Tracheomalacia: Symptoms, Types, Causes and Treatment

Tracheomalacia is a condition marked by abnormal weakness and increased collapsibility of the trachea, the main airway to the lungs. This can result in significant breathing difficulties, noisy breathing, and an array of symptoms that can be subtle or life-threatening. Tracheomalacia can affect both children and adults, is often misdiagnosed, and requires specialized evaluation and management. In this article, we will explore the symptoms, types, causes, and treatment options for tracheomalacia, synthesizing insights from recent research and clinical practice.

Symptoms of Tracheomalacia

Tracheomalacia presents with a wide spectrum of symptoms, from mild respiratory noises to severe, life-threatening events. Recognizing these signs is crucial for timely diagnosis and management, especially since the symptoms often mimic other more common respiratory conditions like asthma or bronchitis.

Symptom	Description	Prevalence/Severity	Sources
Cough	Persistent, barking or brassy	Common, often chronic	1 2 4 6
Stridor	Noisy, harsh breathing (expiratory)	Frequent in children	1 2 4 5
Dyspnea	Shortness of breath	May be intermittent or severe	2 3 7 8
Recurrent Infections	Frequent respiratory tract infections	Indicative of airway compromise	4 6 8
Respiratory Distress	Difficulty breathing, cyanosis	Severe cases, may be episodic	1 4 10
Wheezing	Whistling sound during breathing	Common, may mimic asthma	3 6 8
Hemoptysis	Coughing up blood	Less common, adults	2
Life-Threatening Events	ALTE/BRUE, "dying spells"	Severe, often in infants	1 4 10 12

Table 1: Key Symptoms of Tracheomalacia

Common Respiratory Signs

Tracheomalacia often starts with subtle symptoms, such as a chronic, barking cough or noisy breathing, especially during expiration. These sounds may be more pronounced when the child is crying, feeding, or agitated, and in adults, may worsen with exertion or during respiratory infections 1 4 5 6.

Severe Presentations

In more severe forms, particularly in infants and young children, tracheomalacia can cause acute episodes of respiratory distress, cyanosis (bluish discoloration), or even apparent life-threatening events (ALTE) and brief resolved unexplained events (BRUE). These episodes often require immediate medical attention 1 4 10 12.

Other Associated Symptoms

• Recurrent respiratory infections: The weakened airway makes it easier for pathogens to invade, leading to frequent bronchitis or pneumonia 4 6 8.
• Wheezing and dyspnea: These symptoms closely mimic asthma and are a common reason for misdiagnosis 3 6 8.
• Hemoptysis: Though less common, adults may experience blood-tinged cough due to airway trauma 2.
• Sputum production: Particularly noted in adults, chronic sputum is often associated with underlying infection or inflammation 2 7.

When to Suspect Tracheomalacia

Because the symptoms overlap with several common respiratory illnesses, tracheomalacia should be considered in patients—especially children—with persistent or unexplained respiratory symptoms, recurrent infections, or poor response to standard asthma therapies 2 3 6.

Types of Tracheomalacia

Understanding the different types of tracheomalacia is key to accurate diagnosis and tailored treatment. The types are generally divided by cause (congenital or acquired), location, and severity.

Type	Defining Features	Typical Population	Sources
Primary (Congenital)	Present from birth, structural anomaly	Infants, young children	1 5 6 13
Secondary (Acquired)	Develops after birth, external factors	Children, adults	2 5 7 8
Intrathoracic	Collapse within thoracic trachea	Infants, children	5
Extrathoracic	Collapse above thoracic inlet	Infants, children	5
Localized	Limited segment of trachea affected	Any age	4 7 9
Diffuse	Extensive airway involvement	Any age	4 6
Tracheobronchomalacia	Trachea and main bronchi involved	Severe pediatric/adult	4 6 12

Table 2: Types of Tracheomalacia

Primary (Congenital) Tracheomalacia

This form is present from birth and results from incomplete or abnormal development of the tracheal cartilage or posterior membrane. It is often associated with other congenital anomalies, most notably esophageal atresia and tracheoesophageal fistula 1 5 6 13. Congenital cases may resolve as the child grows and airway structures mature, but severe cases can persist or worsen without intervention.

Secondary (Acquired) Tracheomalacia

Secondary tracheomalacia develops after birth due to external factors such as trauma, chronic inflammation, prolonged intubation, infections, surgical interventions, or compressive lesions (like vascular rings or tumors). Adults are more likely to develop acquired tracheomalacia, though it can also occur in children with underlying risk factors 2 5 7 8.

Anatomical Subtypes

• Intrathoracic tracheomalacia: Collapse occurs within the chest, especially during exhalation or periods of increased intrathoracic pressure 5.
• Extrathoracic tracheomalacia: Collapse happens above the sternal notch, typically during inspiration 5.
• Localized vs. Diffuse: Localized forms affect a small segment, often due to post-surgical changes or external compression. Diffuse types involve longer segments or even the entire trachea and main bronchi (tracheobronchomalacia) 4 7 9 12.

Tracheobronchomalacia

When the main bronchi are involved along with the trachea, the condition is termed tracheobronchomalacia (TBM). This is usually more severe and challenging to treat 4 6 12.

Causes of Tracheomalacia

The underlying causes of tracheomalacia are diverse, ranging from congenital defects to acquired injuries and chronic inflammation.

Cause	Mechanism/Description	Typical Age Group	Sources
Congenital Anomalies	Abnormal tracheal cartilage/posterior membrane	Infants, children	1 5 6 13
Esophageal Atresia/TEF	Associated congenital conditions	Infants	1 10 13
Vascular Compression	Anomalous vessels compressing trachea	Children, adults	9 10 13
Chronic Inflammation	Repeated infections, irritants	Adults, children	7 8
Prolonged Intubation	Mechanical trauma to airway	All ages	7 13
Airway Surgery	Postoperative weakening	Any age	7 9 13
External Trauma	Direct injury to trachea	Any age	7
Neoplasms	Tumor-related compression or invasion	Adults	7
Chronic Vaping/Smoking	Irritant-induced inflammation	Adults	7 8
Idiopathic	Unknown causes	Any age	10

Table 3: Causes of Tracheomalacia

Congenital Causes

Most pediatric cases are due to congenital anomalies in the tracheal structure—either the cartilage rings are softer than normal or the posterior membrane is excessively floppy. Commonly, congenital tracheomalacia is seen with esophageal atresia and tracheoesophageal fistula, where up to 44% of cases are associated 1 5 6 10 13.

Acquired Causes

In older children and adults, tracheomalacia is often acquired:

• Chronic inflammation: Repeated infections or irritant exposure (such as cigarette smoke, industrial pollutants, or vaping) can weaken the tracheal wall 7 8.
• Prolonged intubation or tracheostomy: Extended mechanical ventilation or airway devices can damage and soften the tracheal cartilage 7 13.
• External trauma or surgery: Direct injury or surgical interventions can lead to localized weakening 7 9 13.
• Vascular anomalies: Abnormal blood vessels or conditions like a kyphotic cervical spine can compress the trachea, leading to focal tracheomalacia 9 10 13.
• Neoplasms: Tumors may invade or compress the airway, causing secondary weakness 7.
• Idiopathic: In some cases, no clear cause is identified 10.

Emerging Causes

Recent reports highlight vaping as a possible risk factor for acquired tracheomalacia, particularly in young adults, due to chronic airway inflammation and recurrent infections 8.

Treatment of Tracheomalacia

Treatment strategies for tracheomalacia depend on the severity, underlying cause, and patient age. Approaches range from conservative observation to advanced surgical interventions.

Treatment Option	Indication/Description	Outcomes/Considerations	Sources
Observation	Mild, non-progressive cases	Most resolve with time	1 4 13
Medical Therapy	Cholinergic agents, antibiotics	Symptomatic relief	5 8
Positive Pressure Ventilation	Non-invasive ventilation (CPAP/BiPAP)	Stabilizes airway, avoids surgery	5 8 11
Airway Stenting	Severe, focal cases; not always feasible	Risk of complications	6 9 10
Tracheostomy	Bypass severe obstruction	May cause further malacia	4 13
Aortopexy	Surgical suspension of aorta to relieve tracheal compression	High success in severe cases	10 12 13
Tracheopexy (posterior/anterior)	Surgical fixation of trachea	Effective for posterior collapse	4 11
Airway Reconstruction/Splinting	Surgical repair, external splints	For refractory/severe cases	12 13
Fundoplication	For associated gastroesophageal reflux	Improves outcomes in select pts	10

Table 4: Treatments for Tracheomalacia

Conservative and Medical Management

Most children with mild tracheomalacia improve as their airway matures; thus, observation and supportive care are preferred in these cases 1 4 13. Medical therapy may include bronchodilators, cholinergic agents, and prompt treatment of respiratory infections 5 8.

Positive Pressure Ventilation

Non-invasive positive airway pressure (like CPAP or BiPAP) helps splint the airway open during breathing, reducing symptoms and potentially avoiding surgery, especially in adults or high-risk surgical candidates 5 8 11.

Surgical Interventions

• Aortopexy: The most common surgical option for severe cases, especially those with anterior tracheal compression. Aortopexy involves suspending the aorta to the sternum, pulling the trachea forward and relieving airway collapse. This has a high success rate (>80%) in appropriately selected patients 10 12 13.
• Posterior Tracheopexy: For cases with posterior membranous intrusion, posterior tracheopexy directly addresses the weakened posterior wall, improving airway patency and symptoms 4 11.
• Airway Stenting and Reconstruction: Airway stents may be used in select cases but carry a higher risk of complications, morbidity, and mortality. Surgical splinting or reconstruction is reserved for the most severe or refractory cases 6 9 10 12 13.

Special Considerations

• Tracheostomy: While it can bypass a severely collapsed segment, it may worsen tracheomalacia or cause additional complications and is generally reserved for life-threatening, refractory cases 4 13.
• Associated Gastroesophageal Reflux: In children with both tracheomalacia and reflux, surgical correction (fundoplication) may be required to optimize respiratory outcomes 10.

Outcomes

With appropriate therapy, most patients—especially children—see significant improvement or resolution of symptoms. Surgical approaches like aortopexy and tracheopexy have good success rates, though complications can occur. Severe tracheobronchomalacia remains challenging to treat, with no perfect solution yet devised 10 11 12 13.

Conclusion

Tracheomalacia is a complex disorder that can significantly impact breathing and quality of life. Early recognition and tailored management are essential for optimal outcomes.

Key Points:

• Tracheomalacia causes a wide range of respiratory symptoms, from mild cough to life-threatening events, often mimicking other common diseases.
• There are several types: primary (congenital), secondary (acquired), intrathoracic, extrathoracic, localized, diffuse, and tracheobronchomalacia.
• Causes include congenital defects, vascular anomalies, chronic inflammation, prolonged intubation, trauma, and more.
• Diagnosis is best confirmed by bronchoscopy, with supportive imaging and tests.
• Treatment depends on severity and cause, ranging from observation to advanced surgical intervention (aortopexy, tracheopexy, airway stenting).
• Most mild cases in children resolve with time, while severe cases may require multidisciplinary management.

Understanding tracheomalacia's symptoms, types, causes, and evidence-based treatments empowers patients, families, and clinicians to seek timely and effective care.