title: Impact of long-term nasopharyngeal airway on health-related quality of life of children with obstructive sleep apnea caused by syndromic craniosynostosis.
abstract: Obstructive sleep apnea in children with syndromic craniosynostosis is a well-documented entity, and airway management in these group of children is difficult, with well-documented cardiorespiratory and neurodevelopment consequences. Numerous well-documented techniques are available for airway management in this group of children. In our center, the nasopharyngeal airway (NPA) is the first-line treatment. This study aimed to evaluate the improvement in health-related quality of life in this group of children using the Glasgow Children's Benefit Inventory. We conducted a retrospective postal study of 24 patients with craniofacial disorders who had an NPA inserted as part of their airway management. A 79.2% (n = 19) response rate was obtained. Mean (SD) age was 5.8 (4.1) years. Patients were classified into 3 main groups: Crouzon (n = 11), Apert (n = 6), and Pfeiffer (n = 2) syndromes. There was improvement in 3 of the 4 domains, namely, learning (P = 0.006), vitality (P = 0.003), physical (range, -3 to 8; mean, 2; P = 0.005). There was significant improvement in the sleep study parameters; however, no correlation was found between this and the Glasgow Children's Benefit Inventory findings. Parents also preferred an NPA as opposed to a tracheostomy when given a choice. Our study shows that NPA confers significant improvement not only in health-related quality of life but also in severity of obstructive sleep apnea as judged by sleep study parameters. The NPA is also well tolerated by patients and is much preferred by parents for airway management for their children as opposed to having a tracheostomy. We think that this should be considered in other centers as first-line management in children with syndromic craniosynostosis.
authors.affiliation: Great Ormond Street Hospital for Sick Children, London, UK. prandhawa13@hotmail.com
authors.forename: Premjit S
authors.initials: PS
authors.lastname: Randhawa
authors.forename: Jahangir
authors.initials: J
authors.lastname: Ahmed
authors.forename: Syed Reza
authors.initials: SR
authors.lastname: Nouraei
authors.forename: Michelle E
authors.initials: ME
authors.lastname: Wyatt
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000150:complications; D005260:Female; D006801:Humans; D007442:Intubation, Intratracheal / Q000295:instrumentation*; D008297:Male; D011788:Quality of Life; D012189:Retrospective Studies; D020181:Sleep Apnea, Obstructive / Q000209:etiology* / Q000503:physiopathology / Q000517:prevention & control*; D014139:Tracheostomy