A high index of suspicion is required to diagnose tracheal disorders. Wheezing and a normal chest radiograph may lead to patients being treated for many months or years for presumed adult-onset asthma before the diagnosis of an organic problem is made. Bronchoscopy plays an essential role in the diagnosis and management of tracheal disorders, but may provoke life-threatening airway obstruction as it occludes an already narrow trachea. Physicians treating patients with airway disorders must be aware of the indications and limitations of flexible bronchoscopy and rigid bronchoscopy to avoid potentially dangerous situations. Successful outcome of tracheal surgery requires selection of suitable patients and proper timing of surgery, determining the extent of surgery required, and knowing when additional procedures are needed to relieve excessive anastomotic tension. Postoperative care can be demanding because of the inability to raise secretions and the development of postoperative oedema in compromised airways.

The trachea begins at the lower border of the cricoid cartilage, at which point the uppermost tracheal cartilage is partly inset beneath the cricoid cartilage. It terminates at the point at which the lateral walls of the right and left main bronchi branch from the lower trachea. The carinal spur is useful as a more definite landmark for the termination of the trachea, since it is clearly definable bronchoscopically and radiologically. The average adult human trachea is 11 cm long: length varies, roughly in proportion to the height of the patient. There are approximately two tracheal cartilaginous rings per centimetre of trachea, which contains from 18 to 22 rings. The subglottic laryngeal airway measures 1.5 to 2.0 cm before the trachea is reached. Except for some cases of congenital stenosis with circumferential O rings of the trachea, the only completely circular cartilage in the upper airway is the cricoid cartilage, which has a broad posterior plate.

The potential for displaying the trachea in the neck is of major importance, not only from the standpoint of surgical access to the trachea, but also from the standpoint of the ease of reconstruction following the resection of any length of the trachea. In young people particularly when there is no obesity, hyperextension of the neck delivers more than 50 per cent of the trachea into the neck. In the kyphotic, aged, person, particularly if obesity is present, the cricoid cartilage may be located at the level of the sternal notch, and even the most vigorous hyperextension may fail to deliver any of the trachea into the neck. The anatomical position of the trachea changes from essentially subcutaneous at the cricoid cartilage level to a prevertebral position at the carinal level. The course is thus obliquely caudal and dorsal when an individual is standing in an erect position. In the kyphotic, aged patient, lateral projection becomes increasingly horizontal. The slight extensibility and flexibility of the trachea in youth diminishes with increasing age. Calcification of the cartilage also occurs with age and with injury.

The blood supply is of special importance in resection and reconstruction of the trachea. The upper part of the trachea is principally supplied by branches of the inferior thyroid artery. The lower portion of the trachea is supplied by branches of the bronchial artery, with contributions from the subclavian, supreme intercostal, internal thoracic, and innominate arteries. These vessels supply branches anteriorly to the trachea and posteriorly to the oesophagus, arriving at the trachea through lateral pedicles of tissue. The longitudinal anastomoses between these vessels are very fine. Transverse intercartilaginous arteries branch ultimately into a submucosal capillary network. Excessive division of the lateral tissues by circumferential dissection of the trachea can easily destroy this blood supply, with serious and sometimes disastrous effects.

The relationship of the recurrent laryngeal nerves to the trachea and the oesophagus and the point of entry of these nerves into the larynx need not be repeated here (see Section 11.2.2 84). The relationship of the trachea to the thyroid gland is also well known. The isthmus crosses the trachea at the second and third cartilaginous rings. Intimate adherence of the medial portions of both lobes of the thyroid gland to the trachea is observed at this same level laterally: it may be necessary to remove a lobe, or sometimes the entire thyroid gland, during removal of a tumour in the upper portion of the trachea. Posteriorly, the oesophagus has a common interface through areolar tissue with the membranous tracheal wall. The blood supply of the oesophagus and membranous tracheal wall are intimately linked. Anteriorly, the innominate artery courses obliquely across the anterior surface of the trachea. Below this level the aorta arches backward across the left tracheobronchial angle. Here the left recurrent laryngeal nerve arrives at its place in the tracheo-oesophageal groove.

The lymph nodes adjacent to the trachea are stations in the pathways from the lungs and mediastinum. The lymphatics of the trachea have been less well studied. Gross observations of the clinical behaviour of tumours metastatic from the trachea have been made. Metastases appear to involve the most closely adjacent groups of tracheal lymph nodes. Metastases to the nodes on the side contralateral to the primary lesion or to the carina from lower tracheal tumours are common. More remote metastases to scalene nodes or to other cervical nodes are not often seen.

The primary diagnostic techniques for tracheal abnormalities are radiological imaging and bronchoscopy. A plain chest radiograph is often considered to be normal, but closer inspection will reveal an abnormality of the tracheal air column. Relatively simple radiological techniques without the use of contrast media delineates tracheal disease. The location of the lesion, its linear extent, extratracheal involvement, and the amount of normal airway can be determined. In addition to standard views of the chest taken in various projections, centred high enough to show tracheal detail, anteroposterior filtered tracheal views of the entire airway from the larynx to the carina are required. Lateral neck views using soft-tissue techniques with the patient swallowing and the neck hyperextended to bring the trachea up above the clavicles is useful to define abnormalities in the upper trachea. Fluoroscopy not only demonstrates functional asymmetry of the vocal cords, if present, but also may give additional information about the extent of the lesion and collapse of the airway if malacia is present. Spot films usually are all that are required. In some cases, polytomography (anterior and lateral views) gives additional detail, particularly of mediastinal involvement. Barium oesophagography defines extrinsic compression or invasion of the oesophagus. CT offers little over standard radiological techniques, except to detect extratracheal disease. The role of magnetic resonance imaging is undefined. Its ability to produce sagittal and coronal views, however, has been helpful in certain cases and may give more accurate detail than do standard radiographic techniques.

Bronchoscopy is invaluable in assessing airway pathology. Bronchoscopy should be performed by the surgeon, rather than relying on the findings of others. Flexible and rigid bronchoscopy both have a role to play in the management of airway problems. However, flexible bronchoscopy may precipitate airway obstruction in patients with critical stenosis of the bronchus (<4 mm) by increasing secretions, haemorrhage, oedema, or by just temporarily occluding the airway. Emergency dilatation of the bronchus is required, or death may ensue. It is best not to examine an airway stenosis with the flexible bronchoscope at all. Proper evaluation should be performed in the operating room, with facilities available to dilate the stenosis if necessary. Endoscopy is often best performed as part of the planned surgical procedure.

Bronchoscopy is invaluable in determining the extent of airway disease and, just as importantly, the amount of uninvolved airway. Measurements should be taken and recorded. The quality of the mucosa should be assessed: marked inflammation and erythema may dictate delay of definitive surgical correction to a time when this has subsided. Tumours should be biopsied, and the extent of disease should be determined. As a general rule, the rigid bronchoscope is much more valuable than is the flexible bronchoscope in assessing airway disease.

Most reconstructive procedures on the trachea are performed under spontaneous ventilation,although assistance is given during the intrathoracic portions of these procedures if needed. Prolonged paralysis of respiration is to be avoided, since it is desirable that the patient should resume spontaneous respiration postoperatively without the need for ventilatory support. After the trachea has been divided, in either the anterior or the intrathoracic approach, a sterile, flexible endotracheal tube (Tovell) with attached connecting tubing is introduced into the distal tracheal for maintenance of ventilation. If carinal resection is required, ventilation is usually maintained through the opposite (usually the left) lung after resection of the carina. The use of such tubes is not cumbersome. The alternative of high frequency ventilation through a catheter also works well and is used in special cases. Cardiopulmonary bypass may be used for simple tracheal cases, although it is not needed, and it creates hazards in complicated cases since extensive manipulation of the lung in a heparin-treated patient can lead to intrapulmonary haemorrhage.

The ability to control the airway is crucial to the management of all problems of the trachea. Tracheal tumours and postintubation stenosis may present as emergency airway problems. Endotracheal intubation may be impossible, and even dangerous, leading to complete airway obstruction, especially in patients with high tracheal lesions. Elevation of the head of the patient, administration of cool mist and oxygen, and careful sedation may allow control of the airway in a semielective manner. This goal is best accomplished in the operating room, where an assortment of rigid bronchoscopes, dilators, biopsy forceps, and even instruments to perform emergency tracheostomy are available. Anaesthesia, as in elective tracheal operations, is best accomplished by inhalation technique. Patience is required to allow the patient to become anaesthetized adequately: induction of anaesthesia deep enough to allow rigid bronchoscopy may take as long as 20 min. Paralysing agents should not be used if airway obstruction in an apnoeic patient is to be avoided.

The initial evaluation of neoplasms should be performed with a rigid bronchoscope inserted through the vocal cords and stopping just proximal to the level of obstruction. Rigid telescopes can be used to assess the obstruction: these can be passed beyond most tumours, even those causing nearly total obstruction. Once the status of the distal airway has been assessed, the tumour can be partially removed with biopsy forceps to determine its consistency and vascularity. The tip of the rigid bronchoscope can usually be used to ‘core out’ most of the tumour. The tumour can then be grasped with biopsy forceps and removed. The bronchoscope may be passed into the distal airway for ventilation and to tamponade any bleeding. Direct application of adrenaline-soaked pledgets helps to control persistent oozing. Direct cautery (with insulated electrodes) is rarely required in these situations. Although the laser has become popular in the management of malignant strictures, it is time-consuming, costly, and rarely advantageous compared with other techniques.

Postintubation stenosis poses a slightly different problem in airway control. It may be impossible to pass a large rigid bronchoscope beyond a tough, inflammatory stricture: it may also result in tracheal rupture, or in total airway obstruction secondary to bleeding or oedema. Jackson dilators passed through rigid bronchoscopes under direct vision can be used to dilate postintubation stenoses gradually, minimizing the risk of perforation and bleeding. Administration of racemic adrenaline and steroids in the first 24 to 48 h minimizes postdilatation oedema.

Dilatation or endotracheal removal of malignant and inflammatory strictures, mechanically or by laser, is only a temporary measure: inflammatory strictures usually recur within days to weeks. However, these techniques allow time for more thorough evaluation of the patient and for surgery to be performed electively. Many patients are taking high doses of steroids at the time of presentation, having been treated for refractory asthma. Establishing an airway allows the steroid dosage to be tapered and discontinued, and surgery may then be performed without the threat of impaired healing. Dilations may have to be performed repeatedly during the period of steroid tapering.

The preceding manoeuvres may also be used at the time of elective surgery if the patient has presented with a stable airway. This plan allows assessment of the distal airway, placement of an endotracheal tube, and provision of an adequate lumen to prevent carbon dioxide accumulation early in the procedure. At the time of tracheal resection, this tube can be pulled back or removed and a sterile cuffed endotracheal tube (Tovell tube) can be inserted into the distal airway. Sterile connecting tubing is connected for ventilation. The tube can be removed when necessary, for suctioning or placement of sutures. At the conclusion of the operation, the original endotracheal tube is advanced into the distal airway and the sutures are tied. The patient should be breathing spontaneously at the end of the procedure so that extubation can be performed in the operating room. Intraoperative high-frequency ventilation has been used with equal success, and is especially useful in certain complex carinal reconstructions.

Tracheostomy may be the only way to secure control of the airway. The tracheostomy tube should always be placed through the most damaged portion of the trachea, preserving the maximal amount of normal trachea for subsequent reconstruction. Tracheostomy performed at the completion of tracheal resection should be located at least two rings away from the anastomosis, and the anastomosis should be protected with the thyroid gland or strap muscles to avoid contamination of the suture line. This practice lessens the likelihood of subsequent dehiscence or stenosis. A tracheostomy tube should never be placed through the anastomosis.

The general medical condition must be individually assessed, particularly in patients undergoing extensive intrathoracic resections and reconstructions. Patients should not be receiving steroids or require mechanical ventilation postoperatively: either of these factors could lead to serious anastomotic problems. Pulmonary function testing and quantitative ventilation perfusion scans are invaluable, allowing an accurate prediction of postoperative pulmonary function in patients undergoing complicated intrathoracic resections involving loss of pulmonary parenchyma.

Most tracheal operations can be performed without release manoeuvres: laryngeal release was required in only 27 (8.3 per cent) of 327 tracheal resections for postintubation stenosis and in only 18 (15 per cent) of 119 tracheal resections for primary and secondary tumours of the trachea.

Judicious assessment of each patient's condition is necessary to establish the safe limits of the possible extent of tracheal resection. Previous operations (including mediastinoscopy), disease process, extent of the lesion, age, and body habitus are important factors in deciding which patients are likely to require release manoeuvres. The location of the disease is also important in determining which release procedures will be of benefit. Certain manoeuvres are more effective for achieving additional length when disease is located in the cervical trachea: others are more effective for the intrathoracic trachea. A release manoeuvre is primarily performed to prevent unnecessary tension on the anastomosis and to avoid the need for excessive dissection of the trachea, which might jeopardize the lateral blood supply.

The simplest manoeuvre to minimize tension after tracheal resection is flexion of the neck and mobilization of the pretracheal plane, avoiding the lateral blood supply to the trachea. Flexion of the neck between 15 and 35° may result in downward movement of the trachea by as much as 4.5 cm, or the equivalent of seven tracheal rings. Flexion beyond this may achieve up to 1.5 cm of added length. The amount of trachea that may be removed with simple flexion varies greatly with age and physical habitus. When these simple manoeuvres fail to give sufficient length, a Montgomery suprahyoid laryngeal release is performed, generally before the anastomosis is completed. The release is performed by dividing the muscles that insert on the superior aspect of the hyoid bone. The hyoid bone is then divided just medial to the lesser cornua of the hyoid bone; an additional 1.5 cm of length can be obtained.

Flexion of the head and mobilization of the anterior surface (and posterior to a much lesser degree) of the trachea is also important for lower tracheal lesions. Even in the intrathoracic position, this will allow the trachea to devolve into the thorax. Laryngeal release is no help in gaining additional length for intrathoracic tracheal lesions much beyond the midtrachea.

The right hilum and the inferior pulmonary ligament should be mobilized. A ‘U’-shaped incision in the pericardium below the inferior pulmonary vein allows the hilar structures and the bronchus to advance. Additional length is obtained by completely incising the pericardium around the hilar vessels (Fig. 1) 1939. A posteriorly based pedicle of tissue that includes a bronchial artery and some lymphatics should be preserved whenever possible. Attempts to ‘recreate’ a carina by joining the left and right mainstem bronchi will allow little, if any, advancement of the neo-carina. Length can only be obtained by advancement of the trachea from above. Carinal resections are more commonly reconstructed by end-to-end anastomosis between the distal trachea and either the left or right mainstem and end-to-side anastomosis of the remaining mainstem bronchus.

Lesions located in the midtrachea can benefit from all of the release manoeuvres described. Laryngeal release may predispose the patient to aspiration, particularly of thin liquids, although this problem usually resolves with time. This problem is less common when the Montgomery technique of suprahyoid release is used in preference to a thyrohyoid release.

Tracheostomy is one of the oldest surgical procedures. The ideal method limits loss of tracheal substance, to minimize the possibility of subsequent stomal stenosis. More important is avoidance of excessive leverage on the tracheostomy tube which may erode the stoma. As the defect heals by cicatrization, stenosis may result.

The procedure is best performed in the operating room with a transoral endotracheal tube in place. With the patient supine, a pack 8 to 10 cm thick (3–4 inches) is placed below the shoulders to hyperextend the neck. If local anaesthesia is used (1 per cent lidocaine), care should be taken to anaesthetize all the soft tissues down to the pretracheal fascia. A transverse incision is made 1 cm below the cricoid cartilage and carried through the platysma muscle down to the strap muscles. These are separated in the midline, and the pretracheal fascia is divided vertically.

The thyroid isthmus is usually divided, unless it can be retracted easily to expose the second and third tracheal cartilages. The trachea is cleared of all adventitial tissues. The second and third tracheal rings are incised vertically, along with part of the fourth, if necessary. The oral endotracheal tube is withdrawn until the tip is just proximal to the stoma.

A spreader holds the tracheal incision open, and a tracheostomy cannula of appropriate size is inserted into the trachea. The tracheal balloon is then inflated to the point of preventing air leak, and the tracheostomy tube is tied in place. The flange of the tube can be sutured to the skin for additional security.

The technical and anatomical pitfalls of this operation include injury to anterior cervical veins, left innominate vein, innominate artery, internal jugular vein, and carotid artery, as well as creation of a pneumothorax, especially in children, and placement of the stoma too low, which may result in erosion of the innominate artery. Placement too high may result in damage to the cricoid cartilage, the cricothyroid membrane, or the subglottic larynx. Although tracheostomy is often considered a ‘minor’ procedure, to be delegated to a less experienced surgeon and done in far from ideal circumstances, it should be performed under the guidance of a competent surgeon, ideally in an operating room, or at least in a well-equipped procedure room or intensive care unit.

Strictures of the trachea may result from a number of different causes. Congenital stenoses are rare. Post-traumatic strictures, particularly where there has been tracheal separation, occur. Strictures are most commonly iatrogenic, resulting from intubation, usually for ventilatory support. Infections still cause stenosis of the trachea. A miscellaneous group of diseases that also causes strictures or, at least, tracheal obstruction, includes amyloid disease restricted to the airway and tracheopathia osteoplastica. A small number of idiopathic stenoses occur in patients with no history of previous insult or infection.

Congenital tracheal lesions are rare, but are a subject of interest. Tracheal agenesis or atresia is generally fatal, despite the embryological possibility of normal formation of larynx and lungs. Bronchial communication to the oesophagus may exist, and short-term survival can be achieved by using the oesophagus as an airway. The more conventionally seen tracheo-oesophageal fistulae have been well described and are usually more an oesophageal problem than a tracheal problem. The tracheal portion of the fistula is managed by division and repair.

Congenital tracheal stenosis may present as a web-like diaphragm, most often at the subcricoid level. More lengthy stenoses may involve the entire trachea, sometimes with normal larynx and main bronchi, or variable lengths of the trachea (Fig. 2) 1940. Funnel-like narrowing is sometimes seen, with gradual narrowing to the stenotic segment. Segmental stenosis of the lower trachea may be accompanied by bronchial anomalies, such as origin of all or part of the right upper lobe bronchus from the trachea just above the stenotic segment. Most often the cartilaginous rings are complete circular rings in the area of the stenosis (Fig. 2) 1940. However, the cartilages may be highly disorganized in the area of stenosis. Other anomalies may occur in a patient with congenital tracheal stenosis.

Aberrant pulmonary artery or ‘pulmonary artery sling’ may occur in association with congenital lower tracheal stenosis. The left pulmonary artery originates from the proximal portion of the right pulmonary artery and passes behind the trachea to the left lung. Patients with stenosis must be differentiated from those with a compressed and malacic segment of trachea adjacent to such a pulmonary artery sling.

Congenital vascular rings may also cause compression of the airway. The trachea and oesophagus may be compressed without any associated tracheal anomaly, although secondary malacic changes may occur. Such compromise of the airway may also occur with double aortic arch, with right aortic arch and patent ductus arteriosus or ligamentum arteriosum, with aberrant subclavian artery, and with an abnormal innominate artery.

In contrast to congenital tracheal stenosis, congenital tracheomalacia is rare. Some reported cases may well be secondary to treatment of stenosis in infancy. A rare anomaly leading to airway obstruction is congenital agenesis of the right lung with compression and malacia of the lower trachea and left main bronchus. Malacia occurs because the heart moves into the right axillary line, rotating the aortic arch, and pulling the left pulmonary artery posteriorly against the vertebral column and aorta. The lower end of the trachea and the first part of the left main bronchus pass beneath the aortic arch at this point: the pulmonary artery lies anterior. Marked compression with secondary malacia may follow. Comparable lesions in the adult are seen in ‘post-pneumonectomy syndrome’. Another rare anomaly is laryngotracheo-oesophageal cleft, in which there is an absence of the common wall between the posterior larynx and the upper trachea and the oesophagus.

Tracheobronchiomegaly (Mounier-Kuhn disease) is properly classified as a congenital disease. Despite its presentation in adult life, careful questioning reveals no clear-cut point at which symptoms commenced. Retrospectively, patients and their families are aware that breathing has never been normal. The circumference of the tracheal wall is greatly increased, with irregularities and deformities of the cartilages. This situation may lead to a reverse curve of parts of the anterior wall of the trachea and the main bronchial cartilages, with elongation and redundancy of the membranous wall accompanied by marked thickening of the wall.

The diagnosis should be suspected when respiratory distress is recognized in an infant, often with inspiratory and/or expiratory stridor. Dyspnoea may be paroxysmal rather than continuous. Feeding difficulties, failure of normal development, and frequent and stubborn respiratory infections may be present. Air tracheograms and fluoroscopy describe the anomaly more precisely: contrast medium is not generally necessary if the radiologist is skilled. A CT scan with thin sections provides comparison of the diameter and shape of the airway at various levels. Conventional radiography provides a well-delineated longitudinal picture. Such images are clearer than are sagittal views from MRI.

Bronchoscopy provides excellent delineation of the portion of the airway proximal to the stenotic lesion, and sometimes of the distal airway if the instrument can be passed safely. Rigid paediatric bronchoscopes with magnifying telescopes provide a more precise view than do flexible instruments. Circumferential rings may be clearly seen submucosally in many patients. Disorganized cartilages are, however, less well defined. The bronchoscopist must be alert to the possibility of vascular rings. Now well defined by CT and MRI these lesions do not necessarily require angiography for diagnosis.

A conservative therapeutic approach should be adopted to airway lesions in infants and small children. The juvenile trachea tolerates anastomotic tension less well than the adult trachea, probably because of the delicate nature of the tissues. Furthermore, a small amount of oedema in a tiny airway may be much more obstructive than a similar degree of oedema in an adult. Intubation and ventilation through the juvenile airway is more likely to injure a recent anastomosis or the native trachea. If the child can be treated conservatively, growth of the airway alone may ameliorate the obstructive symptoms. While growth of a stenosis is in general proportional to the growth of the normal tissues and does not actually correct congenital stenosis, improvement may be sufficient to obviate the need for correction or to permit the patient to grow, reducing the risks of surgical reconstruction.

Tracheal webs may be divulsed or removed bronchoscopically with biopsy forceps, cauterizing electrodes, or the laser. A limited stenotic lesion may be resected and an end-to-end anastomosis performed, using the techniques that have evolved for management of the trachea in the adult. The lower trachea in an infant is easily accessible from an anterior approach. Although only a limited amount of information has been obtained in children, animal experimentation, which is probably pertinent in this respect, shows that anastomotic sites grow on average to 80 per cent of the normal sagittal diameter and 75 per cent of the coronal diameter, achieving 80 per cent of a normal lumen. This process permits essentially normal function.

If a congenital stenosis is long and the child is unable to tolerate conservative management (which is not often the case), widening of the stenosis may be achieved by linear incision and insertion of a gusset of pericardium. The trachea is stented for a prolonged period. The mesenchymal patch does not appear to contract with time. Dilatation alone is not useful in treating congenital stenoses. Forceful dilatation may split the circular cartilaginous rings, which cannot stretch. Attempts to manage congenital strictures by injection of steroid or by laser treatment are unavailing.

The trachea and carina may be damaged by blunt or penetrating trauma. The most common causes of closed tracheal trauma to the neck are impact against steering wheel or dashboard in a motor vehicle accident, or an encounter with a cable across the path of a motorcyclist. Injury to the lower trachea and carina comes from impact against the chest wall, most often in a motor vehicle accident.

Gunshot wounds are the most frequent cause of penetrating wounds to the trachea, although rare.

Blunt cervical injury may affect the airway at any level from the hyoid bone to the carina. Crushing and hyperextension injuries may result in crush injuries to the larynx and trachea, separation of the larynx from the trachea, or tracheal rupture. Avulsion of the oesophagus from the pharynx at the cricopharyngeal level or traumatic injury to the oesophageal wall associated with similar injury to the membranous wall of the trachea may occur concomitantly. The presentation of such injuries may be subtle. Contusions and lacerations of the neck or chest may be noted, and subcutaneous emphysema may be detected in the neck. If the injury is lower, radiographs may show air dissecting mediastinal tissues. Unilateral or bilateral pneumothorax may result from tracheal injury within the thorax.

Obstruction may result from crushing of the larynx and from resultant haematoma. Patients may either die immediately from airway obstruction or from tracheal transection, or they may suffer varying severity of dyspnoea. A patient with an initially satisfactory airway may rapidly decompensate and develop obstruction spontaneously or during intubation or examination of the airway. Hoarseness, inspiratory stridor, respiratory distress, or change in voice—either dysphonia or aphonia—are observed. The recurrent laryngeal nerves may be injured unilaterally or bilaterally, either temporarily or permanently. Associated injuries include subluxation of the cervical spine, with or without neurological injury, and injury to the aortic arch or great vessels. Penetrating wounds to the trachea, more frequent in the cervical region or thoracic inlet, may well be accompanied by damage to the great vessels.

When the lower trachea is damaged, prominent injuries to the chest, typical of high-impact trauma, are often seen. These include fractures of the sternum, multiple ribs, or costal cartilage. In children and young adults, severe compression of the chest with resultant injury to the airway may occur without rib fractures. Injuries to the carina and main and lobar bronchi occur from crushing injuries to the chest; these are more common than tracheal injuries. Tracheal and carinal injuries may present with unilateral or bilateral pneumothorax, in addition to mediastinal dissection of air. Rupture at the carina causes a linear, spiralling split upward from the carina, either anteriorly or posteriorly. Bilateral or unilateral pneumothorax from tracheal injury in the mediastinum sometimes responds to tube drainage, with initial sealing of the air leak as a result of approximation of peritracheal tissues.

The site of impact in external injury or the point of entry of a penetrating injury, together with the signs and symptoms described, should announce the probability of a ruptured airway. Respiratory distress due to tension pneumothorax demands prompt diagnosis and treatment. Even if the patient shows little distress, radiological studies should be undertaken, with constant observation for the possibility of sudden deterioration of the airway.

A CT scan can show quite dramatic results, but is not usually justified unless the patient is stable and there are special indications for the study. Usual precautions are observed in moving such patients with respect to possible instability of cervical, dorsal, and lumbar spine, and with respect to possible associated injuries. Endoscopy is important, but may precipitate acute obstruction of an already unstable airway held together only by tenuous peritracheal connective tissue. If tracheal injury is suspected, the surgeon must be prepared to perform immediate tracheostomy if difficulty ensues. It is permissible to attempt bronchoscopy, using either a flexible bronchoscope with an endotracheal tube threaded over it so that it may be inserted directly, or a rigid bronchoscope. Rigid bronchoscopy should only be undertaken after cervical spine injury has been ruled out. If there is damage to the trachea posteriorly or if it is transected, oesophagoscopy is also in order after securing the airway. An unrecognized fistula from the oesophagus may produce lethal mediastinitis.

The laryngotracheal junction is most frequently injured in tracheal trauma. Deceleration injuries can cause long tears in the membranous portion of the lower trachea. Vocal cord function should be assessed, if possible, although one may expect the cords not to function if there is complete transection of the cervical trachea or avulsion from the larynx.

The most important initial aspect of treating a laryngotracheal injury is establishment and maintenance of an airway. If an endotracheal tube can be passed without excessive manipulation, this provides initial stabilization and permits a more measured approach to repair of the injury. If, however, an airway cannot be established, urgent tracheostomy must be performed. The operator encounters masses of clot and contused tissue, and the trachea may not be immediately evident. The separated distal end retracts into the mediastinum and is best identified by the exploring finger. The anterior wall of the separated trachea is grasped with a forceps and is drawn up into the lower cervical region where the disrupted end of the trachea is intubated. The wound is debrided and the extent of damage determined following establishment of a safe airway. A skilled operator familiar with techniques of tracheal repair may directly anastomose the trachea. If the operator is not skilled or if other life-threatening injuries demand treatment, it is preferable to anchor the distal trachea in the base of the neck and to place a tracheostomy tube in the distal separated end. Distal tracheostomy should not be performed, as this will only further injure tracheal tissue and provide no advantage over a tube placed in the already divided end. The wound is closed with drainage after adequate cleansing; secondary repair is undertaken later. If tracheal repair is performed, it is usually necessary to place a small protective tracheostomy approximately 2 cm below the anastomosis, since glottic function is usually severely impaired by recurrent laryngeal nerve paralysis. Later procedures on the glottis will provide an adequate aperture despite the paralysis. It is frequently not recognized that a paralysed larynx, given an adequate glottic aperture, is a functional organ. The aperture permits easy respiration and produces a voice that is hoarse, but completely comprehensible. Such apertures may be established by cord lateralization, arytenoidectomy, or arytenoidesis.

If the larynx has been structurally damaged by the injury, it should be reconstructed. This is often best accomplished by placement of an internal stent, such as the Montgomery moulded laryngeal stent of a T-tube that passes through the vocal cords. These injuries are best handled by a team of specialists that includes an otolaryngologist.

Partial or complete disruption of the oesophagus from the pharynx at the cricopharyngeal level must be repaired or a damaging cervical fistula will result. The closure is buttressed with a pedicled cervical strap muscle to prevent a fistula from tracking along the reparative sutures (Fig. 3) 1941. It is not yet possible to repair the recurrent laryngeal nerves. While laryngeal function will sometimes return, even if a nerve has only been contused, permanent paralysis of the larynx often results.

Lacerations of the lower end of the trachea can often be repaired by those skilled in such techniques by an anterior approach, either through partial sternotomy or, in rare cases, through full sternotomy with an added approach through anterior and posterior pericardiotomy between the superior vena cava and aorta. For those less familiar with this approach, the lowermost trachea, as well as carina and bronchial ruptures, are best approached through a right thoracotomy in the fourth interspace or the bed of the fifth rib.

If tracheal rupture is not recognized or is not treated definitively and acutely, stenosis results. The stenosis will appear to be very long, since the distal separated segment usually retracts into the mediastinum. The functional adequacy of the larynx is first assessed, and an adequate glottic aperture is established. Laryngotracheal continuity is usually re-established at a second procedure. If a oesophageal fistula is also present, it is repaired and supported with a tissue buttress.

Inhalation burns of the larynx, trachea, and bronchi may be particularly difficult to treat. The injurious agent may be chemical, thermal, or a combination. Patients often show little damage to the pharynx or supraglottic larynx once the acute injury has subsided. Persistent damage begins in the subglottis, below the vocal cords, and extends down the airway in a gradually diminishing spectrum of injury. How far down this extends is probably related to the ‘dose’ as well as the injurious potential of the agent. The degree of inflammatory change, granulation tissue response, and scarring depends on the depth of mucosal injury. The tracheal rings are not generally destroyed. Resection is usually precluded, both by total involvement of the subglottic larynx from the undersurface of the vocal cords down as well, and by the length of the tracheal injury. A further caution is that burns of the airway respond to early surgery, even where limited in linear extent, in much the same way as burns of the skin and other tissues. Intense proliferation of scar tissue follows early intervention, with the probability of restenosis. A necessary ventilatory cuff may compound the injury in a localized area.

Airway obstruction caused by burns can be managed initially by a tracheostomy placed at the second or third ring, typically within the area of burn injury, followed later by placement of a silicone T-tube to span the injured area. Proximally, the tube must often extend through the vocal cords. Most patients retain a hoarse but understandable voice and, surprisingly, do not appear to aspirate if they eat carefully. In some patients the T-tube has to remain in place for years while the burn scar gradually subsides. If the original injury is to the more superficial inner layers of the trachea, there is apparent regression and maturation of the connective tissue proliferation with re-epithelialization over time—much the same process as is seen in similar cutaneous burns. T-tubes can be removed without need for further treatment. The larynx and trachea do not return to wholly normal function or diameter, but the airway is usually adequate. If this is not the case, resection of a short segment of stenotic airway is possible.

Following intubation, strictures principally occur at the level of the tracheostomy stomas and at the level of the tracheostomy cuff (Fig. 4) 1942. The upper strictures usually occur in patients who are being maintained on ventilators. Indirect evidence suggests that the aetiology is cicatricial healing of a stomal opening that had once eroded the anterior and lateral walls of the trachea. While this injury can be surgically abetted and while invasive infection may also play a role, it appears to be due most often to leverage of the tracheostomy tube against the stoma because of improperly suspended heavy equipment. Scarring is apt to occur in a patient receiving prolonged support through a tracheostomy tube. Cicatricial stenosis occurs at the anterior and lateral site of the original defect producing an A-shaped stenosis when viewed through the bronchoscope with the patient supine.

In contrast, a high-pressure cuff or a low-pressure cuff used in a high-pressure range by overinflation will produce circumferential pressure injury in the trachea. As healing occurs in a circumferential fashion stenosis results. If erosion has been deep and has destroyed the cartilages, the resulting stenosis will never be amenable to cure, even by the most prolonged stenting, since there is no normal tracheal mural architecture left. Varying depths of erosion are, of course, seen.

Cuffs on endotracheal tubes may also produce this lesion. Patients who develop stenosis after only 48 h of ventilation usually have had only an endotracheal tube in place. In many such patients the injury consists of a massive cicatricial stenosis of scar tissue within the tracheal lumen, although the cartilages are not totally destroyed, and may even be relatively intact. Endotracheal tubes may also cause glottic stenosis and stenosis at the cricoid level, but these are not true tracheal lesions. It is important, however, to be aware of this possibility before considering repair of a tracheal lesion. Corrective surgery on the trachea can be disastrous if there is an inadequately functioning glottis above it. Severe stenoses resulting from cricothyroidostomy may also occur in the subglottic region. In elderly kyphotic patients a tube from a high stoma may gradually erode back through the cricoid cartilage and produce subglottic stenosis. A large-bore endotracheal tube may produce erosion and stenosis at the cricoid level. Repair of strictures involving the subglottic larynx is much more difficult than is repair of strictures of the trachea alone.

Inflammation may cause varying degrees of thinning of cartilages in the segment between the site of a tracheal stoma and the level of a cuff stenosis below. This area may also become malacic. Tracheo-oesophageal fistula is seen most often in patients who require nasogastric feeding tubes for long periods of time in addition to an inflated cuff in the trachea (Fig. 5) 1943. The pressure of these two cuffs acts as an erosive pincer. Anterior erosion of the tracheal wall was seen more often when high-pressure cuffs were routinely used or when the tip of a tube angulated forward. These sometimes caused erosion directly into the innominate artery as it crossed the trachea. A more common cause of innominate artery haemorrhage is the low placement of a tracheostomy tube so that the tube itself rests on the elevated artery and erodes through it at the inferior margin of the stoma. Such lesions are seen most often in children and young adults, since their tracheas are more mobile and rise into the neck along with the innominate artery upon hyperextension. These lesions can be avoided by placing the tracheostomy in an appropriate position at the level of the second and third tracheal rings, rather than with reference to the sternal notch.

Most patients with postintubation stenosis have signs of upper airway obstruction. The patient initially complains of shortness of breath on exertion, followed by progressive shortness of breath even at rest, with wheezing and stridor. Occasionally, episodes of unilateral or bilateral pneumonitis are present. Cyanosis is a very late sign. Any patient who develops signs of upper airway obstruction must be considered to have an organic lesion of the trachea, particularly if he has recently undergone intubation for ventilatory support. Many of these patients are diagnosed as having adult-onset asthma despite their record of recent intubation.

The clinical presentation and history give the presumptive diagnosis in most cases. This may be followed by simple tracheal radiographs of the type described in the section on tumours of the trachea. Fluoroscopy provides evidence that the glottis is functioning adequately and excluding the presence of malacia. Bronchoscopy may be required as a separate procedure in complex cases; in simpler cases it is performed concurrently with the repair if the problem has been radiologically demonstrated.

Surgical treatment of benign strictures of the trachea is standardized and is the treatment of choice if the lesion is not excessively long, if it does not involve other more complex anatomical structures, such as the subglottic larynx, and if the surgeon has had enough experience to promise a good result. Most tracheal strictures may be managed indefinitely by reinstituting a tracheostomy, dilating the stricture, and inserting a silicone rubber T-tube (Montgomery tube). The tube requires changing at infrequent intervals.

An emergency dilatation may be performed through a rigid bronchoscope under general anaesthesia, using just the tips of oesophageal dilators through the bronchoscope. If the stricture is lower down, dilatation with serially larger paediatric bronchoscopes is used to good effect. The period of grace provided by such a dilatation often allows further study of the patient or transport to a centre where tracheal surgery is performed. For longer-term management, a tracheostomy tube or a T-tube may be introduced.

Where there is no contraindication to surgical excision and end-to-end repair, this is the treatment of choice. Morbidity rates are low, and the success rate is very high. Nearly all repairs of postintubation stricture are now undertaken through an anterior approach, using collar incision with or without vertical partial sternal division (Fig. 6(a)–(d)) 1944. Dissection is kept close to the trachea to avoid injury to the recurrent laryngeal nerves. Stenoses are dilated prior to surgical repair if they are less than 6 mm in diameter in order to avoid retention of carbon dioxide and resultant arrhythmias. Division is usually made below a stricture. Unless it is a low one, intubation is carried out across the operative field, and the stricture is dissected from the oesophagus (Fig. 6(c)) 1944. Strictures up to half the length of the trachea may be thus removed, followed by approximation. In older patients, a laryngeal release may be required to provide additional length. Tracheostomy is rarely used. If the stricture involves the subglottic larynx, a single-stage repair of the lesion requires partial removal of the lower anterior subglottic larynx (Figs. 7, 8) 1945,1946. If the stenosis is circumferential, the scar overlying the injured mucosa may be removed from the anterior surface of the cricoid plate. Appropriate tailoring of the distal segment allows repair of both of these defects in a single stage, but the technique is difficult.

Rarely does a postintubation stenotic lesion involve over one-half of the trachea, unless there has been a prior attempt at surgery. If primary reconstruction is impossible because of the extent of damage to the trachea, insertion of a Montgomery silicone T-tube is an excellent long-term alternative. In such a case open surgery is not required for insertion of a prosthesis.

When the neck is hyperextended for performance of tracheostomy in children or young adults, over half the trachea, and the innominate artery, rise into the neck. It follows that if the tracheal stoma is placed in relation to the sternal notch, rather than in relation to the cricoid cartilage, the stoma will actually be in the midtrachea and lie just above the innominate artery. A tube may erode through the artery immediately inferior to the margin of the stoma, producing the lesion shown in Fig. 5(e) 1943. The premonitory haemorrhage that often occurs should lead to inspection of the tracheal stoma, if necessary by bronchoscopy.

In the event of massive haemorrhage, control may be obtained by finger compression down and forward, against the sternum at the site of the bleeding. An endotracheal tube is then slipped into the airway and the cuff inflated to prevent blood from running into the lungs. The surgeon's finger is held in place while the patient is moved to the operating room.

Exposure is best obtained through a collar incision at the level of the stoma and a vertical sternotomy; a haemorrhage is controlled with finger pressure during the initial dissection. During proximal dissection of the origin of the artery care must be taken to avoid extending the injury. Dissection is carried distally on the innominate artery to a level just proximal to its bifurcation; a gentle vascular clamp or tourniquet is applied. The recurrent laryngeal nerve must be avoided as it passes around the subclavian artery. When bleeding has been controlled, the next manoeuvre is usually resection of the damaged segment of the artery and oversewing of the proximal and distal ends with double suture lines. Simple ligation alone is likely to result in secondary haemorrhage. The stumps of the artery proximally and distally are buried under whatever healthy tissue is available: thymus, strap muscles, or even the omentum brought up substernally. An arterial repair would lie in a potentially septic field. Neurological sequelae are rare. It is usually best to create a new tracheal stoma at a higher, more appropriate level (second or third tracheal ring) by inserting a tube long enough to pass beyond the previous stoma. Strap muscle is sutured over the original stoma. Such an arterial fistula is best prevented by correct initial placement of the tracheostomy tube.

A tracheo-innominate artery fistula can also be caused by erosion of a high pressure cuff anteriorly through the tracheal wall and into the overlying innominate artery (Fig. 5(e)) 1943. Occasionally the tip of a tube angulating forward produces a similar erosion. It is impossible to get a finger to the level of this fistula, which lies entirely within the mediastinum. The immediate haemorrhage is controlled by placement of an endotracheal tube with a high-pressure cuff or with a low-pressure cuff inflated sufficiently to compress the opening.

Exposure is then achieved as previously described. The involved arterial segment is excised, and proximal and distal ends are sutured closed. In these now rare cases there is usually circumferential damage to the trachea at cuff level. This situation requires tracheal resection and end-to-end anastomosis. Such patients continue to require the ventilation that they were receiving just prior to the haemorrhage. The tube cuff must be positioned either well above or well below the anastomotic line to avoid pressure on the tracheal anastomosis which could evolve into separation.

Tracheo-oesophageal fistula follows prolonged ventilatory support, usually as a result of pressure on the apposed walls of oesophagus and trachea, between an inflated tracheal cuff and an in-lying oesophageal feeding tube. The fistula usually lies at the level of the cuff, somewhat below the level of the tracheal stoma (Fig. 5(d)) 1943. More often than not, the intratracheal balloon produces a circumferential injury, of which the fistula is just a part. If the fistula is identified while the patient still requires respiratory support, repair is postponed. The oesophagus is kept free of tubes, and the lowest pressure high-volume cuff that will provide a gentle seal is used for continuation of the ventilation. A gastrostomy tube is inserted to drain the stomach and prevent reflux through the fistula. The patient is fed through a jejunostomy. Once the patient has been weaned from the respirator, a single-stage operation is performed.

The collar incision often circumcises the stoma (Fig. 9) 1947,1948. Rarely is the vertical limb needed. The initial dissection is identical to that described for upper tracheal resection. After transection of the trachea below the distal margin of the stenotic segment, the specimen is elevated and the fistulous connection completely isolated circumferentially and dissected from the oesophagus, removing the margin of the fistula. The anterior tracheal stoma is often close enough to the injured segment of trachea to be conveniently included in the resection. If there is a segment of fairly normal trachea between the stoma and the stenotic segment, the stoma may be left in place and allowed to close spontaneously later. If the fistulous opening is long and the tracheal wall is not destroyed anteriorly as far down as the fistula extends, the margin of the tracheal opening is excised as a V, with its point aimed distally, so that the posterior membranous wall may be reconstructed with a vertical suture line before the tracheal ends are anastomosed (not shown). We have seen a granuloma form at the junction of the T closure in such a case. If necessary, some of the oesophageal wall may be left at the sides of the V so that there will be enough tissue for reconstruction of the membranous wall of the trachea.

The oesophagus is closed longitudinally with two layers of 4–0 silk. The sternohyoid muscle, or sometimes the sternothyroid, is used to create a pedicle and is sutured into place over the oesophageal closure, interposing healthy muscle between the vertical suture line in the oesophagus and the transverse suture line of the tracheal anastomosis about to be made. The completed anastomosis of the trachea is shown in Fig. 9 1947,1948.

In the rare case when direct trauma has produced a tracheo-oesophageal fistula without circumferential damage to the trachea, repair does not require resection of the trachea. The trachea and oesophagus are dissected closely until the fistula is fully identified. The recurrent laryngeal nerves are not deliberately exposed. Extra tissue from the anterior oesophageal wall is used to provide sufficient tissue to allow closure of the membranous tracheal wall without tension. The oesophagus is closed in layers, muscle is interposed, and the trachea is then repaired longitudinally. Exposure is difficult because the trachea has not been divided.

Persistent tracheal stomas usually have a mucocutaneous union of trachea and skin. The patient has often had a tracheostomy for a long time, with extended periods of ventilation. Simply drawing a strap muscle over the aperture creates the potential for formation of a granuloma from the raw tissue presented to the interior of the trachea; this is particularly likely to occur with a large stoma. It is better to cover the stomal orifice with a circular flap developed from the peristomal skin. Perioperative antibiotics help to prevent wound infection.

A circular incision is made around the stoma to define the skin that will be used for closure. Wedges of skin are excised on either side to permit linear closure of the circular defect. The collar of skin is carefully elevated, leaving sufficient attached to the trachea to preserve its blood supply. The skin is now inverted on itself by a subcuticular suture that effectively closes the stoma with an epithelial, (skin)-lined surface. Margins of the skin defect are elevated. The strap muscles are dissected free on either side and approximated in the midline in layers. The platysma and skin are closed transversely. Functional and cosmetic results are excellent.

A variety of specific infections can cause stenosis of the trachea. Diphtheria was notorious for producing such injuries, though it is now rare. A small number of patients with a history of diphtheria in childhood develop stenosis later is life: it may be difficult to be certain whether the stenosis is a result of tracheostomies established for the treatment of the diphtheria, or the diphtheria itself. Tuberculosis of the bronchi can cause severe stenosis, particularly of the lower trachea. Typically, the fibrosis is submucosal. An accompanying stricture of either the left or right main bronchus is also seen; upper lobe bronchus may also be involved. If the stenosis is mature and is not excessively long, resection and reconstruction may be definitive. Management becomes difficult in the presence of considerable residual inflammation. It is difficult to manage such a patient by intubation alone because of the involvement of the carina. A period of watching and waiting for the inflammation to subside with chemotherapy is the best cure.

Mediastinal fibrosis may be accompanied by marked narrowing and stenosis of large segments of the trachea and bronchi. At least some patients with mediastinal fibrosis have pathological or bacteriological evidence of previous histoplasmosis. Some of these patients may have problems amenable to extensive surgical resection and reconstruction to save at least some of the lung tissue. In others the mediastinum is so massively fibrotic and the length of trachea and bronchi involved is so great that excision and reconstruction are impossible. There is no known therapy except periodic redilatation.

A number of other infectious disease uncommon in the United States have been implicated in airway obstruction, including scleroma.

Tracheal collapse is seen following a variety of lesions. There are many references to congenital tracheal malacia in the literature. Few of these cases are well documented, however, and many have complicating factors; the malacic changes may well be the result of intubation. The infant trachea is composed of very fine structures, and even slight injury may lead to softening of the already tender rings.

Segmental malacia is seen as a dominant lesion in a number of patients who have had cuff injury to the trachea following intubation. Such patients may have an apparently normal air column on static radiographs. Fluoroscopic observation, however, demonstrates a segment that collapses on cough or forced respiration. Resection is the usual treatment.

Chronic compressive lesions may also lead to collapse of the trachea. A large goitre, a cystic thymus, an aneurysm, or a congenital vascular malformation such as a vascular ring or an anomalous innominate artery may lead to compressive obstruction of the trachea. When the compressing lesion is excised or displaced so that pressure is no longer exerted, malacia may appear because the rings have been so thinned. Transitory intubation is sometimes effective treatment. Other techniques, such as splinting with local prosthetic rings placed external to the lumen or with traction sutures pulled out over buttons placed against the cervical musculature, have been employed. The problems are rare, vary greatly, and require individual solutions.

Two types of tracheal collapse are also seen with chronic obstructive pulmonary disease. One type consists of a softening and flattening of the trachea from front to back. When the patient coughs, the membranous wall becomes approximated to the cartilaginous wall with obstruction of the airway, particularly in the lower half of the trachea. The deformity known as ‘sabre-sheath trachea’, which consists of an increase in the anteroposterior dimension of the trachea with side-to-side narrowing is less common. This may however, cause such extreme narrowing that coughing and approximation of the lateral walls of the trachea causes appreciable obstruction, especially to clearance of secretions. Sabre-sheath trachea affects the lower two-thirds of the trachea.

The various types of surgical procedures that have been devised to correct the first, or C-type, flattening consist of pulling the corners of the cartilages together to shorten the membranous wall. When the patient coughs or breathes forcefully, it is then not possible for the trachea to flatten out.

Relapsing polychondritis is a disease of unknown aetiology, that leads to destruction of cartilage in many parts of the body, including the nasal septum, ears, trachea, and bronchi. The airways soften gradually. The patient is subject not only to difficulties of moving air, but to recurrent infections. The bronchi are involved. No effective surgical procedure has yet been devised.

Stenosis of the airway occasionally occurs in a patient with no history of trauma, infection, inhalation injury, intubation, or ventilation. Biopsy examination discloses no specific organisms or diagnostic characteristics, but acute and chronic inflammation with fibrosis. These patients rarely have a history of systemic disease, and do not develop systemic disease subsequently. They do not have mediastinal fibrosis or processes involving mediastinal lymph nodes. The primary manifestation is upper airway obstruction. The lesions are located principally in the subglottic larynx and upper trachea, less commonly in the upper trachea alone.

One is reluctant to hurry to surgical reconstruction in these patients, since it is impossible to predict the future course of the disease. When airway obstruction becomes severe and does not respond for sufficiently long intervals to dilatation, surgery may be entertained, with, however, the clear caveat that the disease may progress. Since the stenosis often involves the subglottic larynx, complete removal of all inflammatory tissue is often impossible.

Sarcoidosis may, on occasion, cause obstruction of the lower trachea and main bronchi due to a combination of massively enlarged lymph nodes and fibrotic changes in the tracheal wall itself. The circumferential stenosis produced responds to dilatation. Resection and reconstruction are not generally feasible because of the length of airway involved. Amyloid disease may cause a similar extensive process in the airways, which is not amenable to surgical reconstruction. Wegener's granulomatosis may produce tracheal stenosis.

Tracheopathia osteoplastica is a rare condition in which the cartilages become irregularly hypertrophied with nodular overgrowth. Obstruction may follow. Some palliation can be provided by bronchoscopic removal of the most prominent malformations. The disease usually involves the entire trachea and main bronchi; it is not amenable to surgical resection or to reconstruction. Laser treatment has been attempted without success.

Primary tracheal tumours are rare, and they are thus easily overlooked diagnostically. About two-thirds of the primary tracheal tumours are squamous cell carcinoma or adenoid cystic carcinoma (formerly called cylindroma). These two types occur in about equal numbers. The remaining one-third are widely distributed in a heterogeneous group of malignant or benign tumours. A variety of secondary tumours is found in the trachea, including carcinomas of the larynx, thyroid, lung, and oesophagus. Rarely, tumours may metastasize to the submucosa of the trachea or to the mediastinum, with secondary invasion of the trachea. Carcinoma of the breast and mediastinal lymphoma may thus invade the trachea. Incompletely removed neoplasms of the main bronchus, such as carcinoid tumours, also may invade the carina.

Tracheal tumours may appear insidiously. Their most common symptoms and sign are cough (37 per cent), haemoptysis (41 per cent), and signs of progressive airway obstruction, including shortness of breath on exertion (54 per cent), wheezing and stridor (35 per cent) and, less commonly, dysphagia or hoarseness (7 per cent). It is not commonly appreciated that wheezing may be a predominant symptom of a tracheal tumour for a prolonged period. Standard chest radiographs usually show clear lung fields, causing the physician to assume that no organic mass lesion is present. Patients are often treated for adult-onset asthma. Haemoptysis may not be pursued aggressively in a patient with an apparently normal chest radiograph. Presentation may also take the form of unilateral or bilateral recurrent attacks of pneumonitis, which initially respond to antibiotic treatment.

Signs and symptoms vary with the type of tumour. Haemoptysis is prominent in patients with squamous cell carcinoma and usually leads to earlier diagnosis. Hoarseness may signify advanced disease. Adenoid cystic carcinoma more often presents with wheezing or stridor as a predominant symptom, leading to delay in diagnosis. Only a little more than one-quarter of these patients have haemoptysis early in the course of the disease. Dyspnoea, however, may be a prominent symptom. The mean duration of symptoms prior to diagnosis in patients with squamous cell carcinoma of the trachea is only 4 months; in those with adenoid cystic carcinoma, the mean duration is 18 months. In some benign tumours or low-grade malignant tumours of the trachea, the mean duration for carriage of an incorrect diagnosis is up to 4 years. The mean duration of symptoms of miscellaneous malignant tumours is 11 months.

Endoscopy is frequently the means by which a tracheal tumour is discovered in a patient who is being studied for haemoptysis of unknown origin. A high tracheal tumour may be overlooked if a flexible endoscope is passed through a previously introduced endotracheal tube or if the endoscopist is not in the habit of looking carefully at the proximal portion of the trachea. The same hazard faces the endoscopist who uses a rigid bronchoscope. When a lesion is not obstructing or is of such radiological extent that a surgical approach seems indicated in any case, endoscopy is deferred to the time of potential resection. However, when the surgical team is not trained or experienced in the management of tracheal tumours, preliminary bronchoscopy may be done to visualize the tumour. Biopsy specimens must be obtained with care to avoid stimulation of bleeding in an excessively vascular tumour. Haemorrhage in a patient with a very vascular carcinoid tumour, for example, may be life-endangering or may require emergency surgical treatment. Biopsy of the rare haemangiomatous lesion of the trachea can be lethal. If preliminary biopsy examination is not undertaken before surgery, accurate frozen section facilities must be available. Biopsy specimens may need to be taken at a distance from the tumour to determine resectability; this is particularly true for adenoid cystic carcinoma, which is notorious for submucosal spread. Endoscopic examination of the oesophagus is also appropriate in patients with extensive tumours.

Of 198 patients with primary tumours of the trachea treated at the Massachusetts General Hospital between 1962 and 1989, 70 had squamous cell carcinoma, 80 had adenoid cystic carcinoma, and the rest were a mixed group. The mean age of the patients with squamous cell carcinoma was 58 years, in contrast to 43 years for those with adenoid cystic carcinoma. In the latter group, the age spread was much wider.

Squamous cell carcinoma may be exophytic or ulcerative; it may also occur as multiple lesions scattered over a considerable distance in the trachea. The tumour metastasizes to the regional lymph nodes and, in its more aggressive and late forms, invades mediastinal structures. In general, its progress is rapid in comparison with that of adenoid cystic carcinoma. Many of these patients develop a second squamous cell carcinoma of the lung or oropharynx.

Adenoid cystic carcinoma often has a very prolonged course of clinical symptoms, sometimes extending for years. Recurrence may be seen many years after initial treatment. The tumour may extend over long distances submucosally in the airways, and also perineurally. It spreads to regional lymph nodes, although this is more characteristic of squamous cell carcinoma. Although adenoid cystic carcinoma may invade the thyroid gland or the muscular coats of the oesophagus by contiguity, adenoid cystic carcinoma that has not been surgically interfered with frequently displaces mediastinal structures before actually invading them. Metastases to the lungs are common and may grow very slowly over a period of many years, remaining asymptomatic until they reach a large size. Metastases to bone and other organs occur.

Among the other malignant lesions seen in the trachea in the above series of 198 patients were 10 carcinoid tumours clearly originating in the trachea and not in the main bronchi, two spindle cell sarcomas, one adenocarcinoma, one adenosquamous carcinoma, four mucoepidermoid carcinomas, one chondrosarcoma, one carcinosarcoma, one small cell carcinoma, one primary melanoma, and one malignant fibrous histiocytoma.

The benign lesions consisted of neurofibroma, chondroma, chondroblastoma, leiomyoma, granular cell tumour, paragangliomas, haemangioma, and pleomorphic adenoma. Several patients were seen with varieties of squamous papillomas, including solitary squamous papillomas and papillomatosis, either widespread or of a confluent, often verrucous, type.

Secondary tumours involving the trachea were seen in 81 patients. Oesophageal carcinoma (seen in 10 patients) may cause a fistula between the oesophagus and the trachea or the left main bronchus. Aggressive carcinoma of the lung (29 patients) also occasionally results in fistula formation, as both trachea and oesophagus are involved from the mediastinum. Some of the oat cell carcinomas of the trachea that have been reported have probably arisen in the lung and invaded the trachea.

Papillary and follicular carcinomas of the thyroid gland and mixed varieties of the two carcinomas invade the trachea primarily (38 patients), usually at the level of the isthmus. A patient initially presenting with haemoptysis may therefore have carcinoma of the thyroid gland. Invasion of the trachea by thyroid carcinoma is best managed by tracheal resection with airway reconstruction. Localized extension of tumour may require partial oesophageal resection or radical resection, including laryngectomy with mediastinal tracheostomy. More commonly, invasion is seen following prior thyroidectomy for carcinoma in which the surgeon was aware that he was shaving off the tumour from the trachea. In such cases, concurrent or early resection of the involved trachea should be considered.

When the primary tracheal tumour is circumscribed, has not metastasized remotely, does not involve an excessive length of trachea, and has not invaded the mediastinum deeply, the best primary treatment is resection with primary reconstruction of the airway. Considerable experience is required to make the judgement of whether a tumour can be resected safely with sufficient tissue to provide a potentially curative margin, yet allow primary reconstruct of the airway. This judgement is even more crucial when the tumour lies in the lower portion of the trachea or at the carinal level and when an airway has to be finally reconstructed at the time of the original surgery. Patients with adenoid cystic carcinoma in whom frozen sections show microscopic tumours at apparently clear resection margins are particularly difficult to manage.

Both squamous cell carcinoma and adenoid cystic carcinoma of the trachea are usually responsive to irradiation, with varying long-term results. In general, curative irradiation of squamous cell carcinoma produces variable palliation, generally extending for not much longer than a couple of years and with ultimate recurrence. Adenoid cystic carcinoma may respond for 3 to 7 years. Although some investigators have advised preoperative irradiation, particularly in the management of adenoid cystic carcinoma, many prefer to reserve radiation for postoperative treatment, particularly in patients with involved lymph nodes, or microscopic tumour in lymphatic or nerve sheaths or at the resection margin, and when the margins appear to be too small. The same approach has been applied to other primary tracheal tumours.

Experience in the management of tracheal tumours is not large enough to enable definitive statements to be made about optimal treatment. Results to date, however, strongly indicate that the above approach is soundly based. Comparison of results obtained in patients with squamous cell carcinoma and adenoid cystic carcinoma treated by the vigorous protocols described with those of patients treated prior to the institution of extirpational surgery with modern techniques of resection and reconstruction, shows that remarkable progress has been achieved. Excellent long-term results have been obtained in patients with benign tumours amenable to excision and also in those with low-grade malignant tumours of other varieties. Cure has rarely been achieved following resection of recurrent tumours, except for local recurrences of carcinoid tumours at the carina. Long-term palliation, however, has been achieved in patients in whom less malignant thyroid neoplasms have invaded the trachea. In a number of these patients, the ultimate cause of death is the appearance of metastases in the bones or at other remote sites, rather than locally.

When the larynx is extensively involved by tumour, it is not always possible to salvage the organ; laryngotracheal resection is required. Conversely, when only a portion of the larynx is involved, it is possible to salvage a functioning larynx with reconstruction of the airway.

Approximately one-third of all tumours are clearly incurable and not amenable to surgical resection. Two-thirds are amenable to primary resection with reconstruction of the airway. In a rare patient with an extensive tumour affecting a large proportion of the trachea but which has not spread distantly or invaded the mediastinum, resection and reconstruction either by staged procedures or by the use of a prosthetic device may be justified. Such patients usually have adenoid cystic carcinoma. Squamous cell carcinomas extending over a long length of trachea, too often invade the mediastinum beyond the point of possible extirpation.

When extirpation is not possible, because of the extent of the tumour or because of the age or medical condition of the patient, primary irradiation is reasonable.

Some patients with adenoid cystic carcinoma obstructing the trachea and pulmonary metastases benefit from palliative resection if reconstruction can be performed safely.

When the trachea is acutely obstructed by a tumour that is too large to be removed by primary resection, immediate palliation may be achieved by endotracheal removal of the bulk of obstructing tumour endotracheally and radiotherapy. Removal can be undertaken with morcellating biopsy forceps (the older technique) or by the application of newer physical modalities, of which the laser is most useful for destruction of endobronchial tumour. The laser is not applicable as a primary method of treatment of most tracheal tumours, however, since it cannot destroy the base of the tumour without also destroying the tracheal wall. It does, however, have a role in the management of multiple squamous papillomas of the trachea.

Ultimately, as a tumour recurs and laser therapy is no longer possible because of the extension of the tumour through the tracheal wall, additional palliation to prevent strangulation can sometimes be given by the judicious insertion of a silicone rubber T-tube that spans the airway and allows the patient to breathe. Obviously, this method cannot be applied if such a tumour extends below the carina.

When technically feasible, resection and primary reconstruction of the trachea that has been invaded by carcinoma of the thyroid gland should be performed when no extensive metastases are present. It offers prolonged palliation, prevents suffocation from bleeding and obstruction, and provides an opportunity for cure. In carefully selected patients with massive regional disease, radical excision with laryngectomy and oesophagectomy is also appropriate.

Surgical resection of tracheal tumours is often made difficult by the unpredictability of the extent of a lesion.

For many years tracheal resection was limited by the belief that only 2 cm of the trachea (about 4 rings) could be removed and the ends dependably anastomosed by primary suture. Various techniques of anatomical mobilization now permit the dependable and predictable resection of approximately one-half of the trachea. Simple cervical flexion, which delivers the cervical trachea into the mediastinum, is the most useful single manoeuvre for extending the resection of the trachea with primary repair. In a young person who is not obese and who has reasonably supple tissues, more than one-half of the trachea can be removed with primary reconstruction. With increasing age, kyphosis, obesity, and pathological changes, the portion of the trachea that can be so removed and reconstructed is reduced. If additional length is necessary, the tracheal release may be necessary.

In all dissections of the trachea, it is critical to preserve the lateral segmental blood supply, and to ensure gentle and precise handling of all tissues and precision of anastomosis.

Tumours of the upper portion of the trachea are generally approached through a collar incision with, if necessary, a vertical extension through the upper sternum (Fig. 6) 1944. Since the extent of some tumours is not fully predictable even after preoperative radiography and bronchoscopy, it is generally wise to position a patient so that the sternal division can be carried down further and angled into the right fourth interspace to add a thoracotomy to the cervical and mediastinal exposure. Tumours of the lower portion of the trachea are approached most easily through a posterolateral thoracotomy. Laryngeal release adds no additional length for distal tracheal resection. Flexing the neck and freeing the anterior pretracheal plane are the most useful means of gaining additional length, as is intrapericardial release of the pulmonary vessels.

When a tumour involves the carina, various reconstructive techniques are used. Unless the tumour is very small, it is rarely adaptable to reconstruction by approximating the right and left main bronchus to form a new carina and then attaching it to the trachea. Suturing in this fashion anchors the carina low in the mediastinum. If more trachea has been excised, approximation is not possible. More commonly, either the right or left main bronchus is sutured to the trachea, and a lateral anastomosis of the other bronchus to the lower portion of the tracheal wall is performed above the initial anastomosis.

If a recurrent laryngeal nerve is involved by tumour, the nerve is sacrificed, although these nerves are usually identified and carefully saved when possible. Local paratracheal lymph nodes are excised with the specimen when possible: extensive lymph node dissection is likely to destroy the blood supply to the residual portion of the trachea. Partial removal of the lower part of the larynx may be required for tumours high in the trachea. Individually designed procedures are necessary to preserve a functional larynx: portions of the oesophagus and other adjacent structures may need to be resected.

Resection is usually controlled by immediate examination of frozen section specimens in the laboratory to ensure clear margins. Adenoid cystic carcinoma, in particular, may be so extensive as to make resection of all microscopic disease impossible. Postoperative irradiation is then required.

Laryngeal oedema is managed by restricting fluid intake and administering racemic adrenaline and a short course of steroids (25–48 h). The oedema usually regresses within a week. Pneumonia is rare following upper tracheal resections when proper attention is given to intraoperative management and to postoperative physiotherapy. All patients spend 1 day or more in a respiratory intensive care unit whose staff are familiar with the management of such problems.

The most common late complication is the formation of granulations at the suture line (Table 1) 527. Granulations are less of a problem in patients undergoing resections for tumour than in those who have had tracheal reconstructions for inflammatory disease, in whom residual inflammation may be present. Granulations can usually be managed by bronchoscopic removal under light anaesthesia. A suture is often found to have worked its way into the lumen at the base of the granulations: removal of the sutures leads to ultimate healing. Multiple bronchoscopies may be necessary over time. Granulations may be seen radiologically, but are most often manifested by wheezing or minor haemoptysis. The patient must be warned in advance that this is not a cause for alarm or he or she will assume that there is recurrent tumour. Although triamcinolone may be injected into the base of such granulations, there is no evidence supporting its efficacy. Use of absorbable Vicryl sutures appears to prevent the problem.

Separation of anastomosis is usually due to excessive tension following resection of too much trachea or when adjunctive relaxing manoeuvres to lessen the tension have not been made. Excessive circumferential dissection of the trachea, particularly distal to the point of division, may destroy the blood supply and cause separation or stenosis: this is more likely to occur in patients with tumours than in those with intubation stenosis.

Tracheal separation occurring in the immediate postoperative phase suggests that there has been a serious technical error. Reoperation might be considered, to resuture the area and cover with a local muscle flap is the area was small. If the tissues do not seem appropriate for resuturing, a tracheostomy tube may be placed across the defect, to be replaced later by a Montgomery silicone T-tube. A T-tube can be placed initially if a patient does not require a sealed airway. With partial restenosis, the airway that results may be tolerated and may sometimes be improved with endoscopic techniques including laser therapy.

Results of the management of complications are listed in Table 2 528. Good results following complications are possible if handled properly.

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