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///2016 Abstract Details
2016 Abstract Details2019-07-15T10:10:51-05:00

Unexpected airway malformation during neonatal resuscitation

Abstract Number: SU-92
Abstract Type: Case Report/Case Series

Shannon Page MD1 ; Matthew Haight DO2; Pedram Aleshi MD3

Numerous methods of antenatal testing allow early detection of congenital abnormalities and allow for appropriate planning by the perinatal team, including obstetrics, neonatology, surgery, and anesthesia. Presence of undiagnosed life threatening congenital anomalies is rare. We report a case of undiagnosed tracheal agenesis (TA) and subsequent fetal demise.

Our patient was a 43 year old G3P2 with a prior cesarean delivery who transferred her care to us at 29 weeks gestation. Fetal sonography showed duodenal atresia and polyhydramnios, and fetal echocardiogram demonstrated a persistent left SVC with a connection to the coronary sinus. She was admitted at 35 weeks gestation for severe pre-eclampsia and elective repeat cesarean. Operative delivery was without complication, and a 2.5 kg male infant was delivered with a nuchal cord, which was easily reduced. The infant initially had poor tone and no respiratory effort despite stimulation. His nares were suctioned and a repogle was placed without improvement in respiratory effort, and positive pressure ventilation via mask was administered. Chest compressions were initiated and intubation was attempted, during which the vocal cords were visualized, but the endotracheal tube was unable to be passed beyond this point. Video laryngoscopy was attempted with the same difficulty. During laryngoscopy, a membrane was noted past the vocal cords. An LMA was placed while the pericardial and mediastinal spaces were needle decompressed without improvement in respiratory status. Sixteen-minute umbilical artery gas showed a pO2 of <20 and oxygen saturation of 19. Resuscitation continued until the family decided to discontinue efforts at 60 minutes of life. The autopsy report states the larynx ends in a blind pouch. No trachea was identified, just esophagus in its usual anatomic position. Distally there was a 0.1 cm diameter connection from esophagus to trachea approximately 1 cm above the carina.

Tracheal agenesis is a very rare congenital malformation, with an incidence of 1:500,000. Prenatal diagnosis is difficult when a tracheoesophageal fistula (TEF) is present, as pulmonary fluid passes through the fistula to the stomach or amniotic sac, keeping pulmonary fluid pressures normal and giving the lungs a normal appearance. Post-natal airway management poses an extreme challenge. There are reports of successful EXIT procedures as well as ventilation via the esophagus in the presence of a TEF, which may be the only life saving measures in this dire situation.

1.Pratap A, Saha GS, Bhattarai BK, Yadav RP, Nepal A, Bajracharya A, Kumar A, Adhikary S. Tracheal agenesis type B: further evidence to a lethal congenital tracheal malformation. Journal of Ped. Surg. 2007; 42: 1284-1287.

2. Mooren M, Haak M, Lakeman P, Cohen-Overbeek T, van der Voorn J, Bretschneider J, van Elburg R. Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature. Eur J Pediatr. 2012; 171(3): 425-431.

SOAP 2016