NAGER ACROFACIAL DYSOSTOSIS - MANAGEMENT OF A DIFFICULT AIRWAY

Nager acrofacial dysostosis, first described by Nager and deReynier in 1948, is a rare syndrome characterized by mandibulofacial dysostosis with associated radial defects. The facial features include downward s lanting palpebral fissures, absent eyelashes in the medial third of th e lower lids, mandibular and malar hypoplasia, dysplastic ears with co nductive deafness, and variable degrees of palatal clefting. Upper lim b malformation is a constant feature of Nager syndrome and ranges from thumb hypoplasia to absence of the radial ray. The maxillo-mandibular hypoplasia and associated retroplaced tongue set the stage for early and significant upper airway obstruction. The craniofacial anomalies a nd associated trismus make emergent airway intubation challenging. We present a case of Nager syndrome with life threatening airway obstruct ion unresponsive to conservative management. This case and a review of the literature emphasize the importance of early tracheotomy for thes e patients.