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Key Concepts

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  • Epidemiology

    • Primary tracheal tumors are rare, with an estimated 2.7 new cases diagnosed per million persons per year. The many remaining tracheal pathologies comprise heterogeneous epidemiologies.

  • Pathophysiology

    • Congenital tracheal stenosis may present as a web-like diaphragm, most often at the subcricoid level. Primary tracheal tumors are largely (about two-thirds) made up of adenoid cystic and squamous cell carcinomas with roughly equal histologic frequency. Other tumors include pleomorphic adenomas, leiomyomas, chondromas, carcinoid tumors, mucoepidermoid tumors, and sarcomas. The laryngotracheal junction is the most frequent location of injury in airway trauma. Most tracheoesophageal fistulas are complications from mechanical ventilation.

  • Clinical features

    • Patients with tracheal pathology usually present with signs and symptoms of upper airway obstruction: dyspnea on exertion, wheezing, stridor, and obstructive pneumonia. Tracheal tumors most commonly present with cough, hemoptysis, and signs of progressive airway obstruction, although signs and symptoms vary with tumor histology. Tracheoesophageal fistulas (TEFs) typically manifest as a sudden increase in tracheal secretions and ingested material in the trachea.

  • Diagnostics

    • Anteroposterior and lateral tomography is useful to define upper tracheal pathology. Fluoroscopy demonstrates functional vocal cord asymmetry and other details (e.g., airway collapse with malacia). Computed tomography (CT) is helpful in malignant disease to assess extramural extent and lymphadenopathy. Inspiratory/expiratory CT is the easiest, most accurate noninvasive method to diagnose tracheomalacia. Flexible and rigid bronchoscopy is critical in the definitive assessment of the trachea.

  • Treatment

    • Tracheal resection can be performed using a cervical or transthoracic approach, depending on the location and the extent of the lesion. Tracheal release maneuvers are primarily intended to prevent excessive tension on the anastomosis and avoid the need for excessive tracheal dissection.

    • Laryngotracheal injuries should be treated with airway establishment (e.g., tracheostomy). Traumatic lacerations of the lower trachea can be approached via partial or full sternotomy or right posterolateral thoracotomy.

    • Congenital tracheal stenoses may be treated with anterior tracheoplasty and patch repair or slide tracheoplasty.

    • Most TEFs require tracheal resection. Anastomotic complications are reduced if definitive single-stage correction of the fistula is delayed until after the patient has been successfully weaned from mechanical ventilation. Fistula control can be achieved by placing a tracheostomy with the balloon placed below the fistula, draining gastrostomy, and feeding jejunostomy.

    • Idiopathic laryngotracheal stenoses have been treated with complex, multistaged or single-stage procedures involving partial resection of the subglottic larynx and immediate plastic reconstruction.

  • Outcomes/prognosis

    • At the Massachusetts General Hospital, operative mortality for primary tracheal tumor resection has improved from 21 percent in the 1960s to 3 percent in the last decade. The overall 1-, 5-, and 10-year survival rates for primary tracheal carcinoma have been reported to be 84, 45, and 25 percent respectively. All tracheal surgery is subject to anastomotic complications, including suture line granulomas, stenosis, and tracheal separation. Predictors of anastomotic complications include reoperation, diabetes, lengthy resections, laryngotracheal resections, age 17 years or less, and preoperative tracheostomy. Anastomotic complications are associated with a 7.4-percent mortality, compared with 0.06 percent in patients who did not experience anastomotic complications.

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