Tracheomalacia Treatment & Management

Current recommendations for treatment of tracheomalacia include the following:

• Forms of milder primary tracheomalacia are best treated by nonsurgical means ^[13]
• For distal tracheomalacia that is idiopathic, pulsatile, or associated with tracheoesophageal fistula or vascular anomalies, aortopexy with concomitant intraoperative bronchoscopy appears to be the procedure of choice
• For proximal or diffuse tracheomalacia, tracheostomy (despite its related high morbidity) or the use of stents is beneficial ^{[3, 14]}  
• As experience accumulates, a direct surgical approach to treating tracheomalacia may replace tracheostomy in the management of proximal and diffuse tracheomalacia; these procedures include prosthetic stenting, tracheoplasty, and tracheal resection with end-to-end anastomosis

In one study, silicone stents were inserted into the trachea or left main-stem bronchus in 14 children (aged 2-69 months) for tracheomalacia or airway kinking (7 cases), vascular compression (5 cases), and surgically-corrected congenital tracheal stenoses (2 cases). The best results were obtained in tracheomalacia. Six cases out of 14 (43%) were considered successful, and five cases were considered failures, primarily because of stent migration. ^[15]

An earlier study reviewed conservative therapy, tracheostomy, aortopexy, or tracheal reconstruction in 41 infants with tracheomalacia. ^[16] Fifteen patients with mild primary tracheomalacia had resolution of symptoms by the age of 2 years; five treated with tracheostomy developed secondary tracheomalacia at the site. In nine patients with primary tracheomalacia treated with aortopexy, five were symptom-free, one was improved, and three procedures were unsuccessful.

Of the 10 patients in the acquired group treated with aortopexy, six were cured, two were improved, and treatment failed in two. ^[16] Of the 6 patients with tracheostomy, three eventually were extubated, one had major reconstruction, and two had tracheostomies for an extended period.

Tracheomalacia of the milder primary variety is best treated by nonsurgical means.

Most infants who have mild-to-moderate symptoms should be offered conservative therapy because these patients improve by age 18-24 months. ^{[6, 13]}  The majority respond to such therapy, consisting of humidified air, chest physical therapy, slow and careful feedings, and control of infection and secretions with antibiotics.

The use of continuous positive airway pressure (CPAP) has been recommended in patients having respiratory distress and may be successful in patients requiring a short-term intervention as the disorder spontaneously resolves. ^[3]

 

 

General considerations

Tracheomalacia generally is benign; most infants outgrow the symptoms by age 18-24 months. ^[3] Surgical therapy is required when conservative measures are not adequate or when reflex apnea is present. Surgery includes correction of the underlying cause, such as vascular ring when present, tracheostomy, and aortopexy. ^{[6, 17]}

The indications for tracheostomy are severe symptoms, failure of conservative therapy, and proximal or diffuse tracheomalacia. The indications for aortopexy are dying spells or reflex apnea, recurrent pneumonia, intermittent respiratory obstruction, and inability to extubate airway in an infant who is intubated. Surgery only is recommended for severe symptoms and failure of conservative therapy. ^{[6, 3]}

During surgery, a careful search should be made for tracheoesophageal fistula, which should be treated surgically if present. Other causes of tracheal compression, such as mediastinal tumors or vascular rings, also need to be corrected surgically. Patients identified as having vascular anomalies compressing distal trachea should have constricting vessels surgically divided and affixed to other structures to eliminate impingement on the trachea. ^[18]

Tracheomalacia following long-standing tracheotomies may be helped by anterior cricoid/tracheal suspension, where muscular tissue of the overlying trachea is sutured to the fascia of strap muscles.

Acquired tracheomalacia, if severely symptomatic, can be treated by internal stenting, external stenting, or tracheostomy.

The use of various types of tubes and stents for the management of tracheomalacia is helpful. ^[19] Reports exist of success with Montgomery and Dumon tubes in the literature. Short-term satisfactory results also have been reported with the use of expandable metallic stent (Palmaz Stent) placement in patients with intractable respiratory symptoms caused by tracheomalacia.

A report of aortopexy in 28 children with severe and localized tracheomalacia utilized a left lateral muscle-sparing approach. The indications included acute life-threatening events in 22 patients, failure to extubate in five, and recurrent pneumonia in one. Associated esophageal atresia was present in 15 patients, and 13 had primary tracheomalacia. Most symptoms of tracheomalacia resolved in 26 of the 28 patients after aortopexy. ^[20]

Video-assisted posterior tracheopexy, with or without robotic guidance, has been suggested as a possible option for select children with severe tracheomalacia. ^{[21, 22]}

Treatment of tracheomalacia in adult patients

The finding may be incidental in many adults with tracheomalacia; these patients are asymptomatic and do not require therapy.

In symptomatic patients, care is initially supportive. Tracheomalacia frequently occurs in patients who also have chronic obstructive pulmonary disease (COPD), and the obstructive disorder optimally should be treated first. If conservative measures fail, noninvasive, positive-pressure ventilation can be used in the short term to keep the airway open and to facilitate secretion drainage. In selected patients, surgery may be used. Tracheostomy alone may be effective because the tracheostomy tube might bypass the malacic segment, or the tube itself might splint the airway open. If the patient has generalized and extensive disease, a longer tube may be necessary.

Surgical placation of the posterior wall of the trachea with crystalline polypropylene and high-density polyethylene mesh has been used recently. ^[23] Via a right posterolateral thoracotomy, the mesh is fashioned into a 2.5-cm-wide strip, which is sutured to the posterior membranous wall. Thereafter, 2-cm sheets of mesh can be sutured to the right and left mainstem bronchi.

A range of stents can be utilized to keep the airway open mechanically. Metal stents have been used to manage airway obstruction. Such stents can be easily placed by flexible bronchoscopy, are visible on plain radiographs, expand dynamically, and preserve mucociliary function. Formation of granulation tissue, which can cause severe problems including airway obstruction, airway perforation, and death, is a potential complication. Silicone stents are easy to insert, reposition, and remove. However, placing these stents requires rigid bronchoscopy and general anesthesia.

Stents have resulted in both subjective and objective improvement. ^[24] Most patients report immediate improvement in their respiratory symptoms, and airflow improves, but success is not universal. Gotway et al reported long-term pulmonary function improvement with stents placed for both tracheal stenosis and tracheomalacia. ^[19]

Tracheostomy

Tracheostomy helps maintain an airway while the child grows and the trachea regains structural integrity, but the problem with this procedure is that the tracheostomy tube may not support the distal trachea. The tracheostomy can be performed as an open procedure or via a percutaneous approach.

With the patient in supine position, the neck is placed in moderate hypertension. Identify cricoid cartilage and the thyroid isthmus, and aim to place the opening over the third tracheal ring. A transverse incision is made, the pretracheal fascia is divided, and the tracheal rings are counted. The third tracheal ring is identified and divided in the midline; the tracheal incision must be vertical. The second and fourth rings may need to be divided as well. No amount of tracheal tissue is removed during the procedure.

The stoma is enlarged by gently spreading the blades of the hemostat against the margins of the tracheal opening. A lubricated tracheostomy tube is inserted through this opening. Transtracheal injection of lidocaine reduces coughing and eases tube placement. The tube is secured to the neck and adjusted so that the distal end is at least 2 cm above the carina.

Percutaneous tracheostomy

The percutaneous tracheostomy can be performed in the intensive care unit (ICU) and requires specially designed introducer sets. After prepping the patient's neck, a 3-cm longitudinal incision is made over the second and third cartilaginous tracheal rings. The endotracheal tube is withdrawn somewhat, and the introducer catheter is advanced into the tracheal lumen. Confirm the intratracheal location either under bronchoscopic guidance or though the withdrawal of air bubbles. The introducer catheter is advanced into the trachea, and the syringe and steel needle of the introducer catheter are withdrawn.

The flexible J-tipped guide wire is inserted into the trachea through the introducer catheter, and the catheter is removed. Thereafter, an introducing dilator is advanced into the trachea until the black positioning mark. The tapered sequential dilators are used successively to dilate the anterior tracheal wall to a diameter larger than the tracheostomy tube. A tracheostomy tube over the tapered dilator is advanced into the trachea, and dilator, guiding catheter, and wire guide are removed. The inner cannula is inserted, and the patient is attached to the ventilator. A chest radiograph should confirm the correct positioning.

Aortopexy

Aortopexy can provide relief of tracheal compression and relieves the external pressure on the flaccid trachea. This is not a perfect operation, because of the low but significant failure rate and the potential for complications.

The patient is positioned with the left shoulder elevated at a 30º-45º angle. A bronchoscopy is performed to confirm the diagnosis of tracheal compression. Through a left anterior thoracotomy, partial thymectomy improves the exposure and increases the effective cross-sectional area of the upper mediastinum. The apex of the left upper lobe is retracted inferiorly and posteriorly. The search for the vascular ring is conducted, and the esophagus is examined.

A single row of interrupted monofilament sutures is placed from the arch of the aorta to the undersurface of the sternum and tied down to displace the arch anteriorly. The bites into the aorta must be deep enough to include media and adventitia; sometimes, the sutures are passed through the sternum to a subcutaneous pocket. The dissection around the aorta must be avoided because these attachments help to draw open the lumen of the trachea when aortopexy has been achieved.

Aortopexy attaches the aorta to the sternum, pulling the anterior wall of the trachea forward and, therefore, preventing its collapse.

Postoperative care of these patients is very similar to that of patients undergoing thoracic surgery. In the immediate postoperative period, patients may need to be monitored closely in an ICU setting because several days may pass before improvement in airway function occurs. These patients require long-term follow-up for evaluating the success or failure of the surgical procedure and the development of complications.

Long-standing tracheostomies lead to several complications, which include bilateral vocal cord paralysis; compression and erosion of the innominate artery; formation of secondary granulation tissue, which results in protraction of tracheomalacia; and speech delay in several instances.

During aortopexy, as the sutures are placed through aortic wall, there is an immediate risk of hemorrhage and a later potential for postoperative aneurysm formation. Deaths have occurred as a result of operative failures, other structural anomalies, and chronic ventilatory insufficiency.

Complications of percutaneous tracheostomy are bleeding, infection, accidental endotracheal extubation, extratracheal dilator position, esophageal perforation, and mucosal endobronchial flap. Some advantages exist over usual tracheostomy; the procedure is inexpensive and is easy to learn.