Perinatal Management of Unanticipated Congenital Laryngeal Atresia

Abstract

Unless ventilation is achieved within minutes of delivery, patients with congenital laryngeal atresia will not survive. There are 2 settings in which survival is more likely: a tracheotomy may be immediately performed in the delivery room, or a communication may exist between the airway and the pharynx, allowing for air exchange. In the latter case, there are no characteristic findings on prenatal sonography to suggest the diagnosis and to ensure that preparations for immediate tracheotomy are made. We describe a neonate with unanticipated laryngeal atresia and a high tracheoesophageal fistula. Ventilation was maintained first by face mask and then by esophageal intubation until a tracheotomy could be performed. This report provides detailed photodocumentation of the anomaly, discusses the mechanism of air exchange, reviews the relevant embryological development, and outlines a protocol for perinatal management of unanticipated laryngeal atresia.

A case of laryngeal atresia (congenital high airway obstruction syndrome) with chromosome 5p deletion syndrome rescued by ex utero intrapartum treatment

“…10 Exceptionally, a patient with CHAOS with tracheoesophageal fistula (TEF) was relatively stable throughout fetal life because pulmonary fluids were diverted into the alimentary tract, and the intrapulmonary pressure was not so high. 3 Another CHAOS case was reported with a small hole (1 mm) at the subglottic region. The fetus survived without any intervention during fetal life.…”

“…There are previous reports of laryngeal atresia that was treated just after delivery by emergency tracheostomy. 3 However, such patients were very fortunate. Even if patients survived, they had a very high risk of suffering from permanent hypoxic brain damage.…”

“…In those cases, patients experienced complications of tracheoesophageal fistula with poor gas exchange through the fistula before emergency tracheostomy. 3 However, patients with general cases of CHAOS are very difficult to save if it is not diagnosed prenatally.…”

Evidence for autosomal dominant inheritance in prenatally diagnosed CHAOS

“…The finding of congenital high airway obstruction syndrome (CHAOS) on prenatal ultrasound examination is diagnostic of a complete or nearly complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia [2,[4][5][6]11]. CHAOS may be part of various monogenic conditions or chromosomal disorders, but most cases are sporadic with unknown incidence [3,7,9,10].…”

Congenital laryngeal atresia associated with esophageal atresia and tracheoesophageal fistula: a case of long-term survival

“…In contrast, when a fetus has a communication between the airway and the digestive tract such as a tracheoesophageal fistula (TEF), the characteristic sonographic findings of CHAOS may be absent. In such a case of unanticipated laryngeal atresia (LA), partial ventilation may be possible through TEF by esophageal intubation until the airway can be surgically secured [6][7][8]. However, when a patient has esophageal atresia (EA) in addition to LA and TEF, an emergent surgical airway including tracheal puncture and tracheostomy is required to resuscitate.…”