title: Congenital central hypoventilation syndrome; safety of early transition to non-invasive ventilation.
abstract: Congenital central hypoventilation syndrome (CCHS) is a rare disorder of autonomic dysregulation, characterized by alveolar hypoventilation especially during sleep. As a result, lifelong ventilatory assistance is necessary in these patients. Many infants and children initially require positive pressure ventilation via tracheostomy for support. Associated complications and psychosocial pressure may prompt early transition to non-invasive ventilation. We present the details of a patient with CCHS who successfully transitioned from tracheostomy to bilevel positive airway pressure ventilation at an early age of 3 years.
authors.affiliation: Section of Respiratory Medicine, Alberta Children's Hospital, Calgary, Alberta, Canada.
authors.forename: Karen
authors.initials: K
authors.lastname: Kam
authors.forename: Candice
authors.initials: C
authors.lastname: Bjornson
authors.forename: Ian
authors.initials: I
authors.lastname: Mitchell
mesh_terms: D005260:Female; D006801:Humans; D007040:Hypoventilation / Q000151:congenital* / Q000628:therapy; D007231:Infant, Newborn; D063087:Noninvasive Ventilation; D061214:Patient Safety; D020182:Sleep Apnea, Central / Q000628:therapy*; D013997:Time Factors
keywords: central sleep apnea; child; female; hypoventilation/congenital; hypoventilation/therapy; infant; non-invasive ventilation; tracheostomy; transition