fistula

    J Pediatr Surg

    (2007)
  • J. Bokay et al.

    Myoelectrical activity of the stomach after surgical correction of esophageal atresia

    J Pediatr Surg

    (2005)
  • M.R. Di Pace et al.

    Evaluation of esophageal motility and reflux in children treated for esophageal atresia with the use of combined multichannel intraluminal impedance and pH monitoring

    J Pediatr Surg

    (2011)
  • E. Hannon et al.

    Outcomes in adulthood of gastric transposition for complex and long gap esophageal atresia

    J Pediatr Surg

    (2020)
  • N.S. Adzick et al.

    Esophageal adenocarcinoma 20 years after esophageal atresia repair

    J Pediatr Surg

    (1989)
  • L. Alfaro et al.

    Are patients who have had a tracheoesophageal fistula repair during infancy at risk for esophageal adenocarcinoma during adulthood?

    J Pediatr Surg

    (2005)
  • B.B. Pultrum et al.

    Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia

    J Pediatr Surg

    (2005)
  • C.S. Jayasekera et al.

    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)
  • J.A. Deurloo et al.

    Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia

    J Pediatr Surg

    (2001)
  • M. Pesce et al.

    Is There a role for pH impedance monitoring in identifying eosinophilic esophagitis in children with esophageal atresia?

    J Pediatr

    (2019)
  • W. Cheng et al.

    Esophageal atresia and achalasialike esophageal dysmotility

    J Pediatr Surg

    (2004)
  • J. Zacharias et al.

    Congenital tracheoesophageal fistulas presenting in adults: presentation of two cases and a synopsis of the literature

    J Thorac Cardiovasc Surg

    (2004)
  • D. Newberry et al.

    A “little cough” for 40 years

    Lancet

    (1999)
  • D. Azoulay et al.

    Congenital respiratory-esophageal fistula in the adult. Report of nine cases and review of the literature

    J Thorac Cardiovasc Surg

    (1992)
  • M. Kutiyanawala et al.

    CHARGE and esophageal atresia

    J Pediatr Surg

    (1992)
  • C.P. Driver et al.

    Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification

    J Pediatr Surg

    (2001)
  • C.A. Ten Kate et al.

    Patient-driven healthcare recommendations for adults with esophageal atresia and their families

    J Pediatr Surg

    (2021)
  • C. Rabone et al.

    A thematic analysis exploring the psychological well-being of adults born with esophageal atresia

    J Psychosom Res

    (2021)
  • C.T. Mai et al.

    National Birth Defects Prevention Network. National population-based estimates for major birth defects, 2010-2014

    Birth Defects Res

    (2019)
  • J.L. Grosfeld

    Pediatric surgery

    (2006)
  • N. Nassar et al.

    Prevalence of esophageal atresia among 18 international birth defects surveillance programs

    Birth Defects Res A Clin Mol Teratol

    (2012)
  • L. Spitz

    Oesophageal atresia

    Orphanet J Rare Dis

    (2007)
  • P.S. Liebert

    Color atlas of pediatric surgery

    (1996)
  • J. Seo et al.

    An 18-year experience of tracheoesophageal fistula and esophageal atresia

    Korean J Pediatr

    (2010)
  • A.J. Holland et al.

    Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications

    Paediatr Respir Rev

    (2010)
  • G.W. Holcombe et al.

    Ashcraft’s pediatric surgery

    (2014)
  • D. Gibelli et al.

    Prevalence of accessory septations of sphenoid sinus in pediatric population: applications to endoscopic sinus surgery

    Anat Rec (Hoboken)

    (2020)
  • P. Downey et al.

    Recurrent and congenital tracheoesophageal fistula in adults

    Eur J Cardiothorac Surg

    (2017)
  • S. Bairdain et al.

    Long-gap esophageal atresia is a unique entity within the esophageal atresia defect spectrum

    Neonatology

    (2017)
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    Conflicts of interest These authors disclose the following: Julie Khlevner reports speaker’s bureau for Abbott Pediatric Nutrition. Amrita Sethi reports consultant for Boston Scientific, Olympus, Fujifilm, and Medtronic. Julian Abrams reports consulting for Exact Sciences and Castle Biosciences; and research support from Pentax. David A. Katzka reports consulting for Regeneron and Celgene. The remaining authors disclose no conflicts.

    Authors share co-first authorship.

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