[From tracheostomy to non-invasive mask ventilation: a study in children with congenital central hypoventilation syndrome].

BACKGROUND: Children with congenital central hypoventilation syndrome (CCHS) have to be ventilated during sleep due to respiratory insensitivity to CO2. This long-term mechanical ventilation sometimes requires a tracheostomy during infancy, leading to increased risk of infections and of tracheal problems, and later on to stigmatization and restrictions in social life.

PATIENTS AND METHOD: We therefore evaluated non-invasive mask ventilation in 4 children between 6 and 15 years of age, who had been ventilated via tracheal canula since early infancy under polysomnographic control.

RESULTS: Best results were obtained with standard face masks in connection with pressure controlled timed ventilation. In 1 child we used a volume-controlled ventilator. The lack of dyspnea in these patients can worsen the acceptance of a face mask, which is more uncomfortable than a tracheal cannula. In 2 children we waited with the definite closure of the tracheostomy due to pavor-like symptoms and laryngeal closure during sleep and problems in acceptance of the mask, respectively. In the other 2 children we could demonstrate effective non-invasive mask ventilation during temporary tracheal closure for several nights. Therefore the tracheostomy was definitely closed. Long-term follow-up with home monitoring showed effectiveness of non-invasive ventilation in these cases.