Airway Statuses and Nasopharyngeal Airway Use for Airway Obstruction in Syndromic Craniosynostosis

Abstract

Syndromic craniosynostosis is associated with a high rate of respiratory difficulty, due mainly to midfacial hypoplasia. Nasopharyngeal airway establishment has been reported as the first-line approach to airway obstruction and may obviate the need for a highly invasive tracheotomy. No previous studies have compared airway obstruction status in syndromic craniosynostosis between cases requiring and not requiring airway managements. We focus on nasopharyngeal airway use and airway status outcomes to assess respiratory difficulty in patients with syndromic craniosynostosis. A retrospective data analysis of 51 cases with syndromic craniosynostosis was carried out. We divided 30 of the 51 cases with lateral pharyngeal x-rays taken before operations affecting airway diameters into 2 groups, one with neither nasopharyngeal airway insertion nor tracheotomy and the other with one or both of these interventions, and the mean diameters for 8 indices related to the pharyngeal space were compared. Cases with respiratory difficulty due to nasopharyngeal stenosis and requiring airway managements comprised a significantly higher proportion of those with Pfeiffer syndrome than patients with Crouzon or Apert syndrome. Comparative examination of lateral x-ray cephalometry between cases with neither nasopharyngeal airway insertion nor tracheotomy and cases with one or both revealed oropharyngeal diameters tended to be smaller in those with interventions. Cases requiring nasopharyngeal airway insertion were able to continue nasopharyngeal airway use for more than 1 year and a considerable number avoided tracheotomy. It may be worth considering an oropharyngeal-bypass nasopharyngeal airway before performing a tracheotomy.

Perioperative airway complications in infants and children with Crouzon and Pfeiffer syndromes: A single‐center experience

“…In our cohort, just under half had a tracheostomy at some point in their childhood. Some, especially, those who avoid a tracheostomy, may require a long‐term NPA to maintain upper airway patency, 15 and this was the case in one‐third of patients in our study.…”

Craniofacial surgery and specific airway problems

“…Almost half of patients with Apert, Crouzon, or Pfeiffer syndrome develop obstructive sleep apnea, mainly due to midface hypoplasia . Additionally, stenosis of the epipharynx caused by maxillar hypoplasia and stenosis of the mesopharynx caused by mandibular hypoplasia, contribute to airway obstruction even when adenotonsillar hypertrophy is absent . Consequently, adenotonsillectomy often may not relieve the obstruction .…”

Baja talla por déficit selectivo e idiopático de hormona de crecimiento asociado al Síndrome de Crouzon

“…22 Se trata de un síndro-me genéticamente heterogéneo, donde las mutaciones en el gen FGFR1 se asocian casi siempre a manifestaciones más leves. 18,19,23,24 Numerosas dismorfias se han asociado a la baja talla, siendo las causas diversas; se ha observado en los siguientes síndromes: Crouzon, Wolf, Síndrome del brazo largo del cromosoma 12, Asskorg, Aspert, Blom, Cockeyne, Conredi o condrodistrofia punctata, Cornelia-Lange, Donohue o Leprechuanismo y en el Síndrome de Ellis-Van Creveld o displasia condro-ectodérmica.…”