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Congenital Pulmonary Airway Malformation
Hank Baskin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Congenital pulmonary airway malformation (CPAM)
            • Synonyms

              • Congenital cystic adenomatoid malformation (CCAM)
                • Antiquated & unsuitable term: Lesions may be noncystic, & only type 3 lesions have adenomatoid components
            • Definitions

              • Heterogeneous group of cystic & noncystic congenital lung lesions resulting from maldevelopment of fetal airway

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic 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. Adams NC et al: Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions. Pediatr Radiol. 50(13):1839-14, 2020
                                            2. Cortes-Santiago N et al: Pediatric cystic lung lesions: where are we now? Surg Pathol Clin. 13(4):643-55, 2020
                                            3. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                            4. Maneenil G et al: Clinical presentation and outcome in congenital pulmonary malformation: 25 year retrospective study in Thailand. Pediatr Int. 61(8):812-6, 2019
                                            5. Ursini WP et al: Congenital pulmonary airway malformation. Autops Case Rep. 8(2):e2018022, 2018
                                            6. Cook J et al: The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases. Arch Dis Child. 102(9):798-803, 2017
                                            7. 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
                                            8. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                            9. 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
                                            10. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015
                                            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

                                                      • Abbreviations

                                                        • Congenital pulmonary airway malformation (CPAM)
                                                      • Synonyms

                                                        • Congenital cystic adenomatoid malformation (CCAM)
                                                          • Antiquated & unsuitable term: Lesions may be noncystic, & only type 3 lesions have adenomatoid components
                                                      • Definitions

                                                        • Heterogeneous group of cystic & noncystic congenital lung lesions resulting from maldevelopment of fetal airway

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic 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. Adams NC et al: Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions. Pediatr Radiol. 50(13):1839-14, 2020
                                                                                      2. Cortes-Santiago N et al: Pediatric cystic lung lesions: where are we now? Surg Pathol Clin. 13(4):643-55, 2020
                                                                                      3. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                                                                      4. Maneenil G et al: Clinical presentation and outcome in congenital pulmonary malformation: 25 year retrospective study in Thailand. Pediatr Int. 61(8):812-6, 2019
                                                                                      5. Ursini WP et al: Congenital pulmonary airway malformation. Autops Case Rep. 8(2):e2018022, 2018
                                                                                      6. Cook J et al: The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases. Arch Dis Child. 102(9):798-803, 2017
                                                                                      7. 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
                                                                                      8. Fowler DJ et al: The pathology of congenital lung lesions. Semin Pediatr Surg. 24(4):176-82, 2015
                                                                                      9. 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
                                                                                      10. Kunisaki SM et al: Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 50(6):978-82, 2015