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Conservative management of unilateral pulmonary interstitial emphysema under tension
776
November 1975 The Journal o f P E D I A T R I C S
Conservative management of unilateral pulmonary interstitial emphysema under tension J . C. L e o n i d a s , M . D . , * R . T . H a l l , M . D . , a n d P . G. R h o d e s , M . D . , K a n s a s City, M o .
PULMONARY INTERSTITIAL EMPHYSEMA is a frequent complication of idiopathic respiratory distress syndrome in the premature infant?, 2 In m a n y cases the interstitial pulmonary air dissects a l o n g p e r i b r o n c h i a l and perivascular spaces leading to p n e u m o m e d i a s t i n u m or pneumothorax, and less c o m m o n l y to pneumopericardium or pneumoperitoneum. In rare cases, interstitial pulmonary air m a y lead to massive inflation of one lung or lobe with tension causing shift of the mediastinum to the opposite side and compression of the contralateral lung?, 4 In such instances the prevailing dynamics are similar to those o f congenital lobar emphysema. Two recent publications r e c o m m e n d lobectomy f o r the treatment of localized PIE under tension?, 4 An infant with a similar radiographic picture was recently seen at Children's Mercy Hospital. Conservative measures resulted in a dramatic relief of symptoms and radiographic findings. CASE REPORT
Patient B. G. R. was born to a gravida 2, para 1, mother at approximately 34 weeks' gestation by cesarean section because of a placenta praevia. Apgar scores were recorded as 8 and 10 at 1 and 5 minutes of age respectively; the infant, however, received oxygen by face mask immediately following delivery because of "depressed respirations." She was placed in an incubator with increased inspired oxygen. She was then transferred to Children's Mercy Hospital at approximately 26 hours of age. At admission her weight was 1,713 gm, length 45 cm, and head circumference 28.5 era. The general systemic examination revealed severe intercostal and substernal retractions with an audible expiratory grunt. There were no congenital malformaFrom the Departments of Radiology and Pediatrics o f The Children's Mercy Hospital and the University o f Missouri. *Reprint address: The Children's Mercy Hospital, Radiology Department, 24th"and Gillham Rd., Kansas City, Mo. 64108.
Vol. 87, No. 5, pp. 776-778
tions. Arterial blood gases revealed a pH 7:22 pCO~ 43 ram Hg, and pO2 105 mm Hg in 100% inspired oxygen. Roentgenograms of the chest revealed a typical pattern of IRDS. The patient was treated initially with continuous positive airway pressure by face mask. Approximately 4 hours after admission the patient's clinical condition suddenly deteriorated with the development of a left pneumothorax which was relieved with chest tube drainage. The infant was intubated and treated with assisted ventilation because of frequent apneic spells. Ten hours later roentgenograms revealed pulmonary interstitial emphysema of the right lung, with formation of pulmonary pseudocysts, 5 followed by a small right pneumothorax. Subsequently, roentgenograms demonstrated persistence of the characteristic small linear and round lucencies of fight PIE (Fig. 1). The endotracheal tube remained .in place for 65 hours after which she was managed with a face mask. Abbreviations used PIE: pulmonary interstitial emphysema IRDS: idiopathic respiratory distress syndrome CPAP: continuous positive airway pressure Assisted ventilation was required from the second to the sixth hospital day followed by weaning to continuous positive airway pressure and subsequently to a hood on the eighth hospital day. There was a gradual deterioration of her respiratory status beginning at approximately the fourteenth day, which was associated with marked overdistension of the right lung with interstitial emphysema. By the eighteenth day the emphysema became extreme with projection of the right lung across the mediastinum and compression of the left lung (Fig. 2). Progressive respiratory distress made tracheal intubation necessary, and a right upper lobectomy or right pneumonectomy was strongly considered. It was decided to attempt a vigorous form of physical therapy prior to performing a lobectomy in order to relieve the localized PIE. This consisted of instilling 1 ml normal saline hourly into the endotracheal tube followed by endotracheal suctioning, vibration, percussion, and changing the infant's position to favor
Volume 87 Number 5
Pulmonary interstitial emphysema
777
Fig. 1. Small pneumothorax on the fight. Coarse, nonbranching lucencies in the fight lung field indicate early pulmonary interstitial emphysema.
Fig. 2. Extreme overdistension of the right lung (arrowsindicate extension across the mediastinum) with multiple small lucencies scattered throughout: severe pulmonary interstitial emphysema under tension.
drainage from the fight mainstem bronchus. The infant was then ventilated with 100% inspired oxygen for 5 to 10 minutes followed by lowering the inspired oxygen to levels necessary to maintain the Pao2 between 60 and 90 mm Hg. Over the ensuing 18 hours, the inspired oxygen requirements dropped from 60 to 35% and were associated with a dramatic improvement in the clinical condition. A repeat roentgenogram revealed total regression of the overexpanded right lung (Fig. 3). The infant's subsequent hospital course was uncomplicated; however, she continued to have radiographic changes of bronchopulmonary dysplasia. She was discharged on the forty-sixth day of hospitalization with no evidence of unilateral pulmonary emphysema. Indirect opathalmoscopy revealed narrowing of peripheral retinal vessels consistent with stage I retrolental fibroplasia 2 weeks prior to discharge; however, these findings regressed to normal on a follow-up examination at 4 months of age. The remainder of the physical examination was likewise entirely normal at that time.
spaces toward the hilum. It may be postulated that the perivascular spaces near the hilum are compressed by adjacent pulmonary interstitial air pockets preventing escape into the mediastinum. This may result in an interstitial ball valve effect. Administration of 100% inspired oxygen as suggested by Chernick and Avery~ in the management of pneumomediastinum may represent the most important aspect of conservative therapy. Increasing the difference between the partial pressure of gas in the loculated phase and the blood phase for each gas will facilitate the rate of absorption. This must be done with extreme caution in the preterm infant to avoid retrolental fibroplasia. This can probably be accomplished by limiting the exposure to 100% oxygen to 10 minutes followed by lowering the inspired oxygen to levels necessary to maintain the Pao2 between 60 and 90 mm Hg. Pulmonary oxygen toxicity must als0 be considered a potential complication; however, this complication is probably related to the total oxygen dose so that an effective response would lessen the total exposure to higher concentrations of inspired oxygen. Nevertheless, a time limit of 2 to 3 days of
DISCUSSION Localized PIE under tension is relatively rare and incompletely understood. Following alveolar rupture, air dissects into the interstitium and traverses the perivascutar
778
Leonidas, Hall, and Rhodes
The Journal of Pediatrics November 1975
in localized PIE as proposed by Macklin and Macklin. 7 Removal of endotracheal obstruction, when present, will assist in restoring alveolar dynamics and prevent further accumulation of interstitial air. CONCLUSION Vigorous conservative therapy as outlined above is recommended prior to lobectomy when there is unilateral overdistension of the lung in the course of IRDS. Such management may solve the problem without subjecting the patient to the loss of functioning lung tissue. If it is unsuccessful, then lobectomy or pneumonectomy may be contemplated. REFERENCES
Fig. 3. Roentgenogram obtained approximately 48 hours after
the film of Fig. 2. Emphysema is no longer apparent. conservative therapy is probably wise in order to prevent both complications of oxygen toxicity. Vigorous therapy with tracheobronchial suctioning as well as vibration and percussion may alleviate tracheobronchial obstruction. This mechanism may be operative
1. Thiebeault DW, Lachman RS, Laul VR, and Kwong MS: Pulmonary interstitial emphysema, pneumomediastinum and pneumothorax, Am J Dis Child 126:611, 1973. 2. CampbeURE: Intrapulmonary interstitial emphysema: A complication of hyaline membrane disease, Am J Roentgenol Rad Ther Nucl Med 110:449, 1970. 3. Fletcher BD, Outerbfidge EW, Youssef S, and Bolande RP: Pulmonary interstitial emphysema in a newborn infant treated by lobectomy, Pediatrics 54:808, 1974. 4. Magilner AD, Capitanio MA, Wertheimer I, and Burko H: Persistent localized intrapulmonaryinterstitial emphysema: An observation in three infants, Radiology 111:379, 1974. 5. Weller MH: The roentgenographic course and complications of hyaline membrane disease, Pediatr Clin North Am 20:381, 1973. 6. Chernick V, and Avery ME: Spontaneous alveolar rupture at birth, Pediatrics 32:816, 1963. 7. Macklin MT, and Macklin CC: Malignant interstitial emphysema of the lungs and mediasfinum as an important occult complication in many respiratory diseases and other conditions: An interpretation of the clinical literature in the light of laboratory experiment, Medicine 23:281, 1944.
November 1975 The Journal o f P E D I A T R I C S
Conservative management of unilateral pulmonary interstitial emphysema under tension J . C. L e o n i d a s , M . D . , * R . T . H a l l , M . D . , a n d P . G. R h o d e s , M . D . , K a n s a s City, M o .
PULMONARY INTERSTITIAL EMPHYSEMA is a frequent complication of idiopathic respiratory distress syndrome in the premature infant?, 2 In m a n y cases the interstitial pulmonary air dissects a l o n g p e r i b r o n c h i a l and perivascular spaces leading to p n e u m o m e d i a s t i n u m or pneumothorax, and less c o m m o n l y to pneumopericardium or pneumoperitoneum. In rare cases, interstitial pulmonary air m a y lead to massive inflation of one lung or lobe with tension causing shift of the mediastinum to the opposite side and compression of the contralateral lung?, 4 In such instances the prevailing dynamics are similar to those o f congenital lobar emphysema. Two recent publications r e c o m m e n d lobectomy f o r the treatment of localized PIE under tension?, 4 An infant with a similar radiographic picture was recently seen at Children's Mercy Hospital. Conservative measures resulted in a dramatic relief of symptoms and radiographic findings. CASE REPORT
Patient B. G. R. was born to a gravida 2, para 1, mother at approximately 34 weeks' gestation by cesarean section because of a placenta praevia. Apgar scores were recorded as 8 and 10 at 1 and 5 minutes of age respectively; the infant, however, received oxygen by face mask immediately following delivery because of "depressed respirations." She was placed in an incubator with increased inspired oxygen. She was then transferred to Children's Mercy Hospital at approximately 26 hours of age. At admission her weight was 1,713 gm, length 45 cm, and head circumference 28.5 era. The general systemic examination revealed severe intercostal and substernal retractions with an audible expiratory grunt. There were no congenital malformaFrom the Departments of Radiology and Pediatrics o f The Children's Mercy Hospital and the University o f Missouri. *Reprint address: The Children's Mercy Hospital, Radiology Department, 24th"and Gillham Rd., Kansas City, Mo. 64108.
Vol. 87, No. 5, pp. 776-778
tions. Arterial blood gases revealed a pH 7:22 pCO~ 43 ram Hg, and pO2 105 mm Hg in 100% inspired oxygen. Roentgenograms of the chest revealed a typical pattern of IRDS. The patient was treated initially with continuous positive airway pressure by face mask. Approximately 4 hours after admission the patient's clinical condition suddenly deteriorated with the development of a left pneumothorax which was relieved with chest tube drainage. The infant was intubated and treated with assisted ventilation because of frequent apneic spells. Ten hours later roentgenograms revealed pulmonary interstitial emphysema of the right lung, with formation of pulmonary pseudocysts, 5 followed by a small right pneumothorax. Subsequently, roentgenograms demonstrated persistence of the characteristic small linear and round lucencies of fight PIE (Fig. 1). The endotracheal tube remained .in place for 65 hours after which she was managed with a face mask. Abbreviations used PIE: pulmonary interstitial emphysema IRDS: idiopathic respiratory distress syndrome CPAP: continuous positive airway pressure Assisted ventilation was required from the second to the sixth hospital day followed by weaning to continuous positive airway pressure and subsequently to a hood on the eighth hospital day. There was a gradual deterioration of her respiratory status beginning at approximately the fourteenth day, which was associated with marked overdistension of the right lung with interstitial emphysema. By the eighteenth day the emphysema became extreme with projection of the right lung across the mediastinum and compression of the left lung (Fig. 2). Progressive respiratory distress made tracheal intubation necessary, and a right upper lobectomy or right pneumonectomy was strongly considered. It was decided to attempt a vigorous form of physical therapy prior to performing a lobectomy in order to relieve the localized PIE. This consisted of instilling 1 ml normal saline hourly into the endotracheal tube followed by endotracheal suctioning, vibration, percussion, and changing the infant's position to favor
Volume 87 Number 5
Pulmonary interstitial emphysema
777
Fig. 1. Small pneumothorax on the fight. Coarse, nonbranching lucencies in the fight lung field indicate early pulmonary interstitial emphysema.
Fig. 2. Extreme overdistension of the right lung (arrowsindicate extension across the mediastinum) with multiple small lucencies scattered throughout: severe pulmonary interstitial emphysema under tension.
drainage from the fight mainstem bronchus. The infant was then ventilated with 100% inspired oxygen for 5 to 10 minutes followed by lowering the inspired oxygen to levels necessary to maintain the Pao2 between 60 and 90 mm Hg. Over the ensuing 18 hours, the inspired oxygen requirements dropped from 60 to 35% and were associated with a dramatic improvement in the clinical condition. A repeat roentgenogram revealed total regression of the overexpanded right lung (Fig. 3). The infant's subsequent hospital course was uncomplicated; however, she continued to have radiographic changes of bronchopulmonary dysplasia. She was discharged on the forty-sixth day of hospitalization with no evidence of unilateral pulmonary emphysema. Indirect opathalmoscopy revealed narrowing of peripheral retinal vessels consistent with stage I retrolental fibroplasia 2 weeks prior to discharge; however, these findings regressed to normal on a follow-up examination at 4 months of age. The remainder of the physical examination was likewise entirely normal at that time.
spaces toward the hilum. It may be postulated that the perivascular spaces near the hilum are compressed by adjacent pulmonary interstitial air pockets preventing escape into the mediastinum. This may result in an interstitial ball valve effect. Administration of 100% inspired oxygen as suggested by Chernick and Avery~ in the management of pneumomediastinum may represent the most important aspect of conservative therapy. Increasing the difference between the partial pressure of gas in the loculated phase and the blood phase for each gas will facilitate the rate of absorption. This must be done with extreme caution in the preterm infant to avoid retrolental fibroplasia. This can probably be accomplished by limiting the exposure to 100% oxygen to 10 minutes followed by lowering the inspired oxygen to levels necessary to maintain the Pao2 between 60 and 90 mm Hg. Pulmonary oxygen toxicity must als0 be considered a potential complication; however, this complication is probably related to the total oxygen dose so that an effective response would lessen the total exposure to higher concentrations of inspired oxygen. Nevertheless, a time limit of 2 to 3 days of
DISCUSSION Localized PIE under tension is relatively rare and incompletely understood. Following alveolar rupture, air dissects into the interstitium and traverses the perivascutar
778
Leonidas, Hall, and Rhodes
The Journal of Pediatrics November 1975
in localized PIE as proposed by Macklin and Macklin. 7 Removal of endotracheal obstruction, when present, will assist in restoring alveolar dynamics and prevent further accumulation of interstitial air. CONCLUSION Vigorous conservative therapy as outlined above is recommended prior to lobectomy when there is unilateral overdistension of the lung in the course of IRDS. Such management may solve the problem without subjecting the patient to the loss of functioning lung tissue. If it is unsuccessful, then lobectomy or pneumonectomy may be contemplated. REFERENCES
Fig. 3. Roentgenogram obtained approximately 48 hours after
the film of Fig. 2. Emphysema is no longer apparent. conservative therapy is probably wise in order to prevent both complications of oxygen toxicity. Vigorous therapy with tracheobronchial suctioning as well as vibration and percussion may alleviate tracheobronchial obstruction. This mechanism may be operative
1. Thiebeault DW, Lachman RS, Laul VR, and Kwong MS: Pulmonary interstitial emphysema, pneumomediastinum and pneumothorax, Am J Dis Child 126:611, 1973. 2. CampbeURE: Intrapulmonary interstitial emphysema: A complication of hyaline membrane disease, Am J Roentgenol Rad Ther Nucl Med 110:449, 1970. 3. Fletcher BD, Outerbfidge EW, Youssef S, and Bolande RP: Pulmonary interstitial emphysema in a newborn infant treated by lobectomy, Pediatrics 54:808, 1974. 4. Magilner AD, Capitanio MA, Wertheimer I, and Burko H: Persistent localized intrapulmonaryinterstitial emphysema: An observation in three infants, Radiology 111:379, 1974. 5. Weller MH: The roentgenographic course and complications of hyaline membrane disease, Pediatr Clin North Am 20:381, 1973. 6. Chernick V, and Avery ME: Spontaneous alveolar rupture at birth, Pediatrics 32:816, 1963. 7. Macklin MT, and Macklin CC: Malignant interstitial emphysema of the lungs and mediasfinum as an important occult complication in many respiratory diseases and other conditions: An interpretation of the clinical literature in the light of laboratory experiment, Medicine 23:281, 1944.