Laryngomalacia and Tracheomalacia

Overview

Definitions

Laryngomalacia is softening of or redundancy of supraglottic structures leading to collapse and narrowing of the airway Airway ABCDE Assessment during inspiration Inspiration Ventilation: Mechanics of Breathing.

Tracheomalacia is an abnormality in tracheal compliance Compliance Distensibility measure of a chamber such as the lungs (lung compliance) or bladder. Compliance is expressed as a change in volume per unit change in pressure. Veins: Histology caused by a variety of factors, resulting in the dynamic airway Airway ABCDE Assessment narrowing.

Epidemiology

Laryngomalacia:

• Most common congenital Congenital Chorioretinitis laryngeal abnormality
• Estimated incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency 1 in 2,600 children
• 45%–75% of children with stridor have laryngomalacia. 
• No gender Gender Gender Dysphoria propensity
• More common in Hispanic or Black children
• Associated with prematurity Prematurity Neonatal Respiratory Distress Syndrome and low birthweight 
• Can be associated with other congenital Congenital Chorioretinitis anomalies:
  • Down’s syndrome
  • DiGeorge’s syndrome

Tracheomalacia:

• Estimated incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency 1 in 2,100 children
• Can often develop as a complication of other conditions (e.g., vascular rings Vascular rings Vascular rings are a group of rare malformations featuring congenital abnormalities of the aortic arch. The aberrant arteries often form a ring around the esophagus and trachea, putting pressure on these structures. Vascular Rings, post-surgical complication)
• Often accompanied by other upper airway Airway ABCDE Assessment malformations (e.g., tracheoesophageal fistula Tracheoesophageal fistula Tracheoesophageal fistula is an abnormal connection between the trachea and esophagus. Esophageal Atresia and Tracheoesophageal Fistula)
• More frequent in premature Premature Childbirth before 37 weeks of pregnancy (259 days from the first day of the mother’s last menstrual period, or 245 days after fertilization). Necrotizing Enterocolitis infants

Etiology

Laryngomalacia (not exactly known) presumed causes:

• Hypotonia Hypotonia Duchenne Muscular Dystrophy (delayed maturation) of the supportive cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology
• Redundant soft tissue Soft Tissue Soft Tissue Abscess
• Supraglottic inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation
• Neuromuscular disorders
• Gastroesophageal reflux (seen in 60% of children with laryngomalacia)

Tracheomalacia:

• Congenital Congenital Chorioretinitis:
  • Hypotonia Hypotonia Duchenne Muscular Dystrophy/ cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology weakness
    • Ehlers-Danlos syndrome Ehlers-Danlos syndrome Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders that are characterized by hyperextensible skin, hypermobile joints, and fragility of the skin and connective tissue. Ehlers-Danlos Syndrome
    • Bronchopulmonary dysplasia
  • Mechanical intrauterine pressure:
    •   Vascular rings Vascular rings Vascular rings are a group of rare malformations featuring congenital abnormalities of the aortic arch. The aberrant arteries often form a ring around the esophagus and trachea, putting pressure on these structures. Vascular Rings
    •  Tracheoesophageal fistulas
• Acquired:
  • Cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology injury:
    • Tracheostomy or intubation Intubation Peritonsillar Abscess
    • External trauma
    • Surgery
  • External compression Compression Blunt Chest Trauma:
    • Thyroid Thyroid The thyroid gland is one of the largest endocrine glands in the human body. The thyroid gland is a highly vascular, brownish-red gland located in the visceral compartment of the anterior region of the neck. Thyroid Gland: Anatomy goiter Goiter A goiter is a chronic enlargement of the thyroid gland due to nonneoplastic growth occurring in the setting of hypothyroidism, hyperthyroidism, or euthyroidism. Morphologically, thyroid enlargement can be diffuse (smooth consistency) or nodular (uninodular or multinodular). Goiter
    • Tumor Tumor Inflammation
    • Vascular compression Compression Blunt Chest Trauma
    • Abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease/cyst
  • Recurrent infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease:
    • Chronic bronchitis Chronic bronchitis A subcategory of chronic obstructive pulmonary disease. The disease is characterized by hypersecretion of mucus accompanied by a chronic (more than 3 months in 2 consecutive years) productive cough. Infectious agents are a major cause of chronic bronchitis. Rhinovirus
    • Cystic Cystic Fibrocystic Change fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans
  • Chronic inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation:
    • Recurrent polychondritis Polychondritis An acquired disease of unknown etiology, chronic course, and tendency to recur. It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction. Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis
    • Emphysema Emphysema Enlargement of air spaces distal to the terminal bronchioles where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions. Chronic Obstructive Pulmonary Disease (COPD)/ smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases

Pathophysiology

Embryology

1. 25 days: Anlagen of larynx Larynx The larynx, also commonly called the voice box, is a cylindrical space located in the neck at the level of the C3-C6 vertebrae. The major structures forming the framework of the larynx are the thyroid cartilage, cricoid cartilage, and epiglottis. The larynx serves to produce sound (phonation), conducts air to the trachea, and prevents large molecules from reaching the lungs. Larynx: Anatomy, trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy, bronchi Bronchi The larger air passages of the lungs arising from the terminal bifurcation of the trachea. They include the largest two primary bronchi which branch out into secondary bronchi, and tertiary bronchi which extend into bronchioles and pulmonary alveoli. Bronchial Tree: Anatomy, and lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs: Anatomy form from tracheobronchial groove.
2. 33 days: Laryngeal primordia form.
3. 5th–6th week: Tracheoesophageal septum extends to the 1st tracheal ring.
4. 7th week:
   • Cricoid ring is formed.
   • Hyoid bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Bones: Structure and Types is visible.
   • Definitive tracheal cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology appears.

Pathophysiology

Laryngomalacia:

• Abnormally soft laryngeal cartilage Laryngeal cartilage The nine cartilages of the larynx, including the cricoid, thyroid and epiglottic, and two each of arytenoid, corniculate and cuneiform. Larynx: Anatomy:
  • Inspiration Inspiration Ventilation: Mechanics of Breathing → negative airway Airway ABCDE Assessment pressure → airway Airway ABCDE Assessment collapse
  • Narrowed airway Airway ABCDE Assessment → turbulent airflow → inspiratory stridor and increased work of breathing Work of breathing Respiratory muscle contraction during inhalation. The work is accomplished in three phases: lung compliance work, that required to expand the lungs against its elastic forces; tissue resistance work, that required to overcome the viscosity of the lung and chest wall structures; and airway resistance work, that required to overcome airway resistance during the movement of air into the lungs. Work of breathing does not refer to expiration, which is entirely a passive process caused by elastic recoil of the lung and chest cage. Pulmonary Examination
• Dynamic obstruction: fluctuating obstruction with inspiration Inspiration Ventilation: Mechanics of Breathing

Tracheomalacia:

• Cartilaginous:
  • Softening of the cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology
  • Trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy is supported by 16 cartilaginous arches on membranous wall.
  • Weakening of cartilage Cartilage Cartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support. Cartilage: Histology causes collapse: 
    • Intrathoracic trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy during expiration Expiration Ventilation: Mechanics of Breathing
    • Extrathoracic trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy during inspiration Inspiration Ventilation: Mechanics of Breathing
    • Can produce expiratory or biphasic stridor
• Membranous:
  • Anterior displacement Displacement The process by which an emotional or behavioral response that is appropriate for one situation appears in another situation for which it is inappropriate. Defense Mechanisms of the membranous wall
  • Also called excessive dynamic airway Airway ABCDE Assessment collapse (EDAC)
• Classified by tracheal appearance:
  • Crescent Crescent Rapidly Progressive Glomerulonephritis: anteroposterior narrowing
  • Lateral: lateral narrowing (“saber sheath”)
  • Circumferential: anteroposterior and lateral narrowing

Clinical Presentation

Laryngomalacia

• Noisy breathing (stridor):
  • Present by 2 weeks of life
  • Loudest at 4–8 months
  • Mild: present during sleeping/feeding, but disappears with crying
  • Severe: intensifies with crying
• Hoarseness Hoarseness An unnaturally deep or rough quality of voice. Parapharyngeal Abscess is not a feature of laryngomalacia.
• Frequent congestion without infection
• Snoring
• Feeding or swallowing Swallowing The act of taking solids and liquids into the gastrointestinal tract through the mouth and throat. Gastrointestinal Motility difficulties ( dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming “stuck.” Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia)
• Failure to gain appropriate weight (severe cases)
• Laryngopharyngeal reflux: reflux of esophageal contents to the larynx Larynx The larynx, also commonly called the voice box, is a cylindrical space located in the neck at the level of the C3-C6 vertebrae. The major structures forming the framework of the larynx are the thyroid cartilage, cricoid cartilage, and epiglottis. The larynx serves to produce sound (phonation), conducts air to the trachea, and prevents large molecules from reaching the lungs. Larynx: Anatomy, nasopharynx Nasopharynx The top portion of the pharynx situated posterior to the nose and superior to the soft palate. The nasopharynx is the posterior extension of the nasal cavities and has a respiratory function. Pharynx: Anatomy, and mouth

Tracheomalacia

• Can present in children ( congenital Congenital Chorioretinitis) or adults ( congenital Congenital Chorioretinitis or acquired)
• Inspiratory stridor (extrathoracic component)
• Barking cough, expiratory stridor (intrathoracic component)
• Respiratory distress
• Children:
  • Recurrent respiratory infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
  • Prolonged recovery
• Adults:
  • Dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
  • Cough
  • Sputum retention
  • Frequent pneumonia Pneumonia Pneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy. Pneumonia
  • Syncope Syncope Syncope is a short-term loss of consciousness and loss of postural stability followed by spontaneous return of consciousness to the previous neurologic baseline without the need for resuscitation. The condition is caused by transient interruption of cerebral blood flow that may be benign or related to a underlying life-threatening condition. Syncope with forced exhalation/cough
  • Exacerbates other conditions ( chronic obstructive pulmonary disease Chronic obstructive pulmonary disease Chronic obstructive pulmonary disease (COPD) is a lung disease characterized by progressive, largely irreversible airflow obstruction. The condition usually presents in middle-aged or elderly persons with a history of cigarette smoking. Signs and symptoms include prolonged expiration, wheezing, diminished breath sounds, progressive dyspnea, and chronic cough. Chronic Obstructive Pulmonary Disease (COPD) ( COPD COPD Chronic obstructive pulmonary disease (COPD) is a lung disease characterized by progressive, largely irreversible airflow obstruction. The condition usually presents in middle-aged or elderly persons with a history of cigarette smoking. Signs and symptoms include prolonged expiration, wheezing, diminished breath sounds, progressive dyspnea, and chronic cough. Chronic Obstructive Pulmonary Disease (COPD)), asthma Asthma Asthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea. Asthma, bronchiectasis Bronchiectasis Bronchiectasis is a chronic disease of the airways that results from permanent bronchial distortion. This results from a continuous cycle of inflammation, bronchial damage and dilation, impaired clearance of secretions, and recurrent infections. Bronchiectasis)

Diagnosis

Physical examination

Laryngomalacia:

• Lung exam:
  • Stridor occurs during inspiration Inspiration Ventilation: Mechanics of Breathing.
  • Exacerbated by exertion (e.g., crying)
  • May be worse with feeding or supine positioning
• Face exam to rule out other causes of stridor:
  • Evaluate nasal patency to rule out choanal atresia Atresia Hypoplastic Left Heart Syndrome (HLHS).
  • Evaluate jaw Jaw The jaw is made up of the mandible, which comprises the lower jaw, and the maxilla, which comprises the upper jaw. The mandible articulates with the temporal bone via the temporomandibular joint (TMJ). The 4 muscles of mastication produce the movements of the TMJ to ensure the efficient chewing of food. Jaw and Temporomandibular Joint: Anatomy for micrognathia Micrognathia Abnormally small jaw. Pierre Robin Sequence.
• Neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess exam: Assess for masses or vascular engorgement Engorgement Mastitis.

Tracheomalacia:

• Lung exam:
  • Stridor occurs during expiration Expiration Ventilation: Mechanics of Breathing or the stridor can be biphasic.
  • Exacerbated by upper respiratory infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
  • Subcostal retractions Subcostal retractions Respiratory Syncytial Virus when accompanied by:
    • Asthma Asthma Asthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea. Asthma 
    • Bronchiolitis Bronchiolitis Inflammation of the bronchioles. Pediatric Chest Abnormalities
• Signs of respiratory distress in severe cases:
  • Tachypnea Tachypnea Increased respiratory rate. Pulmonary Examination:
    • Age 0–2 months: > 60/min 
    • Age 2–12 months: > 50/min 
    • Age 1–5 years: > 40/min 
    • Age > 5 years: > 20/min 
  • Dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
  • Apnea
  • Retractions (suprasternal, intercostal, or subcostal)
  • Grunting Grunting Physical Examination of the Newborn
  • Nasal flaring
  • Altered mental status Altered Mental Status Sepsis in Children
  • Pulse oximetry < 90%
• Neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess exam:
  • May have visible collapse of the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy during swallowing Swallowing The act of taking solids and liquids into the gastrointestinal tract through the mouth and throat. Gastrointestinal Motility
  • Child may lie with a hyperextended neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess.
• General: failure to thrive Failure to Thrive Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. The majority of cases are due to inadequate caloric intake; however, genetic, infectious, and oncological etiologies are also common. Failure to Thrive

Imaging

Plain X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests of the neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess and chest:

• May or may not show a focal narrowing because the abnormalities are dynamic
• May show the cause of an extrinsic obstruction if an obstruction is present

Airway Airway ABCDE Assessment fluoroscopy Fluoroscopy Production of an image when x-rays strike a fluorescent screen. X-rays:

• Dynamic imaging is performed during inspiration Inspiration Ventilation: Mechanics of Breathing and expiration Expiration Ventilation: Mechanics of Breathing.
• Shows expiratory collapse
• Superior to the plain X-rays X-rays X-rays are high-energy particles of electromagnetic radiation used in the medical field for the generation of anatomical images. X-rays are projected through the body of a patient and onto a film, and this technique is called conventional or projectional radiography. X-rays in demonstrating laryngo- and tracheomalacia

Computed tomography (CT) scan:

• Mostly for adult patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship or children with a suspected external cause of tracheomalacia
• May show external obstruction:
  • Goiters
  • Tumors
  • Vascular rings Vascular rings Vascular rings are a group of rare malformations featuring congenital abnormalities of the aortic arch. The aberrant arteries often form a ring around the esophagus and trachea, putting pressure on these structures. Vascular Rings/slings
• Dynamic expiratory image will show:
  • Posterior tracheal wall flattening or bowing forward
  • Small anteroposterior diameter
  • Increase in lung attenuation

Endoscopy Endoscopy Procedures of applying endoscopes for disease diagnosis and treatment. Endoscopy involves passing an optical instrument through a small incision in the skin i.e., percutaneous; or through a natural orifice and along natural body pathways such as the digestive tract; and/or through an incision in the wall of a tubular structure or organ, i.e. Transluminal, to examine or perform surgery on the interior parts of the body. Gastroesophageal Reflux Disease (GERD) and functional studies

• Flexible intranasal laryngoscopy: shows collapse of the cuneiform cartilages (laryngomalacia)
• Bronchoscopy: airway Airway ABCDE Assessment collapse during expiration Expiration Ventilation: Mechanics of Breathing (< 70% of its original diameter; tracheomalacia)
• Pulmonary function testing Pulmonary Function Testing Pulmonary Function Tests:
  • Decreased peak flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure 
  • Flattening of the flow-volume loop
• Esophageal pH pH The quantitative measurement of the acidity or basicity of a solution. Acid-Base Balance impedance: if the reflux component is present/suspected

Management

Laryngomalacia

Mild:

• Most cases are self-limited and non–life threatening.
• Infants need to be checked for appropriate weight gain.
• Stridor usually resolves by 12–18 months of age.

Moderate/severe:

• Medical management:
  • Acid suppression Suppression Defense Mechanisms for associated reflux
  • Speech and swallow therapy (adjustment of food texture Texture Dermatologic Examination, schedule, pacing)
  • High-calorie formula to help growth and weight gain
• Surgery:
  • Reserved for severe cases unresponsive to medical management
  • Supraglottoplasty: removal of redundant supraglottic tissue
  • Can produce dramatic improvements in breathing and feeding

Tracheomalacia

Children: 

• Self-limited condition in the majority
• Indications for intervention:
  • Episodes of life-threatening airway obstruction Airway obstruction Airway obstruction is a partial or complete blockage of the airways that impedes airflow. An airway obstruction can be classified as upper, central, or lower depending on location. Lower airway obstruction (LAO) is usually a manifestation of chronic disease, such as asthma or chronic obstructive pulmonary disease (COPD). Airway Obstruction
  • Recurrent respiratory infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
  • Respiratory failure Respiratory failure Respiratory failure is a syndrome that develops when the respiratory system is unable to maintain oxygenation and/or ventilation. Respiratory failure may be acute or chronic and is classified as hypoxemic, hypercapnic, or a combination of the two. Respiratory Failure
  • Failure to thrive Failure to Thrive Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. The majority of cases are due to inadequate caloric intake; however, genetic, infectious, and oncological etiologies are also common. Failure to Thrive
• Medical management: continuous positive airway pressure Continuous positive airway pressure A technique of respiratory therapy, in either spontaneously breathing or mechanically ventilated patients, in which airway pressure is maintained above atmospheric pressure throughout the respiratory cycle by pressurization of the ventilatory circuit. Noninvasive Ventilation ( CPAP CPAP A technique of respiratory therapy, in either spontaneously breathing or mechanically ventilated patients, in which airway pressure is maintained above atmospheric pressure throughout the respiratory cycle by pressurization of the ventilatory circuit. Noninvasive Ventilation)
• Surgical interventions:
  • Tracheal stents:
    • Endoluminal stents should only be used short term.
    • External stents: less complications
  • Aortopexy:
    • Suspension of the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy to the sternum Sternum A long, narrow, and flat bone commonly known as breastbone occurring in the midsection of the anterior thoracic segment or chest region, which stabilizes the rib cage and serves as the point of origin for several muscles that move the arms, head, and neck. Chest Wall: Anatomy
    • Reduces pressure on the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy caused by vascular malformations
    • Pulls the anterior tracheal wall toward the sternum Sternum A long, narrow, and flat bone commonly known as breastbone occurring in the midsection of the anterior thoracic segment or chest region, which stabilizes the rib cage and serves as the point of origin for several muscles that move the arms, head, and neck. Chest Wall: Anatomy, opening the airway Airway ABCDE Assessment

Adults:

• Medical:
  • Optimization of associated conditions ( COPD COPD Chronic obstructive pulmonary disease (COPD) is a lung disease characterized by progressive, largely irreversible airflow obstruction. The condition usually presents in middle-aged or elderly persons with a history of cigarette smoking. Signs and symptoms include prolonged expiration, wheezing, diminished breath sounds, progressive dyspnea, and chronic cough. Chronic Obstructive Pulmonary Disease (COPD), asthma Asthma Asthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea. Asthma)
  • Intermittent CPAP CPAP A technique of respiratory therapy, in either spontaneously breathing or mechanically ventilated patients, in which airway pressure is maintained above atmospheric pressure throughout the respiratory cycle by pressurization of the ventilatory circuit. Noninvasive Ventilation
  • Breathing techniques
  • Airway Airway ABCDE Assessment clearance techniques
  • Pulmonary rehabilitation
• Surgical approaches:
  • Tracheal stents
  • Tracheobronchoplasty
  • Tracheal resection and reconstruction
  • Tracheal replacement
  • Tracheostomy: last resort

Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas in children

• Most cases of laryngomalacia and tracheomalacia in children resolve by 2–3 years of age due to airway Airway ABCDE Assessment growth.
• Laryngomalacia and tracheomalacia increase the severity of respiratory infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease.
• Some tracheomalacia cases can persist as exercise intolerance into adulthood.
• Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas is worse in children with:
  • Untreated reflux
  • Associated genetic disorders/ congenital Congenital Chorioretinitis anomalies
  • Neuromuscular disorders

Differential Diagnosis

• Supraglottic webs: thin webs of squamous epithelium Epithelium The epithelium is a complex of specialized cellular organizations arranged into sheets and lining cavities and covering the surfaces of the body. The cells exhibit polarity, having an apical and a basal pole. Structures important for the epithelial integrity and function involve the basement membrane, the semipermeable sheet on which the cells rest, and interdigitations, as well as cellular junctions. Surface Epithelium: Histology due to embryologic arrest of larynx Larynx The larynx, also commonly called the voice box, is a cylindrical space located in the neck at the level of the C3-C6 vertebrae. The major structures forming the framework of the larynx are the thyroid cartilage, cricoid cartilage, and epiglottis. The larynx serves to produce sound (phonation), conducts air to the trachea, and prevents large molecules from reaching the lungs. Larynx: Anatomy development in the area near the vocal cords Vocal cords A pair of cone-shaped elastic mucous membrane projecting from the laryngeal wall and forming a narrow slit between them. Each contains a thickened free edge (vocal ligament) extending from the thyroid cartilage to the arytenoid cartilage, and a vocal muscle that shortens or relaxes the vocal cord to control sound production. Larynx: Anatomy. Cause obstruction of the extrathoracic airway Airway ABCDE Assessment and produce symptoms that are similar to laryngomalacia. 
• Airway Airway ABCDE Assessment hemangioma Hemangioma A vascular anomaly due to proliferation of blood vessels that forms a tumor-like mass. The common types involve capillaries and veins. It can occur anywhere in the body but is most frequently noticed in the skin and subcutaneous tissue. Imaging of the Liver and Biliary Tract: a “blood vessel” overgrowth in the airway Airway ABCDE Assessment. During crying, increased blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure causes swelling Swelling Inflammation and may cause partial obstruction of the airway Airway ABCDE Assessment, creating biphasic stridor, much like tracheomalacia.
• Vascular ring Vascular Ring Pediatric Chest Abnormalities (e.g., double aortic arch Double Aortic Arch Vascular Rings): an anomalous structure that results from the abnormal development of the aortic arch Aortic arch Mediastinum and Great Vessels: Anatomy complex and compresses the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy and/or esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy. Children with vascular rings Vascular rings Vascular rings are a group of rare malformations featuring congenital abnormalities of the aortic arch. The aberrant arteries often form a ring around the esophagus and trachea, putting pressure on these structures. Vascular Rings often present with respiratory distress, dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming “stuck.” Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia, stridor, and a cough similar to that seen in croup Croup Croup, also known as laryngotracheobronchitis, is a disease most commonly caused by a viral infection that leads to severe inflammation of the upper airway. It usually presents in children < 5 years of age. Patients develop a hoarse, "seal-like" barking cough and inspiratory stridor. Croup.
• Thyroglossal duct Thyroglossal duct Thyroid Gland: Anatomy cyst: a benign Benign Fibroadenoma midline neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast created by persistent thyroglossal duct Thyroglossal duct Thyroid Gland: Anatomy cells. Aside from airway obstruction Airway obstruction Airway obstruction is a partial or complete blockage of the airways that impedes airflow. An airway obstruction can be classified as upper, central, or lower depending on location. Lower airway obstruction (LAO) is usually a manifestation of chronic disease, such as asthma or chronic obstructive pulmonary disease (COPD). Airway Obstruction, children with a thyroglossal duct Thyroglossal duct Thyroid Gland: Anatomy cyst may present with dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming “stuck.” Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia.