RT Journal Article
SR Electronic
T1 Impact of cordectomy on nocturnal muscle effort indexes in a patient with respiratory stridor caused by hereditary motor neuropathy
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP P56
DO 10.1183/23120541.sleepandbreathing-2017.P56
VO 3
IS suppl 1
A1 Francesco Mari
A1 Niccolo Nassi
A1 Beatrice Berti
A1 Roberto Baggi
A1 Lorenzo Mirabile
A1 Raffaele Piumelli
A1 Renzo Guerrini
YR 2017
UL http://openres.ersjournals.com/content/3/suppl_1/P56.abstract
AB Introduction respiratory dysfunction during sleep caused by vocal cord involvement with stridor and diaphragmatic paralysis has been described in patients with Charcot-Marie-Tooth hereditary neuropathy type 2C (CMT2C).Aims and objective evaluate the positive effect of early cordectomy on muscular respiratory effort indexes in a pediatric patient with respiratory stridor due to complete bilateral vocal cord paralysis caused by mutation in TRPV4 gene.Methods serial PSG evaluations performed before and immediately after cordectomy.Results Respiratory Mechanical Instability (RMI) parameters evaluation disclosed the need of increase of respiratory muscular effort to obtain the good respiratory function during sleep. Laryngoscopic surgery was performed and serial cardiorespiratory monitorings confirmed the reduction of RMI parameter along the time.Conclusions early cordectomy could be used in patients with bilateral vocal cord paralyses due to CMT2C to reduce the stridor and the nocturnal muscular efforts.