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Laryngomalacia and Tracheomalacia

Laryngomalacia and Tracheomalacia

Laryngomalacia and tracheomalacia are the most common upper airway Airway ABCDE Assessment conditions that produce stridor in newborns. Laryngomalacia and tracheomalacia tend to present in the 1st 2 weeks of life, with symptoms ranging from stridor to respiratory distress. The symptoms are caused by narrowing of the airway Airway ABCDE Assessment, which may be due to weakened 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 tissue, external compression External Compression Blunt Chest Trauma, or hypotonia Hypotonia Duchenne Muscular Dystrophy of the affected area. Most cases are congenital Congenital Chorioretinitis, but tracheomalacia can be acquired in children or adults. Diagnosis is based on history, clinical findings, and confirmation by laryngoscopy or bronchoscopy. Treatment is supportive or surgical, depending on the severity. The majority of cases are self-limiting Self-Limiting Meningitis in Children and resolve by 2–3 years of age, but some tracheomalacia cases can persist into adulthood.

Last updated: 8 Jan, 2021

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Contents

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:

Tracheomalacia:

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:

Anatomical recreation of a vascular ring compared to normal anatomy

Anatomic recreation of a vascular ring Vascular Ring Pediatric Chest Abnormalities compared to normal anatomy:
Double aortic arch Aortic arch Mediastinum and Great Vessels: Anatomy causes compression Compression Blunt Chest Trauma 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 and 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. Vascular rings Vascular Rings Pediatric Vomiting often compress 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, leading to a degree of tracheomalacia.

Image by Lecturio.

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 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:
    • 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
Laryngomalacia

Anatomic and physiologic differences between a healthy 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 and laryngomalacia

Image by Lecturio.
  • Anatomical location of obstruction based on inspiratory vs expiratory stridor

    Anatomic location of obstruction based on inspiratory versus expiratory stridor

    Image by Lecturio.

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:
Tracheomalacia

Cross-sectional view of anatomic changes during inspiration Inspiration Ventilation: Mechanics of Breathing and expiration Expiration Ventilation: Mechanics of Breathing in a healthy 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 versus tracheomalacia:
Due to the structural laxity 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 in tracheomalacia, the already significantly reduced airway Airway ABCDE Assessment lumen further narrows during expiration Expiration Ventilation: Mechanics of Breathing.

Image by Lecturio.

Related videos

6:55
Development of Larynx and Large Airways
5:40
Laryngomalacia and Tracheomalacia

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 Pulmonary disease Diseases involving the respiratory system. Blastomyces/Blastomycosis ( 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:

Tracheomalacia:

  • Lung exam:
  • 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:
  • Dynamic expiratory image will show:
    • Posterior tracheal wall flattening or bowing forward
    • Small anteroposterior diameter
    • Increase in lung attenuation
  • Double aortic arch

    A CT scan showing a double aortic arch Aortic arch Mediastinum and Great Vessels: Anatomy (arrow) compressing 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

    Image: “Double aortic arch Aortic arch Mediastinum and Great Vessels: Anatomy” by the Shandong Medical Imaging Research Research Critical and exhaustive investigation or experimentation, having for its aim the discovery of new facts and their correct interpretation, the revision of accepted conclusions, theories, or laws in the light of newly discovered facts, or the practical application of such new or revised conclusions, theories, or laws. Conflict of Interest Institute, Shandong University, Ji’nan, Shandong, People’s Republic of China. License: CC BY 4.0, edited by Lecturio.

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 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-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
  • Endoscopic features of laryngomalacia

    Endoscopic appearance of laryngomalacia

    Image: “Endoscopic features of laryngomalacia” by Wong Birgitta Yee-Hang et al AL Amyloidosis. License: CC BY 4.0.
  • Bronchoscopy picture of tracheomalacia

    Bronchoscopy picture of tracheomalacia: Notice the narrowing of the lumen.

    Image: “Bronchoscopy picture of tracheomalacia” by Quen Mok. License: CC BY 4.0.

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:
  • 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 Airway ABCDE Assessment 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 Airway ABCDE Assessment pressure ( 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:

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 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 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 Aortic arch Mediastinum and Great Vessels: Anatomy): 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 Pediatric Vomiting 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 Airway ABCDE Assessment 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.

References

  1. D’Souza, J. N., & Schroeder, J. W. (2020). Congenital anomalies of the larynx, trachea, and bronchi. In R. M. Kliegman MD et al., Nelson textbook of pediatrics (pp. 220-2211.e1). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323529501004132
  2. Finder, J. D. (2020). Bronchomalacia and tracheomalacia. In R. M. Kliegman MD et al., Nelson textbook of pediatrics (pp. 221-2215.e1). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323529501004168
  3. Kay, D. J., & Goldsmith, A. J. (2006). Laryngomalacia: A classification system and surgical treatment strategy. Ear, Nose, & Throat Journal, 85(5), 328-336.
  4. Thompson, D. (2007). Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: A new theory of etiology. The Laryngoscope, 117, 1-33.
  5. Kusak B, Cichocka-Jarosz E, Jedynak-Wasowicz U, & Lis G. (2017). Types of laryngomalacia in children: Interrelationship between clinical course and comorbid conditions. Eur Arch Otorhinolaryngol. 274(3):1577-1583.
  6. Edmondson NE, Bent JP 3rd, & Chan C. (2011). Laryngomalacia: The role of gender and ethnicity. Int J Pediatr Otorhinolaryngol.
  7. Boogaard R, Huijsmans SH, Pijnenburg MW, Tiddens HA, de Jongste JC, & Merkus PJ. (2005). Tracheomalacia and bronchomalacia in children: Incidence and patient characteristics.
  8. Management of community-acquired pneumonia (CAP) in infants and children older than 3 months of age. (2011). Pediatrics, 128(6), e1677. DOI:10.1542/peds.2011-2385
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