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Esophageal Atresia
Karen Y. Oh, MD; Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Esophagus atresia (EA) often associated with tracheoesophageal fistula (TEF)
                • > 90% have fistula
                • Proximal atresia with distal TEF most common type

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Acher CW et al: Long-term outcomes of patients with tracheoesophageal fistula/fsophageal atresia: survey results from tracheoesophageal fistula/esophageal atresia online communities. Eur J Pediatr Surg. ePub, 2015
                                    2. Cartabuke RH et al: Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula. Gastroenterol Rep (Oxf). ePub, 2015
                                    3. Hochart V et al: The contribution of fetal MR imaging to the assessment of oesophageal atresia. Eur Radiol. 25(2):306-14, 2015
                                    4. Sfeir R et al: National Esophageal Atresia Register. Eur J Pediatr Surg. 25(6):497-9, 2015
                                    5. Ethun CG et al: Fetal MRI improves diagnostic accuracy in patients referred to a fetal center for suspected esophageal atresia. J Pediatr Surg. 49(5):712-5, 2014
                                    6. Garabedian C et al: Does a combination of ultrasound, MRI, and biochemical amniotic fluid analysis improve prenatal diagnosis of esophageal atresia? Prenat Diagn. 34(9):839-42, 2014
                                    7. Kunisaki SM et al: The diagnosis of fetal esophageal atresia and its implications on perinatal outcome. Pediatr Surg Int. 30(10):971-7, 2014
                                    8. Sfeir R et al: Esophageal atresia: data from a national cohort. J Pediatr Surg. 48(8):1664-9, 2013
                                    9. Pedersen RN et al: Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child. 97(3):227-32, 2012
                                    10. 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
                                    11. de Jong EM et al: Etiology of esophageal atresia and tracheoesophageal fistula: "mind the gap". Curr Gastroenterol Rep. 12(3):215-22, 2010
                                    12. El-Gohary Y et al: Congenital anomalies of the esophagus. Semin Pediatr Surg. 19(3):186-93, 2010
                                    13. González-Hernández J et al: Esophageal atresia: new guidelines in management. Bol Asoc Med P R. 102(1):33-8, 2010
                                    14. Guo W et al: Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula. J Pediatr Surg. 45(12):2351-5, 2010
                                    15. Lacher M et al: Early and long term outcome in children with esophageal atresia treated over the last 22 years. Klin Padiatr. 222(5):296-301, 2010
                                    16. Oddsberg J et al: Maternal diabetes and risk of esophageal atresia. J Pediatr Surg. 45(10):2004-8, 2010
                                    17. Peker E et al: TAR syndrome and esophagial atresia: a concomitant or variant condition? J Matern Fetal Neonatal Med. Epub ahead of print, 2010
                                    18. Quarello E et al: Prenatal diagnosis and evaluation of the length of the defect in esophageal atresia using direct and indirect signs (tracheal print). Ultrasound Obstet Gynecol. Epub ahead of print, 2010
                                    19. Seo J et al: An 18-year experience of tracheoesophageal fistula and esophageal atresia. Korean J Pediatr. 53(6):705-10, 2010
                                    20. Solt I et al: The esophageal 'pouch sign': a benign transient finding. Prenat Diagn. 30(9):845-8, 2010
                                    21. Stringel G et al: Repair of long gap esophageal atresia without anastomosis. J Pediatr Surg. 45(5):872-5, 2010
                                    22. Tröbs RB et al: Embryogenesis of esophageal atresia: Is localized vascular accident a factor? J Indian Assoc Pediatr Surg. 15(3):112-3, 2010
                                    23. Salomon LJ et al: Real-time fetal magnetic resonance imaging for the dynamic visualization of the pouch in esophageal atresia. Ultrasound Obstet Gynecol. 34(4):471-4, 2009
                                    24. Has R et al: Pouch sign in prenatal diagnosis of esophageal atresia. Ultrasound Obstet Gynecol. 23(5):523-4, 2004
                                    25. Kovesi T et al: Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 126(3):915-25, 2004
                                    26. Malinger G et al: The fetal esophagus: anatomical and physiological ultrasonographic characterization using a high-resolution linear transducer. Ultrasound Obstet Gynecol. 24(5):500-5, 2004
                                    27. Marquette GP et al: First-trimester imaging of combined esophageal and duodenal atresia without a tracheoesophageal fistula. J Ultrasound Med. 23(9):1232, 2004
                                    28. Samujh R et al: Oesophageal atresia and biliary atresia: a rare association. Pediatr Surg Int. 20(6):467-8, 2004
                                    29. Tonz M et al: Oesophageal atresia: what has changed in the last 3 decades? Pediatr Surg Int. 20(10):768-72, 2004
                                    30. Kalache KD et al: Prognostic significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia. Am J Obstet Gynecol. 182(4):978-81, 2000
                                    31. Sparey C et al: Esophageal atresia in the Northern Region Congenital Anomaly Survey, 1985-1997: prenatal diagnosis and outcome. Am J Obstet Gynecol. 182(2):427-31, 2000
                                    32. Kalache KD et al: The upper neck pouch sign: a prenatal sonographic marker for esophageal atresia. Ultrasound Obstet Gynecol. 11(2):138-40, 1998
                                    33. Satoh S et al: Antenatal sonographic detection of the proximal esophageal segment: specific evidence for congenital esophageal atresia. J Clin Ultrasound. 23(7):419-23, 1995
                                    34. Stringer MD et al: Prenatal diagnosis of esophageal atresia. J Pediatr Surg. 30(9):1258-63, 1995
                                    35. Estroff JA et al: Second trimester prenatal findings in duodenal and esophageal atresia without tracheoesophageal fistula. J Ultrasound Med. 13(5):375-9, 1994
                                    36. Surana R et al: Small intestinal atresia: effect on fetal nutrition. J Pediatr Surg. 29(9):1250-2, 1994
                                    37. Millener PB et al: Prognostic significance of nonvisualization of the fetal stomach by sonography. AJR Am J Roentgenol. 160(4):827-30, 1993
                                    38. Pretorius DH et al: Tracheoesophageal fistula in utero. Twenty-two cases. J Ultrasound Med. 6(9):509-13, 1987
                                    39. Jolleys A: An examination of the birthweights of babies with some abnormalities of the alimentary tract. J Pediatr Surg. 16(2):160-3, 1981
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Definitions

                                                • Esophagus atresia (EA) often associated with tracheoesophageal fistula (TEF)
                                                  • > 90% have fistula
                                                  • Proximal atresia with distal TEF most common type

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Acher CW et al: Long-term outcomes of patients with tracheoesophageal fistula/fsophageal atresia: survey results from tracheoesophageal fistula/esophageal atresia online communities. Eur J Pediatr Surg. ePub, 2015
                                                                      2. Cartabuke RH et al: Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula. Gastroenterol Rep (Oxf). ePub, 2015
                                                                      3. Hochart V et al: The contribution of fetal MR imaging to the assessment of oesophageal atresia. Eur Radiol. 25(2):306-14, 2015
                                                                      4. Sfeir R et al: National Esophageal Atresia Register. Eur J Pediatr Surg. 25(6):497-9, 2015
                                                                      5. Ethun CG et al: Fetal MRI improves diagnostic accuracy in patients referred to a fetal center for suspected esophageal atresia. J Pediatr Surg. 49(5):712-5, 2014
                                                                      6. Garabedian C et al: Does a combination of ultrasound, MRI, and biochemical amniotic fluid analysis improve prenatal diagnosis of esophageal atresia? Prenat Diagn. 34(9):839-42, 2014
                                                                      7. Kunisaki SM et al: The diagnosis of fetal esophageal atresia and its implications on perinatal outcome. Pediatr Surg Int. 30(10):971-7, 2014
                                                                      8. Sfeir R et al: Esophageal atresia: data from a national cohort. J Pediatr Surg. 48(8):1664-9, 2013
                                                                      9. Pedersen RN et al: Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child. 97(3):227-32, 2012
                                                                      10. 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
                                                                      11. de Jong EM et al: Etiology of esophageal atresia and tracheoesophageal fistula: "mind the gap". Curr Gastroenterol Rep. 12(3):215-22, 2010
                                                                      12. El-Gohary Y et al: Congenital anomalies of the esophagus. Semin Pediatr Surg. 19(3):186-93, 2010
                                                                      13. González-Hernández J et al: Esophageal atresia: new guidelines in management. Bol Asoc Med P R. 102(1):33-8, 2010
                                                                      14. Guo W et al: Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula. J Pediatr Surg. 45(12):2351-5, 2010
                                                                      15. Lacher M et al: Early and long term outcome in children with esophageal atresia treated over the last 22 years. Klin Padiatr. 222(5):296-301, 2010
                                                                      16. Oddsberg J et al: Maternal diabetes and risk of esophageal atresia. J Pediatr Surg. 45(10):2004-8, 2010
                                                                      17. Peker E et al: TAR syndrome and esophagial atresia: a concomitant or variant condition? J Matern Fetal Neonatal Med. Epub ahead of print, 2010
                                                                      18. Quarello E et al: Prenatal diagnosis and evaluation of the length of the defect in esophageal atresia using direct and indirect signs (tracheal print). Ultrasound Obstet Gynecol. Epub ahead of print, 2010
                                                                      19. Seo J et al: An 18-year experience of tracheoesophageal fistula and esophageal atresia. Korean J Pediatr. 53(6):705-10, 2010
                                                                      20. Solt I et al: The esophageal 'pouch sign': a benign transient finding. Prenat Diagn. 30(9):845-8, 2010
                                                                      21. Stringel G et al: Repair of long gap esophageal atresia without anastomosis. J Pediatr Surg. 45(5):872-5, 2010
                                                                      22. Tröbs RB et al: Embryogenesis of esophageal atresia: Is localized vascular accident a factor? J Indian Assoc Pediatr Surg. 15(3):112-3, 2010
                                                                      23. Salomon LJ et al: Real-time fetal magnetic resonance imaging for the dynamic visualization of the pouch in esophageal atresia. Ultrasound Obstet Gynecol. 34(4):471-4, 2009
                                                                      24. Has R et al: Pouch sign in prenatal diagnosis of esophageal atresia. Ultrasound Obstet Gynecol. 23(5):523-4, 2004
                                                                      25. Kovesi T et al: Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 126(3):915-25, 2004
                                                                      26. Malinger G et al: The fetal esophagus: anatomical and physiological ultrasonographic characterization using a high-resolution linear transducer. Ultrasound Obstet Gynecol. 24(5):500-5, 2004
                                                                      27. Marquette GP et al: First-trimester imaging of combined esophageal and duodenal atresia without a tracheoesophageal fistula. J Ultrasound Med. 23(9):1232, 2004
                                                                      28. Samujh R et al: Oesophageal atresia and biliary atresia: a rare association. Pediatr Surg Int. 20(6):467-8, 2004
                                                                      29. Tonz M et al: Oesophageal atresia: what has changed in the last 3 decades? Pediatr Surg Int. 20(10):768-72, 2004
                                                                      30. Kalache KD et al: Prognostic significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia. Am J Obstet Gynecol. 182(4):978-81, 2000
                                                                      31. Sparey C et al: Esophageal atresia in the Northern Region Congenital Anomaly Survey, 1985-1997: prenatal diagnosis and outcome. Am J Obstet Gynecol. 182(2):427-31, 2000
                                                                      32. Kalache KD et al: The upper neck pouch sign: a prenatal sonographic marker for esophageal atresia. Ultrasound Obstet Gynecol. 11(2):138-40, 1998
                                                                      33. Satoh S et al: Antenatal sonographic detection of the proximal esophageal segment: specific evidence for congenital esophageal atresia. J Clin Ultrasound. 23(7):419-23, 1995
                                                                      34. Stringer MD et al: Prenatal diagnosis of esophageal atresia. J Pediatr Surg. 30(9):1258-63, 1995
                                                                      35. Estroff JA et al: Second trimester prenatal findings in duodenal and esophageal atresia without tracheoesophageal fistula. J Ultrasound Med. 13(5):375-9, 1994
                                                                      36. Surana R et al: Small intestinal atresia: effect on fetal nutrition. J Pediatr Surg. 29(9):1250-2, 1994
                                                                      37. Millener PB et al: Prognostic significance of nonvisualization of the fetal stomach by sonography. AJR Am J Roentgenol. 160(4):827-30, 1993
                                                                      38. Pretorius DH et al: Tracheoesophageal fistula in utero. Twenty-two cases. J Ultrasound Med. 6(9):509-13, 1987
                                                                      39. Jolleys A: An examination of the birthweights of babies with some abnormalities of the alimentary tract. J Pediatr Surg. 16(2):160-3, 1981