Table 2.
Summary of clinical manifestations.
| Patient | Assisted Ventilation | Agea at Initiation of Assisted Ventilation | Duration of Assisted Ventilation | Cardiac Arrhythmia | HD | Neurodevelopmental Delay | Neural Crest Tumor |
|---|---|---|---|---|---|---|---|
| 1 | PPV-T | 1 day | Sleep | - | - | Speech delay | - |
| 2 | PPV-T | 4 months | Sleep | Abnormal Holter study at 21 years requiring cardiac pacemaker | - | - | - |
| 3 | PPV-T | 9 months | Sleep | - | Present | - | - |
| 4 | DP (day), PPV-T (sleep) | 1 day | Full-time | - | Present | - | Adrenal ganglioneuroma (resected) |
| 5 | Oxygen | 1.8 months | Sleep | - | - | - | - |
| 6 | PPV-T | 1.4 months | Sleep | - | Present | - | - |
| 7 | PPV-T | 2 months | Sleep | - | Present | Fine motor, speech delay | - |
| 8 | Oxygen | 2 days | Sleep | - | - | - | - |
aAge at initiation of any modality of assisted ventilation including intubation for mechanical ventilation with subsequent failure to wean off ventilatory support necessitating a tracheostomy, noninvasive positive pressure ventilation, or oxygen. DP = diaphragm pacing, HD = Hirschsprung’s disease, PPV-T = positive pressure ventilation via tracheostomy, (-) = negative/normal.