Airway obstruction in congenital central hypoventilation syndrome.
BMJ Case Rep
; 20142014 May 19.
Article
em En
| MEDLINE
| ID: mdl-24842348
ABSTRACT
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Nervo Frênico
/
Traqueostomia
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Terapia por Estimulação Elétrica
/
Apneia do Sono Tipo Central
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Apneia Obstrutiva do Sono
/
Hipoventilação
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Child
/
Female
/
Humans
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article