CHAOS in the Delivery Room: A Dire Emergency

Abstract

A female infant was born by cesarean delivery at 41 weeks gestation to an 18-year-old primigravida mother, who did not receive prenatal care. After delivery, she had chest retractions without audible breath sounds, and soon hypotonia, apnea, and cyanosis with a heart rate of 60 beats per minute developed. Bag and mask ventilation did not cause perceptible chest movement, but improved the heart rate temporarily. Attempts to orally intubate the infant failed, with inability to pass the tube beyond the well-visualized vocal cords. A laryngeal mask airway was inserted, but actually caused a drop in the heart rate. A tracheotomy was unsuccessful, and the infant died shortly after birth. Autopsy results showed complete tracheal agenesis (Type III) with both bronchi joining the esophagus separately (Figure).1Floyd J. Campbell Jr., D.C. Dominy D.E. Agenesis of the trachea.Am Rev Respir Dis. 1962; 86: 557-560PubMed Google Scholar Tracheal agenesis is a group of rare upper airway obstructive anomalies, often associated with other anomalies. An antenatal diagnosis of congenital high airway obstruction syndrome offers an opportunity for palliation through the ex utero intrapartum treatment procedure and subsequent tracheal reconstruction.2Crombleholme T.M. Sylvester K. Flake A.W. Adzick N.S. Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure.Fetal Diagn Ther. 2000; 15: 280-282Crossref PubMed Scopus (77) Google Scholar Patients with a short segment of tracheal atresia or tracheal stenosis and a remnant portion of trachea may survive with prompt surgical intervention such as tracheostomy.3Sankaran K. Bhagirath C.P. Bingham W.T. Hjertaas R. Haight K. Tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula: report of two cases and review of literature.Pediatrics. 1983; 71: 821-823PubMed Google Scholar The occurrence of tracheal agenesis, in the absence of prenatal diagnosis, is a grave medical emergency. Bag and mask ventilation, as in our case, is often the most effective form of ventilation because of the communication between the airway and esophagus. Esophageal intubation in these patients may be helpful because it allows gas exchange and provides support to the esophagus, preventing its collapse.4Chiu T. Cuevas D. Cuevas L. Monteiro C. Tracheal agenesis.South Med J. 1990; 83: 925-930Crossref PubMed Scopus (18) Google Scholar A laryngeal mask airway, although useful in many patients with difficult intubation, may block the esophagus and prevent air exchange in this condition. When all else fails, an autopsy provides an anatomic diagnosis and “closure” to the family.