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Inicio Revista Española de Anestesiología y Reanimación (English Edition) Anaesthetic management of a paediatric patient with congenital fibre type dispro...
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Vol. 65. Issue 8.
Pages 469-472 (October 2018)
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Vol. 65. Issue 8.
Pages 469-472 (October 2018)
Case report
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
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15
F. Buisán
Corresponding author
felix.buisan@gmx.es

Corresponding author.
, O. de la Varga, M. Flores, J. Sánchez-Ruano
Servicio de Anestesiología y Reanimación, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
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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.

Keywords:
Congenital fibre type disproportion
Congenital structural myopathies
Obstructive sleep apnoea hypopnoea syndrome
Intravenous anaesthesia
Rocuronium
Sugammadex
Resumen

La desproporción congénita del tipo de fibras (DCTF) es un raro tipo de miopatía caracterizado por debilidad muscular e hipotonía durante la infancia. Las características clínicas incluyen retraso motor, dificultades en la alimentación, debilidad de las extremidades, contracturas articulares y escoliosis. Se describe el tratamiento anestésico de una paciente de 3 años con miopatía DCTF asociada a mutación del gen TPM3, programada para realización de adenoamigdalectomía por presentar un síndrome de apnea-hipopnea obstructiva del sueño (SAHOS). Nuestras principales preocupaciones fueron la posible susceptibilidad a la hipertermia maligna, el riesgo de rabdomiólisis inducida por anestesia, una mayor sensibilidad a los relajantes musculares no despolarizantes y la presencia de SAHOS. La anestesia total intravenosa con propofol y el empleo de rocuronio/sugammadex parecen ser opciones seguras. Dado el alto riesgo de compromiso respiratorio y otras complicaciones, los pacientes deben controlarse estrechamente en el periodo postoperatorio.

Palabras clave:
Desproporción congénita del tipo de fibras
Miopatías estructurales congénitas
Apnea obstructiva del sueño
Anestesia intravenosa
Rocuronio
Sugammadex

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