Usefulness of dexmedetomidine to prevent emergence agitation in a patient with Krabbe disease: a case report.

We report the case of a child with Krabbe disease who underwent three repeated surgeries and anesthetic management, and we discuss the major concerns about Krabbe disease and the usefulness of a perioperative administration of dexmedetomidine to prevent emergence agitation and hypertension. The patient was scheduled to undergo bilateral orchiopexy, adenotonsillectomy, and knee flexor tendon lengthening under general anesthesia during a 2-year period.

Adenotonsillectomy was scheduled as the second operation when the patient was 4 years old. His height and body weight were 93 cm and 10.3 kg, respectively. Anesthesia was induced with 8% sevoflurane mixed with 6 L/min of O₂ and maintained with N₂O (3.5 L/min), O₂ (1.5 L/min), and sevoflurane (1.5-2.0%). Upon completion of the right tonsillectomy, 1 h before the end of the surgery, a continuous infusion of dexmedetomidine (0.2 μg/kg/h) was started to prevent emergence agitation, irritability, resultant hypertension, and postoperative bleeding. Fentanyl (25 μg) was administered intravenously to reduce postoperative pain. The surgery was uneventful, and the patient's emergence from general anesthesia was prompt. He exhibited no symptoms of emergence agitation or irritability. During his stay in the intensive care unit, 0.2-0.7 μg/kg/h of dexmedetomidine and 6.25 μg/h of fentanyl were continuously administered. The patient was discharged to the ward the following morning without complications.

The perioperative administration of dexmedetomidine was useful to prevent emergence agitation, hypertension, and resultant postoperative bleeding in a pediatric patient with Krabbe disease. Three repeated anesthetic management using inhalation anesthesia were completed uneventfully without muscle relaxants.