Paradoxical vocal cord motion in a child presenting with cyanosis and respiratory failure

The patient was a 10-yr-old male who presented to an outside emergency department with cyanosis and respiratory failure two weeks after removal of his tracheostomy tube. His tracheostomy had been placed early in infancy after a course of extracorporeal membrane oxygenation for persistent pulmonary hypertension of the newborn and subsequent prolonged mechanical ventilation. The diagnoses of subglottic stenosis and tracheomalacia had been made in association with an inability to wean him from mechanical ventilation. The tracheostomy tube was left in place during the ensuing years because of persistent, although mild, tracheomalacia. The patient repeatedly became agitated during airway examination and manipulation of his tracheostomy tube and had failed to appear for numerous scheduled pulmonary clinic visits for follow-up. His medical history was otherwise unrevealing except for mild reactive airways disease that was controlled with aerosolized bronchodilator therapy. Two weeks before admission, in preparation for decannulation of the tracheostomy, the patient underwent fiberoptic bronchoscopy and an overnight sleep study. These were both reported as normal, including vocal cord function, and the tracheostomy was removed. After doing well for one week after decannulation, he presented to a local emergency department with a 1to 2-day history of intermittent stridor during periods of excitement. Hospital admission was recommended, but was refused by the parents. Two days later, he developed severe inspiratory stridor and cyanosis during a family argument. He was taken to the local emergency department where he underwent orotracheal intubation with an uncuffed, 5.0 mm inside diameter endotracheal tube. He was subsequently transported to the pediatric intensive care unit at Lucile Packard Children’s Hospital at Stanford without complication. In the pediatric intensive care unit, the patient was found to be sedated and had normal vital signs. Because of an audible gas leak around the indwelling tracheal tube at a low inflating pressure, his trachea was reintubated with a cuffed, 5.0 mm inside diameter endotracheal tube without difficulty. His supraglottic region and vocal cords seemed normal during direct laryngoscopy. During mechanical ventilation with an FIO2 of 0.30 his oxygen saturation was 100%. Chest radiography revealed no abnormalities. Laboratory values, including complete blood cell count and electrolytes, were normal. The following morning the patient was receiving minimal mechanical ventilatory support, including an FIO2 of 0.30. His trachea was extubated, and shortly afterward he developed severe inspiratory stridor. Fiberoptic examination of the supraglottic region at that time revealed complete adduction of the vocal cords during inhalation and abduction during exhalation, consistent with the diagnosis of PVCM. Tracheoscopy revealed mild, nonobstructive subglottic stenosis and tracheomalacia. No other lesions or inflammation were noted. During the administration of propofol, titrated to sedation with continued spontaneous breathing, the inspiratory stridor resolved, but recurred when the patient began to awaken. A Shiley 4 tracheostomy tube was inserted via the existing stoma (Mallinckrodt Medical, St. Louis, MO). During the next several hours, the patient was weaned from mechanical ventilation. He was transferred to the pediatric ward the following day with the tracheostomy tube in place. A comprehensive inpatient psychiatric evaluation and a developmental assessment were performed. The diagnoses of mild developmental delay, “oppositional defiant disorder,” and “attention deficit disorder” were made. The patient was discharged home one week after admission with his usual bronchodilator medications. In addition to follow-up in the Pulmonary Clinic, recommendations were made for outpatient cognitive and neuropsychiatric assessments, speech therapy, and outpatient family therapy.