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Congenital High Airway Obstruction Syndrome
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Congenital high airway obstruction syndrome (CHAOS)
          • Definitions

            • Rare, highly morbid congenital anomaly of airway with complete, intrinsic laryngeal &/or tracheal obstruction, leading to
              • Dysfunctional, hyperexpanded lungs & everted hemidiaphragms
              • Cardiac & venous compression resulting in ascites or full hydrops

          IMAGING

          • General Features

            • Radiographic Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Fluoroscopic Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Natural History & Prognosis

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                Selected References

                                1. Peiro JL et al: A technical look at fetoscopic laser ablation for fetal laryngeal surgical recanalization in congenital high airway obstruction syndrome. J Laparoendosc Adv Surg Tech A. 30(6):695-700, 2020
                                2. Nolan HR et al: Congenital high airway obstruction syndrome (CHAOS): natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center. J Pediatr Surg. 54(6):1153-8, 2019
                                3. Nicolas CT et al: Fetoscopy-assisted percutaneous decompression of the distal trachea and lungs reverses hydrops fetalis and fetal distress in a fetus with laryngeal atresia. Fetal Diagn Ther. 46(1):75-80, 2019
                                4. Prickett K et al: Fetal evaluation and airway management. Clin Perinatol. 45(4):609-28, 2018
                                5. Gowda M et al: Locating the level and extent of congenital high airway obstruction: fluid in the airway tract as reference points. J Ultrasound Med. 36(10):2179-85, 2017
                                6. Ryan G et al: Airway compromise in the fetus and neonate: prenatal assessment and perinatal management. Semin Fetal Neonatal Med. 21(4):230-9, 2016
                                7. Arthurs OJ et al: Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome. Pediatr Radiol. 45(4):556-61, 2015
                                8. Zamora IJ et al: Mainstem bronchial atresia: a lethal anomaly amenable to fetal surgical treatment. J Pediatr Surg. 49(5):706-11, 2014
                                9. Artunc Ulkumen B et al: Prenatal diagnosis of congenital high airway obstruction syndrome: report of two cases and brief review of the literature. Case Rep Obstet Gynecol. 2013:728974, 2013
                                10. Martínez JM et al: Evaluation of fetal vocal cords to select candidates for successful fetoscopic treatment of congenital high airway obstruction syndrome: preliminary case series. Fetal Diagn Ther. 34(2):77-84, 2013
                                11. Furukawa R et al: Congenital high airway obstruction syndrome without tracheoesophageal fistula and with in utero decrease in relative lung size. Pediatr Radiol. 42(12):1510-3, 2012
                                12. Saadai P et al: Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome. J Pediatr Surg. 47(6):1095-100, 2012
                                13. Sanford E et al: Congenital high airway obstruction sequence (CHAOS): a new case and a review of phenotypic features. Am J Med Genet A. 158A(12):3126-36, 2012
                                14. Courtier J et al: Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI. Pediatr Radiol. 40(11):1800-5, 2010
                                15. Guimaraes CV et al: Prenatal MRI findings of fetuses with congenital high airway obstruction sequence. Korean J Radiol. 10(2):129-34, 2009
                                16. Mong A et al: Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome. Pediatr Radiol. 38(11):1171-9, 2008
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Pathology

                                      • Clinical Issues

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Congenital high airway obstruction syndrome (CHAOS)
                                        • Definitions

                                          • Rare, highly morbid congenital anomaly of airway with complete, intrinsic laryngeal &/or tracheal obstruction, leading to
                                            • Dysfunctional, hyperexpanded lungs & everted hemidiaphragms
                                            • Cardiac & venous compression resulting in ascites or full hydrops

                                        IMAGING

                                        • General Features

                                          • Radiographic Findings

                                            • MR Findings

                                              • Ultrasonographic Findings

                                                • Fluoroscopic Findings

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Microscopic Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Natural History & Prognosis

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              Selected References

                                                              1. Peiro JL et al: A technical look at fetoscopic laser ablation for fetal laryngeal surgical recanalization in congenital high airway obstruction syndrome. J Laparoendosc Adv Surg Tech A. 30(6):695-700, 2020
                                                              2. Nolan HR et al: Congenital high airway obstruction syndrome (CHAOS): natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center. J Pediatr Surg. 54(6):1153-8, 2019
                                                              3. Nicolas CT et al: Fetoscopy-assisted percutaneous decompression of the distal trachea and lungs reverses hydrops fetalis and fetal distress in a fetus with laryngeal atresia. Fetal Diagn Ther. 46(1):75-80, 2019
                                                              4. Prickett K et al: Fetal evaluation and airway management. Clin Perinatol. 45(4):609-28, 2018
                                                              5. Gowda M et al: Locating the level and extent of congenital high airway obstruction: fluid in the airway tract as reference points. J Ultrasound Med. 36(10):2179-85, 2017
                                                              6. Ryan G et al: Airway compromise in the fetus and neonate: prenatal assessment and perinatal management. Semin Fetal Neonatal Med. 21(4):230-9, 2016
                                                              7. Arthurs OJ et al: Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome. Pediatr Radiol. 45(4):556-61, 2015
                                                              8. Zamora IJ et al: Mainstem bronchial atresia: a lethal anomaly amenable to fetal surgical treatment. J Pediatr Surg. 49(5):706-11, 2014
                                                              9. Artunc Ulkumen B et al: Prenatal diagnosis of congenital high airway obstruction syndrome: report of two cases and brief review of the literature. Case Rep Obstet Gynecol. 2013:728974, 2013
                                                              10. Martínez JM et al: Evaluation of fetal vocal cords to select candidates for successful fetoscopic treatment of congenital high airway obstruction syndrome: preliminary case series. Fetal Diagn Ther. 34(2):77-84, 2013
                                                              11. Furukawa R et al: Congenital high airway obstruction syndrome without tracheoesophageal fistula and with in utero decrease in relative lung size. Pediatr Radiol. 42(12):1510-3, 2012
                                                              12. Saadai P et al: Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome. J Pediatr Surg. 47(6):1095-100, 2012
                                                              13. Sanford E et al: Congenital high airway obstruction sequence (CHAOS): a new case and a review of phenotypic features. Am J Med Genet A. 158A(12):3126-36, 2012
                                                              14. Courtier J et al: Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI. Pediatr Radiol. 40(11):1800-5, 2010
                                                              15. Guimaraes CV et al: Prenatal MRI findings of fetuses with congenital high airway obstruction sequence. Korean J Radiol. 10(2):129-34, 2009
                                                              16. Mong A et al: Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome. Pediatr Radiol. 38(11):1171-9, 2008