Clinical Note |

Perinatal Management of Unanticipated Congenital Laryngeal Atresia

Michael S. Cohen, MD; Michael A. Rothschild, MD; Juan Moscoso, MD; Edward Shlasko, MD
Arch Otolaryngol Head Neck Surg. 1998;124(12):1368-1371. doi:10.1001/archotol.124.12.1368.
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Laryngeal atresia is one of the rarest congenital airway anomalies, with approximately 70 documented cases in the world literature. Many of these cases are postmortem reports, with only a few of these neonates having survived delivery, generally in 1 of 2 scenarios.

The first scenario involves prenatal diagnosis by the characteristic sonographic findings of the congenital high airway obstruction syndrome, including distended lungs, dilated airways, flattened diaphragms, and fetal hydrops.1 If airway obstruction is anticipated, the appropriate personnel and equipment to perform a rapid tracheotomy under fetal circulation can be assembled in the delivery room.2

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Figure 1.

The emergency tracheotomy site. A curved endotracheal tube is placed in the distal airway (arrowhead), and a suction catheter is placed in the fistula in the posterior wall of the airway (arrow).

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A contrast esophagram showing the fistula (black arrow) above the tracheotomy tube (white arrow). No distal second fistula was seen.

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Figure 3.

Endoscopic views of the laryngeal atresia and tracheoesophageal fistula. A, The supraglottic larynx, notable for a somewhat stunted epiglottis. B, Close view of the glottis, demonstrating the abnormally shaped cartilages over the posterior cricoid cartilage (arrows). C, An endotracheal tube has been passed through the fistula from the tracheotomy site, superiorly into the pharynx. The fistula communicates with the esophagus at the level of the cricopharyngeus (white arrow), just inferior to the posterior cricoid cartilage (black arrow).

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Figure 4.

Drawing of a sagittal section through the airway, demonstrating the fistula (arrow) passing caudal to the cricoid cartilage from the upper trachea into the esophagus at the level of the cricopharyngeus. There is no discernible pharyngoglottic duct through the glottis itself.

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