Abstract

Abstract Objective This paper discusses two cases of congenital airway malformations that presented in NICU in a four-month period. The aim is to present extremely rare evidences that inevitably lead to the death of the patient if not correctly identified during pregnancy. Case 1: male twin born at 34 weeks by emergency caesarean section to a 37-year-old mother. Antenatal history was notable for in vitro fertilization and renal dysplasia. Immediately after delivery, there was respiratory distress, cyanosis, with a 1- and 5-min Apgar score of 0 and 1, respectively. He required ventilation and was supposed intubated orally with significant difficulty with a 2.0 mm ETT. For persistent ventilation problems and severe combined acidosis, the neonatologists tried without success to reintubate the patient. Some hours later, the otolaryngologist was called and was again unable to intubate with flexible laryngoscopy due to an obstruction that prevented advancement of the endotracheal tube past the vocal folds, but since the baby general conditions had been already deteriorated a decision to withdraw the treatment was made. He died after few hours. Postmortem revealed a polymalformative syndrome with subglottic complete diaphragm, a tracheoesophageal fistula 1 cm caudally to the diaphragm and unilateral multicystic renal dysplasia. Case 2: male vaginally delivery at 35 weeks to a 43-year-old mother with gestational diabetes. Antenatally, ‘VACTERL’ association was suspected on the basis of the US and a MRI was planned but not performed because of the early delivery. Following the delivery, there was severe respiratory distress, no audible cry, and ventilation was not effective in relieving the respiratory distress. Subsequent intubation was unsuccessful. An emergency tracheostomy was attempted: the larynx was identified, but only a tracheal stump was present on neck exploration. Postmortem confirmed type II (according to Floyd) tracheal agenesis with the esophagus connect to the main bronchus, renal dysplasia, anal atresia, and single umbilical artery. Conclusion Clinicians need to be aware of congenital airway malformation and subsequent difficulties upon endotracheal intubation and they have to be prepared to plan a multidisciplinary management at the delivery including emergency intubation through esophageal fistula.

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