UPPER AIRWAY-OBSTRUCTION IN THE SYNDROMAL CRANIOSYNOSTOSIS

This series of consecutive cases details the prevalence and management of upper airway obstruction in the syndromal craniosynostoses (Crouzon, Apert and Pfeiffer syndromes). Upper airway obstruction presents more frequently in Crouzon and Pfeiffer syndrome when presenting early and during the intermediate years. Those patients with Apert syndrome appear relatively free of this problem. Management has been directed toward increasing the size of the nasopharyngeal space by soft tissue alterations (uvulopalatopharyngoplasty, soft palatal split and adenotonsillectomy) with success. Le Fort III advancement osteotomy has been reserved for those more extreme cases, again with objective airway improvement. These techniques have removed the necessity for the progression to tracheostomy in these cases.