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Congenital Pulmonary Airway Malformation
Hank Baskin, MD; A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Congenital pulmonary airway malformation (CPAM)
              • Synonyms

                • Congenital cystic adenomatoid malformation
                  • Antiquated term as lesions may not be cystic or adenomatoid
              • Definitions

                • Heterogeneous group of cystic & noncystic lung lesions resulting from early airway maldevelopment
                  • Overlapping imaging & histopathology with other congenital lung lesions results in blurred lines of classification/nomenclature

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Feinberg A et al: Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg. 51(1):33-7, 2016
                                        2. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                        3. Hall NJ et al: Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations. Pediatr Pulmonol. ePub, 2015
                                        4. Kapralik J et al: Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. J Pediatr Surg. 51(3):508-12, 2015
                                        5. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015
                                        6. 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, 2015
                                        7. Owada K et al: Unusual signal intensity of congenital pulmonary airway malformation on fetal magnetic resonance imaging. Pediatr Radiol. 45(5):763-6, 2015
                                        8. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                        9. Alamo L et al: Prenatal diagnosis of congenital lung malformations. Pediatr Radiol. 42(3):273-83, 2012
                                        10. Barth RA: Imaging of fetal chest masses. Pediatr Radiol. 42 Suppl 1:S62-73, 2012
                                        11. Naito Y et al: Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. J Pediatr Surg. 47(5):852-6, 2012
                                        12. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                        13. Epelman M et al: Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MR. 31(2):141-57, 2010
                                        14. Kocao─člu M et al: Bronchopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings. Diagn Interv Radiol. 16(2):153-61, 2010
                                        15. Stocker JT: Cystic lung disease in infants and children. Fetal Pediatr Pathol. 28(4):155-84, 2009
                                        16. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                        17. Stocker JT: Congenital pulmonary airway malformation: a new name for an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology. 41(S2):424-58, 2002
                                        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

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Congenital pulmonary airway malformation (CPAM)
                                                    • Synonyms

                                                      • Congenital cystic adenomatoid malformation
                                                        • Antiquated term as lesions may not be cystic or adenomatoid
                                                    • Definitions

                                                      • Heterogeneous group of cystic & noncystic lung lesions resulting from early airway maldevelopment
                                                        • Overlapping imaging & histopathology with other congenital lung lesions results in blurred lines of classification/nomenclature

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Ultrasonographic Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Feinberg A et al: Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg. 51(1):33-7, 2016
                                                                              2. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                                                              3. Hall NJ et al: Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations. Pediatr Pulmonol. ePub, 2015
                                                                              4. Kapralik J et al: Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. J Pediatr Surg. 51(3):508-12, 2015
                                                                              5. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015
                                                                              6. 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, 2015
                                                                              7. Owada K et al: Unusual signal intensity of congenital pulmonary airway malformation on fetal magnetic resonance imaging. Pediatr Radiol. 45(5):763-6, 2015
                                                                              8. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                                                              9. Alamo L et al: Prenatal diagnosis of congenital lung malformations. Pediatr Radiol. 42(3):273-83, 2012
                                                                              10. Barth RA: Imaging of fetal chest masses. Pediatr Radiol. 42 Suppl 1:S62-73, 2012
                                                                              11. Naito Y et al: Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. J Pediatr Surg. 47(5):852-6, 2012
                                                                              12. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                                                              13. Epelman M et al: Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MR. 31(2):141-57, 2010
                                                                              14. Kocao─člu M et al: Bronchopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings. Diagn Interv Radiol. 16(2):153-61, 2010
                                                                              15. Stocker JT: Cystic lung disease in infants and children. Fetal Pediatr Pathol. 28(4):155-84, 2009
                                                                              16. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                                                              17. Stocker JT: Congenital pulmonary airway malformation: a new name for an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology. 41(S2):424-58, 2002