Tracheomalacia

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Tracheomalacia
Tracheomalacia.jpg
(A) Tracheal structure with normal C-shape rings. (B) U-shaped rings with a wider posterior membrane, demonstrating posterior intrusion. (C) Bow-shaped rings with a broad posterior membrane and severe posterior intrusion.
Pronunciation
  • "tray·kee·oh·muh·lay·shuh"
Specialty Pulmonology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Wheezing, dyspnea and fatigue upon exertion
Infants may show signs of cyanosis
Complications Inability to propel secretions, leading to repeated cases of pneumonia
TypesCongenital (heart/great vessel malformations or tracheal anomalies)
Acquired (secondary to trauma or tracheostomy) [1]
Diagnostic method Endoscopy (diagnostic); flexible laryngobronchoscopy (confirmation) [1]
PreventionAvoiding hard throat impacts
TreatmentSymptomatic management (PEEP or CPAP) for mild symptoms
Surgery indicated for tracheal compression from large vessel or mass [1]
Prognosis Severe tracheomalacia is associated with significant morbidity and mortality [2]

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. This condition is most commonly seen in infants and young children. [2] The usual symptom is stridor when a person breathes out. This is usually known as a collapsed windpipe.

Contents

The trachea normally opens slightly during breathing in and narrows slightly during breathing out. These processes are exaggerated in tracheomalacia, leading to airway collapse on breathing out.

If the condition extends further to the large airways (bronchi) (if there is also bronchomalacia), it is termed tracheobronchomalacia. The same condition can also affect the larynx, which is called laryngomalacia. The term is from trachea and the Greek μαλακία, softening

Signs and symptoms

Tracheomalacia occurs when the walls of the trachea collapse. This can happen because the walls of the windpipe are weak, or it can happen because something is pressing on it. This may include hypotonia of the trachealis muscle. [3] The whole windpipe can be affected, or only a short piece of it. If the collapsed part of the windpipe goes past the area where the windpipe branches off into the two lungs, it is called bronchomalacia.[ citation needed ]

This problem causes noisy or difficult breathing in the first 1 to 2 months after birth. This is called congenital tracheomalacia (it was present at birth). It is not very common. Babies born with tracheomalacia may have other health issues like a heart defect, reflux or developmental delay. Some children get tracheomalacia because of other health issues. Symptoms can be mild to severe.[ citation needed ]

Symptoms inside the lung include noisy breathing that may get better when you change your baby's position or while he or she is asleep. Breathing problems that get worse during coughing, crying, feeding or colds. High-pitched sound during breathing (stridor). High-pitched cough. Rattling noise or wheezing with breathing. [4]

Diagnosis

There is no standardized, defined set of diagnostic criteria for the diagnosis of tracheomalacia, mainly due to the nonspecific symptoms associated with it. [5] Current diagnostic approaches include pulmonary function testing which shows a characteristic reduction in peak expiratory flow (PEF), physical examination, and imaging such as computed tomography (CT) or magnetic resonance imaging (MRI), fiberoptic bronchoscopy (FB) is considered the best diagnostic method because an inserted camera down the throat shows a direct view of the airways and lungs, detecting changes in the size or appearance of the trachea's lumen and mucosa and any signs of inflammation, fistulas, or external compressions to precisely determine the location and severity of the malacia. [6] Cross-sectional radiological images are important in detecting mediastinal structures involved in TM prior to surgery. Tracheography/Bronchography is no longer a preferred diagnostic method. Bronchography provides an accurate measurement of the airway lumen with a dynamic and morphological evaluation of the tracheobronchial tree. However, this requires the injection of contrast material within the narrowed airway. Risks include allergic reaction, airway plugging, or complete airway obstruction. [5]

Classifications

There are three types of tracheomalacia: [7]

Treatment

According to the 2012 Cochrane review, there is no evidence supporting medical therapy over surgical therapy for significant tracheomalacia, or vice versa. Current recommendations for mild to moderate non-life-threatening tracheomalacia focuses on symptom management. [5]

Life is usually saved if the airway is opened via a hole in the throat. If a person survives, they may have symptoms, but usually will get better after the airway is reopened. If the symptoms are severe enough, treatment may be needed. These range from medical management over mechanical ventilation (both continuous positive airway pressure (CPAP) [8] or bi-level positive airway pressure (BiPAP)),[ citation needed ] to inserting a tracheal stent [8] and surgery. [8]

Surgical techniques include aortopexy, tracheopexy, tracheobronchoplasty, and tracheostomy. [9] [10] The role of the nebulised recombinant human deoxyribonuclease (rhDNase) remains inconclusive. [10]

See also

Related Research Articles

<span class="mw-page-title-main">Trachea</span> Cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, also known as 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 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 overlying ligaments, and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.

<span class="mw-page-title-main">Tracheal intubation</span> Placement of a tube into the trachea

Tracheal intubation, usually simply referred to as intubation, is the placement of a flexible plastic tube into the trachea (windpipe) to maintain an open airway or to serve as a conduit through which to administer certain drugs. It is frequently performed in critically injured, ill, or anesthetized patients to facilitate ventilation of the lungs, including mechanical ventilation, and to prevent the possibility of asphyxiation or airway obstruction.

<span class="mw-page-title-main">Esophageal atresia</span> 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.

<span class="mw-page-title-main">Mechanical ventilation</span> Method to mechanically assist or replace spontaneous breathing

Mechanical ventilation or assisted ventilation is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move air into and out of the lungs, with the main goal of helping the delivery of oxygen and removal of carbon dioxide. Mechanical ventilation is used for many reasons, including to protect the airway due to mechanical or neurologic cause, to ensure adequate oxygenation, or to remove excess carbon dioxide from the lungs. Various healthcare providers are involved with the use of mechanical ventilation and people who require ventilators are typically monitored in an intensive care unit.

<span class="mw-page-title-main">Tracheotomy</span> Temporary surgical incision to create an airway into the trachea

Tracheotomy, or tracheostomy, is a surgical airway management procedure which consists of making an incision (cut) on the anterior aspect (front) of the neck and opening a direct airway through an incision in the trachea (windpipe). The resulting stoma (hole) can serve independently as an airway or as a site for a tracheal tube or tracheostomy tube to be inserted; this tube allows a person to breathe without the use of the nose or mouth.

<span class="mw-page-title-main">Obesity hypoventilation syndrome</span> Condition in which severely overweight people fail to breathe rapidly or deeply enough

Obesity hypoventilation syndrome (OHS) is a condition in which severely overweight people fail to breathe rapidly or deeply enough, resulting in low oxygen levels and high blood carbon dioxide (CO2) levels. The syndrome is often associated with obstructive sleep apnea (OSA), which causes periods of absent or reduced breathing in sleep, resulting in many partial awakenings during the night and sleepiness during the day. The disease puts strain on the heart, which may lead to heart failure and leg swelling.

A tracheal tube is a catheter that is inserted into the trachea for the primary purpose of establishing and maintaining a patent airway and to ensure the adequate exchange of oxygen and carbon dioxide.

<span class="mw-page-title-main">Respiratory arrest</span> Medical condition

Respiratory arrest is a serious medical condition caused by apnea or respiratory dysfunction severe enough that it will not sustain the body. Prolonged apnea refers to a patient who has stopped breathing for a long period of time. If the heart muscle contraction is intact, the condition is known as respiratory arrest. An abrupt stop of pulmonary gas exchange lasting for more than five minutes may permanently damage vital organs, especially the brain. Lack of oxygen to the brain causes loss of consciousness. Brain injury is likely if respiratory arrest goes untreated for more than three minutes, and death is almost certain if more than five minutes.

<span class="mw-page-title-main">Tracheoesophageal fistula</span> Medical condition

A tracheoesophageal fistula is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.

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.

<span class="mw-page-title-main">Obstructive sleep apnea</span> Sleeping and breathing disorder

Obstructive sleep apnea (OSA) is the most common sleep-related breathing disorder and is characterized by recurrent episodes of complete or partial obstruction of the upper airway leading to reduced or absent breathing during sleep. These episodes are termed "apneas" with complete or near-complete cessation of breathing, or "hypopneas" when the reduction in breathing is partial. In either case, a fall in blood oxygen saturation, a disruption in sleep, or both, may result. A high frequency of apneas or hypopneas during sleep may interfere with the quality of sleep, which – in combination with disturbances in blood oxygenation – is thought to contribute to negative consequences to health and quality of life. The terms obstructive sleep apnea syndrome (OSAS) or obstructive sleep apnea–hypopnea syndrome (OSAHS) may be used to refer to OSA when it is associated with symptoms during the daytime.

<span class="mw-page-title-main">Continuous positive airway pressure</span> Form of ventilator which applies mild air pressure continuously to keep airways open

Continuous positive airway pressure (CPAP) is a form of positive airway pressure (PAP) ventilation in which a constant level of pressure greater than atmospheric pressure is continuously applied to the upper respiratory tract of a person. The application of positive pressure may be intended to prevent upper airway collapse, as occurs in obstructive sleep apnea, or to reduce the work of breathing in conditions such as acute decompensated heart failure. CPAP therapy is highly effective for managing obstructive sleep apnea. Compliance and acceptance of use of CPAP therapy can be a limiting factor, with 8% of people stopping use after the first night and 50% within the first year.

<span class="mw-page-title-main">Laryngotracheal stenosis</span> Medical condition

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.

<span class="mw-page-title-main">Bronchomalacia</span> Medical condition

Bronchomalacia is a term for weak cartilage in the walls of the bronchial tubes, often occurring in children under a day. 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 tracheobronchomalacia has been described in older adults.

<span class="mw-page-title-main">Tracheobronchial injury</span> Damage to the tracheobronchial tree

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.

A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall. It occurs in approximately 1 in 10,000 to 20,000 births. It means there is a communication between the oesophagus and the trachea, which allows food or fluid to pass into the airway.

<span class="mw-page-title-main">Tracheobronchomalacia</span> Medical condition

Tracheobronchomalacia (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.

<span class="mw-page-title-main">Tracheal deviation</span>

Tracheal deviation is a clinical sign that results from unequal intrathoracic pressure within the chest cavity. It is most commonly associated with traumatic pneumothorax, but can be caused by a number of both acute and chronic health issues, such as pneumonectomy, atelectasis, pleural effusion, fibrothorax, or some cancers and certain lymphomas associated with the mediastinal lymph nodes.

References

  1. 1 2 3 "Tracheomalacia / Bronchomalacia information". CHUV.
  2. 1 2 McNamara, V. M; Crabbe, D. C. G (1 June 2004). "Tracheomalacia". Paediatric Respiratory Reviews. 5 (2): 147–154. doi:10.1016/j.prrv.2004.01.010. ISSN   1526-0542. PMID   15135125. S2CID   241298489.
  3. ↑ Wert, Susan E. (2017-01-01), Polin, Richard A.; Abman, Steven H.; Rowitch, David H.; Benitz, William E. (eds.), "61 - Normal and Abnormal Structural Development of the Lung", Fetal and Neonatal Physiology (Fifth Edition), Elsevier, pp. 627–641.e3, ISBN   978-0-323-35214-7 , retrieved 2021-01-23
  4. ↑ "Tracheomalacia". Boston Children's Hospital. Esophageal and Airway Treatment Center. Retrieved 23 April 2024.{{cite web}}: |first1= missing |last1= (help)
  5. 1 2 3 4 5 6 7 8 Fraga, Jose Carlos; Jennings, Russell W.; Kim, Peter C. W. (1 June 2016). "Pediatric tracheomalacia". Seminars in Pediatric Surgery. 25 (3): 156–164. doi:10.1053/j.sempedsurg.2016.02.008. PMID   27301602.
  6. ↑ Snijders, Deborah; Barbato, Angelo (15 August 2015). "An Update on Diagnosis of Tracheomalacia in Children" (PDF). European Journal of Pediatric Surgery. 25 (4): 333–335. doi:10.1055/s-0035-1559816. PMID   26276910. S2CID   1259529.
  7. ↑ Austin J, Ali T (January 2003). "Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management". Paediatr Anaesth. 13 (1): 3–11. doi:10.1046/j.1460-9592.2003.00802.x. PMID   12535032. S2CID   35135103.
  8. 1 2 3 "Tracheomalacia - acquired: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 2024-04-19.
  9. ↑ van der Zee DC, Bax NM (2007). "Thoracoscopic tracheoaortopexia for the treatment of life-threatening events in tracheomalacia". Surgical Endoscopy. 21 (11): 2024–2025. doi: 10.1007/s00464-007-9250-8 . PMID   17356936.
  10. 1 2 Goyal, Vikas; Masters, I. Brent; Chang, Anne B. (2012-10-17). "Interventions for primary (intrinsic) tracheomalacia in children" (PDF). The Cochrane Database of Systematic Reviews. 10: CD005304. doi:10.1002/14651858.CD005304.pub3. ISSN   1469-493X. PMID   23076914.
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