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Congenital Pulmonary Airway Malformation
Paula J. Woodward, 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
              • Older name that reflects cystic and adenomatous histologic components in many, but not all, of these masses
              • 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. 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
                                  2. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                  3. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015
                                  4. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                  5. Lee FL et al: Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy. Fetal Diagn Ther. 31(4):264-8, 2012
                                  6. Gupta K et al: Extralobar sequestration associated with congenital pulmonary airway malformation (CPAM), type I: an autopsy report. Fetal Pediatr Pathol. 30(3):167-72, 2011
                                  7. Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med. 15(1):1-8, 2010
                                  8. Curran PF et al: Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg. 45(1):145-50, 2010
                                  9. Adzick NS: Management of fetal lung lesions. Clin Perinatol. 36(2):363-76, x, 2009
                                  10. Lecomte B et al: Hyperechoic congenital lung lesions in a non-selected population: from prenatal detection till perinatal management. Prenat Diagn. 29(13):1222-30, 2009
                                  11. Schott S et al: Cystic adenomatoid malformation of the lung causing hydrops fetalis: case report and review of the literature. Arch Gynecol Obstet. 280(2):293-6, 2009
                                  12. Harmath A et al: Congenital lung malformations in the second trimester: prenatal ultrasound diagnosis and pathologic findings. J Clin Ultrasound. 35(5):250-5, 2007
                                  13. Kunisaki SM et al: Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg. 42(2):404-10, 2007
                                  14. Mann S et al: Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med. 12(6):477-81, 2007
                                  15. Calvert JK et al: Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991-2001. Arch Dis Child Fetal Neonatal Ed. 2006 Jan;91(1):F26-8. Epub 2005 Aug 30. Erratum in: Arch Dis Child Fetal Neonatal Ed. 2006 Jul;91(4):F312. Arch Dis Child Fetal Neonatal Ed. 91(6):547, 2006
                                  16. Wilson RD et al: Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A. 140(2):151-5, 2006
                                  17. Illanes S et al: Prenatal diagnosis of echogenic lung: evolution and outcome. Ultrasound Obstet Gynecol. 26(2):145-9, 2005
                                  18. Achiron R et al: Fetal lung dysplasia: clinical outcome based on a new classification system. Ultrasound Obstet Gynecol. 24(2):127-33, 2004
                                  19. 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
                                  20. Wilson RD et al: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther. 19(5):413-20, 2004
                                  21. 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
                                  22. Cass DL et al: Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg. 32(7):986-90, 1997
                                  23. MacGillivray TE et al: Disappearing fetal lung lesions. J Pediatr Surg. 28(10):1321-4; discussion 1324-5, 1993
                                  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
                                              • Older name that reflects cystic and adenomatous histologic components in many, but not all, of these masses
                                              • 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. 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
                                                                  2. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                                                  3. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015
                                                                  4. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                                                  5. Lee FL et al: Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy. Fetal Diagn Ther. 31(4):264-8, 2012
                                                                  6. Gupta K et al: Extralobar sequestration associated with congenital pulmonary airway malformation (CPAM), type I: an autopsy report. Fetal Pediatr Pathol. 30(3):167-72, 2011
                                                                  7. Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med. 15(1):1-8, 2010
                                                                  8. Curran PF et al: Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg. 45(1):145-50, 2010
                                                                  9. Adzick NS: Management of fetal lung lesions. Clin Perinatol. 36(2):363-76, x, 2009
                                                                  10. Lecomte B et al: Hyperechoic congenital lung lesions in a non-selected population: from prenatal detection till perinatal management. Prenat Diagn. 29(13):1222-30, 2009
                                                                  11. Schott S et al: Cystic adenomatoid malformation of the lung causing hydrops fetalis: case report and review of the literature. Arch Gynecol Obstet. 280(2):293-6, 2009
                                                                  12. Harmath A et al: Congenital lung malformations in the second trimester: prenatal ultrasound diagnosis and pathologic findings. J Clin Ultrasound. 35(5):250-5, 2007
                                                                  13. Kunisaki SM et al: Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg. 42(2):404-10, 2007
                                                                  14. Mann S et al: Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med. 12(6):477-81, 2007
                                                                  15. Calvert JK et al: Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991-2001. Arch Dis Child Fetal Neonatal Ed. 2006 Jan;91(1):F26-8. Epub 2005 Aug 30. Erratum in: Arch Dis Child Fetal Neonatal Ed. 2006 Jul;91(4):F312. Arch Dis Child Fetal Neonatal Ed. 91(6):547, 2006
                                                                  16. Wilson RD et al: Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A. 140(2):151-5, 2006
                                                                  17. Illanes S et al: Prenatal diagnosis of echogenic lung: evolution and outcome. Ultrasound Obstet Gynecol. 26(2):145-9, 2005
                                                                  18. Achiron R et al: Fetal lung dysplasia: clinical outcome based on a new classification system. Ultrasound Obstet Gynecol. 24(2):127-33, 2004
                                                                  19. 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
                                                                  20. Wilson RD et al: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther. 19(5):413-20, 2004
                                                                  21. 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
                                                                  22. Cass DL et al: Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg. 32(7):986-90, 1997
                                                                  23. MacGillivray TE et al: Disappearing fetal lung lesions. J Pediatr Surg. 28(10):1321-4; discussion 1324-5, 1993