Alveolo-pleural fistula (APF)—an abnormal connection between the subsegmental pulmonary parenchyma and the pleura—is a common condition encountered in the pediatric intensive care unit (PICU) and can result from necrotic pneumonia, thoracic surgery, or lung parenchymal abnormalities. APF is associated with persistent air leak and pneumothorax, often resulting in prolonged hospitalization for the affected patients. Standard therapy for APF includes evacuation of the pneumothorax using chest tube, coupled with prolonged use of water seal, intermittent suction, or Heimlich valves to minimize reaccumulation of pleural air until the APF resolves spontaneously.1,2 For some patients, surgical procedures may be required if the resolution is not achieved by the chest tube evacuation alone.3–6
Closure of persistent air leaks using flexible bronchoscopy has historically been reserved for patients who are poor surgical candidates or those for whom surgical approaches have failed.
Bronchoscopic treatment, including instillation of fibrin glue or similar agents into affected lung segments, has demonstrated some success but can be technically challenging.7–11 More recently, the placement of unidirectional intrabronchial valves using flexible bronchoscopy has shown promise as an alternative and less invasive treatment for persistent air leaks.12–14 Herein, we present use of intrabronchial valves in an adolescent patient with Pneumocystis jirovecii pneumonia and APF.
A 21-year-old male patient with congenitally acquired human immunodeficiency virus (HIV) infection and acquired immune deficiency syndrome (AIDS) presented with a pneumonia picture. He had been noncompliant with his HIV medications, and his T-helper cell percentage on admission was 4% (reference range, 33%-65%). Sputum studies did not reveal any specific organism, but he was treated for presumed P. jirovecii pneumonia with atovaquone because of a prior allergic reaction to trimethoprim-sulfamethoxazole. Flexible bronchoscopy with lavage subsequently detected P. jirovecii, after which he underwent a desensitization protocol for his allergy. He completed 1 month of intravenous trimethoprim-sulfamethoxazole and systemic steroid therapy but was left with severe pulmonary impairment, necessitating tracheostomy for chronic ventilator support. Computed tomography (CT) scan of the chest demonstrated multiple cystic lesions in his right and, to a lesser extent, left upper lobes (Fig. 1). Approximately 2 months after the initial presentation, he was weaned to supplemental oxygen using tracheostomy collar during the day and continuous positive end expiratory pressure (CPAP) during sleep, at which time he was discharged to a rehabilitation facility.
Several days after the discharge, he represented with a large right-sided pneumothorax. A chest tube was placed in the emergency room resulting in evacuation of the pneumothorax.
However, a water seal trial 10 days later resulted in reappearance of the pneumothorax and significant respiratory distress (Fig. 2A). Because of the persistent pneumothorax and the multicystic appearance of the right upper lobe, and as he was not a surgical candidate because of his poor clinical condition, a decision was made to insert 1-way endobronchial valves (product HU-VS-5, 6 or 7; Spiration Inc., Redmond, WA) to limit ventilation to the affected area and facilitate healing. Flexible bronchoscopy was performed, and the right upper lobe segmental bronchi were measured using a balloon catheter (product B5-2C; Spiration Inc.) and airway sizing kit (product HU-AS-U; Spiration Inc.). Five-mm-valves were placed in the apical and posterior segments, and a 7 mm valve was placed in the anterior segment. Resolution of the pneumothorax was noted over several days. Early radiographs demonstrated a paucity of air in the right upper lobe and only a small residual pneumothorax, despite accidental dislodgement of the chest tube within 24 hours of the bronchoscopy and an ongoing requirement of nighttime CPAP (Fig. 2B). Subsequent radiographs demonstrated no residual pneumothorax and improved aeration of the right upper lobe (Fig. 2C). CPAP was discontinued approximately 3 weeks following valve placement, and the patient was discharged from the hospital to an inpatient rehabilitation facility. The valves were left in place for approximately 6 weeks, after which they were removed with the flexible bronchoscope using standard biopsy forceps. The pneumothorax did not return, and the patient experienced no adverse events related to the valves.
Intrabronchial unidirectional valves—typically utilized in severe emphysema as a means to prevent ventilation to hyper-inflated lobes that may compromise adjacent normal lung—offer a less invasive and reversible therapeutic option to the volume reduction surgery in select patients.15,16 However, valves are also increasingly utilized in patients with persistent air leaks.17,18 In our institution, placement of intrabronchial valves for persistent air leaks is approved under the institutional review board protocol (HSM#11-00015). Informed consent was obtained from our patient before the procedure.
Intrabronchial valves promote resolution of persistent air leaks presumably by limiting ventilation to the damaged segment of the lung parenchyma or airway, thus allowing the defect to heal spontaneously. Because strategic placement of valves can limit aeration to the entire lobe, these devices may be particularly useful for the leaks resulting from severe infections that cause damage to the large portions of lung parenchyma. Indeed, our patient had significant disease encompassing his entire right upper lobe, and placement of the valves into each of the 3 lobar segments successfully minimized aeration to the entire area, as demonstrated in early radiographs. In comparison, more localized abnormalities (ie, ruptured blebs, congenital cysts, bronchial stump dehiscence following lobectomy) may be more amenable to specific surgical interventions, although valves may represent important adjunctive therapy for these abnormalities as well. Our patient developed APF from P. jirovecii pneumonia, a severe opportunistic infection, which has been associated with cystic lung disease and pneumothorax, particularly in patients with AIDS.6,19,20 Because our patient was deemed to be a poor surgical candidate given his severe muscle wasting, poor immune function, and chronic dependence on mechanical ventilation, it was decided that placement of intrabronchial valves was the most reasonable option. However, we considered the potential risk of subsequent pulmonary infection secondary to the presence of foreign devices in the airway. Fortunately, he did not experience any complications during the 6-week period between placement and removal of the valves.
Chest tube evacuation of intrathoracic air using water seal or intermittent low wall suction remains the standard of care for APF. However, in some patients who have particularly severe disease or an ongoing requirement for positive pressure ventilation (including our patient), resolution of an air leak using chest tube drainage alone could be challenging and can take weeks or even months.21,22 In addition, such patients typically have limited mobility and require long-term hospitalization until the chest tube can be removed. Importantly, our patient developed several complications during his prolonged hospitalization secondary to suboptimal nutrition and poor mobility (including muscle wasting and decubitus ulcer formation). As such, subsequent management plans included transfer to a long-term rehabilitation facility, which could not be initiated until removal of the chest tube. One might speculate that earlier placement of the valves might have minimized these complications and hastened his eventual recovery.
To our knowledge, we are the first to report successful use of intrabronchial unidirectional valves for the treatment of APF in a patient with P. jirovecii pneumonia.
Intrabronchial valves could be considered for similar patients with complicated pulmonary infections and persistent air leaks.
1. Vricella LA, Trachiotis GD. Heimlich valve in the management of pneumothorax
in patients with advanced AIDS. Chest. 2001;120:15–18.
2. Cerfolio RJ. Recent advances in the treatment of air leaks. Curr Opin Pulmon Med. 2005;11:319–323.
3. Takaoka K, Inoue S, Ohira S. Central bronchopleural fistulas closed by bronchoscopic injection of absolute ethanol. Chest. 2002;122:374–378.
4. Woo E, Tan BK, Lim CH. Treatment of recalcitrant air leaks: the combined latissimus dorsi-serratus anterior flap. Ann Plast Surg. 2009;63:188–192.
5. Sakamaki Y, Kido T, Fujiwara T, et al.. A novel procedure using a tissue expander for management of persistent alveolar fistula after lobectomy. Ann Thorac Surg. 2005;79:2130–2132.
6. Crawford BK, Galloway AC, Boyd AD, et al.. Treatment of AIDS-related bronchopleural fistula
by pleurectomy. Ann Thorac Surg. 1992;54:212–214.
7. York EL, Lewall DB, Hirji M, et al.. Endoscopic diagnosis and treatment of postoperative bronchopleural fistula
. Chest. 1990;97:1390–1392.
8. Wood RE, Lacey SR, Azizkhan RG. Endoscopic management of large, postresection bronchopleural fistulae with methacrylate adhesive (Super Glue). J Pediatr Surg. 1992;27:201–202.
9. Varoli F, Roviaro G, Grignani F, et al.. Endoscopic treatment of bronchopleural fistulas. Ann Thorac Surg. 1998;65:807–809.
10. Shah AM, Singhal P, Chhajed PN, et al.. Bronchoscopic closure of bronchopleural fistula
using gelfoam. J Assoc Phys India. 2004;52:508–509.
11. Chang CC, Hsu HH, Kuo SW, et al.. Bronchoscopic gluing for post-lung-transplant bronchopleural fistula
. Eur JCardiothorac Surg. 2007;31:328–330.
12. Abu-Hijleh M, Blundin M. Emergency use of an endobronchial one-way valve in the management of severe air leak and massive subcutaneous emphysema. Lung. 2010;188:253–257.
13. Rosell A, Lopez-Lisbona R, Cubero N, et al.. Endoscopic treatment of persistent alveolar-pleural air leaks with a unidirectional endobronchial valve. Archivos de bronconeumologia. 2011;47:371–373.
14. Brichon PY, Poquet C, Arvieux C, et al.. Successful treatment of a life-threatening air leakage, complicating severe abdominal sepsis, with a one-way endobronchial valve. Interact Cardiovasc Thorac Surg. 2012;15:779–780.
15. Berger RL, Decamp MM, Criner GJ, et al.. Lung volume reduction therapies for advanced emphysema: an update. Chest. 2010;138:407–417.
16. Wood DE, McKenna RJ Jr, Yusen RD, et al.. A multicenter trial of an intrabronchial valve for treatment of severe emphysema. J Thorac Cardiovasc Surg. 2007;133:65–73.
17. Gillespie CT, Sterman DH, Cerfolio RJ, et al.. Endobronchial valve treatment for prolonged air leaks of the lung: a case series. Ann Thorac Surg. 2011;91:270–273.
18. El-Sameed Y, Waness A, Al Shamsi I, et al.. Endobronchial valves in the management of broncho-pleural and alveolo-pleural fistulae. Lung. 2012;190:347–351.
19. Byrnes TA, Brevig JK, Yeoh CB. Pneumothorax
in patients with acquired immunodeficiency syndrome. J Thorac Cardiovasc Surg. 1989;98:546–550.
20. Horowitz MD, Oliva H. Pneumothorax
in AIDS patients: operative management. Am Surg. 1993;59:200–204.
21. Downs JB, Chapman RL Jr. Treatment of bronchopleural fistula
during continuous positive pressure ventilation. Chest. 1976;69:363–366.
22. Filosso PL, Giobbe R, Brussino L, et al.. Patient’s home care management in persistent air leaks and chronic pneumothorax
using a new drainage system. J Cardiovasc Surg. 2010;51:773–775.