eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Tracheomalacia

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Updated: Aug 27, 2009

Introduction

Background

Tracheomalacia is an abnormal collapse of the tracheal walls. It may occur in an isolated lesion or can be found in combination with other lesions that cause compression or damage of the airway. Tracheomalacia is usually benign, with symptoms due to airway obstruction. As such, this condition is often mistaken for chronic asthma or prolonged bronchiolitis.

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

Pathophysiology

Tracheomalacia may occur as a primary lesion, in which case the cartilage of the trachea develops abnormally. This results in tracheal walls that are soft and collapse during respiration. The collapse causes airflow obstruction and wheezing, stridor, or both. If the lesion is extrathoracic, the collapse and airway sounds occur primarily during inspiration. If the lesion is intrathoracic, the collapse and airway sounds occur primarily during exhalation. Because most of the trachea is intrathoracic, exhalatory collapse accounts for most cases of tracheomalacia.

Tracheomalacia may also be found in conjunction with lesions that compress the airway, such as mediastinal masses, vascular slings, and vascular rings. It also occurs with increased frequency in children with chronic inflammation of the proximal airways. Less common in asthma, this etiology of tracheomalacia is more often seen in children with chronic lung disease of infancy, gastroesophageal reflux, or other forms of chronic aspiration.

Primary tracheomalacia is sometimes referred to as type 1, tracheomalacia associated with extrinsic compression is sometimes referred to as type 2, and tracheomalacia associated with intra-airway irritation/inflammation is sometimes referred to as type 3.

Tracheomalacia is frequently found after repair of a tracheoesophageal fistula and may cause significant symptoms for several years after the repair.

Frequency

United States

The frequency of tracheomalacia is unclear. The condition appears to primarily derive from a developmental defect in the cartilage of the tracheal wall. Therefore, the lesion usually occurs in infants and young children. It is frequently found in children who have undergone repair of a tracheoesophageal fistula, chronic lung disease of infancy, vascular compression of the airway, or mediastinal masses of sufficient firmness to compress the airway. Children with gastroesophageal reflux, or aspiration from above, have an increased incidence of tracheomalacia. The problem in this last situation is trying to decide which condition is the cause and which is the effect.

International

Data from the Sophia Children's Hospital in Rotterdam (southwest Netherlands), the only facility performing bronchoscopy in children, suggest an incidence rate of 1 case per 2100 newborns.¹

Mortality/Morbidity

Morbidity and mortality are extremely rare. On occasion, tracheomalacia causes enough obstruction to necessitate intervention. This obstruction generally takes the form of episodic severe airway obstruction causing cyanosis. When infants with chronic lung disease of infancy become irritated, they may have what has been called a "BPD fit." This episode usually involves a cry, with either a breath hold or with a sufficient increase in intrathoracic pressure to partially occlude the airway. If the child has tracheomalacia, the frequency and severity of these episodes is often increased.

Race

No racial predilections are known.

Sex

No sex predilections are known.

Age

Because most cases of tracheomalacia appear to be related to a developmental defect in the cartilage of the tracheal wall, the lesion typically occurs in infants and young children. In most children, the tracheal cartilage normalizes, the airway enlarges, and symptoms resolve by 3 years of age (in many before age 1 y).

Because tracheoesophageal fistula is usually repaired early in life, the associated tracheomalacia also appears in early infancy, usually shortly after surgery.

If the tracheomalacia is a result of compression, the patient's age at presentation depends on the cause of compression. Vascular rings, present from birth, cause tracheomalacia early in life. Other causes of compression, especially tumors, occur later in life.

Clinical

History

• The history of a patient with tracheomalacia typically includes a wheeze that usually begins when the individual is aged 4-8 weeks. Although the congenital lesion is present before this time, airflow is apparently insufficient during the first few weeks of life to generate abnormal sounds. The wheeze generally increases with activity and colds and decreases during quiet periods.
• At first, wheezing may be mistaken for a sign of bronchiolitis, especially during respiratory syncytial virus season. As wheezing continues, it may be mistaken for that of asthma or cystic fibrosis. If bronchodilator therapy has been attempted, the history usually indicates no improvement; however, in some infants, tracheomalacia worsens with bronchodilator therapy.
• Unlike most infants with asthma or cystic fibrosis, infants with tracheomalacia are described as "happy wheezers" (ie, they maintain normal oxygenation and disposition and who grow and develop normally, despite the chronic airway noises).

Physical

• The physical findings suggest the diagnosis.
• The baby looks well and happy despite the chronic wheeze.
• Vital signs, including the patient's respiratory rate and oxygen saturation, are normal.
• Lungs have good air entry. The typical abnormality is of a wheeze throughout exhalation that sounds the same in all lung fields. It is a coarse monophonic exhalatory sound, which has been described as exhalatory stridor. No asymmetry to the breath sounds is present. On occasion, the lesion is intrathoracic, and the airway sounds occur in inspiration.
• The patient's voice and cry are normal, unless gastroesophageal reflux is present.
• Because some children have concomitant laryngomalacia, abnormal sounds may be heard on inspiration.

Causes

• As far as tracheomalacia is understood, most cases are isolated and idiopathic. A recent study proposed a possible neurologic etiology for tracheomalacia.²
• Transient defects in tracheal cartilage development are assumed to be the cause of this condition. This is sometimes referred to as type 1 tracheomalacia.
• Autopsy data are lacking, and no animal model is noted.
• Some children with tracheomalacia have the lesion because of vascular anomalies or other causes of compression of the airway. This is referred to as type 2 tracheomalacia.
• Tracheomalacia is a common finding after repair of a tracheoesophageal fistula.
• Tracheomalacia may occur with and complicate other disorders, including gastroesophageal reflux disease, other forms of recurrent aspiration, and bronchopulmonary dysplasia (chronic lung disease of infancy).

Differential Diagnoses

Other Problems to Be Considered

Anomalous innominate artery
Complete tracheal rings
Tracheal stenosis
Williams-Campbell syndrome

Workup

Laboratory Studies

• Oxygen saturation should be assessed in tracheomalacia.

Imaging Studies

• Fluoroscopy and radiography
  • The diagnosis can often be confirmed by performing fluoroscopy of the airway.
  • During the study, the pediatric radiologist observes the airway collapsing and opening up. This finding may appear on anteroposterior images, on which the tracheal air shadow seems to disappear and reappear with respirations in the affected area of the trachea.
  • The lesion may be observed most easily on lateral images, on which the anterior and posterior walls of the trachea may be observed apposing dynamically.
  • Loss of the tracheal air shadow with no respiratory changes suggests static collapse of the airway.
• CT scanning
  • Chest CT scanning may reveal the abnormal shape of the trachea as the apposition of the walls.
  • "Expiratory frown" shapes are observed in CT scans when the area is scanned during an exhalation or cough.³
  • Chest CT scanning may not reveal the dynamic nature of the lesion and may not depict the lesion at all.
  • A CT scan, especially one obtained with contrast material, may reveal vascular lesions that mimic tracheomalacia and that may lead to tracheomalacia.
• Esophagraphy
  • Esophagraphy can be performed to evaluate for a vascular anomaly, for a bronchogenic cyst, or for esophageal duplication.
  • Esophagraphy does not assist the physician in making a definitive diagnosis.

Other Tests

• The definitive method of diagnosis is bronchoscopy.
  • In one series, when a pediatric pulmonologist suspected tracheomalacia, the correct diagnosis was tracheomalacia in 74% of the cases.¹ However, tracheomalacia was not suspected in 52% of patients who were eventually diagnosed with it.
  • During bronchoscopy, the anterior wall can be observed to collapse against the posterior wall. At the same time, other lesions can be diagnosed or excluded under direct visualization.
  • The authors prefer flexible bronchoscopy to confirm the diagnosis. With flexible bronchoscopy, the baby is breathing spontaneously with light anesthesia. This method preserves respiratory dynamics better than rigid bronchoscopy. In addition, the rigid bronchoscope may serve as a stent to the airway, masking the diagnosis.
• Confirming the diagnosis is important for differential diagnosis. Otherwise, one or more tests may be unnecessarily considered.
  • A sweat test may be performed to reduce the likelihood of cystic fibrosis.
  • Esophagraphy may be performed to eliminate the likelihood of most of the vascular anomalies. These diagnoses are usually eliminated by bronchoscopy.
  • CT scanning to reduce the likelihood of airway foreign body and other lesions that may be compromising the airway. If bronchoscopy is performed, it is an efficient and direct method of looking for foreign bodies.
• If tracheomalacia is diagnosed, a sweat test is not necessary unless other manifestations of cystic fibrosis are present. The adage still holds that a sweat test has no contraindication.
• If gastroesophageal reflux is a concern, evaluate this condition by using a pH probe or esophageal biopsy. If bronchoscopy is performed, bronchoalveolar lavage can be done to assess for lipid-laden macrophages.

Treatment

Medical Care

• After the diagnosis of tracheomalacia is made, the most effective and safest treatment is allowing time to pass ("tincture of time").
• Bronchodilators do not help and sometimes worsen the tracheomalacia.
  • The tone of the smooth muscle helps stent the airway.
  • Administering a beta-agonist relaxes the smooth muscle and may worsen collapse of the airway.
• If the child is having difficulty with retained secretions, chest physiotherapy may be helpful.
• If gastroesophageal reflux is present, appropriate pharmacotherapy should be considered.
• On occasion, systemic corticosteroids are used when the baby has increased symptoms during an acute respiratory tract infection.
  • These drugs should be reserved for episodes in which the tracheomalacia interferes with the child's oral intake or disposition or when the child develops respiratory difficulty.
  • If the child is making more noise but is otherwise doing well, steroids can usually be avoided.
• Continuous positive airway pressure or bilevel positive airway pressure provided by means of tight-fitting face or nasal mask, endotracheal tube, or tracheostomy tube can provide relief from severe obstruction.

Surgical Care

• Surgery may be an option when the baby has one or all of the following:
  • Difficulty gaining weight and developing
  • Recurrent pneumonia or apnea
  • Enough airway obstruction to require long-term airway support
• Tracheostomy can provide internal stenting of the trachea in babies with any of the findings above. With time and growth, the airway obstruction resolves, and the cannula can be removed from the infant.
• In aortopexy, the aortic arch is lifted off the trachea. This has resulted in symptomatic improvement in many children.
• Recent success with metal stents in young children has been described.⁴
• Repair of vascular rings and slings can be done to decompress the trachea and allow healing to begin.

Consultations

• Because the expiratory noise has clinically significant differential diagnoses, refer the baby whose wheeze does not remit with good asthma therapy to a pediatric pulmonologist.
• Bronchoscopy ensures a definitive diagnosis in an infant who is otherwise well. A sweat test, esophagraphy, echocardiography, and/or chest CT scanning may otherwise be necessary.

Diet

• No dietary restrictions or changes are necessary.

Activity

• No restrictions or changes are necessary.
• The abnormal airway dynamics may persist even after the clinical findings have remitted. If symptoms persist with exercise as the patient ages, an exercise test is indicated to make sure that minute ventilation increases appropriately with exercise.

Medication

After the diagnosis of tracheomalacia is made, the most effective and safest treatment is the passage of time. Bronchodilators (eg, albuterol) usually do not help and may worsen tracheomalacia in some infants. The tone of the smooth muscle presumably stents the airway in some babies. Administering a beta-agonist relaxes these muscles and may worsen airway collapse.

Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Babies who have respiratory difficulties during a concomitant upper respiratory tract infection may respond favorably to systemic corticosteroids. If the baby is noisier than usual but acting and eating normally, do not use medications.

Prednisolone (Orapred, Prelone, Pediapred)

May decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) activity. Available in tab and syr; prednisolone syr tastes better than prednisone syr.

Dosing

Adult

5-60 mg/d PO qd or divided bid/tid/qid

Pediatric

2 mg/kg/d PO divided bid for 3-7 d; lower dose as quickly as possible to reduce adverse effects and complications; if used >10 d, attempt dosing qod (must gradually taper for discontinuation)

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, carbamazepine, or rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Prednisone (Deltasone, Meticorten, Orasone)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Dosing

Adult

5-60 mg/d PO qd or divided bid/tid/qid

Pediatric

2 mg/kg/d PO divided bid for 3-7 d; lower dose as quickly as possible to reduce adverse effects and complications; if used >10 d, attempt dosing qod (must gradually taper for discontinuation)

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, carbamazepine, or rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Follow-up

Further Inpatient Care

• Admission is not necessary unless the baby with tracheomalacia is having respiratory distress.

Further Outpatient Care

• No specific therapy or precautions are needed.
• Provide outpatient care if bronchodilators are considered because they worsen the condition in some patients.
• Follow the normal immunization schedule.
• Reassurance during acute respiratory illnesses may be necessary. Babies may need to be seen frequently during these illnesses. During an upper respiratory infection, the normal cough sounds more crouplike in these children because the walls of the trachea appose during the cough. This also causes irritation in the tracheal walls, which prolongs the cough. The added pressures to overcome nasal obstruction adds to the dynamic collapse during the infection. As long as the baby is able to achieve adequate oral intake and is acting normally, intervention is usually not necessary.

Inpatient & Outpatient Medications

• No long-term medications are required.
• Consider systemic corticosteroids during a respiratory tract infection if the baby is having difficulty breathing.

Transfer

• If the baby is having severe respiratory distress, transfer him or her to a pediatric intensive care unit.

Complications

• Severe obstruction requiring acute intervention with mechanical ventilation or positive pressure
• Chronic obstruction necessitating surgical intervention (eg, tracheostomy, stent placement, aortopexy)
• Aortopexy and stent placement have been compared over a 10-year followup.⁵ Both are equally effective in improving symptoms and allowing for normal growth and development. Aortopexy is associated with more perioperative complications, whereas stents are associated with long-term complications and the need for removal.

Prognosis

• The prognosis is excellent. Most patients outgrow this condition by the time they are aged 3 years; many infants outgrow tracheomalacia before they are aged 1 year.
• If gastroesophageal reflux is present, attention to this speeds healing.
• Tracheomalacia after tracheoesophageal fistula repair may take longer to heal than primary tracheomalacia.
• Tracheomalacia after a compressing lesion lasts longer, depending on the length of time of the compression.

Patient Education

• For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.

Miscellaneous

Medicolegal Pitfalls

• If present, gastroesophageal reflux may worsen the tracheomalacia and vice versa.
• Overdiagnosis of asthma may be harmful. Frequent use of beta-agonists may not be helpful because they often fail to provide relief, they increase the risk of drug adverse effects, and/or they worsen the tracheomalacia by relaxing the airway smooth muscle that supports the airway.

Multimedia

Media file 1: This shows the trachea during inspiration and expiration. Tracheal collapse of more than 50% during expiration is diagnostic of tracheomalacia.

References

1. Boogaard R, Huijsmans SH, Pijnenburg MW, et al. Tracheomalacia and bronchomalacia in children: incidence and patient characteristics. Chest. Nov 2005;128(5):3391-7. [Medline].

2. Jamal N, Bent JP, Vicencio AG. A neurologic etiology for tracheomalacia?. Int J Pediatr Otorhinolaryngol. Jun 2009;73(6):885-7. [Medline].

3. Boiselle PM, Ernst A. Tracheal morphology in patients with tracheomalacia: prevalence of inspiratory lunate and expiratory "frown" shapes. J Thorac Imaging. Aug 2006;21(3):190-6. [Medline].

4. Vinograd I, Keidar S, Weinberg M, Silbiger A. Treatment of airway obstruction by metallic stents in infants and children. J Thorac Cardiovasc Surg. Jul 2005;130(1):146-50. [Medline].

5. Valerie EP, Durrant AC, Forte V, et al. A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: is it better than aortopexy?. J Pediatr Surg. Jun 2005;40(6):904-7; discussion 907. [Medline].

6. Austin J, Ali T. Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management. Paediatr Anaesth. Jan 2003;13(1):3-11. [Medline].

7. Casiano RR, Numa WA, Nurko YJ. Efficacy of transoral intraluminal Wallstents for tracheal stenosis or tracheomalacia. Laryngoscope. Oct 2000;110(10 Pt 1):1607-12. [Medline].

8. Dave S, Currie BG. The role of aortopexy in severe tracheomalacia. J Pediatr Surg. Mar 2006;41(3):533-7. [Medline].

9. Furman RH, Backer CL, Dunham ME, et al. The use of balloon-expandable metallic stents in the treatment of pediatric tracheomalacia and bronchomalacia. Arch Otolaryngol Head Neck Surg. Feb 1999;125(2):203-7. [Medline].

10. Inoue K, Yanagihara J, Ono S, et al. Utility of helical CT for diagnosis and operative planning in tracheomalacia after repair of esophageal atresia. Eur J Pediatr Surg. Dec 1998;8(6):355-7. [Medline].

11. Masters IB, Chang AB. Interventions for primary (intrinsic) tracheomalacia in children. Cochrane Database Syst Rev. 2005;CD005304. [Medline].

12. Panitch HB, Keklikian EN, Motley RA, et al. Effect of altering smooth muscle tone on maximal expiratory flows in patients with tracheomalacia. Pediatr Pulmonol. 1990;9(3):170-6. [Medline].

13. Vinograd I, Filler RM, Bahoric A. Long-term functional results of prosthetic airway splinting in tracheomalacia and bronchomalacia. J Pediatr Surg. Jan 1987;22(1):38-41. [Medline].

14. Yalcin E, Dogru D, Ozcelik U, et al. Tracheomalacia and bronchomalacia in 34 children: clinical and radiologic profiles and associations with other diseases. Clin Pediatr (Phila). Nov-Dec 2005;44(9):777-81. [Medline].

Keywords

tracheomalacia, tracheal wall collapse, airway obstruction, airway compression, compression of the airway, asthma, chronic asthma, bronchiolitis, vascular ring and sling, vascular sling and ring, tracheoesophageal fistula, gastroesophageal reflux, GER, gastroesophageal reflux disease, GERD, wheeze, wheezing, happy wheezer, treatment, diagnosis

Contributor Information and Disclosures

Author

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Novartis Honoraria Consulting; Altus  Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting; Gilead  Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center
Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

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