ABSTRACT
Hallermann-Streiff syndrome (HSS) is a rare congenital syndrome with different anomalies including midface hypoplasia, beak nose and micrognathia. The upper airways narrowness can lead to severe respiratory complications such as obstructive sleep apnoea syndrome (OSAS), particularly in infancy. The management of these severe OSAS is difficult and poorly documented in literature. We report the case of an infant with HSS complicated by severe and early OSAS successfully managed with non-invasive ventilation (NIV), provide an overview of respiratory morbidities and discuss treatment options for HSS-related OSAS.
ABSTRACT
Non-invasive ventilation in pressure support (NIV) is well described in the adult and child over 5 years. However, its use in children less than 1 year of age remains anecdotal. We report our preliminary experience with the use of NIV in six children aged from 5 days to 10 months. NIV was delivered with a flow generator (VPAP IIST, Resmed Ltd, North Ryde, NSW, Australia) in association with specific tubings and a nasal mask. The use of NIV resulted in a significant decrease of both the respiratory rate (from 53 to 39 breaths per min, p < 0.01) and the PvCO(2) (from 9.33 to 6.28 kPa, p < 0.01). These results show that NIV can be used in children under 1 year of age with improvement of physiological parameters.