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Congenital Pulmonary Airway Malformation
Melissa L. Rosado-de-Christenson, MD, FACR; Tyler H. Ternes, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Congenital pulmonary airway malformation (CPAM)
                • Spectrum of lesions affecting various portions of tracheobronchial tree & distal airways
              • Abnormal mass of pulmonary tissue with varying degrees of cystic change
              • Communicates with tracheobronchial tree; normal blood supply & venous drainage
              • Congenital cystic adenomatoid malformation (CCAM)
                • Outdated terminology
                  • Some lesions are not cystic
                  • Most lesions are not adenomatoid

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Ultrasonographic Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            Selected References

                                            1. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                            2. Daltro P et al: Congenital chest malformations: a multimodality approach with emphasis on fetal MR imaging. Radiographics. 30(2):385-95, 2010
                                            3. Shimohira M et al: Congenital pulmonary airway malformation: CT-pathologic correlation. J Thorac Imaging. 22(2):149-53, 2007
                                            4. Newman B: Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol. 36(8):773-91, 2006
                                            5. Levine D et al: Fetal thoracic abnormalities: MR imaging. Radiology. 228(2):379-88, 2003
                                            6. Stocker JT et al: Congenital pulmonary airway malformation: a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology. 41(2):424-58, 2002
                                            7. Hubbard AM et al: Fetal magnetic resonance imaging. Top Magn Reson Imaging. 12(2):93-103, 2001
                                            8. Rosado-de-Christenson ML et al: Congenital cystic adenomatoid malformation. Radiographics. 11(5):865-86, 1991
                                            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

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Definitions

                                                        • Congenital pulmonary airway malformation (CPAM)
                                                          • Spectrum of lesions affecting various portions of tracheobronchial tree & distal airways
                                                        • Abnormal mass of pulmonary tissue with varying degrees of cystic change
                                                        • Communicates with tracheobronchial tree; normal blood supply & venous drainage
                                                        • Congenital cystic adenomatoid malformation (CCAM)
                                                          • Outdated terminology
                                                            • Some lesions are not cystic
                                                            • Most lesions are not adenomatoid

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • Ultrasonographic Findings

                                                              • MR Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      Selected References

                                                                                      1. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                                                                      2. Daltro P et al: Congenital chest malformations: a multimodality approach with emphasis on fetal MR imaging. Radiographics. 30(2):385-95, 2010
                                                                                      3. Shimohira M et al: Congenital pulmonary airway malformation: CT-pathologic correlation. J Thorac Imaging. 22(2):149-53, 2007
                                                                                      4. Newman B: Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol. 36(8):773-91, 2006
                                                                                      5. Levine D et al: Fetal thoracic abnormalities: MR imaging. Radiology. 228(2):379-88, 2003
                                                                                      6. Stocker JT et al: Congenital pulmonary airway malformation: a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology. 41(2):424-58, 2002
                                                                                      7. Hubbard AM et al: Fetal magnetic resonance imaging. Top Magn Reson Imaging. 12(2):93-103, 2001
                                                                                      8. Rosado-de-Christenson ML et al: Congenital cystic adenomatoid malformation. Radiographics. 11(5):865-86, 1991