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Table 3 Benefits and risks of different types of ventilation support in CCHS

From: Guidelines for diagnosis and management of congenital central hypoventilation syndrome

  Tracheostomy Ventilation Mask Ventilation Phrenic Nerve Pacing Negative Pressure Ventilation
Benefits • Provides effective ventilation at baseline and during infections
• The airway is secured
• Easy connection and disconnection
• Enables prolonged continuous mechanical ventilation
• Decreases dead space and airway resistance
• Facilitates suctioning of secretions
• Prevents obstructive apnoeas
• Uses portable, battery-operated ventilators (favours mobility)
• Non invasive ventilation
• Easy handling
• Short training, facilitating discharge home
• Avoids tracheostomy
• Uses portable, battery-operated ventilators (favours mobility)
• Reduces stigmatisation, better self-image
• Allows speech development
• Enables mobility during ventilation
• Psychological well-being as the patient is independent and the breathing is “more physiological” breathing.
• Avoids mask-related facial deformation
• Possibility of decannulation
• Non invasive ventilation
• Avoids tracheostomy
• Easy handling
• Short training, facilitating discharge.
• If used just overnight, the patient is device-free during the day
• Costs less than tracheostomy
• The face is free
• Avoids mask-related facial deformation
Risks • Invasive ventilation
• Requires specialised care and long training
• Daily tracheostomy care
• Increases costs
• May interfere with feeding
• Risk of phonation & speech development delays
• Potential stigma
• Complications: infections, accidental decannulation, obstruction, granuloma, tracheomalacia, tracheo-cutaneous fistula after decannulation
• The airway is not secured
• Potential ineffective ventilation due to leakage, upper airway obstruction or asynchrony
• More limited interfaces in infants and younger children
• Difficult to be used more than 18 h a day
• Risk of facial deformation due to prolonged use
• Potential discomfort, pressure sores, pain and non-cooperation that increase risk of self-disconnection
• Potential for aspiration
• Needs high degree of carer surveillance during sleep
• May require temporary intubation when with infection or other challenges
• Surgical procedure for implanting electrodes and receiver
• Requires highly-experienced medical centres
• Can cause OSA requiring mask ventilation, when decannulated
• May require alternative or additional form of ventilation support during illness
• Pacer malfunction
• Surgery is needed for replacement of electrode or receiver
• The airway is not secured
• There is no alarm to alert carers to antenna-receiver uncoupling in some devices
• Not indicated under 1 yo
• Pacing > 12–16 h/day is not recommended
• Ventilation is less effective
• Lack of portability
• The patient is not accessible for assessment and care (according to devices)
• Can cause OSA requiring mask ventilation
• The airway is not secured
• Can cause aspiration in patients with swallowing disorders
• Can cause back/shoulders pain and difficulty sleeping related to supine positioning
• Can cause skin irritation and a sense of feeling chilled
• May require alternative or additional form of ventilation support during illness