Background: Children with congenital central hypoventilation syndrome (CCHS) require life long ventilatory support usually during sleep. Traditionally, they have received either intermittent positive pressure ventilation (IPPV) via tracheostomy or negative pressure ventilation after the diagnosis is made, typically in early infancy. In more recent years, patients have been decannulated and transferred to non invasive positive pressure ventilation (NIPPV) via face/nasal mask later in childhood.
Aims: To review the effectiveness and acceptance of introducing mask ventilation in early infancy, thus avoiding the need for tracheostomy or negative pressure ventilation and to evaluate parent/patient preferences for mask ventilation, tracheostomy or negative pressure ventilation.
Methods: Retrospective case note review and prospective questionnaire to the patients and parents under follow up at the University Hospital of North Staffordshire.
Results: Fifteen patients (10 girls) were identified with a median age 13 years (range 9 months – 21 years). Nine patients had received traditional ventilatory methods with either IPPV via tracheostomy or negative pressure ventilation before being weaned on to mask ventilation at a median age of 8 years (range 1.5 – 11 years). Six patients were established on mask ventilation in early infancy (median 8 weeks, range 5 - 26 weeks) and thus avoided or minimised the duration of tracheostomy or negative pressure ventilation. Mask ventilation was effective in all six patients without major complications. All the nine patients/parents who had received ventilatory support via tracheostomy or negative pressure ventilation prior to the initiation of mask ventilation felt that mask ventilation was a superior method of ventilatory support.
Conclusions: Mask ventilation can be safely commenced at an early age in children affected with CCHS. It is not only effective but is the preferred mode of ventilatory support by parents and patients. Children who are established on other modes of ventilatory support can also be successfully weaned on to mask ventilation within a short period of time.
Key words: Congenital central hypoventilation syndrome, long term ventilation, domiciliary ventilation, tracheostomy, non invasive ventilation.
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