IMPLICATIONS: Abnormal tracheal development causes a spectrum of life-threatening

anomalies. We report a newborn with tracheal agenesis and a common “esophagotrachea.”

Ventilation was achieved first by face mask then with an endotracheal tube. In this report, we
describe the types of tracheal agenesis and discuss initial airway management.

Tracheal agenesis is a rare, congenital anomaly that might confront the anesthesiologist in the
delivery room or operating room. It usually manifests in the newborn with severe respiratory
distress and an absent or exceedingly weak cry. Bag mask ventilation and insertion of an
endotracheal tube (ETT) in the esophagus may enable ventilatory support until a complete
evaluation is made. Long-term survival has not yet been achieved. A distinction between pure
laryngeal agenesis and tracheal agenesis is essential because the airway management in the
delivery room is dramatically different. We report a case of tracheal agenesis in a newborn
infant.

Case Report
A 1700-g male was born at 32-wk gestation by vaginal delivery. The pregnancy was complicated
by multiple fetal anomalies, polyhydramnios, and preterm labor. Fetal echocardiogram revealed
double outlet right ventricle, ventricular septal defect, and a dilated coronary sinus suggestive of
left superior vena cava. The history of polyhydramnios raised the possibility of esophageal
atresia. At birth, Apgar scores were 2 at 1 min, 6 at 5 min, and 7 at 10 min. The patient was
initially resuscitated with mask ventilation, and, despite increased airway pressures, abdominal
distention was not observed. This observation suggested the diagnosis of esophageal atresia. Oral
endotracheal intubation was attempted despite poor visualization of the vocal cords. Satisfactory
ventilation was confirmed by chest elevation, bilateral equal breath sounds, and capnography.
Oxygen saturation by pulse oximetry exceeded 80%, and the initial venous blood gas was pH
7.29 mm Hg, PCO2 53 mm Hg, PO2 47 mm Hg, HCO3 24.8 mm Hg, and base deficit of −2.5
mEq/L. The patient was transferred to the neonatal intensive care unit with a provisional
diagnosis of esophageal atresia and VACTERL association (vertebral, anal, cardiac,
tracheoesophageal, renal, and limb anomalies). An orogastric tube was placed with moderate
difficulty, but position appeared satisfactory on the torso radiograph.

The patient was then transported to the operating room for diagnostic
laryngotracheobronchoscopy. A large gas leak was noted around the existing 3.5-mm ETT,
which was removed immediately before the procedure. Endoscopic evaluation was performed
using a rigid ventilating bronchoscope. During the procedure, oxygen saturation ranged from
94% to 97% with a fraction of inspired oxygen of 1.0. Laryngoscopy revealed a normal-
appearing epiglottis and upper larynx; however, there was no lumen between the vocal cords. A
large foregut pouch was present posterior to the larynx. Stenosis of the distal end of this pouch
prevented visualization of the distal airway. The surgeon concluded that these findings were
consistent with the diagnosis of laryngeal and tracheal atresia. At the conclusion of the
procedure, a 4.0-mm ETT was inserted into the foregut pouch and effective ventilation
confirmed by chest expansion, auscultation, and capnography.

A computed tomography scan was subsequently performed (Fig. 1). Findings were consistent
with esophagotrachea giving rise to bilateral and semi-symmetric main bronchi. The
esophagotrachea had two segments of intrinsic stenosis and the esophagus continued distal to the
origin of the bronchi to join the stomach. The patient was transported back to the neonatal
intensive care unit. The anomaly was determined to be inoperable and incompatible with life.
After family counseling, life support measures were discontinued.

The trachea and the esophagus begin developing from the foregut during the
third and fourth weeks of gestation. Tracheal agenesis seems to result from ventral displacement
of the tracheoesophageal septum that forms during this period. A variety of different anomalies
can result, including laryngeal and tracheal atresia. The distinction between these two anomalies
is critical because of differences in airway and surgical management (Table 1). Tracheal atresia
is a rare, congenital anomaly that usually presents in the delivery room with respiratory distress
and absent cry. Bag-mask ventilation followed by awake insertion of an ETT in the esophagus
will provide adequate ventilation until a definitive diagnosis is achieved and evaluation for
possible surgical intervention is completed. Intubation of the airway while the newborn remains
awake and breathing spontaneously is advisable so as to reduce the risk of complete airway
obstruction and inadequate ventilation.

Between 1900 and 1997, 88 cases of tracheal agenesis

were reported (4). The longest survival period was six weeks after attempted surgical repair.
Floyd et al.(2) Described three types of tracheal agenesis: in type I there is a short segment of
distal trachea arising from the esophagus. In type II, the carina may be identified with a barely
visible segment of trachea attached to the esophagus. In type III, the trachea is absent, and
bilateral main bronchi communicate directly with the esophagus. Faro et al. (3) divided tracheal
atresia into categories “A” through “G” (Fig. 2), representing various types of airway anomalies
decreasing in severity, with “A” representing total pulmonary atresia and “G” representing
tracheal stenosis.
In contrast, laryngeal atresia or agenesis, a rare laryngeal anomaly (1) with fewer than 60 cases
described in the literature, was also described in association with other congenital anomalies,
such as tracheoesophageal fistula and VATER or VACTERL associations. The majority of
patients failed to be diagnosed until postmortem examination. The very few who survived
underwent emergency tracheotomy in the delivery room after failed attempts at mask ventilation
or endotracheal intubation.
The infant with pure laryngeal atresia presents with a normal color at birth, but cyanosis rapidly
develops after clamping of the umbilical cord. Spontaneous respiratory efforts are ineffective, as
are attempts at mask ventilation and tracheal intubation. Without emergency tracheotomy, death
occurs soon after birth. However, because most patients with laryngeal atresia also have
associated tracheoesophageal fistula, survival may be possible by ventilation via the esophagus.
Gatti et al. (1) described three types of laryngeal atresia (Table 1) based on laryngoscopy
findings. A high index of suspicion is required for diagnosis. Hoka et al. (5) described the
management of a newborn with emergency tracheotomy for suspected laryngeal atresia. The
patient survived to undergo reconstructive surgery of his larynx.

Finally, our presentation of this case serves to familiarize our colleagues with this rare and
challenging congenital anomaly for which there is yet no real surgical solution. As
anesthesiologists, we may be confronted with these anomalies in either the labor and delivery
suite or operating room.

Source: http://www.anesthesiaanalgesia.org/content/97/1/50.full