Marshall-Smith 症候群患児の全身麻酔経験

Marshall-Smith syndrome(MSS)is an autosomal dominant inheritance disease characterized by hypoplasia of the middle face, sensory deafness, a tendency for bone fractures, and intellectual disability. For anesthetic management, difficult airway management because of mandibular hypoplasia has been reported. The removal of two mesiodens was planned in a ten-year-old boy with MSS. He had a history of bone fractures, mild intellectual disability, and the facial characteristics of MSS. A CT scan showed the partial obstruction of the upper airway and mandibular hypoplasia. Prior to entry into the operational room, midazolam was injected intravenously for sedation. General anesthesia was induced with propofol, fentanyl, sevoflurane and rocuronium. A nasal airway was effective for airway management, and a McGRATH^® MAC was used for intubation through the nasal cavity. General anesthesia was maintained with total intravenous anesthesia(TIVA), consisting of propofol and fentanyl. The operation was completed in 30 minutes, and no signs of agitation were observed during the emergence period. There were no significant changes in SpO₂ or other parameters throughout the anesthetic management. Leg protectors were used to prevent bone fractures throughout the preoperative period. An assessment of the airway using a CT scan was useful for predicting the difficulty of airway management and intubation. A nasal/oral airway and video laryngeal scope are strongly recommended for the induction of anesthesia in patients with MSS. Since TIVA can enable a calm emergence from anesthesia, it is likely to contribute to the prevention of bone fractures.