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  • Title: [Anesthetic management in a patient with Beckwith-Wiedemann syndrome].
    Author: Fujita A, Okutani R, Fukuda T, Fu K, Okamoto T.
    Journal: Masui; 1994 Sep; 43(9):1389-91. PubMed ID: 7967041.
    Abstract:
    Beckwith-Wiedemann syndrome has three major symptoms, exomphalos, macroglossia and giantism. We report the anesthetic management of a 2-year-old male, weighing 16.1 kg and with a height of 94.6 cm, associated with bilateral retention testis. The anesthesia was maintained with oxygen, nitrous oxide, and sevoflurane. The surgery was completed safely without adverse events such as difficulty in tracheal intubation or refractory hypoglycemia. The anesthetic management of this syndrome requires: an assessment of difficulty in tracheal intubation and treatment, an understanding of the capacity of the patient's glucose metabolism, and assessment of complicated deformities. In particular, it is necessary to carry out a preoperative examination of the cardiovascular system.
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