Index of Suspicion in the Nursery

Abstract

A 34-week preterm baby (triplet C) is born of a triplet gestation to a 29-year-old gravida 2 para 3 mother after conception by in vitro fertilization. The mother is on thyroxine hormone for hypothyroidism and glyburide for diabetes mellitus. Delivery is by cesarean delivery at 34 weeks’ gestation due to progressively worsening maternal hypertension and a low biophysical profile of triplet B. Delivery room stabilization includes positive pressure ventilation, intubation, and surfactant administration. Apgar scores are 6 at 1 minute and 8 at 5 minutes. Birthweight is 2,380 g (50th–75th percentile), length is 46 cm (50th–75th percentile), and head circumference is 33.4 cm (75th–90th percentile). He is extubated to continuous positive airway pressure in a few hours, and respiratory support is weaned to humidified high flow through nasal canula. Feedings are commenced when he is 4 days old and gradually advanced. He vomits and has desaturation with worsening respiratory distress and requires increased respiratory support with each attempt to increase feeds. Radiologic evaluation reveals progressively worsening parenchymal haziness (Figs 1 and 2). After feeds are stopped, his respiratory status improves, and he is weaned down to baseline respiratory support. Radiologic evaluation leads to suspicion of the diagnosis followed by a confirmatory test.An upper gastrointestinal contrast study reveals contrast in the tracheobronchial tree, suggestive of either aspiration or a tracheoesophageal fistula (TEF) (Fig 3). Rigid bronchoscopy and esophagoscopy is deferred to allow for somatic growth while the child is enterally fed through a nasojejunal tube. Subsequent rigid bronchoscopy reveals a normal carina and right and left mainstem bronchi. Inspection of the anterior aspect of the trachea shows no abnormalities. Inspection of the posterior trachea reveals a fistulous opening (Click here to see the video clip) approximately 2.7 cm proximal to the carina that increases in size with positive pressure. A 2F Fogarty catheter is introduced through the working port of the rigid bronchoscope and passes easily through the fistulous opening. Simultaneous flexible esophagoscopy with a neonatal esophagoscope demonstrates a normal-appearing esophagus, an indwelling orogastric tube, and the presence of the Fogarty catheter within the esophageal lumen, confirming the H-type TEF. Echocardiogram shows a small, septum secundum atrial septal defect (ASD). Skeletal survey and head, spinal, and renal ultrasounds are normal.TEF is an abnormal communication between the trachea and the esophagus. It was classified by Gross into the following types:The H type is the least common variant of TEF and accounts for only approximately 4% to 5% of total cases. The commonest level at which it occurs is the second thoracic vertebra. It has the lowest incidence of associated congenital anomalies and the best prognosis among all varieties of TEF. Polyhydramnios is rare, and intrauterine growth is usually normal. Clinically, it is characterized by the classic triad of recurrent episodes of choking and coughing with feeding, lower respiratory tract infections, and abdominal distension as air passes through the fistula into the stomach.In any infant presenting with these symptoms, an H type TEF should be suspected. Bronchoscopy with or without esophagoscopy is the diagnostic gold standard. Surgical treatment consists of ligation and repair of the fistula, usually through a cervical approach although a thoracoscopic approach, or thoracotomy may be required depending on the level of the fistula.The fistula was repaired through a right-sided cervical approach. The patient developed stridor, with increased work of breathing in the immediate postoperative period that steadily improved over the next few days. Oral feedings were commenced on postoperative day 4, well tolerated, and gradually increased to goal. Flexible nasolaryngoscopy was performed 2 weeks after the surgery for persistent stridor, which showed right vocal cord paresis. The stridor continued to improve, and he was discharged home. Follow-up nasolaryngoscopy showed return of right vocal cord function.Recurrent episodes of choking, coughing, and respiratory distress with feeds can be caused by an H-type TEF. It has the best prognosis among all types of TEF.