eMedicine Specialties > Thoracic Surgery > Miscellaneous
Tracheomalacia: Treatment
Updated: Aug 6, 2009
Treatment
Medical Therapy
Supportive therapy is provided to most infants. Most respond to conservative management, 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.2
Current recommendations for treatment of tracheomalacia
- Tracheomalacia of the milder primary variety is best treated by nonsurgical means.
- In distal tracheomalacia that is idiopathic, pulsatile, associated with tracheoesophageal fistula, or from vascular anomalies, aortopexy with concomitant intraoperative bronchoscopy appears to be the procedure of choice.
- In proximal or diffuse tracheomalacia, tracheostomy or the use of stents is beneficial.2,9 In the future, tracheoplasty also may be offered in selected patients. A previous published series of 41 infants reviewed conservative therapy, tracheostomy, aortopexy, or tracheal reconstruction in tracheomalacia. Fifteen (15) patients with mild primary tracheomalacia had resolution of symptoms by the age 2 years, 5 patients treated with tracheostomy developed secondary tracheomalacia at the site. In 9 patients with primary tracheomalacia treated with aortopexy, 5 were symptom free, 1 was improved, and 3 procedures were unsuccessful. Of the 10 patients in the acquired group treated with aortopexy, 6 were cured, 2 were improved, and treatment failed in 2. Of the 6 patients with tracheostomy, 3 eventually were extubated, 1 had major reconstruction, and 2 had tracheostomies for long term.
Surgical Therapy
Tracheomalacia generally is benign; most infants outgrow the symptoms by age 18-24 months.2 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.5,10 Surgery only is recommended for severe symptoms and failure of conservative therapy.5,2
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.
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.11 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 recent 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 5, and recurrent pneumonia in 1. 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.12
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.13 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.14 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.11
Preoperative Details
Surgical therapy is required when conservative measures fail or when reflex apnea is present; this includes correction of the underlying cause, such as a vascular ring when present, tracheostomy, and aortopexy.
Tracheostomy
Tracheostomy helps to 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 percutaneous approach.
Aortopexy
Aortopexy can provide relief of tracheal compression and relieves the external pressure on flaccid trachea. This is not a perfect operation because of a small, but significant, failure rate and potential for complications.
Intraoperative Details
Tracheostomy
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 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
The patient is positioned with the left shoulder elevated at a 30- to 45-degree 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 Details
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.
Follow-up
These patients require long-term follow-up for evaluating the success/failure of the surgical procedure and the development of complications.
For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.
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.
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Further Reading
Keywords
tracheomalacia, esophageal atresia, tracheobronchomalacia, bronchomalacia, tracheoesophageal fistula, swallowing, swallowing difficulty, flaccid tracheal cartilage, wide posterior membranous wall, reduced anterior-posterior airway caliber, tracheal collapse, abnormal tracheal cartilage, airway obstruction, abnormally increased compliance of the trachea, percutaneous tracheostomy, aortopexy, GERD, gastroesophageal reflux, intubation complication, extended intubation, chronic intubation, intubation problem, tracheal injury, trachea, relapsing polychondritis, cartilage inflammation, treatment, diagnosis, medications, disorder
