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Bronchopulmonary Sequestration
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Pulmonary sequestration
            • Definitions

              • Bronchopulmonary sequestration (BPS): Dysplastic nonfunctional lung tissue with no connection to bronchi or pulmonary arteries
              • Intralobar (75%) vs. extralobar (25%) types
                • Intralobar sequestration (ILS) typically has pulmonary venous drainage without separate pleural investment
                • Extralobar sequestration (ELS) typically has systemic venous drainage with separate pleural investment

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Reporting Tips

                                        Selected References

                                        1. Cruz-Martinez R et al: Fetal laser surgery prevents fetal death and avoids the need for neonatal sequestrectomy in cases with bronchopulmonary sequestration. Ultrasound Obstet Gynecol. 46(5):627-8, 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. Singh R et al: The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 24(4):187-95, 2015
                                        5. Tashtoush B et al: Pulmonary sequestration: a 29 patient case series and review. J Clin Diagn Res. 9(12):AC05-8, 2015
                                        6. Nunes C et al: Fetal bronchopulmonary malformations. J Matern Fetal Neonatal Med. 1-5, 2014
                                        7. Walker CM et al: The imaging spectrum of bronchopulmonary sequestration. Curr Probl Diagn Radiol. 43(3):100-14, 2014
                                        8. Alamo L et al: Comparison of foetal US and MRI in the characterisation of congenital lung anomalies. Eur J Radiol. 82(12):e860-6, 2013
                                        9. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                        10. Zucker EJ et al: Paediatric torsed extralobar sequestration containing calcification: Imaging findings with pathological correlation. Clin Radiol. 68(1):94-7, 2013
                                        11. Recio Rodríguez M et al: MR imaging of thoracic abnormalities in the fetus. Radiographics. 32(7):E305-21, 2012
                                        12. Yanagisawa S et al: Intrapericardial extralobar pulmonary sequestration detected as an intrathoracic cystic mass by using prenatal ultrasonography: case report and review of the literature. J Pediatr Surg. 47(12):2327-31, 2012
                                        13. Hadchouel A et al: Factors associated with partial and complete regression of fetal lung lesions. Ultrasound Obstet Gynecol. 38(1):88-93, 2011
                                        14. Wei Y et al: Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 40(1):e39-42, 2011
                                        15. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                        16. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                        17. Kunisaki SM et al: Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses. J Pediatr Surg. 41(1):61-5; discussion 61-5, 2006
                                        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

                                                  • Synonyms

                                                    • Pulmonary sequestration
                                                  • Definitions

                                                    • Bronchopulmonary sequestration (BPS): Dysplastic nonfunctional lung tissue with no connection to bronchi or pulmonary arteries
                                                    • Intralobar (75%) vs. extralobar (25%) types
                                                      • Intralobar sequestration (ILS) typically has pulmonary venous drainage without separate pleural investment
                                                      • Extralobar sequestration (ELS) typically has systemic venous drainage with separate pleural investment

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Cruz-Martinez R et al: Fetal laser surgery prevents fetal death and avoids the need for neonatal sequestrectomy in cases with bronchopulmonary sequestration. Ultrasound Obstet Gynecol. 46(5):627-8, 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. Singh R et al: The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 24(4):187-95, 2015
                                                                              5. Tashtoush B et al: Pulmonary sequestration: a 29 patient case series and review. J Clin Diagn Res. 9(12):AC05-8, 2015
                                                                              6. Nunes C et al: Fetal bronchopulmonary malformations. J Matern Fetal Neonatal Med. 1-5, 2014
                                                                              7. Walker CM et al: The imaging spectrum of bronchopulmonary sequestration. Curr Probl Diagn Radiol. 43(3):100-14, 2014
                                                                              8. Alamo L et al: Comparison of foetal US and MRI in the characterisation of congenital lung anomalies. Eur J Radiol. 82(12):e860-6, 2013
                                                                              9. Pacharn P et al: Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol. 43(9):1136-43, 2013
                                                                              10. Zucker EJ et al: Paediatric torsed extralobar sequestration containing calcification: Imaging findings with pathological correlation. Clin Radiol. 68(1):94-7, 2013
                                                                              11. Recio Rodríguez M et al: MR imaging of thoracic abnormalities in the fetus. Radiographics. 32(7):E305-21, 2012
                                                                              12. Yanagisawa S et al: Intrapericardial extralobar pulmonary sequestration detected as an intrathoracic cystic mass by using prenatal ultrasonography: case report and review of the literature. J Pediatr Surg. 47(12):2327-31, 2012
                                                                              13. Hadchouel A et al: Factors associated with partial and complete regression of fetal lung lesions. Ultrasound Obstet Gynecol. 38(1):88-93, 2011
                                                                              14. Wei Y et al: Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 40(1):e39-42, 2011
                                                                              15. Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
                                                                              16. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                                                              17. Kunisaki SM et al: Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses. J Pediatr Surg. 41(1):61-5; discussion 61-5, 2006