title: [The hereditary motor-sensory neuropathy Charcot-Marie-Tooth disease: anesthesiologic management--case report with literature review].
abstract: A 53-year-old woman diagnosed as having hereditary motor-sensory neuropathy Charcot-Marie-Tooth (CMT) disease Type 2, underwent inguinal hernia surgery. In this patient CMT disease was manifested as distal muscle weakness and wasting. Anaesthetic experience with patients who have CMT disease is limited. Association to malignant hyperthermia is very unlikely although there is one case report that shows that there could be a relationship. We describe a total intravenous anaesthesia (TIVA) protocol with propofol and alfentanil without any muscle relaxants after fiberoptic intubation. The patient made an uneventful recovery and was discharged from the hospital on the fourth postoperative day. TIVA was a safe technique in this patient and should be considered as an alternative for patients presenting with CMT disease.
authors.affiliation: Departement Anästhesie, Kantonsspital Basel, Universitätskliniken, 4031 Basel, Schweiz. hginz@uhbs.ch
authors.forename: H F
authors.initials: HF
authors.lastname: Ginz
authors.forename: W C
authors.initials: WC
authors.lastname: Ummenhofer
authors.forename: T
authors.initials: T
authors.lastname: Erb
authors.forename: A
authors.initials: A
authors.lastname: Urwyler
mesh_terms: D000771:Anesthesia, Intravenous; D002607:Charcot-Marie-Tooth Disease / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006552:Hernia, Inguinal / Q000601:surgery; D006801:Humans; D008305:Malignant Hyperthermia / Q000150:complications / Q000517:prevention & control; D008875:Middle Aged