Document Detail

Airway obstruction in congenital central hypoventilation syndrome.
MedLine Citation:
PMID:  24842348     Owner:  NLM     Status:  In-Data-Review    
Congenital central hypoventilation syndrome (CCHS) is the failure of the autonomic system to control adequate ventilation while asleep with preserved ventilatory response while awake. We report a case of a patient with CCHS who presented with intrathoracic and extrathoracic airway obstruction after tracheostomy tube decannulation and phrenic nerve pacer placement. Nocturnal polysomnography (NPSG) revealed hypoxia, hypercapnia and obstructive sleep apnoea, which required bilevel positive airway pressure titration. Airway endoscopy demonstrated tracheomalacia and paretic true vocal cords in the paramedian position during diaphragmatic pacing. Laryngeal electromyography demonstrated muscular electrical impulses that correlated with diaphragmatic pacer settings. Thus, we surmise that the patient's upper and lower airway obstruction was secondary to diaphragmatic pacer activity. Thorough airway evaluation, including NPSG and endoscopy, may help identify the side effects of diaphragmatic pacing, such as airway obstruction, in patients with CCHS.
Alexandra K Reverdin; Ricardo Mosquera; Giuseppe N Colasurdo; Cindy K Jon; Roya M Clements
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Publication Detail:
Type:  Journal Article     Date:  2014-05-19
Journal Detail:
Title:  BMJ case reports     Volume:  2014     ISSN:  1757-790X     ISO Abbreviation:  BMJ Case Rep     Publication Date:  2014  
Date Detail:
Created Date:  2014-05-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101526291     Medline TA:  BMJ Case Rep     Country:  England    
Other Details:
Languages:  eng     Pagination:  -     Citation Subset:  IM    
Copyright Information:
2014 BMJ Publishing Group Ltd.
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