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INTRODUCTION

Tracheobronchial disease represents a wide variety of pathologies and presents significant diagnostic and therapeutic challenges to practitioners. In part, this is due to the insidious onset of nonspecific clinical symptoms, which commonly lead to delays in definitive diagnosis and appropriate treatment. Additional challenges include the rarity of presentation, diverse pathological findings, and frequency of associated comorbidities. Also owing to the rarity of presentation, treatment algorithms are often nonstandardized and include surgical and interventional as well as systemic modalities. As a result, appropriate diagnosis and management necessitates expertise across multiple subspecialities. While the anatomy of the trachea itself is not complex, its relationship to surrounding structures in the neck and mediastinum mandates expert surgical knowledge and technique to optimize mobility and create a tension-free anastomosis when resection is indicated (Fig. 53-1).

ETIOLOGY

Primary tumors affecting the upper airways are rare, with a reported incidence of 0.14 per 100,000 people per year.1 In adults, the majority of primary tracheal tumors are malignant, whereas benign tumors are more common in children.26 Malignant epithelial tumors, including squamous cell carcinoma (SCC) and adenoid cystic carcinoma (ACC), are the most common primary tracheal malignancies, followed by carcinoid and mucoepidermoid carcinomas.79

Benign tumors of the trachea represent less than 2% of all lung tumors and consist of a wide spectrum of tumor histologies. These include chondromas, hamartomas, squamous papillomas, hemangiomas, glomus tumors, neurogenic tumors, lipomas, and granular cell tumors.4,9,10 These tumors are slow growing and often clinically and radiographically indistinguishable from malignant disease.11,12 Symptoms are usually related to airway obstruction and frequently misdiagnosed as chronic obstructive pulmonary disease (COPD) or asthma. Benign tumors frequently arise from the posterior membrane and most commonly occur in the distal trachea in adults, compared to the upper third of the trachea in children.

Although the true incidence is unknown, the majority of tracheal tumors are likely secondary. This includes either hematogenous or lymphatic metastasis, as well as direct invasion from adjacent structures (i.e., lung, esophagus, thyroid, and mediastinal lymph nodes). Prognosis is usually related to the stage and natural history of the primary disease. In a review of 160 case reports and case series of secondary tracheal tumors, 2242 patients were identified and 78% and 66% of these patients, respectively, underwent segmental surgical resection of the trachea.13

Non-neoplastic disorders of the trachea comprise mainly inflammatory and infectious etiologies, as well as postinterventional (intubation or tracheostomy) tracheal stenosis and tracheomalacia.14,15 The management of these patients represents a unique challenge owing to the frequency and severity of associated comorbidities. In postintubation/tracheostomy patients, the mechanism of injury is related to cuff overinflation, which leads to pressure necrosis, ...

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