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CASE REPORTS;PEDIATRIC ANESTHESIA: Case Report

Tracheal Agenesis: A Rare Disease with Unique Airway Considerations

Saleeby, Manhal G. MD; Vustar, Mirjana MD; Algren, John MD

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doi: 10.1213/01.ANE.0000066358.67483.4D
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In Brief

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.

Figure 1
Figure 1:
Illustration of tracheal agenesis: (1) esophagotrachea; (2) bronchi arising from esophagotrachea; (3) esophagus; (4) stomach; arrow indicates stenosis of esophagotrachea.

Discussion

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.

Table 1
Table 1:
Comparison of Laryngeal and Tracheal Atresia

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.

Figure 2
Figure 2:
Tracheal atresia divided into categories 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.

We thank Dr. Wallace Neblet for his assistance with this case report.

References

1. Gatti WM, MacDonald E, Orfei E. Congenital laryngeal atresia. Laryngoscope 1987; 97: 966–9.
2. Floyd J, Campbell FJ, Dominy DE. Agenesis of the trachea. Am Rev Respir Dis 1962; 86: 557–60.
3. Faro RS, Goodwin CD, Organ CH Jr. Tracheal agenesis. Ann Thorac Surg 1979; 28: 295–9.
4. Kerschner J, Klotch DW. Tracheal agenesis: a case report and review of the literature. Otolaryngol Head Neck Surg 1997; 116: 123–8.
5. Hoka S, Sato M, Yoshitake J. Management of a newborn infant with congenital laryngeal atresia. Anesth Analg 1989; 69: 535–6.

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