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Pulmonary Interstitial Emphysema
Emily S. Orscheln, MD; A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Pulmonary interstitial emphysema (PIE)
          • Definitions

            • PIE: Air within pulmonary interstitium & lymphatics
            • Persistent PIE: Development of persistent, mass-like interstitial cysts
            • Neonatal air leak syndrome: Air escapes from tracheobronchial tree into spaces where it is not normally found
              • PIE, pneumothorax, pneumomediastinum, pneumopericardium, subcutaneous emphysema; rarely pneumoperitoneum

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • Ultrasonographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    • Reporting Tips

                                      Selected References

                                      1. Corsini I et al: Lung ultrasound in the neonatal intensive care unit: review of the literature and future perspectives. Pediatr Pulmonol. 55(7):1550-62, 2020
                                      2. Rocha G: Pulmonary pneumatoceles in neonates. Pediatr Pulmonol. 55(10):2532-41, 2020
                                      3. Williams E et al: Predictors of outcome of prematurely born infants with pulmonary interstitial emphysema. Acta Paediatr. 108(1):106-11, 2019
                                      4. Kim HR et al: Presence of subpleural pulmonary interstitial emphysema as an indication of single or multiple alveolar ruptures on CT in patients with spontaneous pneumomediastinum. Acta Radiol. 57(12):1483-9, 2016
                                      5. Mahapatra S et al: Steroid-induced resolution of refractory pulmonary interstitial emphysema. J Matern Fetal Neonatal Med. 1-4, 2016
                                      6. Toledo Del Castillo B et al: Diffuse persistent pulmonary interstitial emphysema secondary to mechanical ventilation in bronchiolitis. BMC Pulm Med. 16(1):139, 2016
                                      7. Barcia SM et al: Pulmonary interstitial emphysema in adults: a clinicopathologic study of 53 lung explants. Am J Surg Pathol. 38(3):339-45, 2014
                                      8. Corsini I et al: Pulmonary interstitial emphysema after resolution of relapsing pneumothorax and discontinuation of mechanical ventilation. An atypical case in a preterm infant. J Matern Fetal Neonatal Med. 27(15):1610-2, 2014
                                      9. Squires KA et al: High-frequency oscillatory ventilation with low oscillatory frequency in pulmonary interstitial emphysema. Neonatology. 104(4):243-9, 2013
                                      10. Jeng MJ et al: Neonatal air leak syndrome and the role of high-frequency ventilation in its prevention. J Chin Med Assoc. 75(11):551-9, 2012
                                      11. Srinivasan R et al: Persistent pulmonary interstitial emphysema presenting as solitary lung cyst in a preterm infant. BMJ Case Rep, 2012
                                      12. Joseph LJ et al: Unilateral lung intubation for pulmonary air leak syndrome in neonates: a case series and a review of the literature. Am J Perinatol. 28(2):151-6, 2011
                                      13. Gonçalves CA et al: Pulmonary lobar interstitial emphysema. Fetal Pediatr Pathol. 28(4):192-7, 2009
                                      14. Jassal MS et al: Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema. Pediatr Pulmonol. 43(6):615-9, 2008
                                      15. Phatak RS et al: Heliox with inhaled nitric oxide: a novel strategy for severe localized interstitial pulmonary emphysema in preterm neonatal ventilation. Respir Care. 53(12):1731-8, 2008
                                      16. Franquet T et al: Air-leak syndromes in hematopoietic stem cell transplant recipients with chronic GVHD: high-resolution CT findings. J Thorac Imaging. 22(4):335-40, 2007
                                      17. Agrons GA et al: From the archives of the AFIP: lung disease in premature neonates: radiologic-pathologic correlation. Radiographics. 25(4):1047-73, 2005
                                      18. Donnelly LF et al: CT findings and temporal course of persistent pulmonary interstitial emphysema in neonates: a multiinstitutional study. AJR Am J Roentgenol. 180(4):1129-33, 2003
                                      19. Cohen MC et al: Solitary unilocular cyst of the lung with features of persistent interstitial pulmonary emphysema: report of four cases. Pediatr Dev Pathol. 2(6):531-6, 1999
                                      20. Jabra AA et al: Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation. AJR Am J Roentgenol. 169(5):1381-4, 1997
                                      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

                                                • Pulmonary interstitial emphysema (PIE)
                                              • Definitions

                                                • PIE: Air within pulmonary interstitium & lymphatics
                                                • Persistent PIE: Development of persistent, mass-like interstitial cysts
                                                • Neonatal air leak syndrome: Air escapes from tracheobronchial tree into spaces where it is not normally found
                                                  • PIE, pneumothorax, pneumomediastinum, pneumopericardium, subcutaneous emphysema; rarely pneumoperitoneum

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • Ultrasonographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Image Interpretation Pearls

                                                                        • Reporting Tips

                                                                          Selected References

                                                                          1. Corsini I et al: Lung ultrasound in the neonatal intensive care unit: review of the literature and future perspectives. Pediatr Pulmonol. 55(7):1550-62, 2020
                                                                          2. Rocha G: Pulmonary pneumatoceles in neonates. Pediatr Pulmonol. 55(10):2532-41, 2020
                                                                          3. Williams E et al: Predictors of outcome of prematurely born infants with pulmonary interstitial emphysema. Acta Paediatr. 108(1):106-11, 2019
                                                                          4. Kim HR et al: Presence of subpleural pulmonary interstitial emphysema as an indication of single or multiple alveolar ruptures on CT in patients with spontaneous pneumomediastinum. Acta Radiol. 57(12):1483-9, 2016
                                                                          5. Mahapatra S et al: Steroid-induced resolution of refractory pulmonary interstitial emphysema. J Matern Fetal Neonatal Med. 1-4, 2016
                                                                          6. Toledo Del Castillo B et al: Diffuse persistent pulmonary interstitial emphysema secondary to mechanical ventilation in bronchiolitis. BMC Pulm Med. 16(1):139, 2016
                                                                          7. Barcia SM et al: Pulmonary interstitial emphysema in adults: a clinicopathologic study of 53 lung explants. Am J Surg Pathol. 38(3):339-45, 2014
                                                                          8. Corsini I et al: Pulmonary interstitial emphysema after resolution of relapsing pneumothorax and discontinuation of mechanical ventilation. An atypical case in a preterm infant. J Matern Fetal Neonatal Med. 27(15):1610-2, 2014
                                                                          9. Squires KA et al: High-frequency oscillatory ventilation with low oscillatory frequency in pulmonary interstitial emphysema. Neonatology. 104(4):243-9, 2013
                                                                          10. Jeng MJ et al: Neonatal air leak syndrome and the role of high-frequency ventilation in its prevention. J Chin Med Assoc. 75(11):551-9, 2012
                                                                          11. Srinivasan R et al: Persistent pulmonary interstitial emphysema presenting as solitary lung cyst in a preterm infant. BMJ Case Rep, 2012
                                                                          12. Joseph LJ et al: Unilateral lung intubation for pulmonary air leak syndrome in neonates: a case series and a review of the literature. Am J Perinatol. 28(2):151-6, 2011
                                                                          13. Gonçalves CA et al: Pulmonary lobar interstitial emphysema. Fetal Pediatr Pathol. 28(4):192-7, 2009
                                                                          14. Jassal MS et al: Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema. Pediatr Pulmonol. 43(6):615-9, 2008
                                                                          15. Phatak RS et al: Heliox with inhaled nitric oxide: a novel strategy for severe localized interstitial pulmonary emphysema in preterm neonatal ventilation. Respir Care. 53(12):1731-8, 2008
                                                                          16. Franquet T et al: Air-leak syndromes in hematopoietic stem cell transplant recipients with chronic GVHD: high-resolution CT findings. J Thorac Imaging. 22(4):335-40, 2007
                                                                          17. Agrons GA et al: From the archives of the AFIP: lung disease in premature neonates: radiologic-pathologic correlation. Radiographics. 25(4):1047-73, 2005
                                                                          18. Donnelly LF et al: CT findings and temporal course of persistent pulmonary interstitial emphysema in neonates: a multiinstitutional study. AJR Am J Roentgenol. 180(4):1129-33, 2003
                                                                          19. Cohen MC et al: Solitary unilocular cyst of the lung with features of persistent interstitial pulmonary emphysema: report of four cases. Pediatr Dev Pathol. 2(6):531-6, 1999
                                                                          20. Jabra AA et al: Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation. AJR Am J Roentgenol. 169(5):1381-4, 1997