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Tracheal Agenesis
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Tracheal atresia
            • Definitions

              • Absence of portion or entirety of trachea, extending from subglottis to main bronchi

            IMAGING

            • Prenatal imaging

              • Postnatal imaging

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Darouich S et al: Tracheal agenesis with bronchoesophageal fistula. N Engl J Med. 384(9):e27, 2021
                                2. Cristallo Lacalamita M et al: Tracheal agenesis: optimization of computed tomography diagnosis by airway ventilation. Pediatr Radiol. 48(3):427-32, 2018
                                3. Densmore JC et al: Neonatal esophageal trachealization and esophagocarinoplasty in the treatment of flow-limited Floyd II tracheal agenesis. J Thorac Cardiovasc Surg. 153(6):e121-5, 2017
                                4. Windsor A et al: Rare upper airway anomalies. Paediatr Respir Rev. 17:24-8, 2015
                                5. Jungebluth P et al: Airway transplantation. Thorac Surg Clin. 24(1):97-106, 2014
                                6. Coleman AM et al: Tracheal agenesis with tracheoesophageal fistulae: fetal MRI diagnosis with confirmation by ultrasound during an ex utero intrapartum therapy (EXIT) delivery and postdelivery MRI. Pediatr Radiol. 43(10):1385-90, 2013
                                7. de Groot-van der Mooren MD et al: Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature. Eur J Pediatr. 171(3):425-31, 2012
                                8. Usui N et al: Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis. Ann Thorac Surg. 89(6):2019-22, 2010
                                9. Watanabe T et al: A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting. J Pediatr Surg. 43(10):1906-8, 2008
                                10. Heimann K et al: Three new cases of congenital agenesis of the trachea. Eur J Pediatr. 166(1):79-82, 2007
                                11. Felix JF et al: Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients. Int J Pediatr Otorhinolaryngol. 70(2):365-70, 2006
                                12. Strouse PJ et al: CT of tracheal agenesis. Pediatr Radiol. 36(9):920-6, 2006
                                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

                                          • Synonyms

                                            • Tracheal atresia
                                          • Definitions

                                            • Absence of portion or entirety of trachea, extending from subglottis to main bronchi

                                          IMAGING

                                          • Prenatal imaging

                                            • Postnatal imaging

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Staging, Grading, & Classification

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Darouich S et al: Tracheal agenesis with bronchoesophageal fistula. N Engl J Med. 384(9):e27, 2021
                                                              2. Cristallo Lacalamita M et al: Tracheal agenesis: optimization of computed tomography diagnosis by airway ventilation. Pediatr Radiol. 48(3):427-32, 2018
                                                              3. Densmore JC et al: Neonatal esophageal trachealization and esophagocarinoplasty in the treatment of flow-limited Floyd II tracheal agenesis. J Thorac Cardiovasc Surg. 153(6):e121-5, 2017
                                                              4. Windsor A et al: Rare upper airway anomalies. Paediatr Respir Rev. 17:24-8, 2015
                                                              5. Jungebluth P et al: Airway transplantation. Thorac Surg Clin. 24(1):97-106, 2014
                                                              6. Coleman AM et al: Tracheal agenesis with tracheoesophageal fistulae: fetal MRI diagnosis with confirmation by ultrasound during an ex utero intrapartum therapy (EXIT) delivery and postdelivery MRI. Pediatr Radiol. 43(10):1385-90, 2013
                                                              7. de Groot-van der Mooren MD et al: Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature. Eur J Pediatr. 171(3):425-31, 2012
                                                              8. Usui N et al: Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis. Ann Thorac Surg. 89(6):2019-22, 2010
                                                              9. Watanabe T et al: A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting. J Pediatr Surg. 43(10):1906-8, 2008
                                                              10. Heimann K et al: Three new cases of congenital agenesis of the trachea. Eur J Pediatr. 166(1):79-82, 2007
                                                              11. Felix JF et al: Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients. Int J Pediatr Otorhinolaryngol. 70(2):365-70, 2006
                                                              12. Strouse PJ et al: CT of tracheal agenesis. Pediatr Radiol. 36(9):920-6, 2006