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.