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Bilateral congenital lobar emphysema: staged management
Journal of Pediatric Surgery 52 (2017) 1442–1445
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Bilateral congenital lobar emphysema: staged management☆,☆☆ Lindsey Perea a,⁎, Thane Blinman b, Joseph Piccione c, Pablo Laje b a b c
Department of Surgery, Philadelphia College of Osteopathic Medicine, 4170 City Avenue, Philadelphia, PA, 19131, USA Division of Pediatric General, Thoracic and Fetal Surgery, Department of Surgery, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA Division of Pulmonary Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, PA, 19104, USA
a r t i c l e
i n f o
Article history: Received 26 September 2016 Received in revised form 16 January 2017 Accepted 21 January 2017 Key words: Bilateral congenital lobar emphysema Congenital lobar emphysema Congenital hyperlucent lung Pulmonary interstitial glycogenosis
a b s t r a c t Background: Only a few isolated cases in the literature exist to guide management of bilateral congenital lobar emphysema (CLE). Here, we review our experience in infants with bilateral CLE. Methods: A case series of all infants presenting with bilateral CLE from 2014 to 2015 in a single institution. Results: Four patients underwent intervention, with all having right middle lobe (RML) and left upper lobe (LUL) affected. Preoperative planning with computed tomography angiography (CTA) chest allowed a tailored approach based on specific radiologic features. All patients also underwent bronchoscopy to evaluate the anatomy and to assess for alternative causes of airway compression. Three patients underwent unilateral lobectomies, two RML and one LUL. All are growing normally and on room air more than one year later. The last patient underwent a staged procedure beginning with left upper lobectomy followed by right middle lobectomy two weeks later after exhibiting rebound hyperexpansion of the remaining diseased lobe. Thoracoscopy was precluded by mass effect in all patients. No patients underwent emergent lobectomies. One patient had pulmonary interstitial glycogenosis (PIG) in the setting of CLE, first reported case of bilateral CLE with PIG. Conclusions: This study supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow up, and comports with a “the least intervention that is the most effective” philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. Level of evidence: Treatment Study, Level IV. © 2017 Elsevier Inc. All rights reserved.
Congenital lobar emphysema (CLE), or congenital hyperlucent lung, is a rare condition in which over-inflation of one or more lobes occurs secondary to obstruction, either intrinsically or extrinsically. It is thought to result secondary to multiple disruptions in bronchopulmonary development [1]. As air enters the lung, a ball-valve mechanism occurs, trapping air from exiting the lung, further exacerbating the condition [1]. Infants become symptomatic early on in life with about one third symptomatic at birth [2]. Fifty percent of children are symptomatic by one month of life and almost all by six months of age [2]. Single lobe, congenital lobar emphysema occurs with an incidence of 1 in 70–90,000, and a prevalence of 1 in 20–30,000 [2]. The most commonly affected lobe is the left upper lobe (40–50% of cases), followed by the right middle lobe (25–35% of cases) [2]. Oftentimes diagnosis can be made from plain radiographs demonstrating overinflation and mediastinal shift with compression of unaffected lobes/lung. At times, ☆ Poster at the World Federation of Associations of Pediatric Surgeons 2016 Washington D.C. ☆☆ The authors have no conflicts of interest, financial or otherwise, to disclose. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. ⁎ Corresponding author at: 4170 City Avenue, Philadelphia, PA 19131. Tel.: +1 484 678 2686. E-mail addresses: [email protected] (L. Perea), [email protected] (T. Blinman), [email protected] (J. Piccione), [email protected] (P. Laje). http://dx.doi.org/10.1016/j.jpedsurg.2017.01.056 0022-3468/© 2017 Elsevier Inc. All rights reserved.
CLE can be diagnosed prenatally with ultrasound. CLE can be distinguished from other congenital lung lesions such as congenital cystic adenomatoid malformations and bronchopulmonary sequestrations, because of increased echogenicity and reflectivity [3]. Only a few isolated cases in the literature exist to guide management of bilateral CLE [4]. Our institution seems to have acquired a cohort of patients with bilateral CLE. This study looks to report our experience of patients from 2014 to 2015 in what we believe is the largest series of patients found to have bilateral congenital lobar emphysema. Additionally, we look to report on our management and outcomes of these patients.
1. Methods A case series including all infants presenting with bilateral CLE from 2014 to 2015 in a single institution.
2. Results Table 1 summarizes the pertinent findings regarding each patient described in the following cases.
L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
1443
Table 1 Description of patients with pertinent findings. Patient
Lobes affected
CTA findings
Bronchoscopy findings
Lobes resected
Case 1
RML, LUL
Aberrant bronchi
RML
Case 2
RML, LUL
Case 3
RML, LUL
Overinflation of RML with herniation across midline, and overinflation of LUL Overinflation of RML, LUL
Left main stem bronchus compression/malacia Severe RML bronchus compression/malacia (Marked improvement in chest radiograph appearance of adjacent lobes after balloon catheter occlusion) Bronchomalacia of RML and LUL bronchi
LUL and then RML
Case 4
RML, LUL
Compression with near complete effacement of RML and LUL bronchi LUL bronchus compression/malacia
Diffuse emphysema in RML, LUL also with herniation across midline
RML
LUL
RML - right middle lobe. LUL - left upper lobe.
2.1. Case one A 39 1/7-week female presented to our facility as a transfer from an outside hospital at day of life (DOL) 82. She required transfer to the neonatal intensive care unit (NICU) because of tachypnea on her second day of life. At the referring facility, bronchoscopy demonstrated narrow right middle lobe and left main bronchi. Right upper lobe atelectasis was also noted. Plain radiographs demonstrated both right middle and left upper lobe air trapping. Computed tomography angiography (CTA) of the chest noted small aberrant bronchi (Image 1). Because of continued respiratory distress the patient underwent tracheostomy. Her course included inhaled nitric oxide, as well, that was started before transfer to our facility. The patient underwent repeat bronchoscopy with our pulmonary team and was found to have non-pulsatile compression of the left mainstem bronchus at the takeoff of the left upper lobe, which failed to improve despite increasing positive end expiratory pressure (PEEP). The right middle lobe bronchus orifice was severely compressed as well. In order to assess the contribution of these lobes to the patient's alveolar ventilation and their impact on ventilation–perfusion relationships, selective bronchial occlusion was performed under fiberoptic flexible bronchoscopic guidance using a balloon catheter. During occlusion of the right middle lobe, the adjacent lobes re-expanded and the PEEP and peak inspiratory pressure (PIP) requirements decreased dramatically. Given the improvement in right upper and lower lobe re-expansion following balloon catheter occlusion of the right middle lobe, the infant
Image 1. Case one CTA demonstrating left upper and right middle lobe hyperinflation.
underwent a right middle lobectomy via thoracotomy. Prior to discharge her tracheostomy was decannulated and she was breathing room air. Pathology revealed an alveolated lung with congenital lobar emphysema features along with chronic lung disease. She was discharged to home almost three months after surgery. 2.2. Case two A 38 2/7-week male presented to our facility as a transfer from an outside hospital at day of life ten for further management and pulmonary evaluation. At the referring facility, the infant was noted to have intermittent desaturations, tachypnea and increased work of breathing. Chest radiograph at the outside hospital demonstrated right upper lobe atelectasis. CTA chest was obtained demonstrating relative overinflation of both the right middle lobe and left upper lobe without ability to visualize their respective bronchi (Image 2). Of note the right middle lobe extended into the mediastinum anterior to the thymus. These findings were concerning for either bronchial atresia or congenital lobar emphysema. The patient underwent flexible bronchoscopy at 18 days of life demonstrating bronchomalacia of both the right middle lobe and left upper lobe bronchi and apparent non-pulsatile compression. The patient was taken for right middle lobectomy via thoracotomy given the bronchoscopic findings coupled with the herniation of the right middle lobe across midline. Post operatively the patient had continued collapse of the right lung and underwent repeat bronchoscopy to evaluate for mucus impaction and attempt to assist lung re-expansion.
Image 2. Case two CTA demonstrating hyperinflation of right middle lobe with herniation across midline, and hyperinflation of left upper lobe.
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L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
The pathology revealed a combination of patchy areas of distal overinflation along with pulmonary interstitial glycogenosis (PIG) for which he was treated with a three-day steroid course. On postoperative day ten from the thoracotomy the patient was extubated and subsequently discharged 3 weeks later. 2.3. Case three A 40 5/7-week term male presented to our facility as a transfer from an outside facility at day of life 15. The infant was admitted to the outside facility's NICU at birth for respiratory distress, with increasing oxygen requirements. A chest radiograph from two days prior to the transfer demonstrated a hyperinflated right middle lobe as well as left upper lobe. Once at our facility the patient underwent a CTA chest that demonstrated right middle lobe and left upper lobe hyperinflation consistent with congenital lobar emphysema (Image 3). Bronchoscopy revealed non-pulsatile compression with near complete effacement of the right middle and left upper lobe bronchi. Following bronchoscopy, the child underwent a left upper lobectomy through a thoracotomy. Interestingly, an immediate post-operative bronchoscopy demonstrated apparent resolution of the right middle lobe bronchus compression. However, during the postoperative course, rebound hyperexpansion of the right middle lobe occurred and prompted the decision to perform a right middle lobectomy as well. One week postoperatively, the patient was extubated. The pathology of the left upper lobe demonstrated over-distention of the alveolar airspaces. Pathology of the right middle lobe demonstrated mild over distention. After extubation, he was weaned to room air and nutritionally optimized prior to discharge four weeks later.
with increased work of breathing. At this time pulmonology was consulted for management. He was found to have continued right upper lobe atelectasis present on imaging with marked hyperinflation of the right middle and left upper lobes. After discharge he presented to an outside hospital at seven months of age in respiratory distress where he was found to have rhinovirus again, as well as parainfluenza virus. At the outside hospital he was noted to have left lung overinflation with herniation to the right on chest radiograph as well as bronchial tree abnormalities on CTA of the chest. Bronchoscopy revealed left mainstem bronchomalacia, and apparent right upper lobe atresia. The patient's respiratory status continued to decline requiring BiPAP and eventually intubation. He was managed for one month at the referring institution prior to transfer to our institution for further management. After transfer to our facility, the child underwent bronchoscopy and left upper lobe bronchus narrowing improved with high levels of PEEP. Repeat CT scan imaging noted overall progression of disease compared to the prior CT scan with diffuse emphysema in the left upper lobe with new herniation to the right, and emphysema in the right middle and possibly right lower lobes (Image 4). Two weeks after the patient's arrival at our facility, he underwent a left upper lobectomy via thoracotomy given the left upper lobe herniation to the right. He was extubated on post-operative day two. Following extubation the patient was discharged home two weeks later. Pathology revealed emphysematous changes in the left upper lobe along with acute interstitial pneumonia. Repeat imaging one year after resection demonstrated continued right middle lobe hyperinflation but the patient continues to remain stable.
2.4. Case four
3. Discussion
At two months of age, a former 37-week male infant presented to our facility with increased work of breathing and cough and was found to have rhinovirus. Chest radiograph noted right upper lobe atelectasis along with relative hyperinflation of the right middle and left upper lobes. He was diagnosed with asthma during this stay, and symptomatically treated. Unfortunately, at five months of age he returned
As previously noted, congenital lobar emphysema is a very rare disease with bilateral disease only noted in a few cases in the literature. All four patients in this study had right middle lobe and left upper lobe disease. All patients underwent preoperative planning with CTA chest allowing for a tailored treatment approach based on specific radiologic features: lung herniation across midline, mediastinal shift, and/or volumetric enlargement in comparison to the other diseased lobe. The primary rationale for flexible bronchoscopy is to evaluate for alternative causes of lung hyperinflation including vascular compression, foreign bodies, inspissated mucus, airway anatomical anomalies and endobronchial tumors.
Image 3. Case three CTA demonstrating hyperinflation of both right middle and left upper lobes.
Image 4. Case four CTA with left upper and right middle lobe hyperinflation and herniation of left upper lobe across midline.
L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
However, the addition of selective endobronchial occlusion techniques allows further physiologic assessment regarding the potential impact of lobectomy when the etiology of hyperinflation is unclear because of complex respiratory comorbidities. Two patients underwent unilateral right middle lobectomy whereas one patient underwent a unilateral left upper lobectomy, and all are growing normally and breathing room air one year later. All three patients continue to undergo routine follow up with the pulmonary department and remain stable and have not required further intervention for the remaining diseased lobe to this point. The last patient underwent a staged procedure, a left upper lobectomy followed by a right middle lobectomy when he exhibited rebound hyperexpansion of the right middle lobe following the initial surgery. Thoracoscopy was excluded because of mass effect on all patients. None of the patients required emergent lobectomies. Previous case reports evaluated reports of patients with bilateral disease who mostly underwent bilateral lobectomies via thoracotomy either in a single procedure or a staged fashion [4]. Given the evidence that 75% of our small series of patients did not require a subsequent lobectomy, it may be an indication that a staged approach is more favorable than single stage not only in regard to postoperative pain, but more so to see if further surgery is even warranted. One patient, the child in case two, was found to have PIG in the setting of CLE. PIG is a form of interstitial lung disease (ILD) in infants. To our knowledge this is the only reported case of PIG with bilateral CLE.
1445
Hilvers et al., reported a child with a left upper lobe CLE and PIG, the first reported case connecting the two processes in the literature [5]. The pathophysiology and etiology of PIG is poorly understood. Further research may be warranted to see if a true association exists between CLE and PIG. 4. Conclusion This case series supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow-up, and comports with a “the least intervention that is the most effective” philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. References [1] Olutoye OO, Coleman BG, Hubbard AM, et al. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg 2000;35(5):792–5. [2] Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994;41: 453–72. [3] Adzick NS. Management of fetal lung lesions. Clin Perinatol 2009;36:363–76. [4] Maiya S, Clarke JR, More B, et al. Bilateral congenital lobar emphysema: how should we proceed? Pediatr Surg Int 2005;21:659–61. [5] Hilvers P, Polak M, Bacaj P, et al. Coexistence of congenital lobar emphysema and pulmonary interstitial glycogenosis in a neonate. Am J Respir Crit Care Med 2010;181:A6737.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Bilateral congenital lobar emphysema: staged management☆,☆☆ Lindsey Perea a,⁎, Thane Blinman b, Joseph Piccione c, Pablo Laje b a b c
Department of Surgery, Philadelphia College of Osteopathic Medicine, 4170 City Avenue, Philadelphia, PA, 19131, USA Division of Pediatric General, Thoracic and Fetal Surgery, Department of Surgery, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA Division of Pulmonary Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, PA, 19104, USA
a r t i c l e
i n f o
Article history: Received 26 September 2016 Received in revised form 16 January 2017 Accepted 21 January 2017 Key words: Bilateral congenital lobar emphysema Congenital lobar emphysema Congenital hyperlucent lung Pulmonary interstitial glycogenosis
a b s t r a c t Background: Only a few isolated cases in the literature exist to guide management of bilateral congenital lobar emphysema (CLE). Here, we review our experience in infants with bilateral CLE. Methods: A case series of all infants presenting with bilateral CLE from 2014 to 2015 in a single institution. Results: Four patients underwent intervention, with all having right middle lobe (RML) and left upper lobe (LUL) affected. Preoperative planning with computed tomography angiography (CTA) chest allowed a tailored approach based on specific radiologic features. All patients also underwent bronchoscopy to evaluate the anatomy and to assess for alternative causes of airway compression. Three patients underwent unilateral lobectomies, two RML and one LUL. All are growing normally and on room air more than one year later. The last patient underwent a staged procedure beginning with left upper lobectomy followed by right middle lobectomy two weeks later after exhibiting rebound hyperexpansion of the remaining diseased lobe. Thoracoscopy was precluded by mass effect in all patients. No patients underwent emergent lobectomies. One patient had pulmonary interstitial glycogenosis (PIG) in the setting of CLE, first reported case of bilateral CLE with PIG. Conclusions: This study supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow up, and comports with a “the least intervention that is the most effective” philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. Level of evidence: Treatment Study, Level IV. © 2017 Elsevier Inc. All rights reserved.
Congenital lobar emphysema (CLE), or congenital hyperlucent lung, is a rare condition in which over-inflation of one or more lobes occurs secondary to obstruction, either intrinsically or extrinsically. It is thought to result secondary to multiple disruptions in bronchopulmonary development [1]. As air enters the lung, a ball-valve mechanism occurs, trapping air from exiting the lung, further exacerbating the condition [1]. Infants become symptomatic early on in life with about one third symptomatic at birth [2]. Fifty percent of children are symptomatic by one month of life and almost all by six months of age [2]. Single lobe, congenital lobar emphysema occurs with an incidence of 1 in 70–90,000, and a prevalence of 1 in 20–30,000 [2]. The most commonly affected lobe is the left upper lobe (40–50% of cases), followed by the right middle lobe (25–35% of cases) [2]. Oftentimes diagnosis can be made from plain radiographs demonstrating overinflation and mediastinal shift with compression of unaffected lobes/lung. At times, ☆ Poster at the World Federation of Associations of Pediatric Surgeons 2016 Washington D.C. ☆☆ The authors have no conflicts of interest, financial or otherwise, to disclose. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. ⁎ Corresponding author at: 4170 City Avenue, Philadelphia, PA 19131. Tel.: +1 484 678 2686. E-mail addresses: [email protected] (L. Perea), [email protected] (T. Blinman), [email protected] (J. Piccione), [email protected] (P. Laje). http://dx.doi.org/10.1016/j.jpedsurg.2017.01.056 0022-3468/© 2017 Elsevier Inc. All rights reserved.
CLE can be diagnosed prenatally with ultrasound. CLE can be distinguished from other congenital lung lesions such as congenital cystic adenomatoid malformations and bronchopulmonary sequestrations, because of increased echogenicity and reflectivity [3]. Only a few isolated cases in the literature exist to guide management of bilateral CLE [4]. Our institution seems to have acquired a cohort of patients with bilateral CLE. This study looks to report our experience of patients from 2014 to 2015 in what we believe is the largest series of patients found to have bilateral congenital lobar emphysema. Additionally, we look to report on our management and outcomes of these patients.
1. Methods A case series including all infants presenting with bilateral CLE from 2014 to 2015 in a single institution.
2. Results Table 1 summarizes the pertinent findings regarding each patient described in the following cases.
L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
1443
Table 1 Description of patients with pertinent findings. Patient
Lobes affected
CTA findings
Bronchoscopy findings
Lobes resected
Case 1
RML, LUL
Aberrant bronchi
RML
Case 2
RML, LUL
Case 3
RML, LUL
Overinflation of RML with herniation across midline, and overinflation of LUL Overinflation of RML, LUL
Left main stem bronchus compression/malacia Severe RML bronchus compression/malacia (Marked improvement in chest radiograph appearance of adjacent lobes after balloon catheter occlusion) Bronchomalacia of RML and LUL bronchi
LUL and then RML
Case 4
RML, LUL
Compression with near complete effacement of RML and LUL bronchi LUL bronchus compression/malacia
Diffuse emphysema in RML, LUL also with herniation across midline
RML
LUL
RML - right middle lobe. LUL - left upper lobe.
2.1. Case one A 39 1/7-week female presented to our facility as a transfer from an outside hospital at day of life (DOL) 82. She required transfer to the neonatal intensive care unit (NICU) because of tachypnea on her second day of life. At the referring facility, bronchoscopy demonstrated narrow right middle lobe and left main bronchi. Right upper lobe atelectasis was also noted. Plain radiographs demonstrated both right middle and left upper lobe air trapping. Computed tomography angiography (CTA) of the chest noted small aberrant bronchi (Image 1). Because of continued respiratory distress the patient underwent tracheostomy. Her course included inhaled nitric oxide, as well, that was started before transfer to our facility. The patient underwent repeat bronchoscopy with our pulmonary team and was found to have non-pulsatile compression of the left mainstem bronchus at the takeoff of the left upper lobe, which failed to improve despite increasing positive end expiratory pressure (PEEP). The right middle lobe bronchus orifice was severely compressed as well. In order to assess the contribution of these lobes to the patient's alveolar ventilation and their impact on ventilation–perfusion relationships, selective bronchial occlusion was performed under fiberoptic flexible bronchoscopic guidance using a balloon catheter. During occlusion of the right middle lobe, the adjacent lobes re-expanded and the PEEP and peak inspiratory pressure (PIP) requirements decreased dramatically. Given the improvement in right upper and lower lobe re-expansion following balloon catheter occlusion of the right middle lobe, the infant
Image 1. Case one CTA demonstrating left upper and right middle lobe hyperinflation.
underwent a right middle lobectomy via thoracotomy. Prior to discharge her tracheostomy was decannulated and she was breathing room air. Pathology revealed an alveolated lung with congenital lobar emphysema features along with chronic lung disease. She was discharged to home almost three months after surgery. 2.2. Case two A 38 2/7-week male presented to our facility as a transfer from an outside hospital at day of life ten for further management and pulmonary evaluation. At the referring facility, the infant was noted to have intermittent desaturations, tachypnea and increased work of breathing. Chest radiograph at the outside hospital demonstrated right upper lobe atelectasis. CTA chest was obtained demonstrating relative overinflation of both the right middle lobe and left upper lobe without ability to visualize their respective bronchi (Image 2). Of note the right middle lobe extended into the mediastinum anterior to the thymus. These findings were concerning for either bronchial atresia or congenital lobar emphysema. The patient underwent flexible bronchoscopy at 18 days of life demonstrating bronchomalacia of both the right middle lobe and left upper lobe bronchi and apparent non-pulsatile compression. The patient was taken for right middle lobectomy via thoracotomy given the bronchoscopic findings coupled with the herniation of the right middle lobe across midline. Post operatively the patient had continued collapse of the right lung and underwent repeat bronchoscopy to evaluate for mucus impaction and attempt to assist lung re-expansion.
Image 2. Case two CTA demonstrating hyperinflation of right middle lobe with herniation across midline, and hyperinflation of left upper lobe.
1444
L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
The pathology revealed a combination of patchy areas of distal overinflation along with pulmonary interstitial glycogenosis (PIG) for which he was treated with a three-day steroid course. On postoperative day ten from the thoracotomy the patient was extubated and subsequently discharged 3 weeks later. 2.3. Case three A 40 5/7-week term male presented to our facility as a transfer from an outside facility at day of life 15. The infant was admitted to the outside facility's NICU at birth for respiratory distress, with increasing oxygen requirements. A chest radiograph from two days prior to the transfer demonstrated a hyperinflated right middle lobe as well as left upper lobe. Once at our facility the patient underwent a CTA chest that demonstrated right middle lobe and left upper lobe hyperinflation consistent with congenital lobar emphysema (Image 3). Bronchoscopy revealed non-pulsatile compression with near complete effacement of the right middle and left upper lobe bronchi. Following bronchoscopy, the child underwent a left upper lobectomy through a thoracotomy. Interestingly, an immediate post-operative bronchoscopy demonstrated apparent resolution of the right middle lobe bronchus compression. However, during the postoperative course, rebound hyperexpansion of the right middle lobe occurred and prompted the decision to perform a right middle lobectomy as well. One week postoperatively, the patient was extubated. The pathology of the left upper lobe demonstrated over-distention of the alveolar airspaces. Pathology of the right middle lobe demonstrated mild over distention. After extubation, he was weaned to room air and nutritionally optimized prior to discharge four weeks later.
with increased work of breathing. At this time pulmonology was consulted for management. He was found to have continued right upper lobe atelectasis present on imaging with marked hyperinflation of the right middle and left upper lobes. After discharge he presented to an outside hospital at seven months of age in respiratory distress where he was found to have rhinovirus again, as well as parainfluenza virus. At the outside hospital he was noted to have left lung overinflation with herniation to the right on chest radiograph as well as bronchial tree abnormalities on CTA of the chest. Bronchoscopy revealed left mainstem bronchomalacia, and apparent right upper lobe atresia. The patient's respiratory status continued to decline requiring BiPAP and eventually intubation. He was managed for one month at the referring institution prior to transfer to our institution for further management. After transfer to our facility, the child underwent bronchoscopy and left upper lobe bronchus narrowing improved with high levels of PEEP. Repeat CT scan imaging noted overall progression of disease compared to the prior CT scan with diffuse emphysema in the left upper lobe with new herniation to the right, and emphysema in the right middle and possibly right lower lobes (Image 4). Two weeks after the patient's arrival at our facility, he underwent a left upper lobectomy via thoracotomy given the left upper lobe herniation to the right. He was extubated on post-operative day two. Following extubation the patient was discharged home two weeks later. Pathology revealed emphysematous changes in the left upper lobe along with acute interstitial pneumonia. Repeat imaging one year after resection demonstrated continued right middle lobe hyperinflation but the patient continues to remain stable.
2.4. Case four
3. Discussion
At two months of age, a former 37-week male infant presented to our facility with increased work of breathing and cough and was found to have rhinovirus. Chest radiograph noted right upper lobe atelectasis along with relative hyperinflation of the right middle and left upper lobes. He was diagnosed with asthma during this stay, and symptomatically treated. Unfortunately, at five months of age he returned
As previously noted, congenital lobar emphysema is a very rare disease with bilateral disease only noted in a few cases in the literature. All four patients in this study had right middle lobe and left upper lobe disease. All patients underwent preoperative planning with CTA chest allowing for a tailored treatment approach based on specific radiologic features: lung herniation across midline, mediastinal shift, and/or volumetric enlargement in comparison to the other diseased lobe. The primary rationale for flexible bronchoscopy is to evaluate for alternative causes of lung hyperinflation including vascular compression, foreign bodies, inspissated mucus, airway anatomical anomalies and endobronchial tumors.
Image 3. Case three CTA demonstrating hyperinflation of both right middle and left upper lobes.
Image 4. Case four CTA with left upper and right middle lobe hyperinflation and herniation of left upper lobe across midline.
L. Perea et al. / Journal of Pediatric Surgery 52 (2017) 1442–1445
However, the addition of selective endobronchial occlusion techniques allows further physiologic assessment regarding the potential impact of lobectomy when the etiology of hyperinflation is unclear because of complex respiratory comorbidities. Two patients underwent unilateral right middle lobectomy whereas one patient underwent a unilateral left upper lobectomy, and all are growing normally and breathing room air one year later. All three patients continue to undergo routine follow up with the pulmonary department and remain stable and have not required further intervention for the remaining diseased lobe to this point. The last patient underwent a staged procedure, a left upper lobectomy followed by a right middle lobectomy when he exhibited rebound hyperexpansion of the right middle lobe following the initial surgery. Thoracoscopy was excluded because of mass effect on all patients. None of the patients required emergent lobectomies. Previous case reports evaluated reports of patients with bilateral disease who mostly underwent bilateral lobectomies via thoracotomy either in a single procedure or a staged fashion [4]. Given the evidence that 75% of our small series of patients did not require a subsequent lobectomy, it may be an indication that a staged approach is more favorable than single stage not only in regard to postoperative pain, but more so to see if further surgery is even warranted. One patient, the child in case two, was found to have PIG in the setting of CLE. PIG is a form of interstitial lung disease (ILD) in infants. To our knowledge this is the only reported case of PIG with bilateral CLE.
1445
Hilvers et al., reported a child with a left upper lobe CLE and PIG, the first reported case connecting the two processes in the literature [5]. The pathophysiology and etiology of PIG is poorly understood. Further research may be warranted to see if a true association exists between CLE and PIG. 4. Conclusion This case series supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow-up, and comports with a “the least intervention that is the most effective” philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. References [1] Olutoye OO, Coleman BG, Hubbard AM, et al. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg 2000;35(5):792–5. [2] Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994;41: 453–72. [3] Adzick NS. Management of fetal lung lesions. Clin Perinatol 2009;36:363–76. [4] Maiya S, Clarke JR, More B, et al. Bilateral congenital lobar emphysema: how should we proceed? Pediatr Surg Int 2005;21:659–61. [5] Hilvers P, Polak M, Bacaj P, et al. Coexistence of congenital lobar emphysema and pulmonary interstitial glycogenosis in a neonate. Am J Respir Crit Care Med 2010;181:A6737.