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Congenital Pulmonary Airway Malformation
Emily Edwards, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Congenital pulmonary airway malformation (CPAM)
              • Newer terminology reflects developmental disorder of pulmonary airway morphogenesis
          • Synonyms

            • Congenital cystic adenomatoid malformation (CCAM)
              • Older name reflects cystic and adenomatous histologic components in some, but not all, lesions
              • Term still often used in fetal literature

          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. Shulman R et al: Fetal congenital pulmonary airway malformation: the role of an objective measurement of cardiomediastinal shift. Am J Perinatol. 36(3):225-32, 2019
                                  2. David M et al: Prenatal and postnatal management of congenital pulmonary airway malformation. Neonatology. 110(2):101-15, 2016
                                  3. Hellmund A et al: Prenatal diagnosis and evaluation of sonographic predictors for intervention and adverse outcome in congenital pulmonary airway malformation. PLoS One. 11(3):e0150474, 2016
                                  4. Macardle CA et al: Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes. Prenat Diagn. 36(3):282-9, 2016
                                  5. Derderian SC et al: Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg. 50(4):515-8, 2015
                                  6. Euser AG et al: Comparison of congenital pulmonary airway malformation volume ratios calculated by ultrasound and magnetic resonance imaging. J Matern Fetal Neonatal Med. 1-6, 2015
                                  7. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                  8. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                  9. Achiron R et al: Fetal lung lesions: a spectrum of disease. New classification based on pathogenesis, two-dimensional and color Doppler ultrasound. Ultrasound Obstet Gynecol. 24(2):107-14, 2004
                                  10. Wilson RD et al: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther. 19(5):413-20, 2004
                                  11. Crombleholme TM et al: Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg. 37(3):331-8, 2002
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Congenital pulmonary airway malformation (CPAM)
                                              • Newer terminology reflects developmental disorder of pulmonary airway morphogenesis
                                          • Synonyms

                                            • Congenital cystic adenomatoid malformation (CCAM)
                                              • Older name reflects cystic and adenomatous histologic components in some, but not all, lesions
                                              • Term still often used in fetal literature

                                          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. Shulman R et al: Fetal congenital pulmonary airway malformation: the role of an objective measurement of cardiomediastinal shift. Am J Perinatol. 36(3):225-32, 2019
                                                                  2. David M et al: Prenatal and postnatal management of congenital pulmonary airway malformation. Neonatology. 110(2):101-15, 2016
                                                                  3. Hellmund A et al: Prenatal diagnosis and evaluation of sonographic predictors for intervention and adverse outcome in congenital pulmonary airway malformation. PLoS One. 11(3):e0150474, 2016
                                                                  4. Macardle CA et al: Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes. Prenat Diagn. 36(3):282-9, 2016
                                                                  5. Derderian SC et al: Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg. 50(4):515-8, 2015
                                                                  6. Euser AG et al: Comparison of congenital pulmonary airway malformation volume ratios calculated by ultrasound and magnetic resonance imaging. J Matern Fetal Neonatal Med. 1-6, 2015
                                                                  7. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                                                  8. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                                                  9. Achiron R et al: Fetal lung lesions: a spectrum of disease. New classification based on pathogenesis, two-dimensional and color Doppler ultrasound. Ultrasound Obstet Gynecol. 24(2):107-14, 2004
                                                                  10. Wilson RD et al: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther. 19(5):413-20, 2004
                                                                  11. Crombleholme TM et al: Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg. 37(3):331-8, 2002