(tracheal atresia)

Extremely rare malformation: estimated prevalence of 1/50,000; seems more common among males. It is generally associated with other malformations (heart, esophagus), as in VATER or VACTERL syndrome (see this term) or TACRD (acronym for Tracheal Agenesis, Radial malformation, Cardiac malformation and Duodenal atresia) :

-        digestive tract (50 %): duodenal atresia, anorectal malformation

-        urogenital system (35 %)

-        lung (20 %): pulmonary hypoplasia

-        heart (20 %): VSD, ductus arteriosus, hypoplastic left heart

-        large vessels (16 %): hypoplastic aortic arch, coarctation

Floyd's classification is usually used to describe the anomaly: under a  laryngeal or tracheal cul-de-sac, there can be

-         type I (13 %): a short missing subglottic segment of the trachea; the rest of the trachea and bronchi communicates with the esophagus

-         type II (65 %): the bronchi are connected to the esophagus by a short 'carina'

-         type III (22 %): the bronchi opens directly into the esophagus.

Faro's classification is more detailed but its clinical importance is relative: type A matches (lethal) with complete agenesis and the type G with a segmental congenital stenosis (see this term). Forms B (30 %), C (45 %) and E (15 %) are the most common.

The diagnosis is sometimes done in utero: presence of polyhydramnios(80 %) or CHAOS (Complete High Airway Obstruction Syndrome:  hyperechogenic lungs with dil        ted bronchi and flattened diaphragms) if there is no communication with the esophagus. This requires a specialized management at birth during a  EXIT (EXutero Intrapartum Therapy) procedure.

Clinically, the malformation results in acute  respiratory obstruction after the clamping of the umbilical cord. The newborn makes considerable inspiratory efforts but is unable to inhale air or scream. If the lesion is a suspected or known, the baby should be ventilated by mask and intubated via the esophagus or use a supraglottic device. Mortality at 1 year of age: 92,6 %.

 Anesthetic implications: 

It is important to quickly make the differential diagnosis between tracheal agenesis and atresia of the larynx as their immediate support (at birth) is different:

-         laryngeal atresia : cyanosis with fruitless inspiratory efforts; mask ventilation and intubation are difficult or impossible; mask ventilation is possible only in case of associated esophageal-tracheal fistula associated; treatment: emergency tracheotomy.

-         tracheal agenesis : immediate respiratory distress and absence of cry but ventilation by mask is possible; impossible intubation of a apparently normal larynx; treatment: intubate the esophagus and leave the newborn breathing spontaneously  to prevent abdominal distension: this enables survival if the esotracheal or esobronchial communication  is sufficiently large; later, intubation of the trachea by rigid esophagoscopy (type I and II) or feeding gastrostomy and ligation of the lower esophagus (type III).

Complex reconstructive surgery of the trachea.

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-        Willer BL, Bryan KG, Parakininkas DE, Uhing MR, Staudt Sr et al.
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Updated: September 2021