Table 2

Diagnostic categories and subdivision into major subgroups according to ventilatory interface

Interface for support
Cause of ventilationMajor subgroupsTracheostomyMaskOther
Central nervous system (n=168)Congenital central hypoventilation syndrome30251*
Spinal injury2112
Birth injury/cerebral palsy26
Acquired central hypoventilation syndrome812
Other central causes2535
Musculoskeletal (n=402)Duchenne muscular dystrophy1881
Congenital myopathy2334
Other myopathy35
Other dystrophy751
Spinal muscular atrophy type 157
Spinal muscular atrophy II/III562
Other musculoskeletal1779
Respiratory (n=343)Chronic lung disease99
Chronic lung disease (prematurity)95
Airway malacia2613
Prader Willi/obesity syndromes58
Upper airway obstruction6153
Cystic fibrosis/primary ciliary dyskinesia5
Other respiratory12371§
Unclassified (n=20)
  • * One patient has tracheostomy plus diaphragmatic pacing.

  • Two patients have tracheostomy plus diaphragmatic pacing.

  • One child not known.

  • § One child managed with negative pressure device.