Lung volume reduction surgery (LVRS) can be performed in patients with severe emphysematous disease. However, LVRS in pediatric patients has not yet been reported. Here, we report our experience with 2 cases of pediatric LVRS. The first patient was a preterm infant girl with severe bronchopulmonary dysplasia, pulmonary hypertension, and hypothyroidism. The emphysematous portion of the right lung was removed via sternotomy and right hemiclamshell incision. The patient was discharged on full-time home ventilator support for 3 months after the surgery. Since then, her respiratory function has improved continuously. She no longer needs oxygen supplementation or ventilator care. Her T-cannula was removed recently. The second patient was also a preterm infant girl with bronchopulmonary dysplasia. She was born with pulmonary hypertension and multiple congenital anomalies, including an atrial septal defect. Despite receiving the best supportive care, she could not be taken off the mechanical ventilator because of severe hypercapnia. We performed LVRS on the right lung via thoracotomy. She was successfully weaned off the mechanical ventilator 1 month after the surgery. She was discharged without severe complications at 3 months after the operation. At present, she is growing well with the help of intermittent home ventilator support. She can now tolerate an oral diet. Our experience shows that LVRS can be considered as a treatment option for pediatric patients with severe emphysematous lung. It is especially helpful for discontinuing prolonged mechanical ventilator care for patients with respiratory failure.
- BPD —
- bronchopulmonary dysplasia
- CT —
- computed tomography
- LVRS —
- lung volume reduction surgery
Lung volume reduction surgery (LVRS) was reintroduced as a treatment of end-stage emphysema in the mid-1990s.1 LVRS can improve exercise tolerance, lung function, and quality of life in a select group of patients with emphysematous lung.2–4 However, there have been no reports in the literature on LVRS for pediatric patients. Here, we report the cases of 2 infants who underwent LVRS to treat respiratory failure because of severe emphysema accompanied by bronchopulmonary dysplasia (BPD). Our experience suggests that LVRS is an effective treatment modality for children with severe emphysematous lung.
The first patient was a preterm infant girl born at 27 weeks’ gestation with a body weight of 800 g. She had severe BPD, pulmonary hypertension, and hypothyroidism. Prolonged ventilator care was necessary. Intensive medication, including nitric oxide gas, an iloprost nebulizer, sildenafil, and Bosentan were used to control her severe pulmonary hypertension. After 5 months of mechanical ventilator management, multiple air trapping in the right lung was identified on chest radiograph and computed tomography (CT) (Fig 1 A, C, and D). Several attempts at weaning the patient off the ventilator failed because of severe hypercapnia and hypoxia. Her emphysema progressively worsened. To reverse the negative remodeling of the lung parenchyma and restore respiratory mechanics, LVRS was requested by her pediatricians as a rescue treatment. We performed LVRS by sternotomy and combined right hemiclamshell incision through the fifth intercostal space when the patient was 18 months old. Emphysematous changes were observed in the entirety of the right lung. The upper and lower lobes were severely affected. We performed right-upper lobectomy and wide wedge resection of the emphysematous lesion in the right-lower lobe. The resected lung parenchyma was reinforced with double-layer buttressed Teflon felt (DuPont, Wilmington, DE). The patient’s respiratory function improved gradually and persistently, and her pulmonary hypertension reached approximately normal levels after the operation. The patient was discharged with home ventilator support for 3 months after the surgery. Finally, the patient was weaned from the ventilator at postoperative 8 months. Now, she is growing well and has no signs of respiratory distress at 2 years after the operation (Fig 1B).
The second patient was a preterm infant born at 25 weeks’ gestation with a low birth weight (810 g). The patient had severe BPD, pulmonary hypertension, and an atrial septal defect. Mechanical ventilator care was necessary because of severe hypoxia. Extensive fibrotic changes and multiple atelectasis in both lungs, a decreased volume of the apical segment of the right-upper lobe and the lateral segment of the right-middle lobe, and hyperinflation of the right-lower lung field were identified on chest radiograph and CT. Sildenafil, Bosentan, nitric oxide gas, and an iloprost nebulizer were used for 3 months. However, the patient could not be weaned from the mechanical ventilator. We performed LVRS in the 12th month after birth via thoracotomy through the seventh intercostal space. Lung resection was performed at the anterior segment of the right-upper lobe and the superior segment and lateral basal segment of the right-lower lobe. The resected lung parenchyma was buttressed with double-layer Teflon felt as in the previous case. The patient’s respiratory function improved gradually with acceptable blood-gas exchange (Fig 2). The patient was successfully weaned from the mechanical ventilator on the 26th postoperative day. At present, the patient is growing well and shows no signs of respiratory distress at 1 year after the operation and tolerates intermittent home ventilator support. Although the disease severity has decreased, the patient still has a moderate degree of pulmonary hypertension.
According to the pathologic findings of this series, in case patients who would benefit from LVRS, the alveolar spaces are overinflated and the interstitium is mildly thickened, with some smooth muscle formation. There is neither bronchial nor bronchiolar chronic inflammation, nor is there bronchial obstruction by inflammatory exudate, which are differences from adult cases of emphysema produced by chronic obstructive pulmonary disease (Fig 3).
In this case series, we performed LVRS in infants with BPD-related, chronic ventilator dependency. The clinical status of those patients did not improve despite maximal medical treatment. LVRS successfully restored respiratory mechanics in both patients, and they were both eventually weaned from mechanical ventilation. It has been reported that LVRS can improve respiratory function in adults.5–9 However, it has not been reported in pediatric patients. This is the first report on successful LVRS in infants with respiratory failure.
The etiology of emphysematous disease differs between adults and children. In this series, both patients suffered from BPD and were placed on long-term ventilator care. Subsequent emphysematous changes developed owing to ventilator-induced damage. Therefore, effective respiratory mechanics could not be maintained because of hyperinflated lungs. LVRS improves lung function by increasing airway conductance and the ratio of conductance to lung volume, possibly by increasing the elastic recoil of lung tissue.10 Pediatric patients have much less exposure to smoking, dust, and other pollutants in the air and also have greater growth potential of pulmonary elasticity than adults. In addition, there is new evidence for lung repair in preterm infants. Human alveolarization might continue throughout childhood and adolescence and could contribute to alveolar repair and regeneration of the lungs.11–14 These aspects of lung tissue may be key advantages for LVRS in pediatric patients.
In this series, we performed unilateral LVRS, whereas bilateral lung resection is usually done in adults because of the generalized inflammatory nature of emphysematous disease, which is commonly bilateral in adults. Because a worsening of emphysema was mainly induced by long-term ventilator dependency in this series, we decided to resect only the severely deteriorated side of the lungs. Also, this strategy may be helpful for reducing the risk of persistent air leakage and preventing unnecessary complications.
In this study, we reported successful LVRS in pediatric patients with respiratory failure because of severe emphysematous changes. LVRS was performed without major complications and eventually improved respiratory function in both patients. LVRS is a promising treatment option for emphysematous lung disease in children with long-term ventilator dependency despite the availability of other forms of medical treatment.
- Accepted June 8, 2017.
- Address correspondence to Chang Hyun Kang, MD, Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea. E-mail:
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
FUNDING: No external funding.
POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
- Naunheim KS,
- Wood DE,
- Mohsenifar Z, et al; National Emphysema Treatment Trial Research Group
- Dunnill MS
- Copyright © 2018 by the American Academy of Pediatrics