We present a 5-year-old boy with late infantile form of Krabbe disease who required general anesthesia for dental treatment. He was diagnosed with Krabbe disease at 1 year and 6 months old, and bone marrow transplantation was performed to prevent the neurological deterioration at 2 years old. He presented severe neurodegeneration and quadriplegia, and could not move actively except for tossing about from laterel to supine position. A diazepam syrup (2 mg) was administered 90 minutes before the start of anesthesia. After the establishment of a venous route, anesthesia was induced with thiopental sodium (50 mg), and nasotracheal intubation was facilitated with vecuronium bromide (1.4 mg). The anesthesia was maintained with N_2O (3.0l/min), O_2 (1.5l/min) and sevoflurane (1.0～2.0%). Pulpectomy and dental prosthesis for every 20 deciduous teeth was performed, and the anesthesia was completed uneventfully after 5 hours and 50 minutes.
Krabbe病患者の全身麻酔経験
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