Document Detail


Nager's acrofacial dysostosis. Case report and review of the literature.
MedLine Citation:
PMID:  2089020     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
I Danziger; L Brodsky; R Perry; S Nusbaum; J Bernat; L Robinson
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  International journal of pediatric otorhinolaryngology     Volume:  20     ISSN:  0165-5876     ISO Abbreviation:  Int. J. Pediatr. Otorhinolaryngol.     Publication Date:  1990 Dec 
Date Detail:
Created Date:  1991-05-30     Completed Date:  1991-05-30     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  8003603     Medline TA:  Int J Pediatr Otorhinolaryngol     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  225-40     Citation Subset:  IM    
Affiliation:
Department of Otolaryngology, State University of New York, School of Medicine, Buffalo.
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MeSH Terms
Descriptor/Qualifier:
Fingers / abnormalities*
Hand Deformities, Congenital* / pathology
Humans
Infant
Infant, Newborn
Jaw Abnormalities / pathology
Male
Mandibulofacial Dysostosis* / pathology

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