DIAGNOSISThe diagnosis at this point was felt to be:
Bilateral functional diaphragmatic paralysis with unexplained stridor.
The patient remained ventilator dependent
All neuro-imaging of cervical spine and brainstem was normal.
Normal peripheral muscle EMG. Further Approach to Management
It was concluded that the patient would need long ventilatory support until his chest wall compliance falls with growth, which will be around age 9-12 mo. There was failure of non-invasive ventilation, mainly due to problems with the mask. The patient underwent tracheostomy to facilitate prolonged ventilatory management. The reason for the stridor was still not explained, and some of the group felt that this may have been a factor that contributed to the respiratory failure, and might need further investigation.
Within few days of tracheostomy the baby was off any ventilatory support, doing well in room air with no stridor. The chest radiopgraph still showed low lung volumes.