Case Progression:
At 2 years of age, he developed respiratory distress due to rhinovirus bronchiolitis requiring intubation and ventilation for 2 weeks. He was discharged home on overnight non-invasive ventilation having not required ventilator support prior to that time. At 3 years old, he had a three-week admission for aspiration pneumonia. At 3-1/2 years old he again developed respiratory distress and fever due to RSV bronchiolitis requiring 24 h BiPAP support for several weeks before returning to his baseline settings.
At 7-1/2 years old he underwent a sleep study when his mother noted a 6 week history of nocturnal, self-resolving desaturations to the low 70’s, despite consistent overnight non-invasive ventilatory support. On his sleep study, he was found to have increased FiO2 and pressure support requirements, with inspiratory and expiratory positive airway pressure requirements climbing to 22/10 cmH2O, respectively, and an FiO2 reaching the 35-40% range. His transcutaneous CO2 was in the mid-50s. He was observed to have dysynchrony with the ventilator and to have respiratory distress. This presentation of increased pressure support requirements was considered atypical for SMA related respiratory disease. The patient was a high risk for flexible bronchoscopy with anesthesia. CT chest with dynamic airway evaluation subsequently showed bilateral bronchial stenosis (Figure 1). These lesions were not amenable to tracheostomy, as a surgical airway would not bypass the anatomic stenosis. Due to the patient’s underlying conditions and high risk of morbidity and mortality, surgical intervention for the bronchial stenoses was not pursued. The patient was managed medically with adjustment of non-invasive ventilation settings, and supplemental oxygen. The patient’s high risk of aspiration due to bulbar weakness, decreased clearance of secretions, as well as continuous G-J feeds, posed additional challenges when managing his high ventilation pressures and necessitated careful consideration of the risks and benefits of this strategy along with the patient’s caregivers.
Challenge Point: The patient developed sleep disordered breathing, with high pressure support requirements out of keeping with his underlying neuromuscular condition and more suggestive of a stenotic airway.