P3D198: Across the Pacific: The Journey to Diagnose a Term Infant Born at U.S. Naval Hospital Guam with Respiratory Distress and Feeding Intolerance

Introduction: This case presents a neonate born at U.S. Naval Hospital Guam (USNHG) with persisting respiratory distress and feeding intolerance found to have a rare underlying diagnosis after aeromedical evacuation from Guam to Tripler Army Medical Center (TAMC) in Hawaii.

Case Description: The patient is a 39 3/7 week male born to a 35 year old G5 now P4 mother via spontaneous vaginal delivery. The prenatal course is uncomplicated aside from advanced maternal age. He is initially well-appearing with mild retrognathia and no other dysmorphic features. The infant develops respiratory distress after the first breastfeeding attempt. He is placed on non-invasive respiratory support and his symptoms improve. He is weaned to room air by day of life (DOL) 3. Oral feeds are reattempted and the patient has emesis, a bradycardia/desaturation event, and return of respiratory distress. Respiratory support is restarted and oral feeds are discontinued. Serial chest radiographs (Figure 1) show increasing non-specific haziness.

Plans are established to transport the infant to TAMC, the nearest level III NICU. The U.S. Naval Hospital Okinawa Neonatal Transport Team (USNHO NTT) arrives on DOL5 and transports the infant over 3800 miles. At TAMC, flexible bronchoscopy identifies laryngomalacia but is otherwise unremarkable. An esophogram reveals contrast material traversing from the esophagus to the trachea through a defect at the thoracic inlet (Figure 2); establishing the diagnosis of an H-type tracheoesophageal fistula (TEF). On DOL19, the patient undergoes surgical repair and continues to receive care for post-operative esophageal stenosis.

Discussion: As of 2021, Guam does not have an accredited NICU. USNHG has established the Neonatal Stabilization and Transport team as a novel solution which consists of one neonatologist, two NICU nurses, and two respiratory therapists. The purpose of this team is to stabilize infants until the USNHO NTT can evacuate patients to a NICU. This unique mission requires a high level of coordination as military cargo and refueling aircraft are redirected from real-world operations in support of aeromedical evacuation.

Transport of this patient led to the diagnosis of an H-type TEF. This is the rarest variant of TEF, occurring in 1 in 80,000 births. Symptoms include coughing, choking, and cyanosis with feeding due to aspiration through the fistula. Smaller defects can be asymptomatic in newborns and present as recurrent pneumonia into early childhood. The diagnosis is made with identification of the fistula on flexible bronchoscopy or contrast esophogram. Surgical repair is associated with high survival rates although there is risk of recurrent laryngeal nerve damage, fistula recurrence, and esophageal stenosis.

Conclusion: H-type TEF is a rare cause of neonatal respiratory distress characterized by episodic worsening with oral feeds. This case highlights a rare diagnosis and the remarkable efforts to meet the needs of neonates born to American servicemembers in Guam.