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Nager syndrome (nager acrofacial dyostosis)


Nager Syndrome (Nager Acrofacial Dyostosis)

General: Rare congenital syndrome characterized by mandibulofacial dyostosis with associated radial defects.

Ocular: Downward slanting palpebral fissures; absent eyelashes in the medial third of the lower lids.

Clinical: Mandibular and malar hypoplasia; dysplastic ears with conductive deafness; variable degrees of palatal clefting; upper limb malformation (often bilateral hand deformities).

Danziger I, et al. Nager's acrofacial dysostosis. Case report and review of the literature. Int J Pediatr Otorhinolaryngol 1990; 20:225-240.

Friedman RA, et al. Nager acrofacial dysostosis: management of a difficult airway. Int J Pediatr Otorhinolaryngol 1996; 35:69-72.

Pfeiffer RA, Stoess H. Acrofacial dysostosis (Nager syndrome): synopsis and report of a new case. Am J Med Genet 1983; 15:255-260.




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