title: Bradycardia with sevoflurane in siblings with Branchio-oto-renal syndrome.
abstract: Branchio-oto-renal syndrome (BOR, Melnick-Fraser syndrome, MIM#113650) refers to a rare autosomal dominant disorder characterized by branchial cysts or fistulas, hearing loss, external ear malformation, preauricular pits and renal abnormalities. The authors present three episodes of significant bradycardia in two siblings diagnosed with BOR syndrome during the sevoflurane general anesthesia. There is no published experience of anesthesia with this syndrome. Bradycardia occurred variously at induction, maintenance and immediately prior to emergence and required surgical stimulation, atropine, or epinephrine to treat. We seek to raise awareness of the potential for bradycardia during the procedures in patients with this syndrome requiring volatile anesthesia, especially sevoflurane.
authors.affiliation: Department of Anaesthesia and Operating Rooms, Starship Children's Hospital, Auckland, New Zealand. matttnz@gmail.com
authors.forename: Matthew H G
authors.initials: MH
authors.lastname: Taylor
authors.forename: Niall C T
authors.initials: NC
authors.lastname: Wilton
mesh_terms: D000768:Anesthesia, General / Q000009:adverse effects; D018685:Anesthetics, Inhalation / Q000009:adverse effects*; D000889:Anti-Arrhythmia Agents / Q000008:administration & dosage; D001285:Atropine / Q000008:administration & dosage; D001919:Bradycardia / Q000139:chemically induced* / Q000628:therapy; D019280:Branchio-Oto-Renal Syndrome / Q000601:surgery*; D001993:Bronchodilator Agents / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography / Q000379:methods; D004837:Epinephrine / Q000008:administration & dosage; D005260:Female; D006339:Heart Rate / Q000187:drug effects; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008738:Methyl Ethers / Q000009:adverse effects*; D008876:Middle Ear Ventilation / Q000379:methods; D008991:Monitoring, Physiologic / Q000379:methods; D010100:Oxygen; D000077149:Sevoflurane; D035781:Siblings
chemical_list: D018685:Anesthetics, Inhalation; D000889:Anti-Arrhythmia Agents; D001993:Bronchodilator Agents; D008738:Methyl Ethers; D000077149:Sevoflurane; D001285:Atropine; D010100:Oxygen; D004837:Epinephrine