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Esophageal Atresia and Tracheoesophageal Fistula
Steven J. Kraus, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Esophageal atresia (EA): Congenital occlusion of upper esophagus
              • Tracheoesophageal fistula (TEF): Single (less commonly multiple) anomalous congenital connection(s) from esophagus to trachea

            IMAGING

            • General Features

              • Radiographic Findings

                • Fluoroscopic Findings

                  • Ultrasonographic Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Medscape: Imaging in esophageal atresia and tracheoesophageal fistula. http://emedicine.medscape.com/article/414368-overview. Reviewed July, 20, 2016. Accessed July 20, 2016
                                              2. Teague WJ et al: Surgical management of oesophageal atresia. Paediatr Respir Rev. 19:10-5, 2016
                                              3. Muensterer OJ et al: From Vogt to Haight and Holt to now: the history of esophageal atresia over the last century. Pediatr Radiol. 45(8):1230-5, 2015
                                              4. Yalcin S et al: The evaluation of deglutition with videofluoroscopy after repair of esophageal atresia and/or tracheoesophageal fistula. J Pediatr Surg. 50(11):1823-7, 2015
                                              5. Liszewski MC et al: Imaging of long gap esophageal atresia and the Foker process: expected findings and complications. Pediatr Radiol. 44(4):467-75, 2014
                                              6. Smith N: Oesophageal atresia and tracheo-oesophageal fistula. Early Hum Dev. 90(12):947-50, 2014
                                              7. Zani A et al: International survey on the management of esophageal atresia. Eur J Pediatr Surg. 24(1):3-8, 2014
                                              8. Kuti K et al: A rare pitfall in the diagnosis of oesophageal atresia. Pediatr Radiol. 43(8):902-4, 2013
                                              9. Sodhi KS et al: Postoperative appearances of esophageal atresia repair: retrospective study of 210 patients with review of literature - what the radiologist should know. Acta Radiol. 54(2):221-5, 2013
                                              10. Thyoka M et al: Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia: a systematic review. Pediatr Radiol. 43(8):898-901; quiz 896-7, 2013
                                              11. Burjonrappa S et al: Type A esophageal atresia: a critical review of management strategies at a single center. J Pediatr Surg. 45(5):865-71, 2010
                                              12. Serhal L et al: Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations. J Pediatr Surg. 45(7):1459-62, 2010
                                              13. Knight RB et al: Pharyngeal perforation masquerading as esophageal atresia. J Pediatr Surg. 44(11):2216-8, 2009
                                              14. Mortell AE et al: Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg. 18(1):12-9, 2009
                                              15. Stoll C et al: Associated malformations in patients with esophageal atresia. Eur J Med Genet. 52(5):287-90, 2009
                                              16. Houben CH et al: Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat Diagn. 28(7):667-75, 2008
                                              17. Achildi O et al: Congenital anomalies of the esophagus. Otolaryngol Clin North Am. 40(1):219-44, viii, 2007
                                              18. Kovesi T et al: Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 126(3):915-25, 2004
                                              19. Orford J et al: Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int. 20(6):402-7, 2004
                                              20. Crabbe DC: Isolated tracheo-oesophageal fistula. Paediatr Respir Rev. 4(1):74-8, 2003
                                              21. Dahms BB: The Gastrointestinal Tract. In Stocker et al: Pediatric Pathology. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 633-34, 2001
                                              22. Clark DC: Esophageal atresia and tracheoesophageal fistula. Am Fam Physician. 59(4):910-6, 919-20, 1999
                                              23. De Felice C et al: Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 15(3-4):260-3, 1999
                                              24. Imaizumi K et al: Association of microphthalmia with esophageal atresia: report of two new patients and review of the literature. Am J Med Genet. 87(2):180-2, 1999
                                              25. del Rosario JF et al: Common pediatric esophageal disorders. Gastroenterologist. 6(2):104-21, 1998
                                              26. Thakral CL et al: Concurrent right diaphragmatic hernia and esophageal atresia. Pediatr Surg Int. 14(1-2):96-7, 1998
                                              27. al-Salem AH et al: Concurrent left congenital diaphragmatic hernia and esophageal atresia: case report and review of the literature. J Pediatr Surg. 32(5):772-4, 1997
                                              28. Sapin E et al: Coexisting left congenital diaphragmatic hernia and esophageal atresia with tracheoesophageal fistula: successful management in a premature neonate. J Pediatr Surg. 31(7):989-91, 1996
                                              29. Snyder CL et al: Esophageal duplication cyst with esophageal web and tracheoesophageal fistula. J Pediatr Surg. 31(7):968-9, 1996
                                              30. Benjamin B et al: Diagnosis of H-type tracheoesophageal fistula. J Pediatr Surg. 26(6):667-71, 1991
                                              31. Berdon WE et al: Radiographic findings in esophageal atresia with proximal pouch fistula (type B). Pediatr Radiol. 3(2):70-4, 1975
                                              32. Kirkpatrick JA et al: A complex of anomalies associated with tracheoesophageal fistula and esophageal atresia. Am J Roentgenol Radium Ther Nucl Med. 95:208-11, 1965
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Definitions

                                                          • Esophageal atresia (EA): Congenital occlusion of upper esophagus
                                                          • Tracheoesophageal fistula (TEF): Single (less commonly multiple) anomalous congenital connection(s) from esophagus to trachea

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • Fluoroscopic Findings

                                                              • Ultrasonographic Findings

                                                                • MR Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Medscape: Imaging in esophageal atresia and tracheoesophageal fistula. http://emedicine.medscape.com/article/414368-overview. Reviewed July, 20, 2016. Accessed July 20, 2016
                                                                                          2. Teague WJ et al: Surgical management of oesophageal atresia. Paediatr Respir Rev. 19:10-5, 2016
                                                                                          3. Muensterer OJ et al: From Vogt to Haight and Holt to now: the history of esophageal atresia over the last century. Pediatr Radiol. 45(8):1230-5, 2015
                                                                                          4. Yalcin S et al: The evaluation of deglutition with videofluoroscopy after repair of esophageal atresia and/or tracheoesophageal fistula. J Pediatr Surg. 50(11):1823-7, 2015
                                                                                          5. Liszewski MC et al: Imaging of long gap esophageal atresia and the Foker process: expected findings and complications. Pediatr Radiol. 44(4):467-75, 2014
                                                                                          6. Smith N: Oesophageal atresia and tracheo-oesophageal fistula. Early Hum Dev. 90(12):947-50, 2014
                                                                                          7. Zani A et al: International survey on the management of esophageal atresia. Eur J Pediatr Surg. 24(1):3-8, 2014
                                                                                          8. Kuti K et al: A rare pitfall in the diagnosis of oesophageal atresia. Pediatr Radiol. 43(8):902-4, 2013
                                                                                          9. Sodhi KS et al: Postoperative appearances of esophageal atresia repair: retrospective study of 210 patients with review of literature - what the radiologist should know. Acta Radiol. 54(2):221-5, 2013
                                                                                          10. Thyoka M et al: Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia: a systematic review. Pediatr Radiol. 43(8):898-901; quiz 896-7, 2013
                                                                                          11. Burjonrappa S et al: Type A esophageal atresia: a critical review of management strategies at a single center. J Pediatr Surg. 45(5):865-71, 2010
                                                                                          12. Serhal L et al: Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations. J Pediatr Surg. 45(7):1459-62, 2010
                                                                                          13. Knight RB et al: Pharyngeal perforation masquerading as esophageal atresia. J Pediatr Surg. 44(11):2216-8, 2009
                                                                                          14. Mortell AE et al: Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg. 18(1):12-9, 2009
                                                                                          15. Stoll C et al: Associated malformations in patients with esophageal atresia. Eur J Med Genet. 52(5):287-90, 2009
                                                                                          16. Houben CH et al: Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat Diagn. 28(7):667-75, 2008
                                                                                          17. Achildi O et al: Congenital anomalies of the esophagus. Otolaryngol Clin North Am. 40(1):219-44, viii, 2007
                                                                                          18. Kovesi T et al: Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 126(3):915-25, 2004
                                                                                          19. Orford J et al: Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int. 20(6):402-7, 2004
                                                                                          20. Crabbe DC: Isolated tracheo-oesophageal fistula. Paediatr Respir Rev. 4(1):74-8, 2003
                                                                                          21. Dahms BB: The Gastrointestinal Tract. In Stocker et al: Pediatric Pathology. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 633-34, 2001
                                                                                          22. Clark DC: Esophageal atresia and tracheoesophageal fistula. Am Fam Physician. 59(4):910-6, 919-20, 1999
                                                                                          23. De Felice C et al: Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 15(3-4):260-3, 1999
                                                                                          24. Imaizumi K et al: Association of microphthalmia with esophageal atresia: report of two new patients and review of the literature. Am J Med Genet. 87(2):180-2, 1999
                                                                                          25. del Rosario JF et al: Common pediatric esophageal disorders. Gastroenterologist. 6(2):104-21, 1998
                                                                                          26. Thakral CL et al: Concurrent right diaphragmatic hernia and esophageal atresia. Pediatr Surg Int. 14(1-2):96-7, 1998
                                                                                          27. al-Salem AH et al: Concurrent left congenital diaphragmatic hernia and esophageal atresia: case report and review of the literature. J Pediatr Surg. 32(5):772-4, 1997
                                                                                          28. Sapin E et al: Coexisting left congenital diaphragmatic hernia and esophageal atresia with tracheoesophageal fistula: successful management in a premature neonate. J Pediatr Surg. 31(7):989-91, 1996
                                                                                          29. Snyder CL et al: Esophageal duplication cyst with esophageal web and tracheoesophageal fistula. J Pediatr Surg. 31(7):968-9, 1996
                                                                                          30. Benjamin B et al: Diagnosis of H-type tracheoesophageal fistula. J Pediatr Surg. 26(6):667-71, 1991
                                                                                          31. Berdon WE et al: Radiographic findings in esophageal atresia with proximal pouch fistula (type B). Pediatr Radiol. 3(2):70-4, 1975
                                                                                          32. Kirkpatrick JA et al: A complex of anomalies associated with tracheoesophageal fistula and esophageal atresia. Am J Roentgenol Radium Ther Nucl Med. 95:208-11, 1965