Ｂｅｃｋｗｉｔｈ‐Ｗｉｅｄｅｍａｎｎ症候群の周術期における気道確保の問題点について

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  • Perioperative Airway Management of Beckwith-Wiedemann Syndrome.

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Beckwith-Wiedemann syndrome consists of three major symptoms, exomphalos, macroglossia and giantism. A 20-month-old infant with Beckwith-Wiedemann syndrome underwent palatoplasty. Her sister with Beckwith-Wiedemann syndrome had died suddenly after palatoplasty. Anesthesia was induced with nitrous oxide and sevoflurane. When the depth of anesthesia was adequate, tracheal intubation was attempted using RAE tube® (I.D.4.5mm). However, it was impossible to insert it into the trachea because the larynx and vocal cords were swollen. Therefore, we intubatod with a smaller diameter RAE tube®(I.D. 4.0mm). However, it was pulled out. <br>Accidentally when the neck was extended backward for the placement of a mouth gag. A Sweigh-type tracheal tube® (I.D. 4.0mm) was inserted at an adequate depth into the trachea. Anesthesia was maintained with nitrous oxide and sevoflurane. After surgery, the tongue was thrust f oward and fixed with a thread to prevent airway obstruction. The tracheal tube was extubated after the patient had fully recovered from anesthesia. We administered methylprednisolone 125mg intravenously to prevent edema in the mouth. The postoperative course was uneventful, and the thread was removed 2 days after operation.

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