Tracheomalacia is a condition or incident where the

cartilage Cartilage is a resilient and smooth type of connective tissue. In tetrapods, it covers and protects the ends of long bones at the joints as articular cartilage, and is a structural component of many body parts including the rib cage, the neck ...

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. The usual symptom is

stridor Stridor (Latin for "creaking or grating noise") 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 pha ...

when a person breathes out. This is usually known as a collapsed windpipe. 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 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 ...

), it is termed tracheobronchomalacia. The same condition can also affect the

larynx The larynx (), commonly called the voice box, is an organ in the top of the neck involved in breathing, producing sound and protecting the trachea against food aspiration. The opening of larynx into pharynx known as the laryngeal inlet is about ...

, which is called laryngomalacia. The term is from ''trachea'' and the

Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...

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 Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle), often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases ...

of the

trachealis muscle The trachealis muscle is a sheet of smooth muscle in the trachea. Structure The trachealis muscle lies posterior to the trachea and anterior to the oesophagus. It bridges the gap between the free ends of C-shaped rings of cartilage at the poste ...

. 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. 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. 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.

Diagnosis

There is no standardized, defined set of diagnostic criteria for the diagnosis of tracheomalacia, mainly due to the nonspecific symptoms associated with it. Current diagnostic approaches include pulmonary function testing which shows a characteristic reduction in

peak expiratory flow The peak expiratory flow (PEF), also called peak expiratory flow rate (PEFR), is a person's maximum speed of expiration, as measured with a peak flow meter, a small, hand-held device used to monitor a person's ability to breathe out air. It measure ...

(PEF), physical examination, and imaging such as computed tomography (CT) or

magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...

(MRI), fiberoptic

bronchoscopy Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a trac ...

(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 A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It i ...

and any signs of inflammation,

fistula A fistula (plural: fistulas or fistulae ; from Latin ''fistula'', "tube, pipe") in anatomy is an abnormal connection between two hollow spaces (technically, two epithelialized surfaces), such as blood vessels, intestines, or other hollow or ...

s, or external compressions to precisely determine the location and severity of the

malacia Malacia is abnormal softening of a biological tissue, most often cartilage. The word is derived from Greek μαλακός, ''malakos'' = soft. Usually the combining form -malacia suffixed to another combining form that denotes the affected tissue ...

. Cross-sectional radiological images are important in detecting mediastinal structures involved in TM prior to surgery. Tracheography/

Bronchography Bronchography is a radiological technique, which involves x-raying the respiratory tree after coating the airways with contrast. Bronchography is rarely performed, as it has been made obsolete with improvements in computed tomography and bronchos ...

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 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 epithelium as respiratory mucosa. Air is breathed in through the nose t ...

. However, this requires the injection of contrast material within the narrowed airway. Risks include allergic reaction, airway plugging, or complete airway obstruction.

Classifications

There are three types of tracheomalacia: *Type 1—

congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...

, sometimes associated with

tracheoesophageal fistula A tracheoesophageal fistula (TEF, or TOF; see spelling differences) 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 surg ...

esophageal atresia Esophageal can refer to: * The esophagus * Esophageal arteries * Esophageal glands * Esophageal cancer Esophageal cancer is cancer arising from the esophagus—the food pipe that runs between the throat and the stomach. Symptoms often include ...

*Type 2—extrinsic compression sometimes due to vascular rings *Type 3—acquired due to chronic infection or prolonged

intubation Intubation (sometimes entubation) is a medical procedure involving the insertion of a tube into the body. Patients are generally anesthetized beforehand. Examples include tracheal intubation, and the balloon tamponade with a Sengstaken-Blake ...

or inflammatory conditions like relapsing polychondritis

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. * Regular use of hypertonic saline nebulizers * Low dose inhaled steroids may help decrease airway inflammation and swelling * Inhaled Ipratropium may help decrease secretions and stiffen smaller airways * Antibiotics during active respiratory infection may decrease severity and length of symptoms * Continuous Airway Positive Pressure (CPAP) provides additional intraluminal pressure 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 Mechanical ventilation, assisted ventilation or intermittent mandatory ventilation (IMV), is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move a ...

(both

continuous positive airway pressure 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 ...

(CPAP), or bi-level positive airway pressure (BiPAP) to tracheal stenting and

surgery Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pa ...

. Surgical techniques include

aortopexy Aortopexy is a surgical procedure in which the aortic arch is fixated to the sternum. It results in the tracheal lumen being pulled open. It is used to treat severe tracheomalacia Tracheomalacia is a condition or incident where the cartilage that ...

, tracheopexy,

tracheobronchoplasty A tracheobronchoplasty is a surgical procedure performed at limited medical facilities across the United States. It consists of a thoracic surgery during which mesh is sutured to the outside of the patient's trachea through a series of hundreds o ...

, and

tracheostomy 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 r ...

. The role of the nebulised recombinant human deoxyribonuclease (rhDNase) remains inconclusive.

References

External links