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Pediatrics
Diagnosis
Chest
Neonatal Chest Issues
Pulmonary Interstitial Emphysema
Pulmonary Interstitial Emphysema
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

            • Air within pulmonary interstitium & lymphatics
            • Most commonly secondary to barotrauma of positive pressure mechanical ventilation in setting of prematurity, very or extremely low birth weight, & surfactant deficiency disease (SDD)
              • Part of "neonatal air leak syndrome": Air escapes tracheobronchial tree to reside in other spaces
                • Also includes pneumomediastinum, pneumothorax, pneumopericardium, pneumoperitoneum, pneumatocele, subcutaneous emphysema, air embolism
            • May occur in neonate with pulmonary hypoplasia on mechanical ventilation or in setting of meconium aspiration or sepsis
            • Rarely occurs in older patients with underlying lung disease or Valsalva leading to alveolar rupture
              • Stem cell transplant with graft vs. host disease & bronchiolitis obliterans

          IMAGING

          • General Features

            • Radiographic Findings

              • CT 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. 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. ePub, 2016
                                    2. Mahapatra S et al: Steroid-induced resolution of refractory pulmonary interstitial emphysema. J Matern Fetal Neonatal Med. 1-4, 2016
                                    3. 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
                                    4. 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
                                    5. Squires KA et al: High-frequency oscillatory ventilation with low oscillatory frequency in pulmonary interstitial emphysema. Neonatology. 104(4):243-9, 2013
                                    6. 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
                                    7. Srinivasan R et al: Persistent pulmonary interstitial emphysema presenting as solitary lung cyst in a preterm infant. BMJ Case Rep, 2012
                                    8. 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
                                    9. Gonçalves CA et al: Pulmonary lobar interstitial emphysema. Fetal Pediatr Pathol. 28(4):192-7, 2009
                                    10. Jassal MS et al: Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema. Pediatr Pulmonol. 43(6):615-9, 2008
                                    11. 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
                                    12. 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
                                    13. 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
                                    14. 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
                                    15. 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

                                              • Air within pulmonary interstitium & lymphatics
                                              • Most commonly secondary to barotrauma of positive pressure mechanical ventilation in setting of prematurity, very or extremely low birth weight, & surfactant deficiency disease (SDD)
                                                • Part of "neonatal air leak syndrome": Air escapes tracheobronchial tree to reside in other spaces
                                                  • Also includes pneumomediastinum, pneumothorax, pneumopericardium, pneumoperitoneum, pneumatocele, subcutaneous emphysema, air embolism
                                              • May occur in neonate with pulmonary hypoplasia on mechanical ventilation or in setting of meconium aspiration or sepsis
                                              • Rarely occurs in older patients with underlying lung disease or Valsalva leading to alveolar rupture
                                                • Stem cell transplant with graft vs. host disease & bronchiolitis obliterans

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT 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. 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. ePub, 2016
                                                                      2. Mahapatra S et al: Steroid-induced resolution of refractory pulmonary interstitial emphysema. J Matern Fetal Neonatal Med. 1-4, 2016
                                                                      3. 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
                                                                      4. 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
                                                                      5. Squires KA et al: High-frequency oscillatory ventilation with low oscillatory frequency in pulmonary interstitial emphysema. Neonatology. 104(4):243-9, 2013
                                                                      6. 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
                                                                      7. Srinivasan R et al: Persistent pulmonary interstitial emphysema presenting as solitary lung cyst in a preterm infant. BMJ Case Rep, 2012
                                                                      8. 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
                                                                      9. Gonçalves CA et al: Pulmonary lobar interstitial emphysema. Fetal Pediatr Pathol. 28(4):192-7, 2009
                                                                      10. Jassal MS et al: Spontaneous resolution of diffuse persistent pulmonary interstitial emphysema. Pediatr Pulmonol. 43(6):615-9, 2008
                                                                      11. 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
                                                                      12. 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
                                                                      13. 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
                                                                      14. 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
                                                                      15. Jabra AA et al: Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation. AJR Am J Roentgenol. 169(5):1381-4, 1997