Bronchomalacia

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Bronchomalacia
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Larynx, trachea and bronchi.
Specialty Respirology

Bronchomalacia is a term for weak cartilage in the walls of the bronchial tubes, often occurring in children under six months. Bronchomalacia means 'floppiness' of some part of the bronchi. Patients present with noisy breathing and/or wheezing. There is collapse of a main stem bronchus on exhalation. If the trachea is also involved the term tracheobronchomalacia (TBM) is used. If only the upper airway the trachea is involved it is called tracheomalacia (TM). There are two types of bronchomalacia. Primary bronchomalacia is due to a deficiency in the cartilaginous rings. Secondary bronchomalacia may occur by extrinsic compression from an enlarged vessel, a vascular ring or a bronchogenic cyst. Though uncommon, idiopathic (of unknown cause) tracheobronchomalacia has been described in older adults.

Contents

Cause

Bronchomalacia can best be described as a birth defect of the bronchus in the respiratory tract. Congenital malacia of the large airways is one of the few causes of irreversible airways obstruction in children, with symptoms varying from recurrent wheeze and recurrent lower airways infections to severe dyspnea and respiratory insufficiency. It may also be acquired later in life due to chronic or recurring inflammation resulting from infection or other airway disease. [1] [2] [3] [4] [5]

Diagnosis

Classification

Primary bronchomalacia

Secondary bronchomalacia

Treatment

  1. Time
    1. Minimally Invasive, usually in conjunction with Continuous Positive Airflow Pressure.
  2. Continuous Positive Airflow Pressure
    1. A method of respiratory ventilation.
  3. Tracheotomy
    1. Surgical procedures on the neck to open a direct airway through an incision in the trachea (the windpipe).
  4. Prosthesis
    1. Insertion of a prosthesis to keep the bronchial tube open.

Notes

  1. Carden, KA, Boiselle, PM, Waltz, DA, et al. (2005) Tracheomalacia and tracheobronchomalacia in children and adults: an in-depth review. Chest 127,984-1005.
  2. Clements, B Congenital malformations of the lungs and airways. Taussig, LM Landau, LI eds. Pediatric respiratory medicine 1999,1106-1136 Mosby. St. Louis, MO
  3. Austin, J, Ali, T Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management. Paediatr Anaesth 2003;13,3-11
  4. McNamara, VM, Crabbe, DC Tracheomalacia. Paediatr Respir Rev 2004;5,147-154
  5. Carden, K. A.; Boiselle, P. M.; Waltz, D. A.; Ernst, A (2005). "Tracheomalacia and tracheobronchomalacia in children and adults: An in-depth review". Chest. 127 (3): 984–1005. doi:10.1378/chest.127.3.984. PMID   15764786.
  6. Benjamin, B Tracheomalacia in infants and children. Ann Otol Rhinol Laryngol 1984;93,438-442

Related Research Articles

Lung Organ for breathing air

The lungs are the primary organs of the respiratory system in humans and many other animals including a few fish and some snails. In mammals and most other vertebrates, two lungs are located near the backbone on either side of the heart. Their function in the respiratory system is to extract oxygen from the atmosphere and transfer it into the bloodstream, and to release carbon dioxide from the bloodstream into the atmosphere, in a process of gas exchange. Respiration is driven by different muscular systems in different species. Mammals, reptiles and birds use their different muscles to support and foster breathing. In early tetrapods, air was driven into the lungs by the pharyngeal muscles via buccal pumping, a mechanism still seen in amphibians. In humans, the main muscle of respiration that drives breathing is the diaphragm. The lungs also provide airflow that makes vocal sounds including human speech possible.

Trachea Cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, also called the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air-breathing animals with lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea the cricoid cartilage attaches it to the larynx. The trachea is formed by a number of horseshoe-shaped rings, joined together vertically by ligaments over their substance and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.

Esophageal atresia Congenital discontinuity of the oesophagus

Esophageal atresia is a congenital medical condition that affects the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It comprises a variety of congenital anatomic defects that are caused by an abnormal embryological development of the esophagus. It is characterized anatomically by a congenital obstruction of the esophagus with interruption of the continuity of the esophageal wall.

Tracheomalacia is a condition or incident where the cartilage that keeps the airway (trachea) open is soft such that the trachea partly collapses especially during increased airflow. The usual symptom is stridor when a person breathes out. This is usually known as a collapsed windpipe.

Diffusing capacity of the lung (DL) measures the transfer of gas from air in the lung, to the red blood cells in lung blood vessels. It is part of a comprehensive series of pulmonary function tests to determine the overall ability of the lung to transport gas into and out of the blood. DL, especially DLCO, is reduced in certain diseases of the lung and heart. DLCO measurement has been standardized according to a position paper by a task force of the European Respiratory and American Thoracic Societies.

Respiratory tract Organs involved in transmission of air to and from the point where gases diffuse into tissue

The respiratory tract is the subdivision of the respiratory system involved with the process of respiration in mammals. The respiratory tract is lined with respiratory mucosa or respiratory epithelium.

Bronchus Airway in the respiratory tract

A bronchus is a passage or airway in the respiratory system that conducts air into the lungs. The first bronchi to branch from the trachea are the right main bronchus and the left main bronchus, also known as the primary bronchi. These are the widest and enter the lungs at each hilum, where they branch into narrower secondary bronchi or lobar bronchi, and these branch into narrower tertiary bronchi or segmental bronchi. Further divisions of the segmental bronchi are known as 4th order, 5th order, and 6th order segmental bronchi, or grouped together as subsegmental bronchi. The bronchi when too narrow to be supported by cartilage are known as bronchioles. No gas exchange takes place in the bronchi.

Airway management

Airway management includes a set of maneuvers and medical procedures performed to prevent and relieve airway obstruction. This ensures an open pathway for gas exchange between a patient's lungs and the atmosphere. This is accomplished by either clearing a previously obstructed airway; or by preventing airway obstruction in cases such as anaphylaxis, the obtunded patient, or medical sedation. Airway obstruction can be caused by the tongue, foreign objects, the tissues of the airway itself, and bodily fluids such as blood and gastric contents (aspiration).

Stridor is a high-pitched extra-thoracic breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor which is a noise originating in the pharynx. Stridor is a physical sign which is caused by a narrowed or obstructed airway. It can be inspiratory, expiratory or biphasic, although it is usually heard during inspiration. Inspiratory stridor often occurs in children with croup. It may be indicative of serious airway obstruction from severe conditions such as epiglottitis, a foreign body lodged in the airway, or a laryngeal tumor. Stridor should always command attention to establish its cause. Visualization of the airway by medical experts equipped to control the airway may be needed.

Laryngeal papillomatosis

Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis (RRP) or glottal papillomatosis, is a rare medical condition in which benign tumors (papilloma) form along the aerodigestive tract. There are two variants based on the age of onset: juvenile and adult laryngeal papillomatosis. The tumors are caused by human papillomavirus (HPV) infection of the throat. The tumors may lead to narrowing of the airway, which may cause vocal changes or airway obstruction. Laryngeal papillomatosis is initially diagnosed through indirect laryngoscopy upon observation of growths on the larynx and can be confirmed through a biopsy. Treatment for laryngeal papillomatosis aims to remove the papillomas and limit their recurrence. Due to the recurrent nature of the virus, repeated treatments usually are needed. Laryngeal papillomatosis is primarily treated surgically, though supplemental nonsurgical and/or medical treatments may be considered in some cases. The evolution of laryngeal papillomatosis is highly variable. Though total recovery may be observed, it is often persistent despite treatment. The number of new cases of laryngeal papillomatosis cases is at approximately 4.3 cases per 100,000 children and 1.8 cases per 100,000 adults annually.

Ortners syndrome

Ortner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. It was first described by Norbert Ortner (1865–1935), an Austrian physician, in 1897.

High-frequency ventilation is a type of mechanical ventilation which utilizes a respiratory rate greater than four times the normal value. and very small tidal volumes. High frequency ventilation is thought to reduce ventilator-associated lung injury (VALI), especially in the context of ARDS and acute lung injury. This is commonly referred to as lung protective ventilation. There are different types of high-frequency ventilation. Each type has its own unique advantages and disadvantages. The types of HFV are characterized by the delivery system and the type of exhalation phase.

Laryngotracheal stenosis

Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx, trachea, carina or main bronchi. In a small number of patients narrowing may be present in more than one anatomical location.

Williams–Campbell syndrome (WCS) is a disease of the airways where cartilage in the bronchi is defective. It is a form of congenital cystic bronchiectasis. This leads to collapse of the airways and bronchiectasis. It acts as one of the differential to Allergic bronchopulmonary aspergillosis. WCS is a deficiency of the bronchial cartilage distally.

Tracheobronchial injury

Tracheobronchial injury is damage to the tracheobronchial tree. It can result from blunt or penetrating trauma to the neck or chest, inhalation of harmful fumes or smoke, or aspiration of liquids or objects.

Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.

Tracheobronchomalacia

Tracheobronchomalacia or TBM is a condition characterized by flaccidity of the tracheal support cartilage which leads to tracheal collapse. This condition can also affect the bronchi. There are two forms of this condition: primary TBM and secondary TBM. Primary TBM is congenital and starts as early as birth. It is mainly linked to genetic causes. Secondary TBM is acquired and starts in adulthood. It is mainly developed after an accident or chronic inflammation.

Tracheal agenesis is a rare birth defect with a prevalence of less than 1 in 50,000 in which the trachea fails to develop, resulting in an impaired communication between the larynx and the alveoli of the lungs. Although the defect is normally fatal, occasional cases have been reported of long-term survival following surgical intervention.

Advanced airway management

Advanced airway management is the subset of airway management that involves advanced training, skill, and invasiveness. It encompasses various techniques performed to create an open or patent airway – a clear path between a patient's lungs and the outside world.

Pulmonary agenesis

Pulmonary agenesis is an inborn lung underdevelopment that is rare and potentially lethal. The disorder is caused by a complete developmental arrest of the primitive lung during embryonic life, and it is often associated with other developmental defects. Bilateral and unilateral pulmonary agenesis are classified, depending on whether one side of the lung or both sides are affected. Bilateral pulmonary agenesis is lethal, while the mortality rate of unilateral pulmonary agenesis is higher than 50%. Depending on the severity, the symptom ranges from none to various respiratory complaints. It is detectable prenatally, however, its nonspecific clinical features act as the obstacle for diagnosing. The exact cause of pulmonary agenesis is still obscure. However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder. In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.

References

Classification
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