Pulmonary Interstitial Emphysema (PIE) Workup
- Author: Abhay J Bhatt, MD, MBBS; Chief Editor: Ted Rosenkrantz, MD more...
Approach Considerations
Pulmonary interstitial emphysema (PIE) is a radiographic and pathologic diagnosis.
In addition, blood gases should be obtained in these patients, to ensure adequate gas exchange.
Radiography
The classic radiologic appearance of pulmonary interstitial emphysema often provides a clear diagnosis. Pulmonary interstitial emphysema is best visualized in the anteroposterior supine projection. Pulmonary interstitial emphysema has two basic radiographic appearances, linear and cystlike radiolucencies, although both types often appear together.
Linear radiolucencies are coarse and nonbranching, measure from 3-8 mm, and vary in width but rarely exceed 2 mm. Cystlike radiolucencies are small, ranging from 1-4 mm in diameter. Although generally round, they may also appear oval or slightly lobulated.
The disorganized haphazard distribution of pulmonary interstitial emphysema in localized areas is unlike the anatomically organized pattern of the air-bronchogram. The air-bronchogram is a classic radiographic sign of respiratory distress syndrome (RDS), which should not be confused with pulmonary interstitial emphysema.
In RDS, long, smooth, branching, linear radiolucencies decrease in caliber from the hilum and frequently disappear at the lung periphery. Pulmonary interstitial emphysema should be suspected when coarse radiolucencies appear in the lung periphery or when the lucencies do not branch in a pattern consistent with the normal bronchial tree.
In some patients receiving mechanical ventilation, distended airways and alveoli have a somewhat similar radiographic appearance to that of pulmonary interstitial emphysema. Over time, it either progresses to a classic radiographic picture of pulmonary interstitial emphysema or resolves very rapidly as ventilator settings are decreased.
Pulmonary interstitial emphysema can rarely be misinterpreted as normally aerated lung surrounded by exudate as in an aspiration syndrome or pulmonary edema.[15]
This radiograph, obtained from a 1-day-old premature infant at 24 weeks' gestation, shows bilateral pulmonary interstitial emphysema (PIE). Linear radiolucencies extending up to the lung periphery are visible.
This radiograph, obtained from a premature infant at 26 weeks' gestation, shows characteristic radiographic changes of pulmonary interstitial emphysema (PIE) of the right lung.
This radiograph shows pneumothorax and pulmonary interstitial emphysema (PIE) on the right side. Interstitial air prevents collapse of the underlying lung by a tension pneumothorax. In such cases, extreme caution is required during drainage of a pneumothorax to avoid perforation of the underlying lung. Go to Imaging in Pulmonary Interstitial Emphysema for complete information on this topic.
Histologic Findings
The histology of pulmonary interstitial emphysema is well described by Plenat et al.[1] The histology demonstrates interstitial slits preferentially located in perivenous topography.
Sometimes, the peribronchial arterial or arteriolar sheaths are involved. Air dissects through a plane just next to the arterial or arteriolar face, opposite the bronchus, which is pushed into adjoining parenchyma. The bronchoarterial solidarity most often is respected.
Seldom, air can dissect arterioles and bronchioles and isolate them from the adjacent lobules. On the periphery of interstitial slits, the small vessels are compressed but never ruptured, whereas the collagen fibers are constantly broken and squeezed together.
Plenat F, Vert P, Didier F, Andre M. Pulmonary interstitial emphysema. Clin Perinatol. Sep 1978;5(2):351-75. [Medline].
Wood BP, Anderson VM, Mauk JE, Merritt TA. Pulmonary lymphatic air: locating "pulmonary interstitial emphysema" of the premature infant. AJR Am J Roentgenol. May 1982;138(5):809-14. [Medline].
Cunningham K, Paes BA, Symington A. Pulmonary interstitial emphysema: a review. Neonatal Netw. Aug 1992;11(5):7-16, 29-31. [Medline].
Gaylord MS, Thieme RE, Woodall DL, Quissell BJ. Predicting mortality in low-birth-weight infants with pulmonary interstitial emphysema. Pediatrics. Aug 1985;76(2):219-24. [Medline].
Verma RP, Chandra S, Niwas R, Komaroff E. Risk factors and clinical outcomes of pulmonary interstitial emphysema in extremely low birth weight infants. J Perinatol. Mar 2006;26(3):197-200. [Medline].
Kendig JW, Notter RH, Cox C, et al. Surfactant replacement therapy at birth: final analysis of a clinical trial and comparisons with similar trials. Pediatrics. Nov 1988;82(5):756-62. [Medline].
Dunn MS, Shennan AT, Zayack D, Possmayer F. Bovine surfactant replacement therapy in neonates of less than 30 weeks' gestation: a randomized controlled trial of prophylaxis versus treatment. Pediatrics. Mar 1991;87(3):377-86. [Medline].
Kattwinkel J, Bloom BT, Delmore P, et al. Prophylactic administration of calf lung surfactant extract is more effective than early treatment of respiratory distress syndrome in neonates of 29 through 32 weeks' gestation. Pediatrics. Jul 1993;92(1):90-8. [Medline].
Moriette G, Paris-Llado J, Walti H, et al. Prospective randomized multicenter comparison of high-frequency oscillatory ventilation and conventional ventilation in preterm infants of less than 30 weeks with respiratory distress syndrome. Pediatrics. Feb 2001;107(2):363-72. [Medline]. [Full Text].
Heneghan MA, Sosulski R, Alarcon MB. Early pulmonary interstitial emphysema in the newborn: a grave prognostic sign. Clin Pediatr (Phila). Jul 1987;26(7):361-5. [Medline].
Hart SM, McNair M, Gamsu HR, Price JF. Pulmonary interstitial emphysema in very low birthweight infants. Arch Dis Child. Aug 1983;58(8):612-5. [Medline].
Yu VY, Wong PY, Bajuk B, Szymonowicz W. Pulmonary interstitial emphysema in infants less than 1000 g at birth. Aust Paediatr J. Aug 1986;22(3):189-92. [Medline].
Greenough A, Dixon AK, Roberton NR. Pulmonary interstitial emphysema. Arch Dis Child. Nov 1984;59(11):1046-51. [Medline].
Morisot C, Kacet N, Bouchez MC, et al. Risk factors for fatal pulmonary interstitial emphysema in neonates. Eur J Pediatr. Apr 1990;149(7):493-5. [Medline].
Campbell RE. Intrapulmonary interstitial emphysema: a complication of hyaline membrane disease. Am J Roentgenol Radium Ther Nucl Med. Nov 1970;110(3):449-56. [Medline].
Schwartz AN, Graham CB. Neonatal tension pulmonary interstitial emphysema in bronchopulmonary dysplasia: treatment with lateral decubitus positioning. Radiology. Nov 1986;161(2):351-4. [Medline].
Brooks JG, Bustamante SA, Koops BL, et al. Selective bronchial intubation for the treatment of severe localized pulmonary interstitial emphysema in newborn infants. J Pediatr. Oct 1977;91(4):648-52. [Medline].
Chalak LF, Kaiser JR, Arrington RW. Resolution of pulmonary interstitial emphysema following selective left main stem intubation in a premature newborn: an old procedure revisited. Paediatr Anaesth. Feb 2007;17(2):183-6. [Medline].
Weintraub Z, Oliven A, Weissman D, Sonis Z. A new method for selective left main bronchus intubation in premature infants. J Pediatr Surg. Jun 1990;25(6):604-6. [Medline].
Keszler M, Donn SM, Bucciarelli RL, et al. Multicenter controlled trial comparing high-frequency jet ventilation and conventional mechanical ventilation in newborn infants with pulmonary interstitial emphysema. J Pediatr. Jul 1991;119(1 ( Pt 1)):85-93. [Medline].
Clark RH, Gerstmann DR, Null DM, et al. Pulmonary interstitial emphysema treated by high-frequency oscillatory ventilation. Crit Care Med. Nov 1986;14(11):926-30. [Medline].
Ahluwalia JS, Rennie JM, Wells FC. Successful outcome of severe unilateral pulmonary interstitial emphysema after bi-lobectomy in a very low birthweight infant. J R Soc Med. Mar 1996;89(3):167P-8P. [Medline]. [Full Text].
Gessler P, Toenz M, Gugger M, Pfenninger J. Lobar pulmonary interstitial emphysema in a premature infant on continuous positive airway pressure using nasal prongs. Eur J Pediatr. Apr 2001;160(4):263-4. [Medline].
Martinez-Frontanilla LA, Hernandez J, Haase GM, Burrington JD. Surgery of acquired lobar emphysema in the neonate. J Pediatr Surg. Aug 1984;19(4):375-9. [Medline].
Fitzgerald D, Willis D, Usher R, et al. Dexamethasone for pulmonary interstitial emphysema in preterm infants. Biol Neonate. 1998;73(1):34-9. [Medline].
Leonidas JC, Hall RT, Rhodes PG. Conservative management of unilateral pulmonary interstitial emphysema under tension. J Pediatr. Nov 1975;87(5):776-8. [Medline].
Dear PR, Conway SP. Treatment of severe bilateral interstitial emphysema in a baby by artificial pneumothorax and pneumonotomy [letter]. Lancet. Feb 4 1984;1(8371):273-5. [Medline].
Dordelmann M, Schirg E, Poets CF, Ure B, Gluer S, Bohnhorst B. Therapeutic lung puncture for diffuse unilateral pulmonary interstitial emphysema in preterm infants. Eur J Pediatr Surg. Aug 2008;18(4):233-6. [Medline].
Levine DH, Trump DS, Waterkotte G. Unilateral pulmonary interstitial emphysema: a surgical approach to treatment. Pediatrics. Oct 1981;68(4):510-4. [Medline].
Soll RF, Morley CJ. Prophylactic versus selective use of surfactant in preventing morbidity and mortality in preterm infants. Cochrane Database Syst Rev. 2001;CD000510. [Medline].
Stevens TP, Harrington EW, Blennow M, Soll RF. Early surfactant administration with brief ventilation vs. selective surfactant and continued mechanical ventilation for preterm infants with or at risk for respiratory distress syndrome. Cochrane Database Syst Rev. Oct 17 2007;CD003063. [Medline].
[Best Evidence] Soll R, Ozek E. Multiple versus single doses of exogenous surfactant for the prevention or treatment of neonatal respiratory distress syndrome. Cochrane Database Syst Rev. Jan 21 2009;CD000141. [Medline].
Pohlandt F, Saule H, Schroder H, et al. Decreased incidence of extra-alveolar air leakage or death prior to air leakage in high versus low rate positive pressure ventilation: results of a randomised seven-centre trial in preterm infants. Eur J Pediatr. Dec 1992;151(12):904-9. [Medline].
[Best Evidence] Henderson-Smart DJ, Cools F, Bhuta T, Offringa M. Elective high frequency oscillatory ventilation versus conventional ventilation for acute pulmonary dysfunction in preterm infants. Cochrane Database Syst Rev. Jul 18 2007;CD000104. [Medline].
Bhuta T, Henderson-Smart DJ. Rescue high frequency oscillatory ventilation versus conventional ventilation for pulmonary dysfunction in preterm infants. Cochrane Database Syst Rev. 2000;(2):CD000438. [Medline].
Bhuta T, Henderson-Smart DJ. Elective high frequency jet ventilation versus conventional ventilation for respiratory distress syndrome in preterm infants. Cochrane Database Syst Rev. 2000;(2):CD000328. [Medline].
[Best Evidence] McCallion N, Davis PG, Morley CJ. Volume-targeted versus pressure-limited ventilation in the neonate. Cochrane Database Syst Rev. 2005;CD003666. [Medline].

