Age: 0.83
Sex: M
Vaccine: FLU4
Symptoms: Tracheal stenosis, Use of accessory respiratory muscles, Vocal cord disorder
Clinical narrative: Patient is a 10-month-old male born at 37 weeks with a history of aortic coarctation s/p surgical repair and angioplasty, left vocal cord hypomobility, and prior episodes of stridor, who presented to ED on 1/7 with 1 day of progressive respiratory distress. He was found to have significant tracheal stenosis during this hospitalization.    He presented with symptoms of progressively noisy breathing and muted voice which culminated in an episode of severe respiratory distress with increased secretions and work of breathing. He previously had been on a thickened diet but was cleared by OT to take un-thickened breastmilk after a swallow study had been reassuring against aspiration in June. In the week prior, he had also been transitioned from a preemie nipple to larger size nipple. On the day of presentation, he had also incidentally received his 2nd flu shot prior to respiratory decompensation.    In the ED he had oxygen saturation in the low 70s, pronounced work of breathing with tracheal tugging, intercostal and subcostal retractions, and biphasic stridor. He had significant improvement after 1 dose of racemic epi, dexamethasone, and initiation of high-flow nasal cannula on 8 L at 40% FiO2. He was initially transitioned to the floor, but soon became significantly more agitated, tachycardic to the 180s, with recurrence of significant work of breathing, coarse lung sounds, intermittent stridor, self-resolving desaturation to the low 80s.  He was given an additional dose of racemic epinephrine due to concern for possible atypical presentation of anaphylaxis. Vital signs improved but given recurrence of respiratory distress, he was transferred to the PICU.   In the PICU he received 2 further doses of dexamethasone and two nighttime doses of racemic epinephrine for initial concern for croup.  He did not have any further episodes of respiratory distress, continued to improve symptomatically, and vitals stabilized. ENT performed bedside laryngoscopy which revealed a posterior vocal fold notch concerning for glottic and/or subglottic stenosis. He was transferred to the floor on 1/10.   Due to concern for aspiration, on 1/11 Pt underwent a video fluoroscopic swallow study, at which time he was found to have fatigue-related pharyngeal phase dysphagia, vocal fold hypomobility, and VF notch. He also had baseline chest rattle and congestion that increased during feeding. These findings were concerning for potential aspiration, so OT recommended returning to half nectar-thickened feeds. On 1/12 ENT performed an exam under anesthesia with micro laryngoscopy and bronchoscopy which revealed complete tracheal rings and severe tracheal narrowing to 2.9 mm.  CT angiogram on 01/13 was consistent with complete tracheal rings and did not show evidence of vascular ring or extrinsic compression by a mediastinal mass.  Pulmonology was consulted and recommended albuterol (for mucus clearance) and CPT/clapping twice daily, and recommended discharge with racemic epinephrine to be used only in case of respiratory emergency while concurrently calling 911. An EpiPen was also provided as an emergency precaution. After joint decision-making with ENT and family, Pt was discharged home on 01/15 for continued discussion of surgical planning for a slide tracheoplasty.  Pt will also follow-up with pulmonology in 4-8 weeks after discharge. He will need pre-operative CHO and outpatient visit with his cardiologist.  He will need a repeat VFSS in 4-6 months. He is already established for feeding therapy