Tracheal Diseases

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1. Anatomy
A. The trachea
· Average length is 11 cm (range 10-13 cm) and shortens with age
· 18-22 rings (about 2 rings per cm)
· The cricoid cartilage is the only complete ring
· Average diameter is 2.3 cm laterally and 1.8 cm anteroposteriorly
· In infants, the anterioposterior diameter is greater
· COPD will increase the AP diameter ("saber sheath")

B. Anatomic relationships:
· Anterior - thyroid isthmus, innominate artery, aortic arch
· Posterior - esophagus
· Lateral - azygous, pleura, recurrent laryngeal nerves
· Inferior - anterior and posterior subcarinal lymph nodes

C. Arterial supply:
· Three branches of the inferior thyroid artery on either side of the upper trachea
· Subclavian, supreme intercostal, internal thoracic, innominate, superior and middle bronchial arteries all contribute to varying degrees
· Lateral longitudinal vessel anastomoses feed transverse vessels, which penetrate between cartilage rings to supply submucosa
· Mobilize only 1-2 cm circumferentially to avoid ischemic necrosis
· Mobilize anteriorly and posteriorly to avoid significant vessels

2. Congenital Tracheal Anomalies
A. Agenesis or atresia
· Usually fatal at birth

B. Tracheoesophageal fistula
· Tracheal lesion easily closed, esophageal portion more difficult
· Rarely has associated stenosis

C. Congenital stenosis
· Webs and diaphragms most often occur at subcricoid level
· Three types of stenosis: generalized hypoplasia, funnel-like narrowing, and segmental stenosis
· Main bronchi most often normal with generalized hypoplasia, vary in size with funnel-like narrowing, and commonly have anomalies with segmental stenosis
· Associated with a wide range of anomalies throughout the body
· About 50% of pulmonary sling cases are associated with lower tracheal stenosis

D. Diagnosis of congenital stenosis
· Suspicion in infants with inspiratory and expiratory stridor or more severe respiratory distress
· Wheezing, retraction, poor feeding, failure to thrive may also be present

E. Management
· Operations in infants are high risk, as postoperative edema may obstruct small airways and mechanical ventilation may disrupt anastomoses
· A conservative approach is wise, as less than 1/3 of the trachea in a child can be resected
· Webs can be dilated or excised endoscopically
· Stenoses should not be dilated, as this can create longitudinal tears, resulting in recurrent scarring and stricture
· Tracheostomy for palliation is a suitable alternative to allow growth for surgical repair

3. Tracheal Neoplasms
A. Primary neoplasms of the trachea are rare

B. Squamous cell carcinoma
· Most common tracheal neoplasm
· Well localized exophytic or ulcerated lesion
· Multiple lesions or a superficially infiltrating variety also occur
· 1/3 of patients will present with mediastinal or pulmonary metastasis
· Spread is usually to regional tracheal lymph nodes and then directly into mediastinal structures

C. Adenoid cystic carcinoma (cylindroma)
· Grows slowly and displaces mediastinal structures rather than invading
· Submucosal and perineural spread may occur for quite long distances
· Best chance at cure is complete resection at first operation

D. Clinical Features
· Dyspnea on exertion
· Wheezing
· Stridor
· Excessive secretions
· Hemoptysis
· Pneumonia
· Cough

E. Secondary tracheal neoplasms
· Thyroid cancers are the most common indication for tracheal operations for secondary malignancy
· Laryngeal carcinoma involves the upper trachea by direct extension
· Bronchogenic carcinoma more commonly involves the carina or bronchial origin on the right
· Tracheoesophageal fistula can result from radiation therapy for or from invasion by esophageal cancer
· Other tumor include carcinoid, head and neck, breast, and lymphoma

4. Infection and Inflammation
· Tuberculosis may cause strictures of the lower trachea and bronchi
· Strictures are submucosal and the cartilage has a grossly normal caliber
· Fibrosing mediastinitis and histoplasmosis may be so extensive as to preclude reconstruction
· Idiopathic strictures most often occur in the upper trachea
· Other causes include Wegener's granulomatosis, amyloid, sarcoid, relapsing polychondritis, and tracheopathia osteoplastica

5. Trauma
A. Penetrating injuries
· Usually involve the cervical trachea
· Fresh injuries can be closed primarily

B. Blunt injuries
· Closed trauma may lacerate or sever the trachea
· A severed trachea should be re-approximated if possible
· The distal trachea should be intubated for security
· A tracheostomy tube should be inserted in the distal severed end for complex injuries and repair undertaken later
· A vertical split at carina is usually diagnosed by a pneumothorax that fails to resolve with closed suction
· Bronchoscopy, thoracotomy, and occasionally cardiopulmonary bypass are required to repair this lesion
· Traumatic TEF should be promptly repaired, as inflammation makes repair difficult when the diagnosis is delayed
· Always establish the function of the recurrent laryngeal nerves and larynx prior to surgery, if possible

6. Postintubation Injuries
· These are currently the most frequent cause of tracheal stenosis

A. Laryngeal level
· Sealing cuffs may irritate vocal cords, causing granulomas and fusion of the posterior commissure
· Mucosal erosion at the cricoid can cause subglottic stenosis
· Most lesions are reversible with time

B. Stomal level
· Principle factors include a stoma that is too large, loss of tissue from infection (rare), and pressure erosion from rigid connecting systems (most common)
· Avoid injuring the first cartilaginous ring during original tracheostomy to prevent subglottic stenosis
· Avoid low placement of the tracheostomy to prevent innominate artery erosion and supracarinal stenosis

C. Sealing cuff level
· Most common cause is direct pressure necrosis by high-pressure cuff
· This has essentially disappeared with use of low-pressure, high-volume cuffs
· Children may develop granuloma on anterior tracheal wall when ventilated without a cuff

D. Tracheomalacia
· Usually occurs in segment of trachea between stoma and the cuff
· Secretions pool here and inflammation leads to cartilage thinning

E. Tracheoesophageal fistula
· Manifested by sudden appearance of secretions in tracheobronchial tree
· Pneumonitis, pneumonia, abscess, and gastric dilitation may develop
· Methylene blue in tube feedings will appear promptly in the trachea

F. Trachoinnominate fistula
· Manifested by sudden exsanguinating hemorrhage into the tracheobronchial tree
· Emergent tamponade with high-pressure cuff or digital pressure is necessary

7. Diagnosis
· CXR
· Fluoroscopy determines laryngeal function and presence of tracheomalacia
· CT to evaluate extramural extension of tumors
· Bronchoscopy and biopsy at the time of operation

8. Resection and Reconstruction
· All patients should be induced slowly and gently in the OR
· Extubation in the OR is desired in order to avoid intubation injury to the suture line

A. Upper half of trachea
· Collar incision with partial upper sternal split, if necessary, is used for benign and malignant lesions of the upper trachea
· Almost all stenoses above the carina can be reached through this approach as well
· Dissect anteriorly from the cricoid cartilage to the carina, staying close to the trachea
· The recurrent laryngeal nerves must be identified
· Divide the trachea and intubate distally
· About 4.5 cm can be removed without additional mobilization
· Laryngeal release is preferred, if needed, taking care to avoid injury to the superior laryngeal nerves
· Hilar release is more hazardous and is contraindicated in patients with poor pulmonary function
· Once the sutures have been placed, the distal tube is removed and the endotracheal tube is readvanced past the anastomosis
· All sutures are tied and the anastomosis tested under water for leakage

B. Lower half of trachea
· Posterolateral thoracotomy in 4th interspace provides optimal exposure and should be performed on the side contralateral to the aortic arch (usually right)
· About 4.5 to 5 cm can be resected without extreme maneuvers
· Release technique include hilar dissection, loosening of the carina, and cervical flexion
· Laryngeal release does not provide any additional mobilization
· A pleural or pericardial flap is constructed to protect the anastomosis

9. Tracheostomy
· Principal indications include upper airway obstruction, management of secretions, prolonged ventilation, and emergent airway
· Second and third rings are opened vertically
· Avoid high and low stoma placement to prevent subglottic stenosis and innominate artery erosion
· Persistent stoma 3 to 6 months after tracheostomy should be closed surgically

10. Results
· Small numbers of patients make accurate predictions of survival difficult
· Resection of benign tumors and low-grade malignant tumors appears to have high probablity of cure
· Single-stage resection and reconstruction is probably the best approach for squamous cell and adenoid cystic carcinoma
· Adjunctive radiation is usually indicated for squamous cell and adenoid cystic tumors
· Selected patients can be treated effectively with resection and reconstruction for secondary tracheal tumors