We received a spontaneous vaginally delivered male outborn baby at 35 weeks period of gestation, weighing 1900 grams to a 24-years-old primigravida with antenatal risk factors of gestational diabetes mellitus and polyhydramnios. The baby was born limp without any cry and required immediate resuscitation with bag and mask ventilation. Baby continued to have respiratory distress with copious oral secretions. Baby was intubated and shifted to NICU for mechanical ventilation. The initial intubation was reportedly difficult, and baby required frequent ET suctioning for copious secretions.
Clinically, a strong suspicion of esophageal atresia (EA) and tracheo-esophageal fistula (TEF) was made as feeding tube did not pass into the stomach. The chest X-ray revealed lower end of feeding tube going very low almost to T 6/7 level, and serial X rays revealed the feeding tube going sometimes to left side and sometimes to the right side, in retrospect, we came to know that it was in the trachea and was negotiating to left or right main bronchus. Baby also had other associated anomalies at birth i.e. imperforate anus and absent right kidney.
The evaluation with ultrasound revealed dilated left renal pelvis with non-visualized right kidney. After initial stabilization, baby was taken up for emergency surgical exploration. The newborn was then placed in the left lateral decubitus position and a right posterolateral thoracotomy was performed. Tracheo-esophageal fistula was isolated just one cm above the carina and was transfixed and ligated. The upper blind pouch of esophagus could not be localized even after extensive search. The feeding tube could not be seen or felt in the operative field on maneuvering by anesthesiologist. A trial of passing a large bore tube i.e. mucus sucker was attempted, which went through but again could not be traced in the operating field as it was going into the trachea along with the ETT. A trial of suctioning attempt on the mucus sucker made the right lung deflate with desaturation noticed by the anesthesiologist, which gave us a clue as to the connection of the upper end of esophagus with the trachea. An attempt was made by the otolaryngologist intraoperatively to negotiate the fiber-optic scope through the blind upper pouch, which was unsuccessful. Neck exploration for creating a cervical esophagostomy was carried out; however, the procedure was abandoned due to non-visualization of the upper end of esophagus. Peroperatively, we found after neck exploration that there was only one continuous hollow tube containing the ET tube. A feeding gastrostomy and sigmoid colostomy was carried out, and baby was shifted back to neonatal intensive care after the completion of surgery.
Baby was continued on ventilation and other supportive management with antibiotics, IV fluids, and inotropes. However, the baby succumbed on day 4 of life despite aggressive therapeutic and ventilatory support. Direct laryngoscopy revealed only aryepiglottic folds behind the epiglottis, no vocal cords, and there was no visible opening for the trachea suggesting a common upper airway and digestive tract. Fiber-optic scope was passed through the common opening, which revealed normal hypopharynx with normal development of the epiglottis but no evidence of true vocal cords. There was a collapsing lumen opening into a non-collapsible structure i.e. esophagus (collapsible) with absence of proximal trachea continuing with distal trachea (non-collapsible) [Figure 1]. A complete autopsy revealed laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea [Figure 2]. There was also a solitary left kidney with high anorectal anomaly fulfilling VACTERL association.