Tags

Type your tag names separated by a space and hit enter

Nager acrofacial dysostosis: management of a difficult airway.
Int J Pediatr Otorhinolaryngol. 1996 Mar; 35(1):69-72.IJ

Abstract

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 slanting palpebral fissures, absent eyelashes in the medial third of the lower lids, mandibular and malar hypoplasia, dysplastic ears with conductive deafness, and variable degrees of palatal clefting. Upper limb 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 and associated trismus make emergent airway intubation challenging. We present a case of Nager syndrome with life threatening airway obstruction unresponsive to conservative management. This case and a review of the literature emphasize the importance of early tracheotomy for these patients.

Authors+Show Affiliations

Department of Pediatric Otolaryngology, Children's Hospital and Health Center, San Diego, California 92123, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

8882111

Citation

Friedman, R A., et al. "Nager Acrofacial Dysostosis: Management of a Difficult Airway." International Journal of Pediatric Otorhinolaryngology, vol. 35, no. 1, 1996, pp. 69-72.
Friedman RA, Wood E, Pransky SM, et al. Nager acrofacial dysostosis: management of a difficult airway. Int J Pediatr Otorhinolaryngol. 1996;35(1):69-72.
Friedman, R. A., Wood, E., Pransky, S. M., Seid, A. B., & Kearns, D. B. (1996). Nager acrofacial dysostosis: management of a difficult airway. International Journal of Pediatric Otorhinolaryngology, 35(1), 69-72.
Friedman RA, et al. Nager Acrofacial Dysostosis: Management of a Difficult Airway. Int J Pediatr Otorhinolaryngol. 1996;35(1):69-72. PubMed PMID: 8882111.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Nager acrofacial dysostosis: management of a difficult airway. AU - Friedman,R A, AU - Wood,E, AU - Pransky,S M, AU - Seid,A B, AU - Kearns,D B, PY - 1996/3/1/pubmed PY - 1996/3/1/medline PY - 1996/3/1/entrez SP - 69 EP - 72 JF - International journal of pediatric otorhinolaryngology JO - Int J Pediatr Otorhinolaryngol VL - 35 IS - 1 N2 - 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 slanting palpebral fissures, absent eyelashes in the medial third of the lower lids, mandibular and malar hypoplasia, dysplastic ears with conductive deafness, and variable degrees of palatal clefting. Upper limb 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 and associated trismus make emergent airway intubation challenging. We present a case of Nager syndrome with life threatening airway obstruction unresponsive to conservative management. This case and a review of the literature emphasize the importance of early tracheotomy for these patients. SN - 0165-5876 UR - https://www.unboundmedicine.com/medline/citation/8882111/Nager_acrofacial_dysostosis:_management_of_a_difficult_airway_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0165587695013040 DB - PRIME DP - Unbound Medicine ER -