Document Detail


Otolaryngological manifestations of arthrogryposis multiplex congenita.
MedLine Citation:
PMID:  949154     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The important otolaryngological manifestations of arthrogryposis multiplex congenita (AMC), which heretofore have not been described in otolaryngological and other specialty journals, are reviewed. Thirty-seven patients with AMC were studied. Nine of these patients with neurogenic AMC, had otolaryngological manifestations. Six of these had Pierre Robin-like syndromes. Seven patients had severe dysphagia and aspiration pneumonitis. Five patients had voice changes, and three of these required tracheotomy to relieve laryngeal obstruction and prevent aspiration. Two of these patients were aphonic and had laryngeal paralysis. Three of the nine patients who had laryngeal examination showed supraglottic narrowing similar to laryngomalacia. Six patients with otolaryngological problems were autopsied, and two had pharyngeal biopsies. The histological examination demonstrated normal laryngeal and pharyngeal musculature. The cricoarytenoid joints were found normal in all the larynges examined. Central nervous system dysfunction rather than simple muscle weakness, caused the dysphagia and respiratroy difficulties in patients with neurogenic AMC. Dysphagia with aspiration was the single most important cause of death in approximately 20% of patients with AMC. A tracheotomy and feeding gastrostomy appears essential for successful management of the otolaryngological complications of AMC.
Authors:
S R Cohen; H Isaacs
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Annals of otology, rhinology, and laryngology     Volume:  85     ISSN:  0003-4894     ISO Abbreviation:  Ann. Otol. Rhinol. Laryngol.     Publication Date:    1976 Jul-Aug
Date Detail:
Created Date:  1976-10-01     Completed Date:  1976-10-01     Revised Date:  2006-05-15    
Medline Journal Info:
Nlm Unique ID:  0407300     Medline TA:  Ann Otol Rhinol Laryngol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  484-90     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Airway Obstruction / etiology
Arthrogryposis / complications*,  etiology,  pathology
Deglutition Disorders / etiology
Esophagus / pathology
Female
Humans
Infant
Infant, Newborn
Jaw Abnormalities / complications
Laryngeal Cartilages / abnormalities
Laryngoscopy
Larynx / abnormalities,  pathology
Male
Muscle Tonus
Otorhinolaryngologic Diseases / etiology*
Pharynx / pathology
Pneumonia, Aspiration / etiology
Salivation
Voice

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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