title: [Anesthesiologic management of ankle joint osteosynthesis in the 35th week of pregnancy in a patient with Charcot-Marie-Tooth syndrome].
abstract: We describe the case of an extremely overweight woman in her 35 th week of pregnancy with a manifest Charcot-Marie-Tooth syndrome (CMT), on whom a tibial fracture was operated under general anaesthesia. Anaesthesia was induced with thiopental, droperidol, fentanyl and maintained with nitrous oxide and isoflurane. Atracurium was administered as a muscle relaxant agent. Depolarizing muscle relaxants have not been used because of a significantly increased risk of hyperkalemia. The immediate intubation using the principle of a so-called "priming dose" was within normal limits. CMT syndrome is a very rare hereditary autosomal dominant degenerative disease of the peripheral nervous system. The main site of manifestation is the peroneus muscle. In case of affection of the respiratory muscular system, which acutely can occur postoperatively even without any suspicious findings in the preoperatively performed lung function tests, an insufficiency of the respiratory tract is predominant and may require a postoperative intensive care with a prolonged artificial respiration. With the application of non-depolarizing muscle relaxants significant prolongations of the half-life period have occasionally been observed. Therefore we recommend the monitoring of the neuromuscular blockade perioperatively using the train-of-four ratio. An extraordinary progression of the disease frequently occurs during pregnancy.
authors.affiliation: Institut für Anästhesie und Intensivmedizin Chefarzt Prof. G. Lenz, Klinikum Ingolstadt.
authors.forename: U J
authors.initials: UJ
authors.lastname: Kirmayer
authors.forename: A
authors.initials: A
authors.lastname: Preisz
mesh_terms: D000328:Adult; D000768:Anesthesia, General; D016512:Ankle Injuries / Q000601:surgery*; D002607:Charcot-Marie-Tooth Disease / Q000601:surgery*; D005260:Female; D006801:Humans; D008991:Monitoring, Physiologic; D011247:Pregnancy; D011248:Pregnancy Complications / Q000601:surgery*; D013978:Tibial Fractures / Q000601:surgery*
vernacular_title: Anästhesiologisches Management zur Sprunggelenksosteosynthese bei einer in der 35. Woche schwangeren Patientin mit einem Charcot-Marie-Tooth-Syndrom.