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 |