Physical Examination, Complementary Tests, and Functional Status Assessment

Dyspnea, recurrent pneumonia, and inability to raise secretions are nonspecific but common findings in patients with expiratory central airway collapse.^1,2 Symptoms are usually refractory to conventional treatments such as corticosteroids and bronchodilators for suspected asthma and COPD.³ Occasionally, hypercarbic respiratory failure can occur. Once intubated, patients may be difficult to wean from mechanical ventilation.^4,5 In nonintubated patients, spirometry often shows obstructive ventilatory impairment that is proportionate to the severity of the disease.⁶ The expiratory flow-volume loop (FVL) often reveals an airway collapse pattern* (Figure 14-2). Expiratory airway collapse is also seen during dynamic bronchoscopy,^†7 and in fact has been reported in up to 40% of patients with severe COPD.^7,8

Figure 14-2 A, This flow-volume loop shows a sudden decrease in peak expiratory flow, defined as a 50% drop within 10% of forced vital capacity. This airway collapse pattern is commonly seen in patients with severe chronic obstructive pulmonary disease (COPD). B, The corresponding bronchoscopic image during inspiration shows normal tracheal lumen. C, During expiration, evidence of excessive dynamic airway collapse is found (the flow-volume loop and the bronchoscopic images are from the same patient).

In our patient, a diagnosis of diffuse, severe expiratory central airway collapse was made bronchoscopically and on paired dynamic inspiratory-expiratory CT scanning. This was characterized by weakness of the posterior membranous wall, resulting in excessive bulging of the posterior membrane within the airway lumen and causing 100% airway narrowing in the mainstem bronchi and lower trachea (see Figure 14-1).^2,9 No evidence of cartilaginous weakness was noted. Results from studies show that dynamic CT correlates well with bronchoscopy findings in patients with central airway collapse.¹⁰ CT scanning offers complementary information by revealing structures adjacent to the airway and by allowing quantitative measurements of the degree of airway narrowing before and after airway splinting procedures.^11,12 No evidence of extrinsic compression was observed resulting from mediastinal masses or vascular structures (e.g., double aortic arch, aortic aneurysm); also, on expiratory images, no evidence of air trapping and mosaic attenuation suggested bronchiolitis. The patient’s severe limitation of physical activity at rest was consistent with functional impairment class IV based on World Health Organization (WHO) criteria.¹³ Other dyspnea or quality of life (QOL) instruments (St. George’s Respiratory Questionnaire [SGRQ], American Thoracic Society Dyspnea Scale [ATS], Baseline Dyspnea Index [BDI]/Transitional Dyspnea Index [TDI], Karnofsky Performance Scale [KPS], and 6-Minute Walk Test [6MWT]) have been studied in these patients to objectively determine response to interventions.¹⁴

Comorbidities

This patient had no evidence of systolic or diastolic dysfunction on echocardiogram, but evidence revealed moderate pulmonary hypertension, which could result in hemodynamic instability during general anesthesia.¹⁵ She suffered from obesity and GERD, both of which increase the risk for aspiration of gastric contents during general anesthesia,¹⁶ which might be required for any form of bronchoscopic or surgical treatment of EDAC. Additionally, OSA raised concerns regarding difficult mask ventilation, difficult tracheal intubation, hypoxemia, postprocedure airway obstruction, cardiac arrhythmias, and myocardial infarction. The presence of both obesity and hypoventilation perioperatively has been shown to be associated with worse outcomes and higher mortality.¹⁷

Support System

The patient was a resident of a nursing home. Published literature focusing on patient safety in the nursing home environment states that clinical assessments are frequently absent or unsatisfactory at critical points during a nursing home resident’s stay (to ensure prevention and timely treatment of potentially dangerous conditions).¹⁸ Pertinent to this patient’s case is her history of recurrent pneumonias and refractory COPD, both of which should have alerted physicians to a potential alternative diagnosis (i.e., central airway obstruction).

Establishing a differential diagnosis when therapeutic outcomes are not in line with expectations is essential to ensure early diagnosis and treatment for a variety of disorders, including EDAC. In the setting of a long-term care facility, this requires not only an emphasis on resident safety and uniformity of health care delivery, but also consideration of resident-directed, consumer-driven health promotion and quality of life. In this regard, workforce redesign and considerations for resident-centered care are key elements of culture change to ensure a person’s well-being in these institutions.¹⁹

Patient Preferences and Expectations

This patient was motivated to improve her symptoms so that she could increase her mobility and potentially leave the nursing home. She clearly expressed her dissatisfaction with her current care environment, a willingness to consider all available treatment options, including airway stent insertion or open surgical resection if necessary, which might help her regain independent living conditions.

Indications

Treatment of severe diffuse EDAC includes minimally invasive or open surgical procedures.^1,2 Some central airway narrowing during expiration is normal, however, and it has been suggested that airway splinting procedures should be considered only when excessive narrowing results in symptomatic airflow limitation.²⁰ From both anatomic and physiologic perspectives, the boundary between normal and abnormal narrowing of the central airways is far from being established, and the exact degree of expiratory airway narrowing responsible for symptoms and requiring intervention remains unknown. For example, even an 80% reduction in tracheal lumen cross-section at the end of a “forced” expiration, when the flow in central airways physiologically nears zero, may well be within normal limits.* Some authors propose 30%²¹ and others 40%,²² but most investigators use 50% or greater reduction in airway caliber between inspiration and expiration to identify abnormal central airway collapse on dynamic CT or bronchoscopy.^23–25 Upon application of these criteria when a patient is asked to cough or perform a forced expiration, false positives may be noted; in one study, investigators reported that nearly 80% of normal people exceeded the diagnostic criteria of 50% or greater obstruction in the upper and/or lower trachea.²⁶

Patients with concurrent COPD have reduced elastic recoil and peripheral airway obstruction. The expiratory airway collapse seen in the central airways thus represents normal physiology,^20,27,28 because during flow-limited breathing, the central airways become severely compressed, particularly during forced expiration and cough. Both of these maneuvers are routinely used to detect this entity on dynamic imaging studies. The Starling resistor model^† shows that a pressure drop occurs across a very short length of airway, and that proximal airway resistance, downstream from the choke point, mouthward, should not affect airflow. Pressure catheter measurements demonstrate the flow-limiting choke point and lack of further pressure drop in airways between the mouth and the flow-limiting segment.²⁹ Because the choke point in adult humans is often located at the level of the lobar bronchi, and is even more peripheral in patients with COPD,³⁰ central airway collapsibility should not impede airflow.^20,29

Patients with COPD and EDAC, however, may still improve their symptoms and functional status after central airway stabilization with stents or tracheoplasty despite lack of improvement in forced expiratory volume in 1 second (FEV₁).³¹ By stabilizing and reducing central airway flow turbulence, in addition to improving secretion management and preventing excessive cough from chronic airway inflammation, these procedures might result in improved quality of life and dyspnea improvement with exertion caused by a reduction in hyperinflation.^14,31 Our patient had severe (100%) collapse resulting in dyspnea at rest, inability to raise secretions, and recurrent pneumonia. These symptoms were not responding to optimal COPD treatment and CPAP for OSA. For these reasons, after taking into account the patient’s wishes, we elected to perform rigid bronchoscopy under general anesthesia to place an indwelling silicone stent that would splint open the lower trachea and mainstem bronchi. Our goal was to improve airway lumen patency to less than 50% collapse during exhalation, which, by convention, is currently considered within normal limits by most investigators.¹³

Contraindications

No absolute contraindications to rigid bronchoscopy were noted. Evidence of pulmonary artery hypertension was likely due to her COPD and OSA, which could increase the risk of right ventricular dysfunction and hemodynamic instability during general anesthesia.

Expected Results

In the short term (up to 10 to 14 days), airway stabilization with silicone stents in patients with expiratory central airway collapse (malacia and EDAC) improves symptoms, quality of life, and functional status.^4,14 In one study, quality of life and functional status scores improved in 70% of patients and dyspnea scores improved in 91% of patients after stent insertion.¹⁴ Stent-related complications included obstruction from mucus plugging and migration, and almost 10% of patients (5 of 52 patients) had complications related to the bronchoscopic procedure itself. Because the dynamic features of expiratory central airway collapse continuously alter the shape of the central airways, as well as the surface contact between a stent and the airway wall, stent-related complications may occur more frequently in dynamic forms of airway obstruction than in fixed benign obstruction or malignancy.⁴ In one study of 57 patients, 21 partial stent obstructions, 14 infections, and 10 stent migrations were noted.¹⁴ Although not life threatening, these stent-related adverse events required multiple repeat bronchoscopies.

Team Experience

As of this writing, silicone stent insertion requires rigid bronchoscopy. The technique of atraumatic rigid intubation, single stent insertion, or Y stent insertion using the “push” or “pullback” technique* is one that is gradually perfected with practice and experience.

Risk-Benefit Analysis

The rare potential risks of airway injury during rigid bronchoscopy, hemodynamic instability during general anesthesia in the setting of pulmonary hypertension, and postoperative complications from OSA were considered to be outweighed by the potential benefits of improved airway patency, diminished shortness of breath, restored ability to clear secretions, and an enhanced quality of life.

Therapeutic Alternatives for Restoring Airway Patency

Other than silicone stent insertion, therapeutic alternatives for EDAC include noninvasive positive-pressure ventilation, metal stent insertion, and open surgical treatment.