94: Surgery for Bronchiectasis

Since the first description by Laennec in 1819, bronchiectasis has continued to be recognized as a considerable cause of respiratory illness worldwide. It is defined by the permanent dilatation of the bronchi¹ and is caused by a recurrent process of transmural infection and inflammation. Patients suffer from chronic cough, excessive sputum production, a progressive decline in respiratory function despite prolonged antibiotic treatment, and occasionally life-threatening hemoptysis once the disease is entrenched. The majority of patients with this ailment can be treated medically, but those who fail or become intolerant of medical therapy are eligible for surgical intervention.

Early attempts at surgical treatment of bronchiectasis were fraught with complications. Postoperative bronchopleural fistula (BPF) and empyema were common, and perioperative mortality rates were high. The introduction of effective antibiotics in the 1950s paired with improvements in surgical technique led to a decline in perioperative morbidity and mortality. Currently, surgical intervention is mainly reserved for patients with focal disease and persistent symptoms despite optimal medical management. The diffuse form of the disease is best treated with bilateral lung transplantation and will not be discussed extensively in this chapter.

Bronchiectasis is subdivided into specific types based on the pathologic or radiographic appearance of the airways (Fig. 94-1). Cylindrical or tubular bronchiectasis is defined by dilated, slightly tapered airways and often is seen in patients with tuberculosis infections. Varicose bronchiectasis resembles the chronic venous state of the same name, with areas of dilatation and narrowing. Saccular or cystic bronchiectasis is characterized by progressive dilatation of the airways which end in sac-like cystic structures that resemble a cluster of grapes. This subtype is more common after obstruction or bacterial infection. Regardless of the subtype, thick mucoid secretions often are seen pooled in the dilated airways causing a chronic, transmural inflammatory state involving the wall of the airway. Lung parenchyma distal to the dilated, ectatic airways is often damaged as well, with fibrosis and emphysematous changes present. The accompanying bronchial circulation and lymph nodes may be engorged and hypertrophied. The left lower lobe is most commonly affected (Fig. 94-2), followed by the lingula and right middle lobe (Fig. 94-3). Occasionally, the process may progress to multilobe involvement with cavitation and parenchymal destruction, requiring pneumonectomy (Fig. 94-4).

The etiology of bronchiectasis can be divided into obstructive and nonobstructive types (Table 94-1). Obstruction of the airways due to foreign body inhalation is most common in children and shows a predilection for the right lower lobe and posterior segment of the right upper lobe. Both intrinsic narrowing and extrinsic compression of the airway by tumors can lead to bronchiectasis as well. The right middle lobe bronchus is at particular risk because of its relatively narrow lumen. Recurrent mucous plugging can lead to bronchiectasis and is most commonly associated with allergic bronchopulmonary aspergillosis (ABPA).

Infectious agents, both viral and bacterial, make up the most common etiology for the development of bronchiectasis. Children who suffer from measles, adenovirus, or pertussis are at risk of developing bronchiectasis later in life. Viral infections can lead to bronchiectatic airways both through direct infection and through a reduction in host defenses. Bacterial infections, particularly those involving potentially necrotizing agents such as Staphylococcus aureus, Pseudomonas aeruginosa, Streptococcus pneumoniae, and various anaerobes, remain important causes of bronchiectasis, particularly when there is a delay in treatment or other factors that prevent eradication of the infection.

Bronchiectasis in patients with ABPA is caused by an immune reaction to the fungal organism, with production of inflammatory mediators and subsequent direct airway invasion by the fungus. Tuberculosis remains an important cause worldwide, but now is less common in North America. Nontuberculous mycobacterial (NTM) infections represent an important cause of bronchiectasis that is currently on the rise in the United States as well as throughout the world. Primary ciliary dyskinesia and various immune deficiencies such as hypogammaglobulinemia are examples of congenital disorders that lead to bronchiectasis through impairment in host defense mechanisms. Cystic fibrosis is an important cause of bronchiectasis, and it can show a predilection for the upper lobes. Occasionally, the development of bronchiectasis in middle age represents the presenting sign in patients with milder forms of cystic fibrosis. Several autoimmune disorders, such as rheumatoid arthritis and inflammatory bowel disease, have been linked to the presence of recurrent pulmonary infections and the development of bronchiectasis.

For the purposes of surgical planning, it is also important to distinguish the etiologies that lead to focal and diffuse forms of bronchiectasis. The focal variety is often associated with an isolated abnormality causing relative or complete bronchial obstruction. An aspirated foreign body, a slowly growing tumor, and broncholith are examples. Rarely, bronchial compression (as seen with middle lobe syndrome) or angulation of the bronchus (after surgical lobectomy) produces obstruction leading to recurrent infection and the development of localized disease. The process of infection, bronchial inflammation and dilatation, and parenchymal scarring tends to be self-renewing once established, leading to further damage. Bronchiectasis arising from post-infectious causes is also more likely to be localized, whereas disease owing to congenital deficiencies is more likely to be diffuse.

Therapy for bronchiectasis involves treatment of the underlying disorder (if possible), suppression of the bacterial load through appropriate use of antibiotics, encouragement of proper pulmonary hygiene (including the routine use of bronchodilators, mucolytic agents, and postural drainage), and surgery in selected patients. The role of surgery is threefold. First, patients with focal areas of disease that cause unremitting symptoms, associated with localized lung parenchymal destruction, are candidates for resection therapy, usually by means of segmentectomy or lobectomy. Second, the rare patient who presents with massive hemoptysis should be considered for surgical therapy even if less invasive temporizing maneuvers such as bronchial artery embolization are successful. Finally, some patients with end-stage bronchiectasis may be candidates for lung transplantation. As with other patients with end-stage suppurative lung disease, sequential double-lung transplantation is indicated to avoid contamination of the new lung grafts.

Several features or characteristics make a patient with focal bronchiectasis an ideal candidate for resectional therapy. First, the disease should be truly localized and amenable to anatomic lung resection. Nonanatomic or wedge resections should be avoided because they leave behind more proximal dilated bronchi with pooled secretions that, over time, serve to further contaminate adjacent lung parenchyma. Second, adequate pulmonary reserve for the planned resection should be present. This is usually not a major issue because the heavily diseased lung segments tend to contribute little to the patient's overall lung function. Finally, it is preferable to minimize the bacterial load present within the bronchi and lung tissue at the time of surgery with appropriate antimicrobial therapy, often initiated months in advance. This is particularly true in the setting of mycobacterial disease to minimize the risk of subsequent complications such as BPF.

Patients with bronchiectasis present with recurrent pulmonary infections characterized by dyspnea and an unremitting chronic cough productive of thick, tenacious, purulent sputum. Hemoptysis is common and on rare occasion can be massive when there is erosion into the enlarged bronchial vessels. Occasionally, patients with bronchiectasis will describe a nonproductive cough, indicative of upper lobe involvement. Auscultation reveals crackles, wheezes, or rhonchi in most patients.

The radiographic findings in bronchiectasis are understandably important in establishing the diagnosis. Standard radiographs are abnormal in most cases, demonstrating focal areas of consolidation, atelectasis, evidence of thickened bronchi (best noted as ring shadows when seen on end), and in advanced cases, delineation of the dilated cystic changes in the airway. Bronchography using contrast medium, once the standard for establishing the diagnosis of bronchiectasis, has been replaced by computed tomography (CT) scanning as the diagnostic procedure of choice. Evidence of airway dilatation, changes consistent with saccules or varicosities of the airway, and lack of airway tapering toward the periphery are all consistent with bronchiectasis. Evidence of cavitary disease also may be seen. The severity of bronchial wall thickening on CT scan has been linked to the degree of lung impairment and subsequent functional decline.^2,3 Upper lobe involvement suggests the diagnosis of cystic fibrosis or ABPA. Middle lobe and lingular disease is more typical of nontuberculous (environmental) mycobacterial infection such as Mycobacterial avium complex, and lower lobe predominance suggests bacterial involvement.

As mentioned earlier, initiation of targeted antimicrobial therapy before the planned surgical date is crucial to the success of the procedure. This is particularly true in the setting of mycobacterial disease, such as Mycobacterium tuberculosis and the various nontuberculous (previously termed “atypical”, “MOTT”, or “environmental”) mycobacterial species now more common in the United States. For example, patients at our institution with focal bronchiectasis and Mycobacterium avium complex infection are typically started on a three- or four-drug regimen for 2 to 3 months before surgery based on in vitro susceptibility testing of the isolated organism. The regimen is continued through the hospital stay and for several months thereafter, often to a total of 24 months.⁴ The preoperative antibiotic treatment, of course, will vary considerably depending on the offending organisms; routine bacterial pathogens do not require the preoperative treatment duration typical in mycobacterial disease. It is important in most cases to reimage the patient before surgery because effective antimicrobial therapy may improve areas of parenchymal and cavitary disease, particularly in those with normal (noncystic fibrosis) genotypes.⁵

Most patients with chronic suppurative disease of the lungs are malnourished, often to a considerable degree, as a result of the long-standing catabolic state these patients experience. If malnutrition is present, an aggressive preoperative regimen of nutritional supplementation is advised. In many cases, nutritional augmentation may require use of a nasojejunal feeding tube or a percutaneous gastrostomy. In our experience, preoperative improvement in nutritional status in these debilitated patients may lessen the morbidity of the subsequent procedure.⁶

At our institution, all patients being considered for surgical management of bronchiectasis or other infectious disease are discussed at a weekly multidisciplinary conference. The conference is attended by surgeons, pulmonologists, and infectious disease physicians with specialization in respiratory infectious disease. Similar to a multidisciplinary approach common in thoracic oncology, this helps ensure that patients receive the appropriate antimicrobial therapy and helps delineate the optimal timing of surgical intervention. Allowing time for antibiotic therapy to produce a bacterial nadir at the time of surgery minimizes the risk profile in the perioperative period.

Anesthesia

A standard anesthetic technique typical for thoracic surgical procedures is used. Single-lung ventilation is achieved with placement of a double-lumen tube. A thoracic epidural catheter is employed when an open (thoracotomy) approach is planned. In patients in whom a thoracoscopic lobectomy or segmentectomy is performed, the epidural is usually omitted. An arterial line and urinary catheter are placed. Fluid administration is limited, as with other forms of major lung resection. Extubation at the end of the operation is planned.

Bronchoscopy

Preoperative bronchoscopic examination of the airway is essential. Bronchial obstruction owing to tumor or an aspirated foreign body should be ruled out. If severe inflammation of the airway mucosa is found, it may be best to defer definitive resection until better infection control is obtained. Finally, normal variations in bronchial anatomy make preoperative bronchoscopy by the surgeon advisable, particularly if a segmental resection is planned.

Surgical Approach

Our approach to surgical resection for bronchiectasis has been previously reported.^6–8 Traditionally, anatomic lung resection for bronchiectasis (e.g., segmentectomy, lobectomy, pneumonectomy) has been performed via open thoracotomy. The acceptance of a video-assisted thoracoscopic (VATS) approach has been delayed by the impression that dense pleural adhesions, perihilar lymphadenopathy, and a hypertrophic bronchial circulation would make VATS resection unnecessarily challenging and potentially unsafe. However, in the last decade there have been several reports of successful resection using such an approach. We recommend a standard VATS technique utilizing two 10-mm ports and a 3- to 4-cm utility incision based over a rib space in the anterior axillary line (Fig. 94-5). No rib spreading is used. Typically, the initial two ports are placed—one in the seventh intercostal space, anterior axillary line, and the other just posterior to the scapular tip—and the safety and feasibility of a VATS approach are confirmed. The utility incision then can be made, centered over an interspace (usually fourth or fifth) to allow easy access to the area of dissection. The serratus muscle fibers are separated along the axis of the utility incision. The exact placement of the ports and the utility incision depends in part on the planned resection. Muscle transposition (latissimus dorsi, intercostal) is feasible using a thoracoscopic approach.

For patients in whom extensive extrapleural dissection is needed, an open approach is used. A lateral thoracotomy affords excellent exposure to all planned resections, with the possible exception of completion pneumonectomy, where a posterolateral incision is preferred. If possible, the latissimus dorsi muscle should be spared for possible transposition later, if needed.

Extent of Resection

After access is achieved, the anatomic resection is completed in a standard fashion, with individual ligation of the vessels and bronchus to the target lobe or segment. The resection technique is the same whether the approach is VATS or open, although stapling devices are usually mandatory when a VATS approach is used. Situations in which there is concern regarding the bronchial closure, particularly pneumonectomy, may suggest the need for an open approach to facilitate a tailored suture closure of the bronchus and tissue transposition. The diseased segment or lobe often has considerable pleural adhesions that are lysed with cautery, taking care to avoid the usually adjacent phrenic nerve (Fig. 94-6). There is usually considerable bronchial artery hypertrophy and lymphadenopathy surrounding the involved pulmonary hilum, consistent with the chronic infectious state. It is important to achieve complete resection of the diseased bronchi and associated lung tissue, if possible. We prefer in these patients to divide the lung parenchyma (e.g., along the fissures), erring on the side of the uninvolved lobe and thus ensuring complete removal of diseased tissue. In VATS cases, the soft tissues at the utility incision should be retracted and protected from contamination, and the diseased tissue removed with the use of a deployable bag or similar device. As with resections for lung cancer, this latter technique is imperative; certain NTM organisms such as Mycobacterium abscessus can cause devastating chest wall infections if a careless method is adopted for removal of the specimen.

Once the specimen is removed from the field, it is divided on the back table for appropriate cultures to guide subsequent antimicrobial therapy. We typically “double culture” specimens at two different laboratories to minimize sampling or contamination error. The intrathoracic space typically is drained with one or two 28 F thoracostomy tubes.

Use of Tissue Flaps

Tissue transposition is indicated if there is risk of potential breakdown of the bronchial stump or when a significant intrathoracic “space” is present after resection. Postoperative BPFs are more common in the setting of certain poorly controlled infections, such as multidrug-resistant M. tuberculosis, or after certain resections, such as right pneumonectomy. We favor use of either a latissimus dorsi or intercostal muscle flap for bronchial stump coverage in routine circumstances and use of omentum after pneumonectomy, particularly after a right-sided resection.⁹ Use of the serratus anterior muscle is often problematic because of winged scapula-related problems of wound healing and skin necrosis in these chronically malnourished patients.

Mobilization of the latissimus dorsi muscle is completed at the initiation of the procedure, and the muscle is transposed into the chest through the second or third intercostal space after resection. The omentum is mobilized before thoracotomy via laparoscopy or a limited midline abdominal incision, based on the right gastroepiploic artery and vein, and tacked to the undersurface of the appropriate hemidiaphragm for retrieval later after lung resection.

The presence of a significant intrathoracic “space” appears to be more common after major lung resection for infectious lung disease such as bronchiectasis compared with other indications for surgery. The use of transposed muscle such as latissimus dorsi minimizes the potential complications in this setting, including postresection empyema or prolonged air leak.

With few exceptions, patient management after lung resection for bronchiectasis is routine. Appropriate antimicrobial coverage is continued in the postoperative period and often for several months afterwards, as described earlier, depending on the isolated organisms. Special emphasis is placed on pulmonary toilet, chest physiotherapy, early postoperative mobilization, and nutritional supplementation. Postoperative bronchoscopy may be needed to aid secretion clearance. Chest tube management is typical of other indications for lung resection. Most patients after VATS resection are ready for discharge by the second or third postoperative day, whereas patients undergoing more extensive resections by means of open thoracotomy may require hospitalization for 5 to 7 days.

The complications that accompany lung resection for bronchiectasis mirror those that follow similar resections for other indications with a few exceptions. Morbidity following resection ranges from 9% to 20% (Table 94-2). BPF and space problems are more common after resection for bronchiectasis and are discussed in further detail below.

Bronchopleural Fistula

In our practice, development of BPF after segmental or lobar resection is rare. It remains a considerable source of morbidity after pneumonectomy, particularly after right or completion pneumonectomy or in the setting of persistently smear-positive patients with organisms such as multidrug-resistant M. tuberculosis. In these patients, prevention is the key: Appropriate antimicrobial coverage before surgery, a tailored hand-sewn bronchial closure, and use of muscle or omental coverage of the bronchial stump may minimize this disastrous complication.

When a BPF occurs, it will present in a manner similar to that seen after lung resection for other indications. Fever, cough productive of serous followed by purulent sputum, contralateral lung infiltrates, and a dropping air–fluid level on chest radiograph are typical findings. Drainage of the infected space is a key initial step to limit damage to the remaining lung. BPFs noted very early after the initial resection may be treated with primary reclosure and rebuttressing of the stump; later BPFs usually require rib resection and creation of an Eloesser flap, followed by BPF closure (usually with omental transposition, if not used previously) and subsequent Clagett procedure, for successful treatment (see Chapter 82).

Space Problems and Empyema

As mentioned previously, space problems are somewhat more common after lung resection for infectious lung disease than after resection for lung carcinoma. This is due to the relative inability of the remaining lung to fully expand to minimize the residual space, perhaps because of chronic granulomatous changes in the other lobes. In most cases, the space is well tolerated and does not cause problems if the residual lung is fully expanded. However, in difficult resections in which significant pleural soilage or parenchymal injury is observed, the space may lead to a prolonged air leak or, worse, an empyema. Again, prevention is the key: significant space problems should be anticipated, with avoidance of complications through careful dissection technique and liberal use of autologous tissue flaps.

Several large series of open resectional therapy for bronchiectasis have been published in the last decade.^10–14 They report encouraging results, with operative mortality rates of 0% to 1.7% and morbidity rates of 9% to 20% (Table 94-2). The most common reported complications include atelectasis requiring therapeutic bronchoscopy, prolonged air leak, cardiac arrhythmias, space problems, empyema, and BPF. Completion pneumonectomy remains a formidable operation for bronchiectasis with reported mortality as high as 23%.⁹ Failure of medical therapy (recurrent infections despite repeated courses of antibiotics) is the most commonly reported indication for surgical intervention, with only a small percentage of patients presenting with significant hemoptysis or lung abscess. A minority of patients have bilateral disease. All authors stressed the importance of localized disease, early surgical referral and intervention, and complete resection as the keys to successful surgical therapy.

Multiple studies have demonstrated improved safety and shorter hospital stays with a thoracoscopic (VATS) approach to lobectomy and segmentectomy in oncology patients.^15–19 Three series in the last decade^8,20,21 have specifically analyzed the outcomes of patients who undergo a VATS resection for bronchiectasis. Weber et al.²⁰ reported a five trocar method of performing thoracoscopic lobectomy in 76 patients with benign lung disease. The majority of these patients either had bronchiectasis or chronic lung infection alone. They reported a mortality rate of 0%, morbidity rate of 18.7%, and conversion to open procedure in 15.3%. Conversion was mainly due to dense pleural adhesive disease or upper lobe-predominant disease. Compared to a cohort of patients who underwent planned open lobectomy during the same time period, those who underwent a VATS resection suffered fewer post-operative complications, had less blood loss, and had a shorter hospital stay. Zhang et al.²¹ reported 52 patients who underwent VATS lobectomy using two ports and a utility incision. Their results were similar with no mortality, a morbidity of 15.4%, and a conversion rate of 13.5%. Pain was assessed in these patients with an 11 point scale and was significantly lower than that was accessed in a cohort of patients undergoing open lobectomy in the same timeframe. Finally, we have recently reported the experience in VATS resection for bronchiectasis.⁸ It included 212 resections in 171 patients, with a mortality rate of 0%, a complication rate of 9%, a conversion rate of 4.7%, and a mean hospital stay of 3.7 days. The results of the above reports clearly indicate that pulmonary resection for bronchiectasis can feasibly be carried out with a VATS approach with negligible mortality, lower morbidity, shorter hospital stay, and less pain when compared to open thoracotomy.

Surgical treatment of bronchiectasis is usually successful and can be accomplished with minimal morbidity or mortality. Keys to successful surgical intervention are (1) presence of localized disease, (2) relatively early intervention to minimize development of resistant organisms and involvement of dependent lung segments, (3) successful identification of the predominant organisms involved, with targeted antimicrobial therapy based on in vitro sensitivities initiated prior to surgery and continued postoperatively, (4) assessment of preoperative nutritional status and nutritional supplementation where indicated, (5) complete resection of the involved segments of lung containing bronchiectatic airway, and (6) anticipation of potential complications and alteration of the operative plan to minimize subsequent morbidity.

A 62-year-old woman with a long history of pulmonary infections, bronchiectasis, and mycobacterial superinfection was referred for further evaluation and treatment. Previous sputum cultures had yielded M. avium complex. At the time of her initial M. avium complex diagnosis, she had been treated with a three-drug regimen (i.e., clarithromycin, ethambutol, and rifampin) for 18 months. At the time of presentation, she complained of chronic fatigue and dyspnea on exertion and a variably productive cough. Born in New York, she resided for the past decade in Florida. She was a nonsmoker, had no pets, and did not have either a hot tub or a pool. Her family history was notable for a sister also with bronchiectasis and M. avium complex infection, who died of respiratory complications the year before.

A positive sputum smear was obtained at presentation, and cultures grew pan sensitive M. avium complex. Thin-cut CT scan of the chest demonstrated parenchymal destruction and coarse bronchiectasis of the right middle lobe, with minor involvement of the dependent right lower lobe superior segment (Fig. 94-7A to C). Her pulmonary function tests revealed an FEV₁ of 77% and an FVC of 86% of predicted. Although the patient was thin, initial studies did not suggest malnutrition.

The patient was placed on a three-drug outpatient regimen based on in vitro drug sensitivities, in addition to intravenous amikacin 8 weeks before surgery. She underwent an uncomplicated VATS right middle lobectomy, with discharge on the third postoperative day. Her antibiotics were continued through the hospitalization. The surgical specimen demonstrated severe bronchiectasis and chronic granulomatous inflammation consistent with her history of NTM disease. The intravenous antibiotic (amikacin) was stopped following the surgery, and her oral antimicrobial coverage was adjusted based on intraoperative cultures obtained at surgery. Antibiotic coverage was continued for an additional 12 months once she was noted to be sputum culture negative. She remains alive and well and is essentially asymptomatic with respect to her pulmonary disease.

This is an excellent review on the etiology, management, and indications for surgical intervention in bronchiectasis. The authors have clearly outlined the role of limited anatomic surgical resection in unremitting localized disease or massive hemoptysis and potential double-lung transplantation for end-stage disease. The detailed description of the surgical approach and common pitfalls highlights that this chapter is written by surgeons for surgeons.