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 CO 2. This long-term mechanical ventilation sometimes requires a tracheostomy during infancy, leading to increased risk of infections and of tracheal pro blems, and later on to stigmatization and restrictions in social life. Patients and Method: We therefore evaluated non-invasive mask ventilation i n 4 children between 6 and 15 years of age, who had been ventilated via tra cheal canula since early infancy under polysomnographic control. Results: Best results were obtained with standard face masks in connection with pressure controled timed ventilation. In 1 child we used a volume-cont rolled ventilator. The lack of dyspnea in thee patients can worsen the acce ptance of a face mask, which is more uncomfortable than a tracheal canula. In 2 children we waited with the definite closure of the tracheostomy due t o pavor-like symptoms and laryngeal closure during sleep and problems in ac ceptance of the mask, respectively. In the other 2 children we could demons trate effective non-invasive mask ventilation during temporary tracheal clo sure for several nights. Therefore the tracheostomy was definitely closed. Long-term follow-up with home monitoring showed effectiveness of non-invasi ve ventilation in these cases.