Saito Junichi, Kimura Futoshi, Hashimoto Hiroshi, Sakai Tetsuhiro, Hirota Kazuyoshi
Department of Anesthesiology, Hirosaki University Graduate School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Japan.
Department of Anesthesiology, Odate Municipal General Hospital, Odate, Japan.
JA Clin Rep. 2018 Apr 21;4(1):34. doi: 10.1186/s40981-018-0171-4.
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 O and maintained with NO (3.5 L/min), O (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.
我们报告了一名患有克拉伯病的儿童接受三次重复手术及麻醉管理的病例，并讨论了关于克拉伯病的主要关注点以及围手术期使用右美托咪定预防苏醒期躁动和高血压的有效性。该患者计划在两年内接受全身麻醉下的双侧睾丸固定术、腺样体扁桃体切除术和膝部屈肌腱延长术。
患者4岁时计划进行腺样体扁桃体切除术作为第二次手术。他的身高和体重分别为93厘米和10.3千克。使用8%七氟醚与6升/分钟的氧气混合诱导麻醉，并使用一氧化氮（3.5升/分钟）、氧气（1.5升/分钟）和七氟醚（1.5 - 2.0%）维持麻醉。在右侧扁桃体切除术后，手术结束前1小时，开始持续输注右美托咪定（0.2微克/千克/小时）以预防苏醒期躁动、易怒、由此导致的高血压和术后出血。静脉注射芬太尼（25微克）以减轻术后疼痛。手术过程顺利，患者从全身麻醉中迅速苏醒。他没有表现出苏醒期躁动或易怒的症状。在重症监护病房期间，持续给予0.2 - 0.7微克/千克/小时的右美托咪定和6.25微克/小时的芬太尼。患者次日早晨无并发症地被送回病房。
围手术期使用右美托咪定有助于预防患有克拉伯病的儿科患者出现苏醒期躁动、高血压及由此导致的术后出血。在不使用肌肉松弛剂的情况下，使用吸入麻醉顺利完成了三次重复的麻醉管理。
