Continuing Education Activity

The trapped lung is defined as the inability of the lung to expand and fill the thoracic cavity because of a fibrinous restrictive pleural layer that prevents normal visceral and parietal pleural apposition. It is caused by remote inflammation of the pleura and typically presents as chronic stable pleural effusion. To avoid the high morbidity associated with multiple invasive diagnostic procedures, it must be promptly diagnosed and treated. This activity reviews the etiology, evaluation, and treatment of trapped lung and highlights the role of interprofessional diagnosis, timely evaluation, and management.

Objectives:

• Outline the concept of the non-expandable lung.
• Describe the evaluation of the patient the trapped lung.
• Explain the treatment and management options available for trapped lung.

Introduction

Non-expandable lung (NEL) is a term used to describe entities that are characterized by the inability of the lung to expand into the chest cavity or respective pleural space. There are three distinct causes of NEL: a) An endobronchial lesion obstructing the lumen of the bronchus leading to the distal lobar collapse, b) chronic atelectasis, and c) visceral pleural restriction due to pleural disease resulting in the trapped lung. The trapped lung, first described in 1967, is a clinical entity that is characterized by the presence of a restrictive visceral pleura.[1] 

Trapped lung occurs as a mature fibrous strip encircles the visceral pleura restricting the lung expansion, which develops from inflammatory sequelae. The usual primary causes of pleural inflammation are pneumonia or hemothorax, but other causes like pneumothorax, thoracic surgical interventions, uremia, autoimmune diseases like rheumatoid pleuritis are also well established.[2][3][4]

In clinical practice, a patient with trapped lung commonly presents with chronic pleural effusion. Repeated thoracenteses, without lung re-expansion post-procedure, can result in adverse events.

Etiology

Historically, pneumothorax therapy, used to treat tuberculosis, had caused trapped lung.[2] The most common etiologies include immunologic diseases such as rheumatoid pleurisy, inflammatory pleuritis, improperly drained hemothorax, parapneumonic effusions, and infections like bacterial empyema, tuberculous pleurisy, malignant pleural effusions, asbestos exposure, uremic pleurisy, post-cardiac surgery, chest radiation, and medications.[5][6]

Epidemiology

It is unclear why some patients with pleural inflammation heal without any sequela while others develop a restrictive fibrous visceral peel leading to the trapped lung. The exact incidence of trapped lung is poorly documented, but it is expected to be higher than is generally recognized. A study by Dresler et al suggests that among patients who undergo thoracentesis, approximately 10% have non-expandable lung, 20% of patients who undergo therapeutic thoracentesis and 30% of those who undergo therapeutic thoracentesis for malignant pleural effusion have non-expandable lung.[7]

Pathophysiology

A restricting peel prevents normal parietal and visceral pleural layer apposition in a trapped lung. The formation of this fibrous restrictive peel requires the presence of a long-standing stable pleuro-pulmonary disease, either from an inflamed lung or a malignant visceral pleural tumor.[3] This fibrotic restrictive visceral pleura prevents the lung from expanding when pleural fluid is removed during thoracocenteses. Negative pleural pressure within the pleural space increases the entry of fluid into pleural space and reduces pleural fluid exit through pleural lymphatics thus leading to the formation of a chronic pleural effusion. So, a steady state of fluid formation and removal is established, leading to persistent negative pressure pleural effusion of constant volume. This persistence of the pleural effusion is mainly mechanical and can be considered a "pleural effusion ex-vacuo".[2][4]

History and Physical

Symptoms vary depending upon the size of the pleural effusion. Patients can be asymptomatic with small effusions and are noticed incidentally on imaging studies like a chest x-ray and CT chest or abdomen. Clinical exam shows ipsilateral: decreased breath sounds, stony dull percussion, and decreased fremitus. When the effusion is significant enough, it can cause respiratory distress and warrant immediate relief through thoracentesis or even a surgical approach.

Patients experience discomfort during the thoracentesis owing to further reductions in intrathoracic pressure. Lung entrapment, on the other hand, is an active inflammatory process presenting as chest pain and severe dyspnea. Trapped lung poses a significant risk for inadvertent multiple diagnostic, and therapeutic procedures attempted for chronic stable pleural effusion, leading to further complications. Hence, trapped lung should be considered early in the differential diagnosis of any case of chronic stable pleural effusion when no clinically apparent active disease is found.[2][5]

Evaluation

The pleural fluid analysis in a trapped lung is consistent with a transudate or a protein-discordant exudate and has a paucicellular mononuclear cell predominance. Pleural fluid LDH is low, and protein may be in the exudative range. The elevated total pleural fluid protein may be related to factors other than active pleural inflammation or malignancy and does not exclude the diagnosis. The characteristic diagnostic clue is that the pleural effusion reaccumulates rapidly after thoracocenteses and the effusion volume due to trapped lung remains remarkably constant over time.

A high index of suspicion should be maintained in the diagnosis and management of trapped lungs, particularly in the setting of malignant pleural effusions so as to prevent complications from repeated thoracenteses. Repeated thoracenteses can result in various complications like chest pain, the formation of loculations, bleeding, development of hemothorax, and also recurrent effusions. Trapped lung should be suspected in any patient who presents with a stable chronic pleural effusion, particularly if there is an antecedent history of pneumonia, pneumothorax, thoracic surgery, or hemothorax.[8][9][10]

Pleural Manometry 

The trapped lung is can be diagnosed via pleural manometry during thoracentesis. As it is time-consuming and requires technical expertise, currently manometry is not routinely used during thoracentesis. With large-volume thoracentesis, pleural elastance changes throughout the procedure. It is used as a predictor of successful pleurodesis, by measuring the absolute closing pressure and overall elastance. When the elastance is high, the probability of the pleural layers being pulled apart is increased, which interferes with pleurodesis. In the setting of trapped lung, despite the presence of a pleural effusion, the pleural pressure is low, and it drops significantly with the removal of fluid. This high pleural elastance (change in pressure/change in volume) is a hallmark of trapped lung. The presence of these characteristic pleural pressure changes is highly suggestive of a trapped lung. It may aid in the diagnosis of patients with minimal or absent symptoms. However, confirmation with direct visualization via video-assisted thoracoscopy or air contrast CT showing visceral pleural thickening (a pleural rind) is required.

Imaging Studies

Upright and decubitus radiographs and chest computed tomography (CT) often show pleural thickening and loculations. Pleural fluid may shift a bit with postural changes but will not be free-flowing. The paradoxical finding that the hemithorax with pleural effusion is reduced in size when compared to the contralateral side is suggestive of significantly higher negative pleural pressure on the affected side than on the opposite side, provides further evidence for the diagnosis of trapped lung. Post-thoracentesis radiographs may show air in the pleural space with the same size and shape as the prior effusion.[10]

Ultrasound

Point-of-care ultrasound (POCUS)is a rapid, bedside, cost-effective way to diagnose pleural pathologies and must be performed before any pleural intervention. With the patient positioned upright or supine, a low-frequency, 2- to 5-MHz transducer probe is placed in a longitudinal orientation. With the probe marker held cranially from either mid-axillary or posterior mid-scapular views, images are viewed in brightness mode and motion mode. In motion mode, the respiratory movement of an atelectatic lung occurs in a sinusoidal pattern within the surrounding pleural fluid. This represents a “sinusoid sign" which is suggestive of the expandable lung. The absence of this sinusoidal respirophasic lung is known as an “absent sinusoid sign". It suggests an unexpandable lung and coincides with pleural manometry findings.[11] Apart from this, thoracic ultrasound is useful to differentiate between malignant and benign diseases, pleural thickening, nodularity, and lung consolidation with or without effusion.[12]

Treatment / Management

There are various treatment options for trapped Lung, and the patient's symptoms determine the choice of treatment modality. Observation is warranted in an asymptomatic patient if the effusion is not worsening.[4] If the patient has dyspnea, symptom alleviation can be achieved through fluid removal via thoracentesis.[13] In patients with a poor prognosis and a shorter expected survival period, repeated thoracentesis or a pleural catheter is advised as the least invasive best approach to provide immediate symptom relief. Complications include rapid re-accumulation requiring multiple visits, infection leading to empyema, worsening pneumothorax, hemothorax, and bleeding.[14][15]

Implantable Pleural Catheter

Pleurodesis is considered an effective therapeutic choice for patients with malignant, recurrent pleural effusion. However, pleurodesis is ineffective and not the management of choice in cases of trapped lungs.[16] In these patients, a chronic indwelling pleural catheter can be considered for symptomatic relief. This is a small-bore catheter 66 cm long, 15 F silicone rubber catheter with fenestrations along the distal 24 cm, placed within the pleural space. It aids in partial lung expansion and alleviates symptoms, as dyspnea in these patients is predominantly due to pleural space tension relative to chest wall and diaphragm rather than parenchymal lung pathology or lung collapse.[17][15][18]

Pleuroperitoneal Shunt

The pleuroperitoneal shunt, first proposed in 1982 by Weese and Schouten, can provide effective and safe palliation in select patients. It can transport up to 1.5 L of pleural fluid into the abdominal cavity with each compression. Complications include failure of shunt and infection and are not routinely recommended or preferred.[19][20][21]

Surgical Decortication 

When a more definitive approach is indicated, surgical decortication is the only curative treatment modality.[4] It involves removing a fibrous visceral pleural layer covering the lung in an attempt to allow lung expansion.[22] Pleura can now oppose against the chest wall, and this relieves restrictive ventilatory defect, thus having a positive impact on hypoxia and ventilation-perfusion mismatch. It also reduces chest wall pain and discomfort and dyspnea and prevents recurrent pleural effusions. It is important to exclude other diseases and underlying cardiopulmonary conditions before attempting surgical decortication. Visceral pleurectomy can be performed by either open thoracotomy or the most preferred minimally invasive approach, Video-assisted thoracoscopic surgery (VATS).[23][24] VATS is a minimally invasive surgical technique used to diagnose and treat pleuro-parenchymal diseases. A tiny camera (thoracoscope) and surgical instruments are inserted into your chest through one or more small incisions in the chest wall during a VATS procedure.

Intrapleural Fibrinolytic Therapy

In select cases, fibrinolytic drugs such as tPA, urokinase, and streptokinase can be introduced into the pleural space to break down fibrin strands and adhesions and promote drainage and expansion of the lung. These can be used as palliative care options in inoperable scenarios.[25]

All of these treatment options are, in essence, palliative. Re-expansion of the lung even after the removal of this peel is difficult when the lung has been trapped for a long period of time.[4]

Differential Diagnosis

It is important to differentiate the trapped lung from lung entrapment so as to identify active processes that require specific treatment and to avoid unnecessary diagnostic and therapeutic pleural procedures in patients with trapped lung. Lung entrapment may result from either pleural or non-pleural causes while the trapped lung is caused only by pleural causes. Non-pleural causes that cause lung entrapment include conditions that increase elastic recoil pressure of the lung. They include endobronchial obstruction due to bronchogenic carcinoma or interstitial diseases like lymphangitic carcinomatosis[26] 

The pleural fluid analysis is usually consistent with exudate in lung entrapment while patients with trapped lung have a transudate or a protein-discordant exudate with a mononuclear cell predominance. Furthermore, these entities can be differentiated by the particular patterns of pleural elastance. Unlike trapped lung, pleural manometry in lung entrapment shows normal elastance during initial pleural fluid removal, but the lung doesn't expand fully as more fluid is removed and the slope of the elastance curve increases. While most cases of lung entrapment resolve with resolution of the inflammatory process, the resolution is incomplete in a trapped lung. Therefore, a trapped lung and lung entrapment can represent a continuum of the same process.

Other differentials include pneumothorax ex-vacuo (particularly right upper lobe collapse), cryptogenic organizing pneumonia, chronic pulmonary consolidations, and bronchiolitis obliterans organizing pneumonia.[27]

Prognosis

The trapped lung is an independent risk factor for short survival compared to patients without non-expandable lung in mesothelioma patients.[28] Bibby and colleagues found that in a cohort of 229 patients, 33% of patients with Malignant Pleural Mesothelioma have a non-expandable lung. Perhaps most importantly, they have found for the first time that non-expandable lung is an independent predictor of poor survival.[29] It is unclear as to why it has an effect on the survival rate, and further studies are warranted.

Complications

Undiagnosed trapped lung typically presents as chronic stable pleural effusion, and these patients might undergo multiple thoracocenteses without any significant improvement in respiratory symptoms. Repeated thoracenteses can lead to many adverse events related to the procedure, delay in diagnosis and adequate treatment, improper use of resources, worsening of the underlying disease, and above all, significant patient discomfort.[11]

Deterrence and Patient Education

The main risk in trapped lung patients is multiple diagnostic and therapeutic procedures for a persistent pleural effusion. This can be prevented by clinical awareness of this condition. The patient should be counseled regarding the benign nature of trapped lung once underlying malignancy is excluded and the irreversible outcome of this pleural abnormality. Further diagnostic or therapeutic interventions should be avoided unless the radiographical appearance changes and patients should be educated to reduce the risk of undergoing an ill-advised intervention in the future.

Pearls and Other Issues

Here are important points to take note of:

• Knowledge of trapped lung is crucial for physicians as many of these patients are subjected to repeated thoracentesis, managed with chest tubes, resulting in prolonged hospitalization.
• Trapped lung should be suspected in any patient with a chronic stable pleural effusion, particularly if there is a history of pneumonia, pneumothorax, hemothorax, thoracic surgery, or malignancy.
• Ultrasound is a rapid and cost-effective way of diagnosing pleural effusion. 
• Once the diagnosis of a trapped lung is made, the need for pleural drainage must be assessed based on the clinical circumstances on a case-by-case basis.[11] 
• High pleural space elastance (change in pressure/change in volume) exceeding 14.5 cm HO/L is the hallmark of a trapped lung.[5]
• Decortication of visceral pleura by VATS might allow reexpansion of the trapped lung to some degree and aid in the resolution of the trapped lung.[22][30]

Enhancing Healthcare Team Outcomes

The outcomes of a trapped lung depend on the underlying cause. To improve outcomes, prompt consultation with a pulmonologist, thoracic surgeon, and/or an intervention radiologist is highly recommended. Clinicians should have a high index of suspicion for trapped lung when they come across a case of chronic stable pleural effusion with no apparent diagnosis. Radiologists play a vital role in identifying the presence of thickened rind of visceral pleura on imaging studies. Patient education is extremely important and an interdisciplinary discussion must be held with the patient and their caregivers for better outcomes. In patients with underlying malignancy oncologists and surgical pathologists must be involved in the care of these patients.[3]