LAUSR

Our patient is a full-term male diagnosed at birth with a soft palate cleft. He was discharged home after routine monitoring on room air, consuming formula via a specialty feeding system. At 6 days of age, he presented to our hospital with cyanosis and unresponsiveness associated with an emesis event after feeding. Physical examination demonstrated mild inspiratory stridor, but his bedside flexible fiberoptic laryngoscopy (FFL) revealed a normal nasopharynx (other than known soft palate cleft), normal oropharynx, patent supraglottis, and patent glottis. Due to audible inspiratory stridor both at rest and during oral feeds, a videofluoroscopic swallow study (VFSS) was performed, revealing only retrograde flow of the thin liquid into the nasopharynx consistent with a cleft palate and several shallow laryngeal penetration events. He was discharged from the hospital, but readmitted at 26 days of age for increase in stridor, apnea, and cyanosis for 2 days. A repeat FFL again revealed a normal upper airway. Speech Language Pathology (SLP) was consulted for a clinical feeding/swallowing assessment. Bedside assessment revealed inspiratory stridor, breath holding, and gasping during the oral feeding trial, with him consuming < 5 ml. The patient’s inspiratory stridor and breath holding increased when he was held in near upright and reclined positions, with notable improvement when positioned in sidelying and prone concerning for upper airway obstruction. He was diagnosed with reflux and positioned in prone throughout the majority of the day and night with Intensive Care Unit monitoring. The patient’s stridor and increased work of breathing continued, with additional retractions at baseline despite reflux management and positional supports. The patient’s lateral images of his previous VFSS were re-reviewed with attention given to the tongue base and mandible and noted to be atypical when compared to similar aged infants by the SLP team (Figs. 1 and 2). What is the cause of his symptoms both at rest and during feeding?