Document Detail

Cephalometric findings in facioscapulohumeral muscular dystrophy patients with obstructive sleep apneas.
MedLine Citation:
PMID:  20174877     Owner:  NLM     Status:  In-Data-Review    
PURPOSES: The purposes of the study are: (1) to establish if cephalometry and upper airway examination may provide tools for detecting facioscapulohumeral (FSHD) patients at risk for obstructive sleep apnea syndrome (OSAS); and (2) to correlate cephalometry and otorhinolaryngologic evaluation with clinical and polysomnographic features of FHSD patients with OSAS.
METHODS: Patients were 13 adults affected by genetically confirmed FSHD and OSAS, 11 men, with mean age 47.1 ± 12.8 years (range, 33-72 years). All underwent clinical evaluation, Manual Muscle Test, Clinical Severity Scale for FSHD, Epworth Sleepiness Scale, polysomnography, otorhinolaryngologic evaluation, and cephalometry.
RESULTS: Cephalometric evidence of pharyngeal narrowing [posterior airways space (PAS) < 10 mm] was present in only one patient. The mandibular planus and hyoid (MP-H) distance ranged from 6.5 to 33.1 mm (mean, 17.5 ± 7.8 mm). The mean length of soft palate (PNS-P) was 31.9 ± 4.8 mm (range, 22.2 to 39.7 mm). No patient presented an ANB angle > 7°. There was no significant correlation between cephalometric measures, clinical scores, and PSG indexes. PAS and MP-H were not related to the severity of the disease.
CONCLUSIONS: Upper airway morphological evaluation is of poor utility in the clinical assessment of FSHD patients and do not allow to predict the occurrence of sleep-related upper airway obstruction. This suggests that the pathogenesis of OSAS in FSHD is dependent on the muscular impairment, rather than to the anatomy of upper airways.
Giacomo Della Marca; Francesca Pantanali; Roberto Frusciante; Emanuele Scarano; Alessandro Cianfoni; Lea Calò; Serena Dittoni; Catello Vollono; Anna Losurdo; Elisa Testani; Salvatore Colicchio; Valentina Gnoni; Elisabetta Iannaccone; Benedetto Farina; Tommaso Pirronti; Pietro A Tonali; Enzo Ricci
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Publication Detail:
Type:  Journal Article     Date:  2010-02-20
Journal Detail:
Title:  Sleep & breathing = Schlaf & Atmung     Volume:  15     ISSN:  1522-1709     ISO Abbreviation:  Sleep Breath     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-05-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9804161     Medline TA:  Sleep Breath     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  99-106     Citation Subset:  IM    
Department of Neurosciences, Catholic University, Rome, Italy,
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