title: [Anesthetic management for a patient with scoliosis combined with central core disease].
abstract: We gave general anesthesia to a patient with scoliosis combined with central core disease (CCD). CCD is a slowly progressive autosomal dominant congenital myopathy. CCD is presented typically in infancy with hypotonia and delay of motor development, characterized by predominantly proximal weakness pronounced in the hip girdle. Orthopedic complications are common with congenital dislocation of the hips, scoliosis and foot deformity. CCD is due to mutations in the skeletal muscle ryanodine receptor (RYR1) gene at chromosome 19q13.1, which is also implicated in the malignant hyperthermia (MH). A patient with CCD is at risk of MH, with an abnormal response to suxamethonium and volatile anesthetics. The anesthetist ought to be aware of the diagnosis of CCD and to plan anesthetic management accordingly, avoiding potentially MH-triggering agents. We used total intravenous anesthesia (TIVA) in this case, and he showed no MH symptoms perioperatively. This report demonstrates that anesthesia in a patient with CCD could be successfully maintained with TIVA.
authors.affiliation: Department of Anesthesiology, Iwate Medical University, Morioka 020-8505.
authors.forename: Naoto
authors.initials: N
authors.lastname: Yamada
authors.forename: Yuuichirou
authors.initials: Y
authors.lastname: Tamura
authors.forename: Kou
authors.initials: K
authors.lastname: Ishikawa
authors.forename: Tasuku
authors.initials: T
authors.lastname: Suzuki
authors.forename: Haruo
authors.initials: H
authors.lastname: Ookawa
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Nagata
mesh_terms: D000293:Adolescent; D000771:Anesthesia, Intravenous / Q000379:methods*; D018686:Anesthetics, Intravenous / Q000008:administration & dosage; D006801:Humans; D008297:Male; D020512:Myopathy, Central Core / Q000150:complications*; D010880:Piperidines / Q000008:administration & dosage; D015742:Propofol / Q000008:administration & dosage; D000077208:Remifentanil; D012600:Scoliosis / Q000150:complications / Q000601:surgery*
chemical_list: D018686:Anesthetics, Intravenous; D010880:Piperidines; D000077208:Remifentanil; D015742:Propofol