Rare type of tracheal agenesis: Unexpected presentation and immediate consideration of emergent esophageal intubation in neonatal resuscitation program. Case reports and review of the literature

Abstract

BackgroundTracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature.MethodsThis study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature.ResultsWe identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation.ConclusionEsophageal intubation may be a life‐sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.

Tracheal agenesis: the importance of teamwork in an uncommon pathology, challenging diagnosis, and high mortality—a case report

“…Survival in cases of tracheal agenesis depends on airway management and the possibility of selective intubation of the fistula through the esophagus ( 17 ). In fact, in a recently published article by Wu et al, regarding three cases of tracheal agenesis, when faced with a patient clinically suggestive of tracheal agenesis, immediate esophageal intubation should be attempted to ventilate the patient through the tracheoesophageal fistula as a life support measure ( 19 ). In our case, unintentional intubation in the esophagus was performed in the delivery room, and survival for a few hours was possible thanks to teamwork with anesthesia and surgical teams to maintain a patent airway.…”