title: [Arthrogryposis multiplex congenita: special anesthesiological aspects].
abstract: Case report on a 2.5-year old girl suffering from arthrogryposis multiplex congenita (AMC) who was admitted for an extensive orthopaedic operation of equinovarus. The patient showed typical AMC-related problems such as skin and subcutaneous tissue abnormalities, lack of veins, contractural deformities of all four limbs and microgenia. Problems associated with anaesthesia in this patient were difficult intubation and venipuncture and a potential risk of developing malignant hyperthermia when using volatile anaesthetics. For preoperative blood chemistry sampling and intravenous induction of general anaesthesia, the patient received a central venous catheter under local and N2O/O2 anaesthesia on the day before surgery. Following intravenous induction of trigger-free anaesthesia using fentanyl, thiopental and vecuronium, the child was intubated and ventilated with 30% O2 in N2O the next day. A caudal catheter was inserted for intraoperative reduction of anaesthetics and postoperative pain relief. Intraoperatively, caudal anaesthesia was performed with 2 ml of 2% mepivacaine every 90 min. No inadvertent reactions were seen during a 7 h operation. In the recovery room, the patient received 4 ml of plain 0.25% bupivacaine per 4 h via the caudal catheter and had excellent analgesia during 24 postoperative hours. The following course was uneventful and the child was discharged from hospital two weeks later. AMC-related problems concerning the management of anaesthesia are discussed.
authors.affiliation: Abteilung für Anästhesiologie, Hamburg.
authors.forename: T
authors.initials: T
authors.lastname: Standl
authors.forename: F
authors.initials: F
authors.lastname: Wappler
mesh_terms: D000763:Anesthesia, Caudal; D001176:Arthrogryposis / Q000601:surgery*; D002675:Child, Preschool; D003025:Clubfoot / Q000601:surgery*; D005260:Female; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control*; D010102:Oxygen Inhalation Therapy; D011300:Preoperative Care; D012307:Risk Factors