ORIGIN AND DEVELOPMENT

I. The trachea: the entodermal lining of the middle segment of the

laryngotracheal tube forms the epithelium and glands of the trachea.
Mesenchymal cells (from splanchnic mesenchyme) surround the
tracheal tube and ultimately form the cartilage, connective tissue,
and smooth muscles of its walls
A. BY WEEK 8 (28-30 mm EMBRYO): mesenchymal rudiments of
the 16-20 tracheal cartilages are seen and, in the following 2
weeks, the masses form cartilage beginning cranially and
extending caudally. Simultaneously, fibroelastic tissue of the
tracheal wall arises from mesenchyme between the cartilage
and, posteriorly, between the ends of the embryonic rings
smooth muscle (the trachealis) arises
1. Cilia appear at 10 weeks (51-53 mm embryo)
2. By week 12, mucosal glands are seen and develop in a
craniocaudal direction
3. By the end of week 20, all major microscopic features of
the trachea are visible, but it is short and narrow while
the larynx is relatively lon This relationship remains until
after birth when the trachea outgrows the larynx to reach
its final form
II. The epithelium and glands of the trachea and pulmonary epithelium are
derived from the endodermal lining of the laryngotracheal tube.
III. The splanchnic mesoderm surrounding the laryngotracheal tube gives rise
to the cartilage, connective tissue and muscles of the trachea.

CLINICAL ASPECT

There are many kinds of pathological disturbances that can affect the trachea. Here
several disorders shall be mentioned with a brief summary of the disease itself.

Two non-pathological but serious ailments of the trachea include choking and a tracheal
foreign body. Choking could be due to the accidental inspiration of food or fluid into
the laryngeal inlet and coughing is the natural reflex in order to remove these remnants.
An actual foreign body that ends up in the trachea however is much more serious.
Depending on the size of the object that is inspirated it can travel down into the bronchi
and obstruct the breathing completely, resulting in death if immediate action is not taken.

Congenital birth defects of the trachea include tracheal agenesis and

a tracheoesophageal fistula. In the first case, the trachea fails to develop and has fatal
consequences. As for the fistula, this is an embryonic malformation where a persisting
channel connects the trachea and the esophagus together. Food swallowed is able to
pass into the trachea and has serious consequences if left untreated.

Lastly, some acquired diseases include tracheal stenosis, tracheomalacia and tracheal
cancer. Tracheal stenosis is the narrowing of the tracheal tube due to inflammation of the
inner respiratory lining. Tracheomalacia is a disorder that can be brought on by smoking
or an injury and causes the trachea to lose its rigidity. In children however this disorder is
congenital. Tracheal cancer is quite rare but is usually diagnosed after a prolonged bout
of coughing or difficulty breathing due to the obstructional growth.

Tracheoesophageal fistula is a congenital defect in which the trachea and

esophagus are abnormally connected (a fistula). This is because of
abnormalities in the separation between the trachea and oesophagus
during development.[11] This occurs in approximately 1 in 3000 births, and
the most common abnormalities is a separation of the upper and lower
ends of the oesophagus, with the upper end finishing in a closed
pouch.[11] Other abnormalities may associated with this, including cardiac
abnormalities, or VACTERL syndrome.[11] Such fistulas may be detected
before a baby is born because of excess amniotic fluid; after birth, they are
often associated with pneumonitis and pneumonia because of aspiration of
food contents.[11]

Tracheal agenesis (also known as tracheal atresia) is a rare birth

defect[2] with a prevalence of less than 1 in 50,000[3] in which
the trachea fails to develop, resulting in an impaired communication
between the larynx and the alveoli of the lungs[1]. Although the defect is
normally fatal[3][4], occasional cases have been reported of long-term
survival following surgical intervention[4][5].

In tracheal agenesis, a delay in the development of the primary bronchial

buds causes a transient arrest in the growth of the laryngotracheal tube,
hindering the normal development of the trachea[3]. The failure of the lung
buds to develop from the primitive foregut leads to tracheoesophageal
malformations.[19] The development of the buds occurs after the elongation
of the oesophagus, causing a dissociation in the growth of the two organs.
Due to this dissociation, the buds directly develop to form the lungs, without
forming the trachea, resulting in tracheal agenesis.[20] Different degrees of
this malformation result in different types of tracheal agenesis.

The diagnosis of the disease is dependent on observable signs and

symptoms and on the stage of gestation. There are two ways to diagnose
tracheal agenesis: early in utero, or later at birth. Recognisable clinical
patterns are associated with tracheal agenesis.
Most commonly, the in-utero diagnosis of the disease requires
using radiography and ultrasound scans.
At birth, the diagnosis is suspected clinically through the observations of
characteristic signs and symptoms of the disease. Tracheal agenesis
should be suspected when tracheal intubation is impossible. The
confirmation of the diagnosis relies on results of endoscopic laryngoscopies
and oesophagoscopies at birth[15]. X-ray studies are necessary to
determine the class and the severity of the disease. CT scans remain an
option to provide more information if required[15].

PATHOLOGY

A wide variety of disorders can affect the trachea and central bronchi. Computed
tomography is the imaging modality of choice in the evaluation of tracheobronchial
disease. Tracheobronchial abnormalities are sometimes incidentally detected on
routine imaging or when imaging is performed for another reason. Abnormalities of
the tracheobronchial tree, however, can be easily missed because they can be subtle.
Furthermore, because symptoms in patients with tracheobronchial disorders often
overlap symptoms of lung disease, radiologists may focus their attention on the lungs
and overlook the tracheobronchial tree. In this article, we review a wide range of
tracheobronchial diseases with emphasis on their computed tomographic appearances.

Extraluminal paratracheal air bubbles are occasionally seen on computed

tomography (CT) studies that include the thoracic inlet (ie, CT of the neck, of
the cervical spine, and of the chest). In most cases, these paratracheal air bubbles are
tracheal diverticula (TD), sometimes also referred to as tracheal pouch, tracheocele,
and tracheogenic cyst. TD are most commonly seen at the right posterolateral aspect
of the upper trachea at the level T1-T3. Because of their typical location and
appearance, they are easily recognized and should not be confused
with pneumomediastinum or other causes of air bubbles in the same region. This
article describes the prevalence, possible pathophysiology, and associated
complications of TD and illustrates the spectrum of their appearance on CT.

Malignant involvement of the trachea predominantly results from direct

spread of neighboring tumors to the tracheal wall. Primary tracheal
malignancies show a low incidence of approximately 0.1 in every 100,000
persons per year, squamous cell carcinomas and adenoid cystic
carcinomas accounting for about two-thirds of adult primary tracheal
tumors. The etiology of squamous cell carcinoma and its premalignant
lesions is strongly associated with tobacco smoking. Patients with tracheal
malignancies show an unfavorable prognosis, with reported 5- and 10-year
survival rates of 5% to 15% and 6% to 7%, respectively, for all types of
tracheal carcinoma.