title: Stuve Wiedemann syndrome and related syndromes: case report and possible anesthetic complications.
abstract: Stuve Wiedemann syndrome (SWS) is an autosomal recessively inherited syndrome which is characterized by bowing of the long bones, camptodactyly, facial dysmorphism, hypotonia, feeding and swallowing difficulties, and respiratory distress. In most cases episodes of unexplained hyperthermia are present. Patients with SWS can develop hyperthermia in conjunction with anesthesia and surgery, and a relationship has been suggested between the syndrome and malignant hyperthermia. We describe a 3-year-old child diagnosed with SWS to whom we administered general anesthesia during the removal of a corneal ulcer and dilatation of the lacrimal duct. Our patient had received, uncomplicated, inhalational anesthesia five times previously for different operations. There were no anesthesia-related complications in the present or previous perioperative periods. On one occasion the patient developed mild postoperative hyperthermia. We believe that this hyperthermia is different from the specific disorder of malignant hyperthermia and that sevoflurane can be safely used in patients with SWS. We also describe symptomatically related syndromes and their theoretical risks for anesthesia.
authors.affiliation: Department of Anesthesiology, Pain and Palliative Care, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands.
authors.forename: Dominique
authors.initials: D
authors.lastname: Bonthuis
authors.forename: Eva
authors.initials: E
authors.lastname: Morava
authors.forename: Leo H D J
authors.initials: LH
authors.lastname: Booij
authors.forename: Jacques J
authors.initials: JJ
authors.lastname: Driessen
mesh_terms: D000758:Anesthesia; D001842:Bone and Bones / Q000002:abnormalities; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000601:surgery*; D004569:Electroencephalography; D005145:Face / Q000002:abnormalities; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009123:Muscle Hypotonia / Q000151:congenital; D013577:Syndrome