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  • Title: [Propofol-fentanyl anesthesia for a 13-year-old patient with Beckwith-Wiedemann syndrome].
    Author: Anraku S, Ushijima K, Terasaki H.
    Journal: Masui; 2001 Nov; 50(11):1224-6. PubMed ID: 11758329.
    Abstract:
    We report anesthetic management of a 13-year-old boy with Beckwith-Wiedemann syndrome for urethral fistulectomy using propofol-fentanyl anesthesia. The syndrome has three major symptoms; exomphalos, macroglossia and gigantism. Other complications including neonatal hypoglycemia, visceromegaly, metabolic disorders, malignant tumors, and cardiovascular abnormalities may also be seen. This patient was complicated with gigantism, exomphalos, hypospadias, cryptorchidism, hepatoblastoma, accessory mammae, and left axis deviation with left anterior fascicular block. A comprehensive preoperative examination should include assessment of the airway, cardiovascular conditions, endocrine status, blood glucose, and electrolytes. In this patient, airway management was easy by laryngeal mask airway and the anesthetic course was uneventful.
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