title: [Anesthetic management for partial tongue resection in a patient with Beckwith-Wiedemann syndrome].
abstract: A 14-month-old baby weighing 4300 g was a giant infant with macroglossia. Exomphalos was not present, but diastasis recti abdominis was observed. The patient was therefore diagnosed as having Beckwith-Wiedemann syndrome (EMG syndrome). Other characteristic signs such as neonatal hypoglycemia, hemihypertrophy, and a small ventricular septal defect were also recognized, but nephromegaly or hepatomegaly was not present. Tongue reduction by wedge resection was performed under general anesthesia. Some of the problems associated with anesthetic management in this syndrome are hypoglycemia, airway obstruction and cardiovascular status. After induction with increasing concentration of halothane (0.5-4.0%) and 66% nitrous oxide in oxygen, a nasotracheal tube was inserted. Endotracheal intubation was easy without using a neuromuscular blocking agent. Anesthetic maintenance was accomplished with nitrous oxide 66% in oxygen and halothane 0.5-1.0% and no neuromuscular blocking agent was used. The plasma glucose level was kept within normal ranges during and after the operation by infusion of acetate Ringer's solution with 5% glucose. The postoperative progress was uneventful.
authors.affiliation: Department of Anesthesiology, Toyokawa City Hospital.
authors.forename: T
authors.initials: T
authors.lastname: Kato
authors.forename: Y
authors.initials: Y
authors.lastname: Ochiai
authors.forename: Y
authors.initials: Y
authors.lastname: Naganawa
authors.forename: I
authors.initials: I
authors.lastname: Maki
authors.forename: Y
authors.initials: Y
authors.lastname: Ozawa
authors.forename: M
authors.initials: M
authors.lastname: Ohnishi
authors.forename: T
authors.initials: T
authors.lastname: Hata
mesh_terms: D000769:Anesthesia, Inhalation; D001506:Beckwith-Wiedemann Syndrome / Q000601:surgery*; D006221:Halothane; D006801:Humans; D007223:Infant; D008260:Macroglossia / Q000601:surgery*; D008297:Male; D009609:Nitrous Oxide; D010100:Oxygen
chemical_list: D009609:Nitrous Oxide; D010100:Oxygen; D006221:Halothane