脳血管芽腫摘出術と褐色細胞腫摘出術を緊急で行ったvon Hippel-Lindau病の麻酔経験

コントロール不良の褐色細胞腫を合併した状態で，小脳血管芽腫による著明な頭蓋内圧亢進のため緊急手術が必要となったvon Hippel-Lindau病患者に対する麻酔を経験した．症例は37歳男性．全身麻酔下に，副腎腫瘍切除術，小脳腫瘍切除術の順で手術が行われた．副腎操作時は高用量レミフェンタニル（1.0μg/kg/min）と降圧薬にて，血圧を良好に管理することができた．また褐色細胞腫摘出後に速やかに血圧は正常化し，脳外科手術中も循環動態は安定していた．術前にコントロールされていない褐色細胞腫を合併した患者に対する循環管理において，高用量レミフェンタニルを用いた麻酔管理は一つの選択肢となりうる．

We present an anesthesia case of a 37-year-old male patient with von Hippel-Lindau syndrome who was complicated with uncontrolled pheochromocytoma and cerebellar hemangioblastoma. On the fifth day after admission, because the patient showed clinical signs of high intracranial pressure, emergency surgery was decided. First, under general anesthesia, pheochromocytectomy was performed and then hemangioblastoma were removed on the same day. We initially were able to successfully control the patient's blood pressure with high dose remifentanil (1.0 μg/kg/min) and vasodilators. Following pheochromocytectomy, we lowered the dose of remifentanil (0.2-0.3 μg/kg/min) and the patient's blood pressure was able to be maintained in the normal range perioperatively. In conclusion, we found that a high dose of remifentanil can adequately control hemodynamics in patients with uncontrolled pheochromocytoma.

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