Breathing Easy: Dr. Cable’s Guide to Pediatric Airway Disorders

Pediatric airway disorders encompass a range of conditions that can obstruct or damage a child’s breathing passages, which include the larynx (voice box), trachea (windpipe), and bronchi (the air passages into the lungs). These disorders can be congenital, meaning they are present at birth due to developmental anomalies, or they can be acquired later as a result of illness, injury, or medical interventions. One of the most common presenting symptoms across many of these conditions is stridor, which is a term for noisy breathing, often high-pitched, that results from turbulent airflow through a narrowed airway segment. Diagnosing and managing these often complex conditions requires specialized expertise.

The pediatric airway is notably different from that of an adult; it is significantly smaller in diameter and more pliable. Consequently, even relatively minor degrees of narrowing or inflammation can cause substantial breathing difficulty in an infant or young child⁴ This inherent vulnerability is why many airway disorders present early in life and can sometimes be quite serious, necessitating prompt evaluation and care by a pediatric ENT specialist. Furthermore, the impact of these disorders often extends beyond just breathing. Because the airway is intricately linked with functions like swallowing and voice production, significant airway obstruction can interfere with feeding, potentially leading to poor weight gain or failure to thrive, and can also affect voice quality and development.

Common Pediatric Airway Disorders: Causes, Symptoms, and What Parents Should Know

Several distinct conditions can affect a child’s airway. Understanding the specific disorder is the first step toward effective management. While many airway disorders can cause stridor, the specific characteristics of the noisy breathing (e.g., whether it occurs during inhalation, exhalation, or both) along with other associated symptoms like feeding difficulties, voice changes, or episodes of cyanosis (bluish discoloration of the skin), can provide important clues to the underlying cause and the precise location of the obstruction. This differentiation requires expert diagnostic skills and specialized tools.

A. Laryngomalacia

• Definition: Laryngomalacia is the most common congenital abnormality of the larynx and the most frequent cause of stridor in infants. It occurs when the soft, immature cartilages and tissues above the vocal cords (the supraglottic larynx) are floppy and collapse inward during inspiration (breathing in), partially obstructing the airway.
• Causes: The exact cause is often unknown, but it is thought to be related to delayed maturation of the laryngeal structures or a neuromuscular incoordination of the muscles supporting the larynx. Gastroesophageal reflux (GER), where stomach contents flow back into the esophagus and potentially irritate the larynx, may also play a role or can exacerbate the symptoms of laryngomalacia.
• Symptoms: The hallmark symptom is inspiratory stridor – a harsh, squeaky, or crowing sound heard when the baby breathes in. This noisy breathing often starts at birth or within the first few weeks of life and typically worsens over the initial several months. The stridor is characteristically louder when the baby is agitated, crying, feeding, sleeping on their back, or has an upper respiratory infection. Despite the noisy breathing, most infants with laryngomalacia have mild symptoms and do not experience significant trouble breathing or feeding, and they generally gain weight well.
  
  However, in more severe cases, symptoms can include:

• Noticeable difficulty breathing, with visible “tugging” or retractions at the neck, between the ribs, or at the stomach during inhalation.
• Feeding problems, such as choking, gagging, or prolonged feeding times.
• Poor weight gain or failure to thrive.
• Pauses in breathing (apnea).
• Cyanosis (a bluish tinge to the skin or lips due to low oxygen levels).
• Onset and Course: Symptoms usually appear from birth to a few weeks of age, may peak in severity around 4-8 months, and then typically begin to improve spontaneously. In most cases, laryngomalacia resolves on its own by the time the child is 12 to 18 months old as the laryngeal structures mature and firm up. 

B. Subglottic Stenosis (SGS)

• Definition: Subglottic stenosis refers to a narrowing of the airway in the subglottic region, which is the area just below the vocal cords and above the trachea. This is anatomically the narrowest part of a young child’s airway, making it particularly vulnerable to obstruction. The severity of SGS is often graded on a scale from 1 (mildest) to 4 (complete obstruction) based on the percentage of airway narrowing.
• Causes:

• Congenital SGS: This form is present at birth and results from abnormal development of the cricoid cartilage (the ring-shaped cartilage that forms the lower part of the larynx) during pregnancy. The cricoid cartilage may be abnormally small, elliptical (oval-shaped instead of round), or unusually thick, leading to a congenitally narrow subglottic lumen.
• Acquired SGS: This type develops after birth. The most common cause of acquired SGS in infants is prolonged endotracheal intubation (having a breathing tube placed in the airway), particularly in premature babies or those requiring mechanical ventilation for extended periods in the Neonatal Intensive Care Unit (NICU). The breathing tube can cause irritation, inflammation, and scarring in the delicate subglottic mucosa, leading to narrowing. Other causes of acquired SGS include direct trauma to the larynx or trachea, severe infections (like bacterial tracheitis), or chronic irritation from severe gastroesophageal reflux disease (GERD). Acquired airway disorders, such as SGS and tracheomalacia, are frequently linked to prematurity and the associated need for prolonged respiratory support, highlighting a potential iatrogenic (medically induced) component, even when such interventions are life-saving.
• Symptoms: Symptoms of SGS can vary depending on the grade of narrowing. Common signs include:

• Stridor: This is often biphasic, meaning it is heard during both inspiration (breathing in) and expiration (breathing out), though it can be primarily inspiratory.
• Shortness of breath or respiratory distress, especially during exertion (like crying or activity) or with an upper respiratory infection.
• Recurrent or prolonged episodes of croup (a harsh, barking cough with noisy breathing). Children with underlying SGS may experience more severe or frequent croup than other children.
• Difficulty feeding and poor weight gain or failure to thrive in severe cases.
• The voice may also sound abnormal or hoarse.
• Subglottic stenosis is a recognized indication for tracheotomy if severe and is a condition managed by pediatric airway specialists.

C. Tracheomalacia

• Definition: Tracheomalacia is a condition characterized by weakness or floppiness of the tracheal cartilage, the C-shaped rings that support the windpipe. This structural weakness allows the trachea to collapse or narrow excessively, particularly during exhalation, coughing, or crying, when intrathoracic pressure increases.
• Causes:

• Congenital (Primary) Tracheomalacia: This occurs when the tracheal cartilage does not develop properly in utero, resulting in an inherently weak or floppy windpipe. It can sometimes be associated with other congenital anomalies, such as esophageal atresia (a condition where the esophagus doesn’t connect properly to the stomach), tracheoesophageal fistula (an abnormal connection between the trachea and esophagus), heart defects, or certain genetic syndromes like Ehlers-Danlos syndrome.
• Acquired (Secondary) Tracheomalacia: This type develops after birth due to external compression of the trachea (e.g., by an abnormal blood vessel like a vascular ring, a tumor, or a cyst), chronic inflammation, prolonged intubation or tracheostomy, or as a consequence of previous surgery in the chest or on the esophagus.
• Symptoms: Symptoms can range from mild to severe:

• Noisy breathing, which may be described as a wheeze, rattle, or stridor. The noise may be more prominent during exhalation.
• A characteristic harsh, barking, or “seal-like” chronic cough, often referred to as a “tracheal bark.”
• Recurrent respiratory infections, such as bronchitis or pneumonia, because the collapsed airway makes it difficult to effectively clear secretions from the lungs.
• Exercise intolerance or increased breathing difficulty with activity.
• Breathing issues that worsen with feeding, crying, or coughing.
• In more severe cases: choking or cyanotic spells (turning blue) during feeding, apparent life-threatening events (ALTEs) or brief resolved unexplained events (BRUEs), difficulty swallowing (dysphagia), or failure to thrive.
• Onset and Course: Congenital tracheomalacia often becomes symptomatic within the first one to two months of life. Many mild cases tend to improve gradually as the child grows and the tracheal cartilage strengthens, often resolving by age two or three. 

D. Vocal Cord Paralysis

• Definition: Vocal cord paralysis occurs when one (unilateral) or both (bilateral) vocal cords are unable to move properly. The vocal cords are located within the larynx (voice box) and are essential for breathing, voice production (phonation), and protecting the airway during swallowing.
• Causes: Vocal cord paralysis can result from a variety of factors:

• Congenital: Some infants are born with vocal cord paralysis, which may be due to birth trauma (e.g., stretching of the recurrent laryngeal nerve during delivery), neurological conditions (like hydrocephalus or Arnold-Chiari malformation), or idiopathic (unknown cause).
• Acquired: Paralysis can occur due to injury to the recurrent laryngeal nerves, which control vocal cord movement. This injury can happen during surgical procedures (especially cardiothoracic surgery, thyroid surgery, or neck surgery), as a result of prolonged endotracheal intubation causing scarring or pressure on the nerves, from trauma to the neck or chest, due to viral infections affecting the nerves, or from tumors compressing the nerves.
• Symptoms: The symptoms depend largely on whether the paralysis is unilateral or bilateral:

• Unilateral Vocal Cord Paralysis: If only one vocal cord is paralyzed, the primary symptom is often a change in voice quality, such as hoarseness, a weak or breathy voice, or a soft cry in infants. There may also be some risk of aspiration (food or liquids entering the airway during swallowing) if the paralyzed cord cannot close effectively against the mobile one to protect the airway.
• Bilateral Vocal Cord Paralysis: If both vocal cords are paralyzed, particularly if they are stuck in a relatively closed position (median or paramedian), the main concern is airway obstruction. This can cause significant stridor (noisy breathing, often inspiratory), shortness of breath, and respiratory distress, especially with exertion. The voice, paradoxically, may be relatively normal or only mildly affected because the cords are close together. Aspiration is less common than with unilateral paralysis if the cords are fixed near the midline. Infants may present with a weak cry.  Bilateral vocal cord paralysis is a serious condition that can be life-threatening if the airway is severely compromised and is a recognized indication for tracheotomy. 

E. Other Airway Issues:

A variety of other less common congenital or acquired conditions can also cause airway obstruction in children. These include choanal atresia (blockage of the back of the nasal passage), laryngeal webs (a membrane across the larynx), laryngeal or tracheal cysts, subglottic hemangiomas (benign tumors made of blood vessels that can grow in the airway), and foreign bodies aspirated into the airway. 

Gastroesophageal reflux (GER/GERD) is a condition that frequently appears as a potential contributor or an exacerbating factor in several pediatric airway disorders. For example, in laryngomalacia, refluxed stomach acid can irritate the already sensitive laryngeal tissues, potentially worsening the stridor and inflammation. Similarly, GERD has been implicated as a factor that can contribute to the development or persistence of acquired subglottic stenosis by causing chronic inflammation and irritation in the subglottic area. This suggests that comprehensive management of some airway disorders may also involve addressing and treating GERD.

How Dr. Cable Diagnoses Pediatric Airway Disorders

Diagnosing pediatric airway disorders requires a meticulous and often multimodal approach, combining careful clinical assessment with specialized diagnostic procedures. The goal is to accurately identify the location, nature, and severity of the airway problem.

• Comprehensive Medical History: The evaluation begins with a detailed history, including the onset and specific characteristics of the symptoms (e.g., type of stridor, timing, triggers), feeding history (difficulties, choking, poor weight gain), birth history (gestational age, any complications, history of intubation or NICU stay), previous illnesses or surgeries, and overall growth and development.
• Physical Examination: A thorough physical examination includes observing the child’s breathing pattern at rest and during activity, noting the rate and effort of breathing, and looking for signs of respiratory distress such as nasal flaring, indrawing of the chest wall (retractions at the neck, between the ribs, or below the sternum), and cyanosis. Auscultation (listening with a stethoscope) of the chest and neck can help characterize airway sounds.
• Flexible Nasopharyngolaryngoscopy (NPL) or Flexible Laryngoscopy: This is a key diagnostic tool for evaluating the upper airway, particularly the nasal passages, pharynx (throat), and larynx (voice box). It is typically performed in the office while the child is awake and breathing spontaneously. A very thin, flexible tube with a light and camera at its tip (an endoscope) is gently passed through the child’s nostril into the airway. This allows for a dynamic assessment of the laryngeal structures and their movement during breathing and vocalization. It is particularly useful for diagnosing conditions like laryngomalacia (visualizing the collapse of supraglottic tissues during inspiration) and vocal cord paralysis (observing impaired vocal cord movement).
• Microlaryngoscopy and Bronchoscopy (MLB): This procedure involves examining the larynx, trachea, and bronchi (lower airways) using either rigid or flexible endoscopes while the child is under general anesthesia in an operating room setting. MLB provides a more detailed and stable view of the entire airway than NPL and allows for precise assessment and grading of conditions like subglottic stenosis or tracheomalacia. It also permits procedures such as biopsies, removal of lesions, or therapeutic interventions like dilation if indicated. The choice between an awake flexible laryngoscopy and an MLB under anesthesia depends on the suspected condition, the child’s age and clinical stability, and the specific information required for diagnosis and treatment planning.
• Imaging Studies:

• Chest and Neck X-rays: Standard X-rays can provide information about the overall structure of the chest and neck, and may reveal tracheal narrowing, foreign bodies, or other gross abnormalities.
• Computed Tomography (CT) Scan or Magnetic Resonance Imaging (MRI): These advanced imaging techniques provide detailed cross-sectional images of the airway and surrounding structures. They may be used for complex cases of stenosis, to evaluate for external compression of the airway (e.g., by blood vessels or masses), or to plan surgical reconstruction.

• Swallow Studies: If there are concerns about aspiration (food or liquid entering the airway) or swallowing difficulties (dysphagia), specialized swallow studies may be performed. These can include a Modified Barium Swallow Study (MBSS or videofluoroscopic swallow study – VFSS) or a Fiberoptic Endoscopic Evaluation of Swallowing (FEES).
• Pulmonary Function Tests (PFTs): In older children who can cooperate, PFTs can measure lung volumes, airflow rates, and other parameters to assess the functional impact of an airway disorder on breathing.
• Esophageal pH or Impedance Probe Study: If GERD is suspected as a significant contributing factor to airway symptoms, a probe study may be done to measure the frequency and duration of acid or non-acid reflux into the esophagus and potentially the pharynx/larynx.

The diagnostic process for pediatric airway disorders often requires this combination of clinical acumen, direct endoscopic visualization, and sometimes sophisticated imaging or functional tests to arrive at an accurate diagnosis and formulate the best treatment plan.

Treatment Strategies for Pediatric Airway Disorders: Dr. Cable’s Approach

The treatment for pediatric airway disorders is highly individualized and depends critically on the specific diagnosis, the severity of the condition, the child’s age and overall health, and the degree to which the disorder impacts breathing, feeding, growth, and quality of life. The approach can range from conservative observation to complex surgical interventions.

A. Laryngomalacia Treatment:

• Observation and Conservative Management: The majority of infants with laryngomalacia have mild symptoms that do not interfere significantly with breathing or feeding. These cases typically resolve spontaneously by 12-18 months of age as the laryngeal structures mature. Management involves regular monitoring of the child’s breathing, feeding patterns, and weight gain.
• Medical Management: Since gastroesophageal reflux (GER) is common in infants with laryngomalacia and can exacerbate symptoms, anti-reflux medications (such as H2 blockers or proton pump inhibitors) are often prescribed. Feeding modifications, such as thickened feeds or more frequent, smaller volume feeds, may also be helpful for infants with associated feeding difficulties.
• Surgical Intervention (Supraglottoplasty): Surgery is reserved for severe cases of laryngomalacia where the child experiences significant breathing problems (e.g., severe stridor with retractions, oxygen desaturations), feeding difficulties leading to poor weight gain or failure to thrive, apneic episodes (pauses in breathing), or cyanosis. The surgical procedure is called a supraglottoplasty. It is performed endoscopically (through the mouth using instruments alongside a laryngoscope) to trim or tighten the excessive or floppy aryepiglottic folds or remove redundant tissue from the arytenoid cartilages, thereby opening the laryngeal inlet and relieving the obstruction.

B. Subglottic Stenosis (SGS) Treatment:

• Observation: For mild (Grade 1) SGS with minimal or no symptoms, a period of observation with regular follow-up examinations, often including periodic microlaryngoscopy and bronchoscopy (MLB) to monitor the airway, may be appropriate. Some mild congenital stenoses may improve with growth.
• Endoscopic Dilation: For less severe stenoses, or as an initial approach, the narrowed segment of the subglottis can be dilated (stretched open) endoscopically using special balloons or rigid dilators. This may need to be repeated multiple times. 
• Laryngotracheal Reconstruction (LTR): This is an open surgical procedure used for more significant or persistent SGS that does not respond to endoscopic management. In LTR, the narrowed segment of the airway is surgically expanded, often using cartilage grafts harvested from the child’s rib, thyroid cartilage, or (less commonly) ear to widen the cricoid ring and subglottis. 
• Cricotracheal Resection (CTR): For very severe, complex, or long-segment stenoses, CTR may be performed. This involves surgically removing the entire stenotic (narrowed) segment of the cricoid and upper trachea and then rejoining the healthy airway ends (anastomosis). This is a more complex procedure but can offer a definitive cure in selected cases.
• Tracheostomy: In cases of severe SGS that cause life-threatening airway obstruction, or if other treatments are unsuccessful or need to be staged, a tracheostomy may be necessary to provide a safe and stable airway. The tracheostomy tube is placed below the level of the stenosis, bypassing the obstruction. It can serve as a temporary measure while awaiting definitive airway reconstruction or, in some rare cases, as a long-term solution.

C. Tracheomalacia Treatment:

• Conservative Management: Many infants with congenital tracheomalacia improve as they grow and their tracheal cartilage strengthens, often by age 2 or 3. Management may include providing humidified air to keep secretions moist, chest physiotherapy to help clear mucus from the lungs, and, in some cases, Continuous Positive Airway Pressure (CPAP), especially during sleep, to help stent the airway open. Medications may be used to manage associated symptoms or complications, such as antibiotics for respiratory infections, bronchodilators to relax airway muscles, corticosteroids to reduce inflammation during acute exacerbations, and mucolytics to thin secretions.
• Surgical Interventions: Surgery is considered for severe, symptomatic tracheomalacia that does not improve with conservative measures or growth, or if there are life-threatening events (e.g., severe blue spells, recurrent aspiration pneumonia, failure to thrive). Several surgical approaches exist:

• Aortopexy: If the trachea is being compressed by the aorta (the large artery from the heart), this procedure involves surgically lifting the aorta and suturing it to the back of the sternum (breastbone), thereby relieving the compression on the trachea.
• Tracheopexy: This procedure involves suturing the front wall of the floppy trachea directly to the back of the sternum or other stable structures to suspend it and prevent collapse.
• Bronchopexy: Similar to tracheopexy, but performed on the bronchi if they are also involved in the collapse.
• Tracheostomy: For the most severe cases of tracheomalacia causing intractable respiratory failure or inability to protect the airway, a tracheostomy may be required.

D. Vocal Cord Paralysis Treatment:

• Observation: Some cases of vocal cord paralysis, particularly if acquired (e.g., post-viral or post-surgical) or unilateral, may show spontaneous recovery of vocal cord movement over time (months to a year or more). Therefore, a period of observation is often warranted.
• Speech and Swallowing Therapy: Speech-language pathologists play a crucial role in managing voice changes and any associated swallowing difficulties (aspiration risk). Therapy can help optimize voice production with the remaining mobile cord (in unilateral cases) and teach safe swallowing strategies.
• Surgical Interventions: Surgery is considered if there is no spontaneous recovery and symptoms are significant. The type of surgery depends on whether the paralysis is unilateral or bilateral and the nature of the symptoms:

• Unilateral Vocal Cord Paralysis: If the main issue is a weak, breathy voice or mild aspiration due to an incompletely closing glottis (the opening between the vocal cords), procedures to “medialize” or “bulk up” the paralyzed vocal cord can be performed. This helps the mobile cord make better contact with the paralyzed one during speech and swallowing. Injection laryngoplasty involves injecting a substance into the paralyzed vocal cord to add volume.
• Bilateral Vocal Cord Paralysis: If both vocal cords are paralyzed in a position that significantly narrows the airway and causes breathing difficulty (stridor, respiratory distress), the primary goal of surgery is to create a more adequate airway. This might involve procedures like a cordotomy (making an incision in one vocal cord), an arytenoidectomy (removing part or all of the arytenoid cartilage to which the vocal cord attaches), or other airway expansion techniques. In severe cases of bilateral paralysis causing critical airway obstruction, a tracheostomy may be necessary to ensure safe breathing.

The decision for surgical intervention in any pediatric airway disorder is complex and carefully considered, weighing the potential benefits against the risks, the natural history of the specific condition (e.g., the high likelihood of spontaneous resolution in mild laryngomalacia), and the overall impact on the child’s health and development. Advances in endoscopic and open surgical techniques continue to improve outcomes for children with these challenging conditions, with a focus on establishing a stable, natural airway whenever possible and minimizing the need for long-term tracheostomy.

Living with a Pediatric Airway Disorder: Long-Term Outlook and Follow-Up

The long-term outlook for children with airway disorders varies greatly depending on the specific condition, its underlying cause, its severity, and the effectiveness of treatment.

• Many children with mild congenital conditions like laryngomalacia or some forms of tracheomalacia will see their symptoms improve and resolve with growth as their airway structures mature and strengthen.
• For more significant or complex disorders, such as severe subglottic stenosis or bilateral vocal cord paralysis, the journey may be longer and involve multiple interventions or staged surgical repairs. Even after successful treatment, ongoing monitoring by an ENT specialist is crucial. This follow-up is essential to assess airway function, monitor for any recurrence of narrowing or other complications, and ensure continued healthy growth and development. Repeat endoscopic examinations (MLB) may be part of this long-term surveillance.
• Some airway conditions or their surgical treatments can have a lasting impact on voice quality. Speech therapy can be very beneficial in these cases to help optimize vocal function and intelligibility.
• The management of complex pediatric airway disorders often requires a multidisciplinary team approach. This team may include pediatric otolaryngologists, pulmonologists (lung specialists), gastroenterologists (if GERD or swallowing issues are involved), speech-language pathologists, nutritionists, and other specialists as needed to address all aspects of the child’s health. This holistic approach aims not just to establish a patent airway but also to optimize overall function for breathing, feeding, communication, and quality of life.

The path for a child with a significant airway disorder and their family can be challenging, often requiring long-term specialist care and resilience. However, with advances in diagnostic techniques and treatment modalities, the outcomes for many of these children have improved significantly.

When to Seek Urgent Care for Airway Symptoms

Parents of children with known or suspected airway disorders should be vigilant for signs of acute respiratory distress. Seek immediate emergency medical attention if your child exhibits any of the following:

• Significant Difficulty Breathing: This may include rapid breathing, visible retractions (tugging in of the skin at the neck, between the ribs, or below the ribcage), nasal flaring, or grunting sounds with breathing.
• Cyanosis: A bluish discoloration of the lips, tongue, or skin, indicating insufficient oxygen.
• Apneic Episodes: Any observed pauses in breathing, especially if prolonged (e.g., more than 10-15 seconds) or associated with color change or limpness.
• Severe Stridor: Particularly if it is new, suddenly worsens, or is present even when the child is at rest and calm.
• Choking Episodes or Inability to Swallow:
• Extreme Lethargy, Unresponsiveness, or Altered Mental Status:

These are red-flag symptoms that may indicate a critically compromised airway requiring immediate intervention.

The following table provides a quick comparison of common pediatric airway disorders:

Table 3: Common Pediatric Airway Disorders: A Quick Comparison

Disorder	Primary Problem Location	Typical Key Symptom(s)	Usual Onset	Common Cause Type
Laryngomalacia	Larynx (supraglottis)	Inspiratory stridor, noisy breathing (worse with feeding/crying)	Birth to few weeks	Congenital (floppy laryngeal tissue)
Subglottic Stenosis	Larynx (subglottis)	Biphasic or inspiratory stridor, recurrent croup, shortness of breath	Congenital or Acquired	Congenital (cartilage malformation) or Acquired (injury/inflammation, e.g., post-intubation)
Tracheomalacia	Trachea	Expiratory wheeze/rattle, chronic “barking” cough, recurrent infections	Congenital (often by 1-2 months) or Acquired	Congenital (weak tracheal cartilage) or Acquired (compression/injury)
Vocal Cord Paralysis (Bilateral)	Larynx (vocal cords)	Stridor/shortness of breath, often normal voice; weak cry in infants	Congenital or Acquired	Nerve injury/dysfunction

Partnering with Dr. Cable for Your Child’s Airway Health

Dr. Cable has specialized training and extensive experience in the diagnosis and management of the full spectrum of pediatric airway disorders. A collaborative approach, often involving other pediatric specialists, ensures that all aspects of your child’s health are addressed.

If your child experiences noisy breathing, difficulty breathing, chronic cough, recurrent croup, or any other concerning airway symptoms, an expert evaluation is essential.

If your child experiences noisy breathing, difficulty breathing, or other concerning airway symptoms, contact Dr. Cable’s office for an expert evaluation.

Further Reading:

1. The Evaluation of Stridor in Pediatric Patients