Tracheomalacia

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Background

Tracheomalacia is a process characterized by flaccidity of the supporting tracheal cartilage, widening of the posterior membranous wall, and reduced anterior-posterior airway caliber. These factors cause tracheal collapse, especially during times of increased airflow, such as coughing, crying, or feeding.[1, 2, 3] Tracheomalacia most commonly affects the distal third of the trachea and can be associated with various congenital anomalies, including cardiovascular defects, developmental delay, gastroesophageal reflux, and tracheoesophageal fistula.

Tracheomalacia can be categorized into 3 groups based on histological, endoscopic, and clinical presentation.

Problem

Tracheomalacia is a structural abnormality of the tracheal cartilage allowing collapse of its walls and airway obstruction. A deficiency and/or malformation of the supporting cartilage exists, with a decrease in the cartilage-to-muscle ratio.

Immaturity of the tracheobronchial cartilage is thought to be the cause in type I, whereas degeneration of previously healthy cartilage is thought to produce other types. Inflammatory processes, extrinsic compression from vascular anomalies, or neoplasms may produce degeneration.

Diffuse malacia of the airway of the congenital origin improves by age 6-12 months as the structural integrity of the trachea is restored gradually with resolution of the process. See the images below.


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Healthy trachea is visualized endoscopically.


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This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.

Epidemiology

Frequency

All types of tracheomalacia are extremely rare; no definite incidence rates are available.[4]

In a total of 512 bronchoscopies, airway malacia was diagnosed in 160 children (94 males) at a median age of 4.0 years (range, 0-17 y). Airway malacia was classified as primary in 136 children and as secondary in 24 children. The incidence of primary airway malacia was estimated to be at least 1 in 2,100.[5]

Etiology

Tracheomalacia can be associated with a variety of congenital anomalies, including cardiovascular defects, developmental delay, esophageal anomalies, and gastroesophageal reflux. Tracheomalacia can be caused by a diffuse process of congenital origin or by a localized abnormality such as a vascular ring, anomalous innominate artery, esophageal atresia,[1] and tracheoesophageal fistula. Internal compression by an endobronchial or tracheostomy tube also may be the culprit. Tracheal cartilage deficiency may be present in 75% of the patients with tracheoesophageal fistula. Tracheomalacia rarely is found in combination with laryngomalacia.

The entire cartilaginous structure of the upper airway is diffusely involved in congenital abnormality, or a localized area of decreased rigidity may be observed secondary to abnormal development of foregut and vasculature in embryonic life. A vascular ring around the trachea does not allow normal development in that area of trachea, and tracheomalacia is observed in the area of impingement.

The cases of acquired tracheomalacia occur with increasing frequency both in children and in adults, and the tracheomalacia often is not recognized clearly. These lesions usually cause focal tracheomalacia and may result from indwelling tracheostomy[6] and endobronchial tube, chest trauma, chronic tracheobronchitis, and inflammation (relapsing polychondritis). They may be secondary to pulmonary resection and tracheal malignancy (cylindroma), and they may be idiopathic.

Classification of adult tracheomalacia[2]

Pathophysiology

Tracheomalacia most commonly affects the distal third of the trachea. By virtue of its intrinsic flexibility, or compliance, the trachea changes caliber during the respiratory cycle. Tracheal dilatation and lengthening occurs during inspiration; narrowing and shortening occurs during expiration. Accentuation of this cyclic process may cause excessive narrowing of tracheal lumen, thus deforming the entire length or a localized segment. However, it is rarely found in combination with laryngomalacia because of the separate developmental pathways for the trachea and the larynx.

In general, abnormal collapsibility denotes a loss of structural rigidity, such as softening, better expressed as abnormally increased compliance. Any disease process affecting the integrity of the tracheal wall is apt to cause a change in tracheal compliance. The anatomic defect may be trivial or even may escape detection. The functional interference with ventilation may cause expiratory flow obstruction and interfere with clearance of secretions.

Functional impairment is proportional to the length of the involved segment and the degree of stenosis. Furthermore, kinking may occur at the transition between healthy tracheal wall and the indurated segment, as well as in the malacic segment. In diffuse tracheal disease or extensive peritracheal adhesions, the trachea usually distends unevenly during inspiration and collapses during expiration, thus interfering with the tracheal function.

Presentation

History

Physical

Differential Diagnosis

The differential diagnosis of tracheomalacia includes laryngomalacia, subglottic stenosis, congenital cysts, vocal cord paralysis, and hypocalcemic tetany. Complications include problems with acute airway obstruction and perioperative morbidity and mortality.

According to a recent study by Boogaard in 2005, when pediatric pulmonologists diagnosed airway malacia (based on symptoms, history, and lung function) prior to bronchoscopy, a correct diagnosis was made in 74% of the cases. However, in 52% of the diagnoses of airway malacia, the diagnosis was not suspected prior to bronchoscopy. The children with tracheomalacia present with atypical and variable clinical features; considerable overlap occurs with features of allergic asthma.[5, 8]

Indications

Surgical therapy is indicated when conservative measures fail. 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.

Relevant Anatomy

Contraindications

Most infants who have mild-to-moderate symptoms should be offered conservative therapy because these patients improve by age 18-24 months.[6, 9]

Imaging Studies

Other Tests

Diagnostic Procedures

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

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.[6, 12] Surgery only is recommended for severe symptoms and failure of conservative therapy.[6, 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.[13]

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.[14] 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.[15]

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.[16] 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.[17] 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.[14]

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, see eMedicineHealth'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.

Outcome and Prognosis

With conservative measures, the symptoms often resolve spontaneously by age 18-24 months.[2]

Tracheostomy has been used to stent the airway until natural maturation of cartilage occurs. This often imposes a heavy penalty on the child; therefore, treatment alternatives should be explored.

Aortopexy has proven to be a safe, expedient way to relieve the problem of tracheomalacia in most patients. The success of aortopexy has been reported at about 75% in several small studies. Aortopexy has less long-term morbidity than tracheostomy. While not altering the structural characteristics of the tracheal wall, it widens the anterior-posterior tracheal dimension to maintain a patent lumen. The only treatment failures with aortopexy were patients with diffuse or proximal tracheal involvement.

Future and Controversies

Current recommendations for treatment of tracheomalacia include the following:

Author

Daniel S Schwartz, MD, FACS, Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Disclosure: Nothing to disclose.

Specialty Editors

Richard Thurer, MD, B and Donald Carlin Professor of Thoracic Surgical Oncology, University of Miami, Leonard M Miller School of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Shreekanth V Karwande, MBBS, Chair, Professor, Department of Surgery, Division of Cardiothoracic Surgery, University of Utah School of Medicine and Medical Center

Disclosure: Nothing to disclose.

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Disclosure: Nothing to disclose.

Chief Editor

Mary C Mancini, MD, PhD, Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Disclosure: Nothing to disclose.

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Healthy trachea is visualized endoscopically.

This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.

Lateral chest radiograph shows excessive tracheal narrowing.

This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.

A 58-year-old woman with a history of polychondritis presented with inspiratory stridor and respiratory difficulties. The chest radiograph shows narrowing of the distal trachea on bronchoscopy. More than a 50% decrease in tracheal lumen occurred during expiration (see CT images).

The CT scan of a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties shows tracheal narrowing of the distal trachea.

CT image showing tracheal narrowing in a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties.

A 3-dimensional reconstruction of CT scan images confirms the presence of tracheomalacia in a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties.

Lateral chest radiograph shows excessive tracheal narrowing.

This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.

The mechanism of tracheal narrowing is shown here in healthy cases and in cases of tracheomalacia. Adapted from Feist JH, et al. Chest 68:3, Sept, 1975.

Healthy trachea is visualized endoscopically.

A 58-year-old woman with a history of polychondritis presented with inspiratory stridor and respiratory difficulties. The chest radiograph shows narrowing of the distal trachea on bronchoscopy. More than a 50% decrease in tracheal lumen occurred during expiration (see CT images).

The CT scan of a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties shows tracheal narrowing of the distal trachea.

CT image showing tracheal narrowing in a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties.

A 3-dimensional reconstruction of CT scan images confirms the presence of tracheomalacia in a 58-year-old woman with a history of polychondritis who presented with inspiratory stridor and respiratory difficulties.

Patterns of upper airway obstruction are presented here. Patient A has fixed upper airway obstruction. Patient B has variable extrathoracic obstruction, eg, vocal cord dysfunction. Patient C has variable intrathoracic obstruction, eg tracheomalacia.

A flow volume loop shows a pattern of variable extrathoracic obstruction. Truncation of the expiratory limb is present. As the pleural pressure exceeds the airway pressure, airway collapse occurs due to flow limitation during expiration and not during inspiration.

A flow volume loop shows the classic pattern of fixed upper airway obstruction. Truncation of both inspiratory and expiratory limbs is present.