Introduction Congenital Central Hypoventilation Syndrome (CCHS) results from mutation of PHOX2B gene causing alveolar hypoventilation especially during sleep1. In babies with severe CCHS, tracheostomy ventilation (TrLTV) is often required due to their dependence on the ventilator for most of the day, entailing high burden of care2. As the patients get older, many see a reduction in daytime ventilatory support. To date, published data on transitioning TrLTV CCHS patients to non-invasive ventilation (NIV) is limited.1 2
Methods A retrospective review of electronic records summarising the experience of decannulating and transitioning CCHS patients from TrLTV to NIV in a large tertiary centre over a 10 year-period (May 2012- Jan 2023).
Results The LTV service looks after 13 CCHS patients on ventilation (8NIV;5TrLTV). Seven children (4 female; median age[range]7 yrs [5–8] yrs) underwent decannulation to NIV. Six out of 7 children were successfully decannulated to NIV and all remain so since. The median length of decannulation process[range]=8.5[3–14 ] months. Five patients successfully decannulated to NIV at 1st attempt. Of the 2 who failed the 1st attempt, 1 child succeeded at 2nd attempt (1 waiting for 2nd trial). In our cohort, switching from nasal to full face mask aided the success of the transition due to improved leak, less nasal congestion, comfort and overall tolerance to the mask ventilation. Contributing factors to failure included age of child, level of understanding, anxiety and choice of mask.
Discussions Our study shows decannulation and successful transition from TrLTV to NIV are feasible and safe in CCHS children. Further research to identify practical barriers and characterise patients with most difficulties helps to optimise the decannulation pathway and best direct resources.
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