Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy. / Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras.
Rev Esp Anestesiol Reanim (Engl Ed)
; 65(8): 469-472, 2018 Oct.
Article
in En, Es
| MEDLINE
| ID: mdl-29699707
ABSTRACT
Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period.
Key words
Anestesia intravenosa; Apnea obstructiva del sueño; Congenital fibre type disproportion; Congenital structural myopathies; Desproporción congénita del tipo de fibras; Intravenous anaesthesia; Miopatías estructurales congénitas; Obstructive sleep apnoea hypopnoea syndrome; Rocuronio; Rocuronium; Sugammadex
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Tonsillectomy
/
Adenoidectomy
/
Sleep Apnea, Obstructive
/
Anesthesia
Limits:
Child, preschool
/
Female
/
Humans
Language:
En
/
Es
Journal:
Rev Esp Anestesiol Reanim (Engl Ed)
Year:
2018
Document type:
Article