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Nager's acrofacial dysostosis. Case report and review of the literature.
Int J Pediatr Otorhinolaryngol. 1990 Dec; 20(3):225-40.IJ

Abstract

Nager's acrofacial dysostosis is a rare congenital anomaly in which mandibulofacial dysostosis is seen in combination with limb deformities, particularly hypoplasia of the radial aspect of the hand. A boy with a severe manifestation of this syndrome is presented. Deformities include severe bilateral mandibular hypoplasia with trismus, malar hypoplasia, downward sloping palpebral fissures, high-arched hard palate, absent soft palate, external auditory and middle ear abnormalities and bilateral hand deformities. The diagnosis and treatment difficulties in this patient are discussed in light of those reported in the literature. An interdisciplinary team approach to the management of patients with Nager syndrome is emphasized.

Authors+Show Affiliations

Department of Otolaryngology, State University of New York, School of Medicine, Buffalo.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

2089020

Citation

Danziger, I, et al. "Nager's Acrofacial Dysostosis. Case Report and Review of the Literature." International Journal of Pediatric Otorhinolaryngology, vol. 20, no. 3, 1990, pp. 225-40.
Danziger I, Brodsky L, Perry R, et al. Nager's acrofacial dysostosis. Case report and review of the literature. Int J Pediatr Otorhinolaryngol. 1990;20(3):225-40.
Danziger, I., Brodsky, L., Perry, R., Nusbaum, S., Bernat, J., & Robinson, L. (1990). Nager's acrofacial dysostosis. Case report and review of the literature. International Journal of Pediatric Otorhinolaryngology, 20(3), 225-40.
Danziger I, et al. Nager's Acrofacial Dysostosis. Case Report and Review of the Literature. Int J Pediatr Otorhinolaryngol. 1990;20(3):225-40. PubMed PMID: 2089020.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Nager's acrofacial dysostosis. Case report and review of the literature. AU - Danziger,I, AU - Brodsky,L, AU - Perry,R, AU - Nusbaum,S, AU - Bernat,J, AU - Robinson,L, PY - 1990/12/1/pubmed PY - 1990/12/1/medline PY - 1990/12/1/entrez SP - 225 EP - 40 JF - International journal of pediatric otorhinolaryngology JO - Int J Pediatr Otorhinolaryngol VL - 20 IS - 3 N2 - Nager's acrofacial dysostosis is a rare congenital anomaly in which mandibulofacial dysostosis is seen in combination with limb deformities, particularly hypoplasia of the radial aspect of the hand. A boy with a severe manifestation of this syndrome is presented. Deformities include severe bilateral mandibular hypoplasia with trismus, malar hypoplasia, downward sloping palpebral fissures, high-arched hard palate, absent soft palate, external auditory and middle ear abnormalities and bilateral hand deformities. The diagnosis and treatment difficulties in this patient are discussed in light of those reported in the literature. An interdisciplinary team approach to the management of patients with Nager syndrome is emphasized. SN - 0165-5876 UR - https://www.unboundmedicine.com/medline/citation/2089020/Nager's_acrofacial_dysostosis__Case_report_and_review_of_the_literature_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0165-5876(90)90353-S DB - PRIME DP - Unbound Medicine ER -